Database: EMBASE <: international biomedical and pharmaceutical literature, 1988 - Aug 2000. [Trial access until 3/2001. Feedback welcome to medical.library@umich.edu] Search Strategy (You Saved Citations 1-300 From Set 78): ----------------------------------------------------------------------------- 1 exp Hereditary diseases/ 157059 2 exp Skin diseases, genetic/ 190419 3 exp Bone diseases, developmental/ 5659 4 exp Tooth abnormalities/ 729 5 (amelogenesis adj imperfecta).mp. 57 6 (dens adj3 dente).mp. 1 7 odontodysplasia.mp. 5 8 (tricho adj dento adj osseous).mp. 10 9 (TDO adj syndrom:).mp. 4 10 anodontia.mp. 25 11 hypodontia.mp. 143 12 Osteogenesis imperfecta/ 906 13 exp Tooth diseases/ 7753 14 12 and 13 15 15 Dyskeratosis congenita/ 141 16 dyskeratosis.mp. 468 17 "Zinsser-Engman-Cole syndrome".mp. 2 18 HBID.mp. 1 19 or/15-18 468 20 Celiac disease/ 2827 21 (celiac or coeliac).mp. 5138 22 steatorrhea.mp. 625 23 sprue.mp. 270 24 (gluten adj enteropath:).mp. 30 25 or/20-24 5792 26 ((teeth or tooth or dent: or odonto: or enamel:) adj3 328 (imperfect: or hypoplasia or dysplasia or hypocalcificat:)).mp. 27 or/1-11,14,19,25-26 341133 28 exp Tooth demineralization/ 7753 29 demineralization.mp. 907 30 caries.mp. 1846 31 caires.mp. 0 32 craies.mp. 0 33 careis.mp. 1 34 carise.mp. 0 35 (teeth adj3 cavit:).mp. 32 36 (tooth adj3 cavit:).mp. 31 37 (dental adj3 cavit:).mp. 55 38 (dentin adj3 cavit:).mp. 14 39 (enamel adj3 cavit:).mp. 6 40 (teeth adj3 decay:).mp. 60 41 (tooth adj3 decay:).mp. 59 42 (dental adj3 decay:).mp. 48 43 (dentin adj3 decay:).mp. 0 44 (enamel adj3 decay:).mp. 1 45 (active adj decay).mp. 5 46 (rampant adj3 decay:).mp. 4 47 (recurrent adj3 decay:).mp. 3 48 (white adj spot:).mp. 229 49 carious.mp. 114 50 cariology.ti,ab. 2 51 (non-cavitated adj3 lesion:).mp. 0 52 (noncavitated adj3 lesion:).mp. 1 53 Tooth remineralization/ 819 54 (dental adj3 fissure:).mp. 7 55 (tooth adj3 fissure:).mp. 3 56 (teeth adj3 fissure:).mp. 1 57 caries-free.mp. 30 58 cariesfree.mp. 0 59 Cariogenic agents/ 3 60 precavit:.mp. 2 61 (filled adj3 teeth).mp. 47 62 (filled adj3 tooth).mp. 9 63 (oral adj fissure:).mp. 4 64 (tooth adj3 remineraliz:).mp. 1 65 (teeth adj3 remineraliz:).mp. 4 66 dft.mp. 583 67 dfs.mp. 1015 68 dmf:.mp. 1290 69 cariogeni:.mp. 169 70 or/28-69 12701 71 27 and 70 2074 72 limit 71 to english language 1846 73 limit 72 to human 1744 74 exp Dentistry/ 2199 75 exp Dental care/ 6313 76 dent:.mp. 20519 77 or/74-76 22634 78 73 and 77 569 79 from 78 keep 1-300 300 *************************** <1> UI - 2000284318 AU - Davies R AU - Bedi R AU - Scully C TI - Oral health care for patients with special needs. SO - British Medical Journal 19 AUG 2000Vol 321(7259) (pp 495-498), 2000. <2> UI - 2000282862 AU - Nunn JH AU - Sharp J AU - Lambert HJ AU - Plant ND AU - Coulthard MG IN - J.H. Nunn, School of Dentistry, University of Newcastle, Framlington Place, Newcastle upon Tyne NE2 4BW; United Kingdom. E-Mail: j.h.nunn@ncl.ac.uk. TI - Oral health in children with renal disease. SO - Pediatric Nephrology Vol 14(10-11) (pp 997-1001), 2000. AB - Thirty-eight children (aged 2-16 years) attending a regional kidney unit had a full clinical and radiological dental examination. Twenty had previously undergone a renal transplant, 11 had chronic renal failure and 7 had other renal diseases. Periodontal disease was uncommon. The presence of gingival hyperplasia (gum overgrowth), as recorded in 22 of the children, did not show any relationship with the use of immunosuppressant therapy. However, gingival overgrowth was so excessive in 2 patients that surgical removal was required. The prevalence of dental caries was low. Enamel defects were common, and of an unusual pattern, with a much higher prevalence of diffuse opacities and enamel hypoplasia than in the normal child population, 83% and 22%, respectively. This increased prevalence is probably due to disordered calcium and phosphate metabolism. The prevalence of these defects may reflect an early onset of renal disease, since there were a number of very young children in the programme. Dental and medical care should be closely integrated for children with renal disease to avoid the undesirable dental sequelae of, in particular, gingival overgrowth, carcinoma and enamel hypoplasia. [References: 23] <3> UI - 2000263682 AU - Gendron R AU - Grenier D AU - Maheu-Robert L-F IN - D. Grenier, Groupe de Recherche/Ecologie Buccale, Faculte de Medicine Dentaire, Universite Laval, Cite universitaire, Laval, Que. G1K 7P4; Canada. TI - The oral cavity as a reservoir of bacterial pathogens for focal infections. SO - Microbes & Infection Vol 2(8) (pp 897-906), 2000. AB - Dental procedures, but more importantly, oral infections and poor oral health can provoke the introduction of oral microorganisms into the bloodstream or the lymphatic system. The subsequent attachment and multiplication of these bacteria on tissues or organs can lead to focal oral infections. Pathogenic agents may also remain at their primary oral site but the toxins liberated can reach an organ or tissue via the bloodstream and cause metastatic injury. Finally, metastatic inflammation may result from an immunological injury caused by oral bacteria or their soluble products that enter the bloodstream and react with circulating specific antibodies to form macromolecular complexes. (C) 2000 Editions scientifiques et medicales Elsevier SAS. [References: 108] <4> UI - 2000273497 AU - Oettinger-Barak O AU - Machtei EE AU - Peled M AU - Barak S AU - L-Naaj IA AU - Laufer D IN - Dr. O. Oettinger-Barak, Periodontal Unit, Dept. of Oral/Maxillofacial Surgery, Rambam Medical Center 31096, P.O. Box 9602, Haifa; Israel. E-Mail: barakori@zahav.net.il. TI - Cyclosporine A-induced gingival hyperplasia pemphigus vulgaris: Literature review and report of a case. SO - Journal of Periodontology Vol 71(4) (pp 650-656), 2000. AB - Gingival hyperplasia appears in 8% to 85% of patients treated with cyclosporine. Most studies show an association between oral hygiene status and the prevalence and severity of this gingival overgrowth. Thus, besides attempting to substitute this drug with another whenever possible, treatment usually involves maintenance of strict oral hygiene coupled with scaling and root planing and removal of iatrogenic factors. Sometimes a second treatment phase involving periodontal surgery is necessary. Cyclosporine-induced gingival overgrowth has been mainly described in post-organ transplant patients. The present case describes, for the first time, a severe form of cyclosporine-induced gingival overgrowth arising in a 15 year-old male with pemphigus vulgaris. Periodontal treatment included oral hygiene and scaling and root planing under local anesthesia. There was a significant reduction in gingival enlargement, as well as a reduction in plaque levels and inflammation. Cessation of drug administration, combined with continuous periodontal treatment, brought further improvement. This successful conservative treatment of cyclosporine-induced gingival overgrowth in a pemphigus vulgaris patient suggests that early diagnosis and comprehensive treatment of these lesions may yield good response and reduce the need for periodontal surgery. [References: 57] <5> UI - 2000266551 AU - Kanerva L AU - Zwanenburg R IN - L. Kanerva, Section of Dermatology, Finnish Inst. of Occupational Health, Topeliuksenkatu 41aA, FIN-00250 Helsinki; Finland. TI - Allergic contact reactions to poly(oxy-1,2-ethanediyl) alpha,alpha'-[(1- methylethylidene)di-4,1-phenylene]bis[omega-[(2-methyl-1-oxo-2- propenyl)oxy] (BIS-EMA). SO - Contact Dermatitis Vol 43(2) (pp 115), 2000. <6> UI - 2000261988 AU - Moiseiwitsch JRD IN - J.R.D. Moiseiwitsch, Department of Endodontics, School of Dentistry, University of North Carolina, Chapel Hill, NC 27599-7450; United States. E-Mail: julian@dentistry.unc.edu. TI - The role of serotonin and during craniofacial development. SO - Critical Reviews in Oral Biology & Medicine Vol 11(2) (pp 230-239), 2000. AB - Several neurotransmitters, in particular serotonin (5-HT), have demonstrated multiple functions during early development and mid-gestational craniofacial morphogenesis. Early studies indicated that 5-HT is present in the oocyte, where it appears to function as a regulator of cell cleavage. Later, it has a significant role during gastrulation, during which there are significant areas of 5-HT uptake in the primitive streak. Subsequently, in association with neurulation, 5-HT uptake is seen in the floor plate of the developing neural tube. During neural crest formation and branchial arch formation, 5-HT has been demonstrated to facilitate cell migration and stimulate cell differentiation. During morphogenesis of the craniofacial structures, 5-HT stimulates dental development and may aid in cusp formation. All of the most commonly prescribed antidepressant drugs inhibit serotonin uptake, yet they do not appear to cause major craniofacial malformations in vivo. Given the wide spectrum of effects that 5-HT has during development, it is difficult to understand why these anti-depressants are not major teratogens. Redundancy within the system may allow receptor and uptake pathways to function normally even with lower than normal levels of circulating serotonin. Serotonin-binding proteins, that are expressed in most craniofacial regions at critical times during craniofacial development, may have a buffering capacity that maintains adequate 5-HT tissue concentrations over a wide range of 5-HT serum concentrations. Dental development appears to be particularly sensitive to even small fluctuations in concentrations of 5-HT. Therefore, it may be that children of patients who have received selective serotonergic reuptake inhibitors (such as Prozac and Zoloft) or the less selective tricyclic anti-depressant drugs (such as Elavil) would be at a higher risk for developmental dental defects such as anodontia and hypodontia. In this review, the evidence supporting a role for 5-HT during mammalian craniofacial development is discussed. A series of models is proposed that may explain how the craniofacial effects of 5-HT are mediated. [References: 63] <7> UI - 2000257157 AU - Harrington B IN - B. Harrington, World Federation of Hemophilia, 1425 Rene Levesque Boulevard West, Montreal, Que. H3G 1T7; Canada. TI - Primary dental care of patients with haemophilia. SO - Haemophilia Vol 6(SUPPL. 1) (pp 7-12), 2000. <8> UI - 2000244763 AU - Bowles JT IN - J.T. Bowles, 60 East Chestnut 395, Chicago, IL 60611; United States. E-Mail: JeftBo@aol.com. TI - Sex, kings and serial killers and other group-selected human traits. SO - Medical Hypotheses Vol 54(6) (pp 864-894), 2000. AB - (Note: This unorthodox paper contains the first argument for heart disease being a programmed age change and promoted by the dramatic, post age-40 increases in the hormones FSH and hCG seen in some individuals.) A recent issue of Science suggests that the evolutionary purpose of sex is unknown. Paradox: Surviving to adulthood implies a valuable gene combination which is destroyed by sexual recombination. This should be detrimental to offspring. Proposed: Sex is group-selected in prey to allow coalescence of beneficial, and disposal of detrimental, mutations in single individuals enabling rapid adaptation to novel predation. Group selection is a universal force driven by local inter-species (not intra-species) competition. Aging, metabolism, litter size, and fixed body size are directly linked. Sexual recombination and chromosomes destroy gene linkage and exist because mutations are usually detrimental, rarely positive, and occur in linked groups. In unevolving environments, sex is selected against and asexuality emerges. Periodic evolution of novel predators, like man, can explain the 'punctuated equilibria' fossil record. Genes inhibited by methylation or chromatin condensation, expressed at older ages in predation-minimized environments, allow for group selection. Stress increases mutation rates and beneficial mutation likelihood. Females select bigger, brighter, louder, or stronger males that can survive predator attention. Size approximates age and thus predator encounters; male traits represent predation-survival potential. Human male traits include, balding, acne, beard-length, wrinkling, graying, nose/ear growth. Progeria accelerates development of most male traits. Domination of groups by single males allows rapid predation-defense evolution: adolescent males are expelled, brave the wild, and expel another group's male to mate. If expelled and dominant males are culled by predation, males reaching puberty first will reproduce. Hormonal acceleration of puberty accelerates aging/population turnover, induces smaller bodies, larger litters. With a fixed group biomass, more, smaller, stressed individuals with faster aging/turnover, increase beneficial mutation likelihood. 'Kin selection', where dominant families are supported by celibate relatives, allow the best group genes to survive famine. Dominant families gorge while others starve. Equal food sharing results in group extinction leading to group-evolved human traits of social hierarchy, greed, king/queen/God worship. Menstrual hormone cycling parallels aging. FSH and DHT promote ovarian, hair, acne, dental, and arterial follicle development causing ovulation, hair growth, pimples, dental caries, and atherosclerotic soft plaques. Soft plaques contain macrophages and LDL plug; upper plaque layers thin and rupture, releasing LDL plug, causing thrombosis. FSH withdrawal or LH/hCG increases trigger ovulation and thrombosis. Artery narrowing atherosclerotic hard plaques are stress-induced through cortisol-promoted necrotic calcification. LH/hCG-induced apoptosis promotes ovulation and aging-related somatic atrophy. Long-term estradiol stimulates, while progesterone suppresses, gonadotropin levels. Estradiol protects by inhibiting gonadotropin bioactivity and has extracellular antioxidant, but intranuclear free radical, effects. Female X-linked gene mosaicism conserves evolved aging systems. Maternal age factors for chromosomal trisomy suggest menopause prevents human parthenogenesis. Homosexuality and serial killing inhibit genetic contribution by individuals evolutionarily perceived as stressed. Smoking during pregnancy may induce homosexual offspring. Nitric oxide, a free radical, stimulates cGMP, but not cAMP. cGMP likely first evolved as an antioxidant defense to free radicals. Human aging syndromes might reflect human evolution progression. AS4 affects tissues evolved from plant ancestors, AS5a - from predators, AS5b-immune system, and AS6-sex tissues. AS4 is driven by senescent gene expression induction of necrosis, AS5a by caspase-induced apoptosis, and AS5b and AS6 by p53-mediated apoptosis. Learning the difference between necrosis and p53-mediated apoptosis should reveal the basic cause of carcinogenesis. Lipofuscin may represent nonfunctional mitochondria and also appears in progeria (AS4). Lipofuscin and/or mitochondria link the non-telomeric aging systems. Variations in universal DNA code suggest mitochondria were the first predators. Post-40 TSH/hCG increases suggest these hormones also drive aging systems. LH/hCG receptor stimulation increases COX-2 levels. Lifelong telomere shortening has no effect on mice until the fourth generation. Conclusion: Only by accepting group selection as an important evolutionary force can the remaining paradoxes of biology, including sex and aging, be resolved. (C) 2000 Harcourt Publishers Ltd. [References: 111] <9> UI - 2000234927 AU - Bachanek T AU - Staroslawska E AU - Wolanska E AU - Jarmolinska K IN - Dr. T. Bachanek, Department of Conservative Dentistry, Medical Academy, Karmelicka 7, 20-081 Lublin; Poland. TI - Heavy metal poisoning in glass worker characterised by severe dental changes. SO - Annals of Agriculture and Environmental Medicine, Vol 7(1) (pp 51-53), 2000. AB - The paper presents the clinical description of the masticatory organ and biochemical assessment of dental tissue in a patient employed in a glassworks for 20 years. During 12 years the patient has suffered baldness ('Alopecia areata') and atypical extensive and non-healing cutaneous lesions. Dental examination revealed changes typical of chronic poisoning by cadmium and bismuth compounds. [References: 20] <10> UI - 2000225972 AU - Coventry J AU - Griffiths G AU - Scully C AU - Tonetti M TI - Periodontal disease. SO - British Medical Journal 01 JUL 2000Vol 321(7252) (pp 36-39), 2000. <11> UI - 2000219852 AU - Da Fonseca MA IN - Dr. M.A. Da Fonseca, Dept. of Orthodont./Pediatric Dent., Univ. of Michigan Sch. of Dentistry, 1011 N. University Avenue, Ann Arbor, MI 48109; United States. E-Mail: marcio@umich.edu. TI - Dental findings in the Schimke immuno-osseous dysplasia. SO - American Journal of Medical Genetics 17 JUL 2000Vol 93(2) (pp 158-160), 2000. AB - Schimke immuno-osseous dysplasia is a rare autosomal recessive disorder that affects primarily bone, T lymphocytes, kidneys, and skin. The patients have a triangular face, broad nasal bridge, bulbous nose tip, small palpebral fissures, short neck, long upper lip, and low hairline. Dental abnormalities of affected patients have not been discussed in detail. The patient described in this clinical report presented with clinical and radiographic abnormalities that may constitute a diagnostic characteristic in this condition. (C) 2000 Wiley-Liss, Inc. [References: 19] <12> UI - 2000200610 AU - Walls AWG AU - Pack ARC IN - Prof. A.W.G. Walls, Department of Restorative Dentistry, Dental School, University of Newcastle upon Tyne, Framlington Place, Newcastle upon Tyne NE2 4BW; United Kingdom. TI - Age, gender, oestrogen replacement therapy and dentition. SO - Journal of the British Menopause Society Vol 6(2) (pp 67-73), 2000. AB - An increasing number of older people are retaining their teeth in the UK. Edentulism is gradually declining and will increasingly become a feature of social deprivation. This review covers the processes of ageing in teeth, the peridontium, the oral mucosa, muscles of mastication, salivary glands and taste. Diseases of the oral mucosa, in particular cancer, are examined. Tooth decay and peridontal disease are discussed. The relationship between systemic health and oral health status are becoming increasingly important. The effect of diet and tobacco smoking are explored. Finally, the association between peridontal disease and osteoporosis, atherosclerosis, venous thromboembolism are discussed. [References: 55] <13> UI - 2000197417 AU - Scarel RM AU - Trevilatto PC AU - Di Hipolito O Jr AU - Camargo LEA AU - Line SRP IN - S.R.P. Line, Faculdade de Odontol. de Piracicaba, UNICAMP, Av. Limeira 901, 13414018 Piracicaba, SP; Brazil. E-Mail: serglin@fop.unicamp.br. TI - Absence of mutations in the homeodomain of the MSX1 gene in patients with hypodontia. SO - American Journal of Medical Genetics 19 JUN 2000Vol 92(5) (pp 346-349), 2000. AB - Hypodontia, the congenital absence of one or a few permanent teeth, is one of the most frequent alterations of the human dentition. Although hypodontia does not represent a public health problem, it may cause both speech and masticatory dysfunction and esthetic problems. A missense mutation in the homeodomain of MSX1 gene has been associated with hypodontia of second premolars and third molars in humans. However, another study excluded this gene as causative locus for hypodontia of incisors and premolars. To further investigate the role of the MSX1 gene in human hypodontia, we analyzed the homeobox region of the MSX1 gene in 20 individuals with different patterns of familial or isolated hypodontia. The direct sequencing of PCR products did not show any polymorphisms or mutations in the human MSX1 gene. Our results indicate that inactivation of MSX1 gene in humans must have a highly selective effect on dentition, and other genes must be involved in the cause of hypodontia in humans. (C) 2000 Wiley-Liss, Inc. [References: 15] <14> UI - 2000193228 AU - Glorio R AU - Allevato M AU - De Pablo A AU - Abbruzzese M AU - Carmona L AU - Savarin M AU - Ibarra M AU - Busso C AU - Mordoh A AU - Llopis C AU - Haas R AU - Bello M AU - Woscoff A IN - Dr. R. Glorio, Hosp. de Clin. 'Jose de San Martin', Division Dermatologia, Avenida Cordoba 2351, 4to piso, Buenos Aires 1120; Argentina. TI - Prevalence of cutaneous manifestations in 200 patients with eating disorders. SO - International Journal of Dermatology Vol 39(5) (pp 348-353), 2000. AB - Background: Eating disorder are increasing and show a variety of symptoms. They mainly include anorexia nervosa (AN), bulimia nervosa (BN), and eating disorders not specified (EDNOS). They predominate in females and represent an important danger, especially in teenagers. In serious cases, they may be life-threatening. Objective: To determine the prevalence of cutaneous findings in patients with eating disorders and to compare the results with those found in the literature. Methods: An observational, transverse, and prospective study was performed. Two hundred patients of recent admission to ALUBA (association that fights against BN and AN), a psychiatric unit for eating disorders, were included: 122 BN; 62 AN; 16 EDNOS. Results: Patients with eating disorders show dermatologic manifestations (alopecia, xerosis, hypertrichosis, caries, nail fragility) that are secondary to starvation. Russell's sign, seen as calluses on the dorsal aspect of the hands, is a consequence of self-induced vomiting and the local trauma of the superior incisors. This sign represents a compensatory behavior to overeating and predominates in the BN group. Conclusion: The recognition of dermatologic signs could be of immense value and could lead to the early diagnosis and treatment of these eating disorders. [References: 30] <15> UI - 2000167955 AU - Cole BOI AU - Welbury RR AU - Bond E AU - Abinun M IN - B.O.I. Cole, Dept. of Child Dental Health, Dental Hospital and School, Richardson Road, Newcastle upon Tyne NE2 4AZ; United Kingdom. TI - Dental manifestations in severe combined immunodeficiency following bone marrow transplantation. SO - Bone Marrow Transplantation Vol 25(9) (pp 1007-1009), 2000. AB - Severe combined immunodeficiency (SCID) is a rare primary immunodeficiency disorder with an estimated overall frequency of 1 in 75,000 live births. Bone marrow transplantation is the only curative treatment available. Using T cell-depleted HLA non-identical bone marrow requires preconditioning with a short course of cytotoxic chemotherapy. We report severe dental developmental anomalies in three such patients under longterm follow up. [References: 12] <16> UI - 2000165606 AU - Okada K AU - Yamashiro T AU - Tenshin S AU - Takano-Yamamoto T IN - Dr. T. Takano-Yamamoto, Department of Orthodontics, Okayama University Dental School, Shikata-cho 2-5-1, Okayama City, Okayama 700-8525; Japan. E-Mail: L-yamamo@dent.okayama-u.ac.jp. TI - Orthodontic treatment for a patient with Pierre-Robin sequence complicated by juvenile periodontitis. SO - Cleft Palate-Craniofacial Journal Vol 37(3) (pp 318), 2000. AB - Objective: To arrest the advancement of periodontitis. Patient: A 17- year-old boy diagnosed with Pierre-Robin sequence at birth exhibited localized juvenile periodontitis. Severe bone loss and mobile teeth were localized in the incisors and molars, which were irregularly positioned, possibly associated with a residual scar from palatoplasty for a soft palate cleft at an early age. Conclusion: Combined orthodontic-periodontic- prosthodontic treatment arrested the advancement of the periodontitis and established a stable occlusion. [References: 25] <17> UI - 2000161484 AU - Glass IA AU - Trenholme A AU - Mildenhall L AU - Bailey RJ AU - Cotter PD IN - Dr. P.D. Cotter, Division of Medical Genetics, Children's Hospital Oakland, 747 Fifty Second Street, Oakland, CA 94609-1809; United States. E-Mail: pcotter@itsa.ucsf.edu. TI - Mild phenotype in two siblings with distal monosomy 12p13.31 [right arrow] pter. SO - Clinical Genetics Vol 57(5) (pp 401-405), 2000. AB - We report two sibs with trisomy for the region 2p25.1 [right arrow] pter and monosomy for the region 12p13.31 [right arrow] pter, due to adjacent-1 segregation of a maternal balanced reciprocal translocation, 46,XX,t(2;12)(p25.1;p13.31). These sibs presented with a mild phenotype, but nevertheless showed features of each of the contributing aneusomies. Monosomy 12p has previously been considered to have a variable and indistinct phenotype. Comparison of these patients with previous reports showed that many features, including microcephaly, facial dysmorphia, developmental and growth delay and dental and digital anomalies are frequently associated with monosomy for 12p. Many of these features are common to other aneusomies, thereby mitigating against a distinct 12p monosomy syndrome at this time. However, the combination of digital and dental anomalies may suggest the presence of this particular monosomy. The proband and his sister had some of the more non-specific features of 2p trisomy syndrome, and comparison with previous reports suggested that the characteristic 2p trisomy syndrome is more usually associated with larger or more proximal trisomies of 2p. [References: 17] <18> UI - 2000146058 AU - Kanerva L AU - Estlander T AU - Jolanki R AU - Alanko K IN - L. Kanerva, Section of Dermatology, Finnish Inst. of Occupational Health, Topeliuksenkatu 41aA, FIN-00250 Helsinki; Finland. TI - False-negative patch test reactions due to a lower concentration of patch test substance than declared. SO - Contact Dermatitis Vol 42(5) (pp 289-291), 2000. <19> UI - 2000138873 AU - Franklin D AU - Senior N AU - James I AU - Roberts G IN - D. Franklin, Division of Child Dental Health, Bristol Dental Hospital, Lower Maudlin Street, Bristol BS1 2LY; United Kingdom. TI - Oral health status of children in a paediatric intensive care unit. SO - Intensive Care Medicine Vol 26(3) (pp 319-324), 2000. AB - Objective: The aim of the present study was to examine the dental status of critically ill children in a Paediatric Intensive Care Unit (PICU) and determine the efficacy of the mouth care received. Design: Prospective nonrandomized study. Setting: The study was carried out in the PICU at Great Ormond Street Hospital for Children. Patients and participants: All children admitted to the PICU during the period of the study were eligible for inclusion. A total of 54 children completed the study. Measurements and results: Children were examined for dental caries, plaque accumulation, gingival inflammation and gingival bleeding on admission to the PICU. The examination was repeated on discharge from the Unit. The levels of dental caries found on examination were compared with results from the most recent national surveys carried out in the United Kingdom, and analysed using the one sample t-test. The levels of plaque accumulation, gingival inflammation and gingival bleeding on admission were compared to those on discharge and analysed using the paired sample t-test. Results revealed a very highly significant increase in plaque accumulation (p = 0.001), and a highly significant increase in gingival inflammation (p = 0.006) between admission to the PICU and discharge. Conclusions: Results indicate that the present mouth care regimen is not effective in preventing the build up of plaque or maintaining gingival health. These children may therefore be at unnecessary risk from local or systemic spread of oral microorganisms. [References: 39] <20> UI - 2000131285 AU - Stellingsma C AU - Meijer HJA AU - Raghoebar GM IN - Dr. C. Stellingsma, Department of Oral/Maxfac. Surg., University Hospital Groningen, PO Box 30.001, 9700 RB Groningen; Netherlands. E-Mail: c.stellingsma@kchir.azg.nl. TI - Use of short endosseous implants and an overdenture in the extremely resorbed mandible: A five-year retrospective study. SO - Journal of Oral & Maxillofacial Surgery Vol 58(4) (pp 382-387), 2000. AB - Purpose: A retrospective study was performed to assess the success of short endosseous implants in combination with an implant-retained overdenture in the extremely resorbed mandible. Patients and Methods: Patients included had a mandible not exceeding a median height of 12 mm measured on a standardized lateral cephalometric radiograph. Seventeen patients (14 women, 3 men; mean age, 65 years) with 68 endosseous implants were clinically and radiographically evaluated. Results: During the follow-up period (mean, 77 months; range, 60 to 97 months), 8 implants were lost, bringing the cumulative survival rate to 88%. The peri-implant tissues were in good condition, and the bone loss around the implants was minimal. Conclusion: Because of the relative simplicity and low morbidity of this treatment strategy, it is a justified treatment option. [References: 35] <21> UI - 2000121251 AU - Mason C AU - Azam N AU - Holt RD AU - Rule DC IN - C. Mason, Department of Paediatric Dentistry, Eastman Dental Institute, Oral Healthcare Sciences, 256 Gray's Inn Road, London WC1X 8LD; United Kingdom. TI - A retrospective study of unerupted maxillary incisors associated with supernumerary teeth. SO - British Journal of Oral & Maxillofacial Surgery Vol 38(1) (pp 62-65), 2000. AB - We retrospectively reviewed the clinical records and radiographs of 100 patients who had had supernumerary teeth removed. Their mean (SD) age was 9 years 3 months. The 100 patients had a total of 127 unerupted maxillary incisors associated with supernumerary teeth. The supernumerary tooth was removed without exposure of the permanent tooth more often among the 79 younger children (aged 10 years or less) (SND = 3.52, P < 0.01), and when the incisors were less mature (Cvek classification 1,2, and 3) (SND = 5.27, P < 0.01). Of these incisors with immature roots that were treated conservatively, 60 (72%) erupted and 24 (28%) required further operation. Of the mature incisors treated conservatively, 10 (63%) required further operation. In this study, almost three-quarters of immature incisors erupted spontaneously after removal of the associated supernumerary teeth. Over half of the mature teeth required further operation. Mature teeth should be exposed with or without bonding at the time of removal of the supernumerary teeth. [References: 24] <22> UI - 2000115288 AU - Hervas Garcia A AU - Forner Navarro L AU - Llena Puy MC IN - A. Hervas Garcia, Unidad Docente de PTD, Clinica Odontologica, c/ Gasco Oliag 1, 46010 - Valencia; Spain. E-Mail: forner@uv.es. TI - Dental manifestations of systemic disease. SO - Medicina Oral Vol 5(2) (pp 109-117), 2000. AB - A review is made of those systemic disorders that may associate dental pathology, grouped as follows: congenital dental developmental disorders, chromosomal anomalies, radiations, immune disorders, intoxications, neurological alterations, gastrointestinal diseases, osteodystrophy and associated conditions, skin diseases, metabolic and endocrine disorders, craniofacial malformation syndromes and other congenital general malformations. The associated dental pathology is described in each case. [References: 106] <23> UI - 2000115285 AU - Alaejos Algarra CC AU - Contreras Martinez MA AU - Buenechea Imaz R AU - Berini Aytes L AU - Gay Escoda C IN - Dr. C. Gay Escoda, C/ Ganduxer 140, 4., 08022 Barcelona; Spain. TI - Mesiodens: A retrospective study of 44 patients. SO - Medicina Oral Vol 5(2) (pp 81-88), 2000. AB - The presence of supernumerary teeth in the midline zone of the upper maxilla - a condition known as mesiodens- can cause a number of alterations, including displacement of the adjacent teeth, anomalous or delayed eruption of the permanent teeth, median diastema, or cyst formation around the unerupted supernumerary tooth, etc. A retrospective study is made of 44 patients with a total of 64 mesiodens surgically treated in our center. Surgery involved the localization of the supernumerary tooth; those mesiodens located on the palatinal side were treated using a palatinal approach, whereas those included in the vestibular zone were extracted via a vestibular approach. Sixty-two of the supernumerary teeth were located palatine to the permanent central incisor. In 56 cases surgical extraction of the mesiodens via a palatinal approach proved sufficient, while the remaining 6 cases furthermore required surgical exposure with an apical repositioning flap. Finally, two mesiodens were situated labially to the permanent central incisor, and surgical exposure of the latter was required using an apical repositioning flap. A review is made of the literature, and the clinical, etiological and diagnostic aspects, as well as treatment options are discussed. It is concluded that mesiodens extraction is a simple and frequent technique, in view of the high incidence of supernumerary teeth, though in all cases the surgical approach employed should be based on a correct diagnosis. [References: 39] <24> UI - 2000115190 AU - Wang H-C AU - Chen C-C AU - Wang W-J AU - Ho WL IN - Dr. H.-C. Wang, Department of Dermatology, Taichung Veterans General Hospital, 160, Section 3, Taichung-Kang Road, Taichung 407; Taiwan. TI - Hypohidrotic ectodermal dysplasia. SO - Chinese Medical Journal (Taipei) Vol 63(3) (pp 230-233), 2000. AB - Hypohidrotic ectodermal dysplasia was first described by Thurnam in 1848. It is a rare, X-linked, recessive disorder characterized by anhidrosis or hypohidrosis, hypotrichosis, dental hypoplasia and characteristic facial features. Herein, we report a typical case of hypohidrotic ectodermal dysplasia. A 20-year-old male presented with the above symptoms at birth. When a family history was taken, it was discovered that his uncle (mother's brother) had the same characteristic facial features and hypotrichosis. [References: 15] <25> UI - 2000113966 AU - Liou EJW AU - Chen PKT AU - Huang CS AU - Chen YR IN - E.J.W. Liou, Orthodon./Craniofacial Dent. Dept., Chang Gung Memorial Hospital, 199 Tung-Hwa North Road, 105 Taipei; Taiwan. E-Mail: lioueric@ms19.hinet.net. TI - Interdental distraction osteogenesis and rapid orthodontic tooth movement: A novel approach to approximate a wide alveolar cleft or bony defect. SO - Plastic & Reconstructive Surgery Vol 105(4) (pp 1262-1272), 2000. AB - The closure of a wide alveolar cleft and fistula in cleft patients and the reconstruction of a maxillary dentoalveolar defect in traumatic patients are challenging for both orthodontists and surgeons. This is due to the difficulty in achieving complete closure by using local attached gingiva and the great volume of bone required for the graft. In this article, the authors propose using interdental distraction osteogenesis to create a segment of new alveolar bone and attached gingiva for the complete approximation of a wide alveolar cleft/fistula and the reconstruction of a maxillary dentoalveolar defect. They performed this procedure on one patient with a traumatic maxillary dentoalveolar defect and 10 patients with unilateral or bilateral cleft lips and palates who had varied dentoalveolar clefts/fistulas. Interdental and maxillary osteotomies were performed on one side of the dental arch by the cleft or defect. After a latency period of 3 days, the osteotomized distal segment of the dental arch was then distracted and transported toward the cleft or defect by using a tooth-borne intraoral distraction device. The alveoli and gingivae on both ends of the cleft or defect were approximated after distraction osteogenesis. The need for extensive alveolar bone grafting was eliminated. A segment of new edentulous alveolus and attached gingiva was created interdentally at a site distant to the cleft or defect. In the cleft patients, teeth were moved orthodontically into the regenerate (newly formed alveolar bone) dental crowding 1 week after distraction. The orthodontic tooth movement was rapidly completed in 3 months, and the edentulous space was eliminated. Interdental distraction osteogenesis minimizes an alveolar cleft/fistula and helps reconstruct a maxillary dentoalveolar defect by approximating the native alveoli and gingivae; it also creates new alveolar bone and gingiva for rapid orthodontic tooth movement. [References: 26] <26> UI - 2000101613 AU - Aurouze C AU - Moller ET AU - Bevis RR AU - Rehm K AU - Rudney J IN - Dr. C. Aurouze, Le mont d'or, 83110 Sanary Sur Mer; France. E-Mail: cath005@yahoo.com. TI - The presurgical status of the alveolar cleft and success of secondary bone grafting. SO - Cleft Palate-Craniofacial Journal Vol 37(2) (pp 179-184), 2000. AB - Objective: The primary purpose of this study was to evaluate presurgical status of the alveolar cleft site and success of secondary alveolar bone grafting. Design: Thirty patient records were retrospectively reviewed. Patients selected for inclusion had isolated cleft of at least the primary palate. Patients with additional anomalies were not selected. The study population consisted of 15 female sites and 16 male cleft sites. There were two bilateral cleft lip and palate (CLP) patients and 28 unilateral CLP patients. The age at the time of the secondary alveolar bone grafting ranged from 7 years to 14 years, 4 months. Setting: The study was conducted at the Cleft Palate Clinic at the University of Minnesota, School of Dentistry. Method: Presurgical radiographs taken at least 1 month prior to the secondary bone grafting and postsurgical radiographs taken at least 6 months after bone surgery were measured. Measurements included size of the cleft defect and bone support for distal and mesial teeth adjacent to the cleft. Evaluation of success was determined on the basis of postsurgical measurements of satisfactory, intermediate, and unsatisfactory outcomes. Results and Conclusion: The size of the cleft defect was not correlated with the success rate of the secondary alveolar bone grafting. If the amount of distal bone support for the mesial tooth was the same as those in a periodontally healthy individual, a satisfactory outcome was 5.8 times more likely. If the amount of mesial bone support for the distal tooth was the same as those in a periodontally healthy individual, the satisfactory outcome was 3.8 times more likely. Although not a primary purpose of the study, it was found that in this study population, if the patient was female, a satisfactory outcome was 3.8 times more likely. [References: 18] <27> UI - 2000079257 AU - Kirby B AU - Rogers S IN - B. Kirby, Dept. of Dermatology, Hope Hospital, Salford, Manchester M6 8HD; United Kingdom. E-Mail: bkirby1997@yahoo.com. TI - Gingival hyperplasia in psoriasis patients treated with cyclosporin [2]. SO - Clinical & Experimental Dermatology Vol 25(1) (pp 97-98), 2000. <28> UI - 2000075062 AU - Karrer S AU - Landthaler M AU - Schmalz G IN - S. Karrer, Department of Dermatology, University of Regensburg, D-93042 Regensburg; Germany. E-Mail: Sigrid.Karrer@klinik.uni-regensburg.de. TI - Ehlers-Danlos syndrome type VIII with severe periodontitis and apical root resorption after orthodontic treatment [7]. SO - Acta Dermato-Venereologica Vol 80(1) (pp 56-57), 2000. <29> UI - 2000065111 AU - Bacon W AU - Hall RK AU - Roset J-P AU - Boukari A AU - Tenenbaum H AU - Walter B IN - Prof. W. Bacon, Dept. Orthopedie Dento-Faciale, Universite Louis Pasteur, 1 Place de l'Hopital, F-67000 Strasbourg; France. E-Mail: william.bacon@chru.strasbourg.fr. TI - GAPO syndrome: A new case of this rare syndrome and a review of the relative importance of different phenotypic features in diagnosis. SO - Journal of Craniofacial Genetics & Developmental Biology Vol 19(4) (pp 189-200), 1999. AB - The case of GAPO syndrome reported here is the 24th recorded case, 23 cases having been published previously. The 29-year-old male under discussion presents all the typical features of the syndrome, having short stature, dysmorphic craniofacial features, total alopecia and pseudoanodontia. Orally, the erupted primary dentition was extremely worn and on radiographic examination, the second mandibular molars were found to be unerupted, together with the entire permanent dentition. Cephalometry revealed the absence of facial pneumatisation, a deficient cranial base with diminished upper face height and maxillary and mandibular hypoplasia with a prognathic skeletal pattern. Histological examination of an extracted primary incisor and its surrounding root bone revealed extensive ankylosis. This paper describes in detail the clinical findings and reviews, and discusses previously published cases in relation to the present one. As with prior cases, parental consanguinity was present in the pedigree. [References: 30] <30> UI - 2000055473 AU - Hart TC AU - Marazita ML AU - Wright JT IN - T.C. Hart, Pittsburgh Univ. Sch. of Dental Med., Division of Oral Biology, Pittsburgh, PA 15261; United States. TI - The impact of molecular genetics on oral health paradigms. SO - Critical Reviews in Oral Biology & Medicine Vol 11(1) (pp 26-56), 2000. AB - As a result of our increased understanding of the human genome, and the functional interrelationships of gene products with each other and with the environment, it is becoming increasingly evident that many human diseases are influenced by heritable alterations in the structure or function of genes. Significant advances in research methods and newly emerging partnerships between private and public sector interests are creating new possibilities for utilization of genetic information for the diagnosis and treatment of human diseases. The availability and application of genetic information to the understanding of normal and abnormal human growth and development are fundamentally changing the way we approach the study of human diseases. As a result, the issues and principles of medical genetics are coming to bear across all disciplines of health care, in this review, we discuss some of the potential applications of human molecular genetics for the diagnosis and treatment of oral diseases. This discussion is presented in the context of the ongoing technological advances and conceptual changes that are occurring in the field of medical genetics. To realize the promise of this new molecular genetics, we must be prepared to foresee the possibilities and to incorporate these newly emergent technologies into the evolving discipline of dentistry. By using examples of human conditions, we illustrate the broad application of this emerging technology to the study of simple as well as complex genetic diseases. Throughout this paper, we will use the following terminology: Penetrance - In a population, defined as the proportion of individuals possessing a disease-causing genotype who express the disease phenotype. When this proportion is less than 100%, the disease is said to have reduced or incomplete penetrance. Polymerase chain reaction (PCR) - A technique for amplifying a large number of copies of a specific DNA sequence flanked by two oligonucleotide primers. The DNA is alternately heated and cooled in the presence of DNA polymerase and free nucleotides, so that the specified DNA segment is denatured, hybridized with primers, and extended by DNA polymerase. MIM - Mendelian Inheritance in Man catalogue number from V. McKusick's Mendelian Inheritance in man (OMIM, 1998). [References: 239] <31> UI - 2000054938 AU - Bondarets N AU - McDonald F IN - Dr. F. McDonald, Department of Orthodontics, Guys Tower, Kings College, St. Thomas St., London SE1 9RT; United Kingdom. E-Mail: fraser.mcdonald@kcl.ac.uk. TI - Analysis of the vertical facial form in patients with severe hypodontia. SO - American Journal of Physical Anthropology Vol 111(2) (pp 177-184), 2000. AB - We examined the lateral cephalograms of Russian patients in the following categories: control with acceptable occlusions (group 1); severe hypodontia with absence of six or more teeth (group 2); and severe hypodontia associated with hypohidrotic ectodermal dysplasia (HED) (group 3). Analysis was in a cross-sectional manner, comparing dimensions at the start of the mixed dentition phase (age 6-10) and in the permanent dentition (age 12-18). The groups were matched for age and sex. Thirty-one hard- and soft-tissue landmarks were traced, and 35 linear, 19 angular, and 7 ratioed measurements were taken and compared, using analysis of variance to compare the means of each group. A reduced anterior face height was found in groups 2 and 3 as a consequence of a reduced anterior lower face height. In group 2 in the mixed dentition, the posterior face height was also reduced. The inclination of the mandible ( UI - 2000047801 AU - Kanerva L AU - Alanko K AU - Estlander T IN - L. Kanerva, Section of Dermatology, Finnish Inst. of Occupational Health, Topeliuksenkatu 41 aA, FIN-00250 Helsinki; Finland. TI - Allergic contact gingivostomatitis from a temporary crown made of methacrylates and epoxy diacrylates. SO - Allergy: European Journal of Allergy and Clinical Immunology, Vol 54(12) (pp 1316-1321), 1999. AB - Occupational allergic contact dermatitis caused by (meth)acrylates is common in dental personnel, whereas dental acrylic fillings and crowns have rarely been reported to cause problems in dental patients. Here we report on a 48-year-old woman who developed gingivitis, stomatitis, and perioral dermatitis after a temporary crown made of restorative, two-component material had been inserted. The manufacturer stated that the temporary crown base paste and catalyst contained three (meth)acrylates, namely, a proacrylate, which is a modification of 2,2-bis[4-(2-hydroxy-3- methacryloxypropoxy)phenyl]propane (BIS-GMA); a tricyclate, which is a saturated, aliphatic, tricyclic methacrylate; and urethane methacrylate. The manufacturer refused to give more exact information on the (meth)acrylates. Patch testing revealed that the patient was highly allergic to BIS-GMA, other epoxy diacrylates, and (meth)acrylates, as well as to the base paste and catalyst of the temporary crown. Accordingly, it was concluded that the allergic reaction was caused by BIS-GMA, or a cross-reacting (meth)acrylate, or other (meth)acrylates in the temporary crown. [References: 28] <33> UI - 2000043965 AU - Lekkas C AU - Latief BS AU - Ter Rahe SPN AU - Kuijpers-Jagtman AM IN - Dr. C. Lekkas, University Hospital Leiden, Dept. of Oral/Maxillofacial Surg., Postbox 9600, 2300 RC Leiden; Netherlands. TI - The adult unoperated cleft patient: Absence of maxillary teeth outside the cleft area. SO - Cleft Palate-Craniofacial Journal Vol 37(1) (pp 17-20), 2000. AB - Objective: The purpose of this study was to investigate the possible absence of teeth in the postcanine region of the upper jaw of the unoperated adult cleft patient. Method: The study was performed on 266 dental casts of fully unoperated adult cleft patients. The patients were divided into four groups according to the type of the cleft: unilateral cleft lip and alveolus, unilateral cleft lip and palate, bilateral cleft lip and alveolus, and bilateral cleft lip and palate. Results: No absence of permanent teeth in the canine and postcanine area of the upper jaw could be found. Conclusion: The results are in contradiction with the established hypothesis that absence of teeth outside the cleft area of the maxilla is due to an unknown congenital factor. On the contrary, the findings support the hypothesis that surgery for the closure of the hard palate in early childhood is the most important etiological factor for the absence of teeth outside the cleft area in the early operated cleft patient. The superficial position of the tooth germs (at the time of the palatal surgery), especially those of the premolars, supports this hypothesis. [References: 16] <34> UI - 2000035691 AU - Wang H AU - Zhao S AU - Zhao W AU - Feng G AU - Jiang S AU - Liu W AU - Li S AU - Xue H AU - He L IN - Dr. L. He, Shanghai Institute of Physiology, Chinese Academy of Sciences, 320 Yue-Yang Road, Shanghai 200031; China. E-Mail: helinanna@hotmail.com. TI - Congenital absence of permanent teeth in a six-generation Chinese kindred. SO - American Journal of Medical Genetics 31 JAN 2000Vol 90(3) (pp 193-198), 2000. AB - We report on rare, heritable, permanent tooth agenesis in a large Chinese kindred. The congenital absence of permanent teeth except the first and second accessory teeth was observed in 52 individuals through six successive generations in the kindred comprising 328 members. Clinical assessments were carried out, and inheritance mode and spousal influence of the anomaly on their offspring were analyzed. Consequently, the anomaly was transmitted in an autosomal dominant fashion with incomplete penetrance (P = 0.88), and no significant clinical manifestations other than the oligodontia were found. A geographical or environmental effect on the affected individuals was obviously eliminated, because any who are related to the kindred but live under the same conditions are fully healthy. The disorder we describe, therefore, differs from any previously reported oligodontia/anodontia syndromes. The oligodontia ranged from a few teeth to the whole set of teeth, and usually occurred at a period from age 7 or 8 years, the time when primary teeth are normally replaced by permanent teeth, to the forties. Roentgenography of the affected persons indicated that only the first and/or second accessory teeth with tooth buds developed as permanent teeth. In fact, the diphyodontic germination sometimes occurred in the oral cavity of the affected individuals. [References: 15] <35> UI - 2000027231 AU - Shashikala M AU - Surendra VHH TI - Pharmaceutical Industries : Medical screening report. SO - Indian Journal of Industrial Medicine Vol 3(3) (pp 143-145), 1999. AB - This programme involves screening of employees for morbidity and further recommendations to improve the Occupational Health Services within the industrial sector. Methodology includes preliminary walkthrough survey of entire factory and Interview (Oral questionnaire) and Medical Examination of the employees (788) of Pharmaceutical Industries in Bangalore (pooled data of different Pharmaceutical Sections - Pharmaceutical Formulations, Bulk drugs, Laboratory, Administration and Canteen). Analysis revealed refractory error 112 (14.2%), acid peptic disease 110 (14%), psychosocial stress 96 (12.2%), irritation of eyes 77 (9.8%), hypertension 62 (7.9%), anaemia 57 (7.2%), vitamin B complex deficiency 52 (6.6%), nasal allergy 51 (6.5%), muscle and joint pain 48 (6.1%), dental caries 44 (5.6%), nasobronchial allergy 23 (2.9%), diabetes 19 (2.4%), respiratory tract infection 18 (2.3%), dermatitis 18 (2.3%), impaired hearing 8 (1%) etc. [References: 3] <36> UI - 2000002757 AU - Vajdovich I AU - Fazekas A IN - I. Vajdovich, Dept. of Dentistry and Oral Surgery, County Hospital, Sima Ferenc u. 44-58, H-6600 Szentes; Hungary. TI - A ten-year clinical follow-up study of prosthetic rehabilitation of the edentulous lower jaw with endosteal dental implants. SO - Journal of Long-Term Effects of Medical Implants Vol 9(3) (pp 171-183), 1999. AB - This paper reports on 10 years of clinical experience with implant prosthodontic treatment of total edentulism. During this period, of the 735 implants that were inserted, only 19 were lost, a 97.4% success rate. The authors believe that evaluating the success of implant treatment requires examining the health of the peripilastrium with periodontologic methods. The indices the authors used for such an evaluation were plaque accumulation, crevicular fluid flow rate, and sulcus bleeding; crevicular fluid flow proved the most satisfactory for estimating the health of periimplant tissues. The results demonstrate that periimplant-tissue health is similar to that of the tissues surrounding nontreated natural teeth and better than that of the tissues surrounding natural teeth wearing crowns. [References: 29] <37> UI - 2000002476 AU - Hart TC AU - Hart PS AU - Bowden DW AU - Michalec MD AU - Callison SA AU - Walker SJ AU - Zhang Y AU - Firatli E IN - Dr. T.C. Hart, Division of Oral Biology, University of Pittsburgh, 3501 Terrace Street, 618 Salk Hall, Pittsburgh, PA 15261; United States. TI - Mutations of the cathepsin C gene are responsible for Papillon-Lefevre syndrome. SO - Journal of Medical Genetics Vol 36(12) (pp 881-887), 1999. AB - Papillon-Lefevre syndrome (PLS) is an autosomal recessive disorder characterised by palmoplantar hyperkeratosis and severe early onset periodontitis that results in the premature loss of the primary and secondary dentitions. A major gene locus for PLS has been mapped to a 2.8 cM interval on chromosome 11q14. Correlation of physical and genetic maps of this interval indicate it includes at least 40 ESTs and six known genes including the lysosomal protease cathepsin C gene (CTSC). The CTSC message is expressed at high levels in a variety of immune cells including polymorphonuclear leucocytes, macrophages, and their precursors. By RT-PCR, we found CTSC is also expressed in epithelial regions commonly affected by PLS, including the palms, soles, knees, and oral keratinised gingiva. The 4.7 kb CTSC gene consists of two exons. Sequence analysis of CTSC from subjects affected with PLS from five consanguineous Turkish families identified four different mutations. An exon 1 nonsense mutation (856C [right arrow] T) introduces a premature stop codon at amino acid 286. Three exon 2 mutations were identified, including a single nucleotide deletion (2692delA) of codon 349 introducing a frameshift and premature termination codon, a 2 bp deletion (2673-2674delCT) that results in introduction of a stop codon at amino acid 343, and a G [right arrow] A substitution in codon 429 (2931G [right arrow] A) introducing a premature termination codon. All PLS patients were homozygous for cathepsin C mutations inherited from a common ancestor. Parents and sibs heterozygous for cathepsin C mutations do not show either the palmoplantar hyperkeratosis or severe early onset periodontitis characteristic of PLS. A more complete understanding of the functional physiology of cathepsin C carries significant implications for understanding normal and abnormal skin development and periodontal disease susceptibility. [References: 23] <38> UI - 1999426538 AU - Anonymous TI - Oral defense with cathepsin C. SO - Nature Medicine Vol 5(12) (pp 1358), 1999. <39> UI - 1999425822 AU - Toomes C AU - James J AU - Wood AJ AU - Wu CL AU - McCormick D AU - Lench N AU - Hewitt C AU - Moynihan L AU - Roberts E AU - Woods CG AU - Markham A AU - Wong M AU - Widmer R AU - Ghaffar KA AU - Pemberton M AU - Hussein IR AU - Temtamy SA AU - Davies R AU - Read AP AU - Sloan P AU - Dixon MJ AU - Thakker NS IN - N.S. Thakker, St. Mary's Hospital, Turner Dental School, University of Manchester, Manchester; United Kingdom. E-Mail: nthakker@man.ac.uk. TI - Loss-of-function mutations in the cathepsin C gene result in periodontal disease and palmoplantar keratosis. SO - Nature Genetics Vol 23(4) (pp 421-424), 1999. AB - Papillon-Lefevre syndrome, or keratosis palmoplantaris with periodontopathia (PLS, MIM 245000), is an autosomal recessive disorder that is mainly ascertained by dentists because of the severe periodontitis that afflicts patients. Both the deciduous and permanent dentitions are affected, resulting in premature tooth loss. Palmoplantar keratosis, varying from mild psoriasiform scaly skin to overt hyperkeratosis, typically develops within the first three years of life. Keratosis also affects other sites such as elbows and knees. Most PLS patients display both periodontitis and hyperkeratosis. Some patients have only palmoplantar keratosis or periodontitis, and in rare individuals the periodontitis is mild and of late onset. The PLS locus has been mapped to chromosome 11q14-q21 (refs 7-9). Using homozygosity mapping in eight small consanguineous families, we have narrowed the candidate region to a 1.2-cM interval between D11S4082 and D11S931. The gene (CTSC) encoding the lysosomal protease cathepsin C (or dipeptidyl aminopeptidase I) lies within this interval. We defined the genomic structure of CTSC and found mutations in all eight families. In two of these families we used a functional assay to demonstrate an almost total loss of cathepsin C activity in PLS patients and reduced activity in obligate carriers. [References: 25] <40> UI - 1999420615 AU - Shimizu H AU - Takizawa Y AU - Pulkkinen L AU - Murata S AU - Kawai M AU - Hachisuka H AU - Udono M AU - Uitto J AU - Nishikawa T IN - Dr. H. Shimizu, Department of Dermatology, Hokkaido Univ. School of Medicine, Nishi-7, Kita-15, Kita-ku, Sapporo 060-8638; Japan. TI - Epidermolysis bullosa simplex associated with muscular dystrophy: Phenotype-genotype correlations and review of the literature. SO - Journal of the American Academy of Dermatology Vol 41(6) (pp 950-956), 1999. AB - Background: Epidermolysis bullosa simplex associated with muscular dystrophy (EBS-MD; OMIM 226670) is an autosomal recessive disorder caused by genetic defects in the plectin gene. Because EBS-MD is relatively rare, and gene defects have been elucidated only in a limited number of patients, the precise phenotype-genotype correlations have not yet been fully elucidated. Objective: The purpose of this study was to define clinical features of EBS- MD and to clarify its phenotype-genotype correlations. Methods: Clinical, ultrastructural, immunohistochemical, and molecular features of 4 unrelated Japanese patients with EBS-MD were recorded. In addition, 6 cases with defined plectin gene mutations reported in the literature were reviewed. Results: In skin of the EBS-MD patients, the blister formation always occurs just above the hemidesmosomes, and expression of plectin is absent or markedly reduced in all cases examined. All 10 patients, including 6 cases in the literature, showed generalized blistering at birth or soon thereafter, and experienced nail deformities. In addition, decayed teeth (5 cases), urethral strictures (3), mild palmoplantar hyperkeratosis (2), infantile respiratory complications (2), alopecia (1), and laryngeal webs (1) were present. All 8 patients who were older than 9 years demonstrated considerable muscle weakness, and the majority of them ended up being wheelchair bound. Among the 10 patients, 7 were products of consanguineous marriage, 9 have premature termination codon (PTC) mutations in both alleles of the plectin gene, and 7 cases were homozygous for the mutation. One patient who is homozygous for a 2719del9 in-frame deletion mutation that resulted in elimination of 3 amino acids, QEA, could still walk at the age of 46 and showed milder clinical severity. Conclusion: EBS-MD reveals clinical features not only characteristic of EBS and MD, but also other manifestations including urethral, dental, and respiratory complications. The majority of patients are products of consanguineous marriage and have homozygous plectin gene mutations. Whereas patients with PTC mutations in both alleles typically showed severe clinical features of EBS-MD and ended up being wheelchair bound, a homozygous patient for an in-frame deletion mutation showed positive, yet attenuated, plectin expression and milder clinical phenotype. Thus plectin immunofluorescence, combined with identification of the underlying plectin mutations, is of value in predicting the severity of the muscle involvement that occurs later in life of patients with EBS-MD. [References: 22] <41> UI - 1999402929 AU - Patton LL AU - Van der Horst C IN - L.L. Patton, Department of Dental Ecology, 388 Dental Office Building, University of North Carolina, Chapel Hill, NC 27599-7450; United States. TI - Oral infections and other manifestations of HIV disease. SO - Infectious Disease Clinics of North America Vol 13(4) (pp 879-900), 1999. AB - Oral lesions are important in the clinical spectrum of HIV/AIDS, arousing suspicion of acute seroconversion illness (aphthous ulceration and candidiasis), suggesting HIV infection in the undiagnosed individual (candidiasis, hairy leukoplakia, Kaposi's sarcoma, necrotizing ulcerative gingivitis), indicating clinical disease progression and predicting development of AIDS (candidiasis, hairy leukoplakia), and marking immune suppression in HIV-infected individuals (candidiasis, hairy leukoplakia, necrotizing periodontal disease, Kaposi's sarcoma, long-standing herpes infection, major aphthous ulcers). In addition, oral lesions are included in staging systems for HIV disease progressions and as entry criteria or endpoints in clinical trials of antiretroviral drugs. Recognition and management of these oral conditions is important for the health and quality of life of the individual with HIV/AIDS. In keeping with this, the U.S. Department of Health Services Clinical Practice Guideline for Evaluation and Management of Early HIV Infection includes recommendations that an oral examination, emphasizing oral mucosal surfaces, be conducted by the primary care provider at each visit, a dental examination by a dentist should be done at least two times a year, and patients should be informed of the importance of oral care and educated about common HIV-related oral lesions and associated symptoms. [References: 138] <42> UI - 1999398682 AU - Lin Y-TJ AU - Tsai C-L IN - Dr. Y.-T.J. Lin, Department of Dentistry, Chang Gung Memorial Hospital, Kaohsiung Medical Center, 123, Ta Pei Rd, Niao Sung Hsiang, Kaohsiung Hsien; Taiwan. TI - Caries prevalence and bottle-feeding practices in 2-year-old children with cleft lip, cleft palate, or both in Taiwan. SO - Cleft Palate-Craniofacial Journal Vol 36(6) (pp 522-526), 1999. AB - Objective: The purposes of this study were to investigate the caries prevalence in cleft lip, cleft palate, or both in children under the age of 2 years and to evaluate parental attitudes toward bottle-feeding, dental care, and their relationship to baby bottle tooth decay (BBTD) in Taiwan. Design: Randomized and prospective study. Setting: Institutional setting. Patients and Methods: One hundred twenty-three 2-year-old children (68 boys and 55 girls) with cleft lip, cleft palate, or both were selected for this study. A questionnaire that asked questions about knowledge of oral health, knowledge and beliefs about BBTD, children's feeding habits, children's dental care, and parenting attitudes toward children with clefts was completed by the parents or caretakers. Children were divided into bottle-feeding and non- bottle-feeding groups according to the questionnaire responses of parents or caretakers. Each child was examined with a dental mirror and explorer under focused flashlight using defs index to determine the presence of BBTD. Results: Thirty-nine percent (48) subjects reported a bottle-feeding habit; the overall prevalence of BBTD was 15.4%. The habit of bottle-feeding was significantly related to BBTD (p = .019). The defs score for children who were bottle-fed was significantly higher than children who were not bottle- fed (p = .045). Parents or caretakers of both bottle-feeding and non-bottle- feeding children showed no significant differences in their attitudes toward bottle-feeding and feeding habits (p > .05). However, parents of non-bottle- fed children had significantly better dental care than parents of bottle-fed children in brushing frequency (p < .001) and brushing before bed (p < .001). Conclusions: Children with clefts who took a bottle to bed showed an increased risk of developing BBTD. The parents or caretakers of bottle-fed children also showed a lack of motivation to perform regular preventive dental home care for their children. This suggests that oral health promotion programs should begin in infancy for children with clefts and their parents. [References: 21] <43> UI - 1999390858 AU - Ramon-Fluixa C AU - Bagan-Sebastian JV AU - Milian-Masanet MA AU - Scully C IN - Prof. J.V. Bagan-Sebastian, Hospital General Universitario, Servicio de Estomatologia, Avda. Tres Cruces, s/n., 46014-Valencia; Spain. TI - Periodontal status in patients with oral lichen planus: A study of 90 cases. SO - Oral Diseases Vol 5(4) (pp 303-306), 1999. AB - OBJECTIVE: This study was carried out to relate periodontal status to the oral lesions in patients with oral lichen planus (OLP). MATERIALS AND METHODS: Periodontal status was evaluated in 90 patients with OLP and in 52 controls, in terms of the plaque index (PII), simplified calculus index (CIS) and periodontal disease index (PDI). RESULTS: No significant differences were observed between the two groups as regards the different periodontal indices. The plaque and calculus indices were higher in the more extensive forms of OLP (P = 0.02 and P = 0.012, respectively), and in the presence of gingival involvement (P = 0.004 and P = 0.04). A significant association was also observed between the presence of atrophic-erosive lesions and increased periodontal deterioration (P = 0.037). CONCLUSIONS: Increased plaque and calculus deposits are associated to a significantly higher incidence of atrophic-erosive gingival lesions in patients with OLP. [References: 53] <44> UI - 1999390316 AU - Zhou G AU - Chen Y AU - Zhou L AU - Thirunavukkarasu K AU - Hecht J AU - Chitayat D AU - Gelb BD AU - Pirinen S AU - Berry SA AU - Greenberg CR AU - Karsenty G AU - Lee B IN - B. Lee, Department Molecular Human Genetics, Baylor College of Medicine, BCM225, One Baylor Plaza, Houston, TX 77030; United States. E-Mail: blee@bcm.tmc.edu. TI - CBFA1 mutation analysis and functional correlation with phenotypic variability in cleidocranial dysplasia. SO - Human Molecular Genetics Vol 8(12) (pp 2311-2316), 1999. AB - Cleidocranial dysplasia (CCD) is a dominantly inherited skeletal dysplasia caused by mutations in the osteoblast-specific transcription factor CBFA1. To correlate CBFA1 mutations in different functional domains with the CCD clinical spectrum, we studied 26 independent cases of CCD and a total of 16 new mutations were identified in 17 families. The majority of mutations were de novo missense mutations that affected conserved residues in the runt domain and completely abolished both DNA binding and transactivation of a reporter gene. These, and mutations which result in premature termination in the runt domain, produced a classic CCD phenotype by abolishing transactivation of the mutant protein with consequent haploinsufficiency. We further identified three putative hypomorphic mutations (R391X, T200A and 90insC) which result in a clinical spectrum including classic and mild CCD, as well as an isolated dental phenotype characterized by delayed eruption of permanent teeth. Functional studies show that two of the three mutations were hypomorphic in nature and two were associated with significant intrafamilial variable expressivity, including isolated dental anomalies without the skeletal features of CCD. Together these data show that variable loss of function due to alterations in the runt and PST domains of CBFA1 may give rise to clinical variability, including classic CCD, mild CCD and isolated primary dental anomalies. [References: 29] <45> UI - 1999390074 AU - Cartwright AR AU - Kula K AU - Wright TJ IN - T.J. Wright, Department of Pediatric Dentistry, School of Dentistry, CB 7450, The University of North Carolina, Chapel Hill, NC 27599-7450; United States. E-Mail: twright@email.unc.edu. TI - Craniofacial features associated with amelogenesis imperfecta. SO - Journal of Craniofacial Genetics & Developmental Biology Vol 19(3) (pp 148-156), 1999. AB - Craniofacial alterations occur with increased frequency in patients with amelogenesis imperfecta (AI). The purpose of this study was to characterize the craniofacial features associated with AI in families from the US. Twenty-seven people with AI and 14 unaffected family members from nine separate kindreds were evaluated. The diagnosis was established by history, clinical, and radiographic examination, and histological and/or biochemical analysis of enamel. The kindreds were generally classified as hypoplastic AI (HPAI), hypocalcified AI (HCAI), or hypomaturation AI (HMAI) and then further subclassified based on phenotype and mode of inheritance. Linear and angular cephalometric measures were converted to z-scores using gender/age matched values from the Bolton and Behrent's standards. Statistical analyses included t-tests and ANOVA accepting P <= 0.05 as significant. The vertical dimension of the lower face was significantly increased (ANSMe; P = 0.001), especially in affected individuals compared with unaffected relatives, in all kindreds with HCAI and HMAI but in only one kindred with autosomal recessive rough AI. Clinically, an anterior open bite (overbite < 0 mm) was observed in 26% of all dentate individuals with AI and none of their unaffected relatives. Skeletal morphology was highly variable depending on the AI type and kindred. While this study shows an association of altered craniofacial morphology with certain AI kindreds, the relationship of the AI genotype to the observed malocclusions remains to be defined. [References: 34] <46> UI - 1999386744 AU - Moog U AU - Maroteaux P AU - Schrander-Stumpel CTRM AU - Van Ooij A AU - Schrander JJP AU - Fryns JP IN - Dr. U. Moog, Department of Clinical Genetics, Maastricht University, PO Box 1475, 6201 BL Maastricht; Netherlands. TI - Two sibs with an unusual pattern of skeletal malformations resembling osteogenesis imperfecta: A new type of skeletal dysplasia?. SO - Journal of Medical Genetics Vol 36(11) (pp 856-858), 1999. AB - We report a 6 year old boy with multiple fractures owing to bilateral, peculiar, wave-like defects of the tibial corticalis with alternative hyperostosis and thinning. Furthermore, he had Wormian bones of the skull, dentinogenesis imperfecta, and a distinct facial phenotype with hypertelorism and periorbital fullness. Collagen studies showed normal results. His sister, aged 2 years, showed the same facial phenotype and dental abnormalities as well as Wormian bones, but no radiographical abnormalities of the tubular bones so far. The mother also had dentine abnormalities but no skeletal abnormalities on x ray. This entity is probably the same as that described in a sporadic case by Suarez and Stickler in 1974. In spite of the considerable overlap with osteogenesis imperfecta (bone fragility, Wormian bones, and dentinogenesis imperfecta), we believe this disorder to be a different entity, in particular because of the unique cortical defects, missing osteopenia, and normal results of collagen studies. [References: 11] <47> UI - 1999342979 AU - Kozma C AU - Chong SS AU - Meck JM IN - Dr. C. Kozma, Child Development Center, Georgetown University Medical Center, 3307 M Street, Washington, DC 20007-3935; United States. TI - Interstitial deletion of 4p15.32p16.3 in a boy with minor anomalies, hearing loss, borderline intelligence, and oligodontia. SO - American Journal of Medical Genetics Vol 86(4) (pp 316-320), 1999. AB - We describe an 11-year-old boy of Saudi origin with an interstitial deletion in the short arm of chromosome 4 (p15.32p16.3) as determined by G- banding and fluorescent in situ hybridization. His clinical manifestations Were similar but not identical to previously reported cases of interstitial deletion in the same chromosomal region, and were not those associated with Wolf-Hirschhorn syndrome. The boy had normal facial characteristics, short stature, minor anomalies of hands and feet, amblyopia of the right eye, bilateral hearing loss, and hypotonia. On developmental testing, he had borderline intelligence, with a severe sensory integration and motor planning disorder, and severe deficits in the communication domain. In addition, he had severe oligodontia affecting his secondary dentition. This finding supports the presence of one or more genes involved in dentition in this chromosomal region. [References: 19] <48> UI - 1999333864 AU - Raney RB AU - Asmar L AU - Vassilopoulou-Sellin R AU - Klein MJ AU - Donaldson SS AU - Green J AU - Heyn R AU - Wharam M AU - Glicksman AS AU - Gehan EA AU - Anderson J AU - Maurer HM IN - Dr. R.B. Raney, Department of Pediatrics, Box 87, Texas Univ. M.D. Anderson Can. Ctr., 1515 Holcombe Blvd., Houston, TX 77030; United States. TI - Late complications of therapy in 213 children with localized, nonorbital soft-tissue sarcoma of the head and neck: A descriptive report from the intergroup rhabdomyosarcoma studies (IRS)-II and - III. SO - Medical & Pediatric Oncology Vol 33(4) (pp 362-371), 1999. AB - Background. This review of children and adolescents with nonorbital soft-tissue sarcoma of the head and neck was undertaken to describe late sequelae of treatment, as manifested primarily by problems with statural growth, facial and nuchal symmetry, dentition, vision and hearing, and school performance. Procedure. Four hundred sixty-nine patients entered the IRS-II and -III protocols with localized, nonorbital soft-tissue sarcomas of the head and neck from 1978 through 1987. Their overall survival rate was 53% (250/469) at 5 years. Two hundred thirteen patients were surviving relapse- free 5 or more years after diagnosis, for whom there were serial height measurements at 2 or more years after initiation of therapy. Their median age at diagnosis was 5 years: the median length of follow-up was 7 years. All received multiple-agent chemotherapy, and all but 3 received irradiation to the primary tumor volume. Sixty-eight percent of the tumors arose in cranial parameningeal sites, 22% in nonparameningeal sites, and 10% in the neck. We reviewed flow sheets submitted to the IRS Group Statistical Office to ascertain which late sequelae were recorded. Results. One hundred sixty-four patients (77%) had one or more problems recorded. One hundred ninety of the two hundred thirteen patients (89%) were under 15 years of age at study entry, and at follow-up 92 (48%) had failed to maintain their initial height velocity, which had decreased by more than 25 percentile points from the original value. Thirty-six of the one hundred ninety patients (19%) were receiving growth hormone injections. Hypoplasia or asymmetry of tissues in the primary tumor site was reported in 74 patients, and 13 underwent reconstructive surgery. Poor dentition or malformed teeth were noted in 61 patients. Impaired vision developed in 37 patients, owing primarily to cataracts, corneal changes, and optic atrophy. Thirty-six patients had decreased hearing acuity, and 9 were fitted with hearing aids; 5 of these 9 had received cisplatin. Thirty-five patients were noted to have problems learning in school. Four patients developed a second malignancy (two sarcomas, one carcinoma, one leukemia). Conclusions. Late sequelae affected the majority of these patients treated for soft-tissue sarcoma of the head and neck on IRS-II and -III. The potential impact of certain sequelae could be reduced by specific measures, such as surgical reconstruction and hormonal therapy. Late sequelae must be taken into account in designing future curative treatments. [References: 29] <49> UI - 1999329205 AU - Anonymous TI - Linear gingival erythema. SO - Consultant Vol 38(1) (pp 200), 1998. <50> UI - 1999321923 AU - Mhanni AA AU - Cross HG AU - Chudley AE IN - Dr. A.E. Chudley, Section of Genetics and Metabolism, Children's Hospital, 840 Sherbrook St., Winnipeg, Man. R3A 1S1; Canada. E-Mail: chudley@cc.umanitoba.ca. TI - Kabuki syndrome: Description of dental findings in 8 patients. SO - Clinical Genetics Vol 56(2) (pp 154-157), 1999. AB - The cardinal features of Kabuki (Niikawa-Kuroki) syndrome (KS) include characteristic facial dysmorphic features, mild to moderate mental deficiency, skeletal abnormalities, dermatoglyphic abnormalities, and postnatal growth retardation. We identified 8 patients with KS in a genetics clinic over the past 5 years. All were Caucasians, except for 2 who were of mixed Aboriginal and Caucasian descent. All had the facial gestalt, the dermatoglyphic abnormalities characteristic of the syndrome, and developmental delay. Dental abnormalities of permanent teeth were seen in all 8 cases; 6 had missing lower incisors. Five patients had uniquely abnormal upper incisor teeth shape; the upper incisors had a 'flat head' screwdriver-shaped appearance. Other dental abnormalities included missing lower lateral incisors, missing second premolars, and ectopic upper 6-year molars. We believe the presence of the unique dental findings will prove useful in the diagnostic assessment of individuals with KS. [References: 14] <51> UI - 1999313107 AU - Delattre VF AU - Stimson PG IN - V.F. Delattre, University of Texas Dental Branch, 6516 John Freeman Avenue, Houston, TX 77030; United States. TI - Self-assessment of the forensic value of dental records. SO - Journal of Forensic Sciences Vol 44(5) (pp 906-909), 1999. AB - One of the most important aspects of a person's dental record may well prove to be it's potential value should the forensic dental identification of their remains become necessary. The better the quality of the antemortem dental records, the easier and faster the identification of the remains will be. The forensic dentist must be able to select identifying features by decoding the deceased's antemortem dental records. A study was conducted on two groups of dentists who were asked to self-assess the forensic dental value of the dental records maintained in their own practices. The three most frequently recorded identifying dental features, other than caries and restorations, were the presence of diastemas, displaced or rotated teeth, and dental anomalies. Surveyed dentists imbedded identifying information into the removable prosthetic devices fabricated for their patients an average of only 64% of the time. Only 56% of the two groups combined felt that their dental chartings and written records would be extremely useful in dental identifications. It is concluded that the quality of antemortem dental records available for comparison to postmortem remains varies from inadequate to extremely useful. Practicing dentists can become valuable members of the dental identification process by developing and maintaining standards of record keeping which would be valuable in restoring their patients' identity. [References: 2] <52> UI - 1999311955 AU - Lovell NC AU - Whyte I IN - N.C. Lovell, Department of Anthropology, 13-15 Tory Building, University of Alberta, Edmonton, Alta. T6G 2H4; Canada. E-Mail: Nancy.Lovell@ualberta.ca. TI - Patterns of dental enamel defects at ancient Mendes, Egypt. SO - American Journal of Physical Anthropology Vol 110(1) (pp 69-80), 1999. AB - The dental remains of 88 individuals from Old Kingdom, First Intermediate, and Greco-Roman periods at the ancient Egyptian site of Mendes (Tell er-Rub(c)a) were examined for dental enamel hypoplasia, and the results reported here provide some of the first comparative data on enamel defects in ancient Egypt. Overall, 48% of the individuals in the sample have one or more teeth with hypoplasia, with 17% of permanent teeth and 8% of deciduous teeth affected. The permanent teeth account for 87% of the total number of affected teeth, a prevalence over deciduous teeth that is significant at alpha = 0.05. Permanent and deciduous teeth display different patterns of hypoplasia, with the former exhibiting both discrete pitting and linear furrowing, and the latter exhibiting only pits. Teeth with linear defects significantly outnumber those with pits by a factor of more than three to one. Only permanent canines display more than one lesion on a tooth, with a mean of 1.4 defects per affected tooth. Although calculation of the age of insult from lesion position is imprecise, it appears that stress episodes occurred most commonly between approximately 3-5 years of age. The presence of pits in the deciduous dentition, however, suggests that physiological stresses began in utero. There is no statistically significant difference in the frequency of enamel defects between males and females. An observed decrease in the frequency of defects from the Old Kingdom period to the subsequent First Intermediate and Greco-Roman periods is not significant at alpha = 0.05, although such a decrease is expected given epigraphic and other data that refer to prolonged drought and malnutrition in the late Old Kingdom. The calculated chi2 value of 3.83 is significant at the 0.10 level, however, and since our sample is rather small and the magnitude of the chi-square statistic is a function of sample size, we recommend that future research investigate further the relationship between the frequency of enamel defects and the time period in which they occur. [References: 67] <53> UI - 1999303318 AU - Nakamura Y AU - Hossain M AU - Hirayama K AU - Matsumoto K IN - Dr. Y. Nakamura, Department of Endodontics, School of Dentistry, Showa University, 2-1-1, Kitasenzoku, Ohta-ku, Tokyo 145-8515; Japan. E-Mail: yukio@senzoku.showa-u.ac.jp. TI - A clinical study on the removal of gingival melanin pigmentation with the CO2 laser. SO - Lasers in Surgery & Medicine Vol 25(2) (pp 140-147), 1999. AB - Background and Objective: In a previous study, the possibility of removal of dog gingival melanin pigmentation with CO2 laser therapy was reported. The present study was designed to investigate the effect of the CO2 laser on human gingival pigmentation and evaluate the clinical outcome. Study Design/Materials and Methods: A CO2 laser (output: 6-8 W, pulse duration: 0.2 seconds) was irradiated on the melanin pigmented gingival surface of 10 patients, aged 20-49 years. Followup clinical and histopathological evaluations were performed. Results: The CO2 laser was effective in removing melanin pigmentation in all patients. In the histopathological study, no pigmented-laden cells nor any inflammatory cell infiltration was observed following laser irradiation. No re-pigmentation was seen in any case in the first year. However, four of seven cases showed re- pigmentation at 24 months. The re-pigmentation was almost equal to the preoperative state. Conclusions: The CO2 laser has proved to be another effective, safe, and easily applicable therapy for the removal of gingival melanin pigmentation. [References: 20] <54> UI - 1999301989 AU - Ozeki H AU - Shirai S AU - Ikeda K AU - Ogura Y IN - H. Ozeki, Department of Ophthalmology, Nagoya City Univ. Medical School, 1-Kawasumi, Mizuho-ku, Nagoya 467-8601; Japan. E-Mail: ozeki@med.nagoya-cu.ac.jp. TI - Anomalies associated with Axenfeld-Rieger syndrome. SO - Graefes Archive for Clinical & Experimental Ophthalmology Vol 237(9) (pp 730-734), 1999. AB - Background: To detect the associated anomalies in patients with Axenfeld-Rieger syndrome is clinically important, because early treatment for such anomalies is crucial to both visual and systemic development. This study was conducted to clarify the associated anomalies in the syndrome. Methods: We evaluated 21 patients with Axenfeld-Rieger syndrome encountered at Nagoya City University Hospital over a 16-year period. Patients who presented with a prominent Schwalbe's line accompanying the iris strands were diagnosed as having Axenfeld-Rieger syndrome. Results: The series consisted of 9 males and 12 females, ranging in age from 1 month to 41 years, mean 15.4 +/- 12.7 (SD) years. The syndrome was bilateral in 17 cases and unilateral in 4 cases. Hypoplasia of the iris was observed in 10 eyes of 6 patients. The associated ocular anomalies included sclerocornea in 6 eyes of 3 patients, developmental glaucoma in 5 eyes of 3 patients, persistent pupillary membrane in 4 eyes of 2 patients, microphthalmos in 3 eyes of 2 patients, and typical iris coloboma in 1 eye. Of 10 eyes with hypoplasia of the iris, 5 exhibited glaucoma. The accompanying systemic anomalies included 9 cases of dental anomalies, 5 of facial anomalies, and 3 of Alagille syndrome. Conclusions: All of the associated ocular and systemic anomalies appeared to arise from the maldevelopment of the neural crest cells. Patients with Axenfeld-Rieger syndrome should therefore be examined for the presence of anomalies in the tissues of neural crest origin. Patients with hypoplasia of the iris should be checked for glaucoma. [References: 20] <55> UI - 1999291835 AU - Silva VB AU - Simoes AM AU - Marques-de-Faria AP IN - Dr. A.M. Simoes, Faculdade de Ciencias Medicas, Dept. Oftalmologia H.C. UNICAMP, Cidade Universitaria 'Zeferino Vaz', CP 61111, Campinas, Sao Paulo, CEP 13081-970; Brazil. E-Mail: simoestorigoe@uol.com.br. TI - EEM syndrome: Report of a family and results of a ten-year follow-up. SO - Ophthalmic Genetics Vol 20(2) (pp 95-99), 1999. AB - We report on a Brazilian kindred in which two sibs presented with the complete form of EEM (ectodermal dysplasia, ectrodactyly and macular dystrophy) syndrome with hypotrichosis, dental anomalies, syndactyly, and retinal changes with prominent pigmentation in the posterior pole of the retina. In this family, we also observed another sib with syndactyly, as well as a first cousin with ectrodactyly. A 10-year follow-up demonstrated gradually decreasing visual acuity and progression of retinal degenerative anomalies. [References: 5] <56> UI - 1999290027 AU - Anonymous TI - Nonsyphilitic dental dysplasia. SO - Consultant Vol 39(7) (pp 2132), 1999. <57> UI - 1999287388 AU - Taieb A IN - Prof. A. Taieb, Unite de Dermatologie Pediatrique, Hopital Pellegrin-Enfants, Bordeaux; France. TI - X-linked hypohidrotic ectodermal dysplasia in the newborn: A diagnostic challenge. SO - European Journal of Pediatric Dermatology Vol 8(4) (pp 201-204), 1998. AB - X-linked hypohidrotic ectodermal dysplasia is the most common phenotype in the group of ectodermal dysplasia. It is characterized by hypohidrosis, hypodontia and hypotrichosis. The Author underlines the need for an early diagnosis, namely in the neonatal period, of this disorder. The latter is in fact particularly dangerous in this period, when not recognized, due to the impaired thermolysis. Unfortunately, the most characteristic signs of the disorder, namely hypotrichosis and hypodontia, are not obvious at this age. Therefore, the diagnosis should be suspected at this age in case of minor signs such as unexplained fever, collodion-like membranes, prominent forehead, periorbital darkening and saddle nose in a male infant. The carrier mother usually presents minor dental and hair problems. [References: 6] <58> UI - 1999263766 AU - Price JA AU - Wright JT AU - Walker SJ AU - Crawford PJM AU - Aldred MJ AU - Hart TC IN - Dr. T.C. Hart, Department of Pediatrics, Wake Forest University, School of Medicine, Medical Center Boulevard, Winston-Salem, NC 27157; United States. E-Mail: thart@wfubmc.edu. TI - Tricho-dento-osseous syndrome and amelogenesis inperfecta with taurodontism are genetically distinct conditions. SO - Clinical Genetics Vol 56(1) (pp 35-40), 1999. AB - Amelogenesis imperfecta of the hypomaturation-hypoplasia type with taurodontism (AIHHT) is inherited as a highly penetrant autosomal dominant trait. These dental findings are similar to those of another autosomal dominant condition, the tricho-dento-osseous syndrome (TDO), from which AIHHT differs primarily by lack of changes in the hair and bones. TDO is characterized by a highly variable clinical phenotype. While enamel hypoplasia and taurodontism appear to be present in all TDO cases, non-dental features may be absent, with approximately half of TDO cases losing the kinky/curly hair phenotype seen in infancy by adolescence, and in almost 20% of cases, osseous changes are not evident. The genetic basis for AIHHT is unknown and it has been questioned whether AIHHT and TDO are separate conditions or a spectrum of disease. The genetic basis for TDO has recently been identified as a deletion mutation in the distal-less 3 (DLX3) transcription factor gene. To determine if AIHHT and TDO represent variable expression of a common DLX3 gene mutation, allelic mutations of the DLX3 gene, or mutations in DLX7 (the linked paralogue to DLX3 on chromosome 17), we have performed mutational analysis and sequencing studies of the DLX3 and DLX7 genes in three individuals (two affected and one unaffected) from a family with AIHHT. Results of the analysis demonstrate that AIHHT and TDO are not due to a common DLX3 gene mutation. Sequence analyses of the DLX3 and DLX7 genes suggest AIHHT is not due to genetic mutations or polymorphisms in the exons of these genes. These results suggest that AIHHT and TDO are two genetically distinct conditions. [References: 19] <59> UI - 1999235512 AU - Schultes G AU - Gaggl A AU - Karcher H IN - Dr. G. Schultes, Clinical Department of OMS, Auenbruggerplatz 7, A-8036 Graz; Austria. TI - Comparison of periodontal disease in patients with clefts of palate and patients with unilateral clefts of lip, palate, and alveolus. SO - Cleft Palate-Craniofacial Journal Vol 36(4) (pp 322-327), 1999. AB - Objective: Long-term health of the stomatognathic system as well as esthetic aspects are the therapeutic goals in patients with orofacial clefts. The aim of this study was to analyze the periodontal condition of patients with cleft palate (CP) and cleft lip, palate, and alveolus (CLP) and to determine the differing degrees and localization of periodontal disease. Design: In 30 patients with unilateral cleft lip, palate, and alveolus and 30 patients with cleft palate (CP), periodontal lesions were identified and classified according to the Community Periodontal Index of Treatment Needs (CPITN) and pathological mobility of teeth was noted. The state of oral hygiene was recorded by the Approximal Plaque Index. Results: In general, poor oral hygiene was found in all patients. Patients with CLP were classified as CPITN code 0 in 0%, codes 1 and 2 in 7%, code 3 in 43%, and code 4 in 50% of cases. Patients with cleft palate showed code 0 in 0%, codes 1 and 2 in 30%, code 3 in 45%, and code 4 in 25% of cases. In this study, periodontal disease was found in patients with cleft palate to a similar extent to that in the general population, whereas patients with cleft lip, palate, and alveolus had a predisposition to deep peridontal destruction of teeth adjacent to the cleft. The registration of pathological mobility of teeth and resulting loss of attachment corresponded to the periodontal disease classification according to the CPITN. Conclusion: A critical periodontal situation was found in patients with unilateral cleft lip, palate, and alveolus. In contrast, patients with cleft palate exhibited a similar periodontal situation to that found in the general population, with additional damage that may be attributed to orthodontic treatment. [References: 44] <60> UI - 1999237639 AU - Kreiborg S AU - Jensen BL AU - Larsen P AU - Schleidt DT AU - Darvann T IN - Prof. S. Kreiborg, Department of Pediatric Dentistry, School of Dentistry, University of Copenhagen, Norre Alle 20, DK-2200 Copenhagen N; Denmark. TI - Anomalies of craniofacial skeleton and teeth in cleidocranial dysplasia. SO - Journal of Craniofacial Genetics & Developmental Biology Vol 19(2) (pp 75-79), 1999. AB - Mutations involving the transcription factor CBFA1 cause cleidocranial dysplasia (CCD) in man. Recently, a mouse model of CCD has been generated (Cbfa1 +/-), and disturbances of osteoclast differentiation have been documented. It has been shown that these animals exhibit hypoplastic clavicles and nasal bones, and retarded ossification of parietal, interparietal, and supraoccipital bones. Humans with CCD show all these features, including severely retarded ossification of the cranial base, strongly suggesting that both intramembranous ossification and endochondral ossification are affected. In addition, CCD patients have multiple supernumerary teeth and delayed tooth eruption. The present report presents 3D reconstructions of computerised tomography (CT) scans of the craniofacial region of a CCD boy examined at both 1 and 7 years of age. The anomalies in craniofacial skeleton and teeth are analysed and compared to the findings of our previous clinical studies and to the findings in the animal model. Based on the available information, we suggest that osteoblast, osteoclast, and dentinoclast differentiation may be disturbed in CCD. [References: 13] <61> UI - 1999231908 AU - Hori R AU - Sato M AU - Kohno S AU - Hoshino E IN - E. Hoshino, Department of Oral Microbiology, Niigata University Sch. of Dentistry, Niigata; Japan. E-Mail: hoshino@dent.niigata-u.ac.jp. TI - Tongue microflora in edentulous geriatric denture-wearers. SO - Microbial Ecology in Health & Disease Vol 11(2) (pp 89-95), 1999. AB - The aim of this study was to investigate the bacterial composition of tongue plaque of healthy edentate geriatric individuals wearing dentures. One male of 67 years-old and four females ranging from 68 to 73 years-old were involved. Plaque was obtained from an area of 10 mm2 on the dorsal surface at the anterior two-thirds of the tongue. Total numbers of colony-forming units (CFU) were determined and the predominant bacteria were isolated for identification. Significantly more bacteria (p < 0.05) were recovered after anaerobic incubation (mean; 2.0 x 107 CFU/mg) than after aerobic (mean; 1.1 x 107 CFU/mg) or microaerophilic (mean; 1.0 x 107 CFU/mg) incubation. Out of 210 predominant strains isolated, 33% were obligate anaerobes and 66% were facultatively anaerobic. Veillonella (8% of total isolates) and Streptococcus (35% of total isolates) were the most major genera identified among obligate and facultative anaerobes, respectively. Actinomyces strains represented 27% of total isolates. Gram negative anaerobic rods and asaccharolytic Eubacterium were also detected, comprising 14% and 3% of total isolates, respectively. The bacterial composition of the tongue was somewhat similar to that of saliva and denture plaque reported in geriatric edentulous persons, suggesting that tongue plaque in geriatric edentulous persons who wear dentures may function as a major bacterial reservoir. [References: 33] <62> UI - 1999230693 AU - Geis GS IN - G.S. Geis, G. D. Searle and Co., 4901 Searle Parkway, Skokie, IL 60077; United States. TI - Update on clinical developments with celecoxib, a new specific COX-2 inhibitor: What can we expect?. SO - Scandinavian Journal of Rheumatology - Supplement Vol 28(109) (pp 31-37), 1999. AB - Nonsteroidal anti-inflammatory drugs (NSAIDs) are effective for the relief of pain and inflammation, yet their use is tempered by the development of side effects, primarily in the gastrointestinal (GI) tract. It is now known that inhibition of the enzyme cyclooxygenase (COX) is the principal mechanism for both the efficacy and the toxicity of NSAIDs. Recent research has shown that COX exists as at least two isoenzymes, COX-1 and COX-2. Compelling evidence suggests that COX-1 synthesizes prostaglandins that are involved in the regulation of normal cell activity (including GI cytoprotection), whereas COX-2 appears to produce prostaglandins mainly at sites of inflammation. These findings led to the search for compounds that would inhibit COX-2 without affecting COX-1. Several agents are under investigation in this new therapeutic category, including celecoxib (SC-58635). Celecoxib was developed as an anti-inflammatory and analgesic agent, and has been studied in preclinical studies and in clinical trials. This paper focuses on the results of five key clinical trials of celecoxib: an efficacy trial in dental pain, a 2-week osteoarthritis (OA) efficacy trial, a 4-week rheumatoid arthritis (RA) efficacy trial, a 1-week endoscopic study of GI mucosal effects, and a 10-day study of effects on platelet function. The arthritis trials identified celecoxib doses that were effective in treating OA and RA and that were distinguished from placebo on standard arthritis scales. In the upper GI endoscopy study, no ulcers occurred in subjects receiving celecoxib or placebo, whereas 19% of subjects receiving naproxen developed gastric ulcers. In the platelet effects trial, no statistically significant difference from placebo was seen in the effect of celecoxib on platelet aggregation or bleeding time. In contrast, naproxen caused statistically significant reductions in platelet aggregation and a statistically significant increase in bleeding time. These preliminary trials show that celecoxib achieves analgesic and anti-inflammatory efficacy in arthritis through specific COX-2 inhibition without showing evidence of two of the toxic effects of COX-1 inhibition associated with NSAIDs. [References: 19] <63> UI - 1999215839 AU - Walpole SM AU - Ronce N AU - Grayson C AU - Dessay B AU - Yates JRW AU - Trump D AU - Toutain A IN - D. Trump, Department of Medical Genetics, Cambridge Institute Medical Research, Addenbrooke's Hospital, Cambridge CB2 2XY; United Kingdom. E-Mail: dorothy.trump@cimr.cam.ac.uk. TI - Exclusion of RAI2 as the causative gene for Nance-Horan syndrome. SO - Human Genetics Vol 104(5) (pp 410-411), 1999. AB - Nance-Horan syndrome (NHS) is an X-linked condition characterised by congenital cataracts, microphthalmia and/or microcornea, unusual dental morphology, dysmorphic facial features, and developmental delay in some cases. Recent linkage studies have mapped the NHS disease gene to a 3.5-cM interval on Xp22.2 between DXS1053 and DXS443. We previously identified a human homologue of a mouse retinoic-acid-induced gene (RAI2) within the NHS critical flanking interval and have tested the gene as a candidate for Nance-Horan syndrome in nine NHS-affected families. Direct sequencing of the RAI2 gene and predicted promoter region has revealed no mutations in the families screened; RAI2 is therefore unlikely to be associated with NHS. [References: 6] <64> UI - 1999213518 AU - Shiboski CH AU - Palacio H AU - Neuhaus JM AU - Greenblatt RM IN - Dr. C.H. Shiboski, Department of Stomatology, Box 0422, University of California, 513 Parnassus Ave., San Francisco, CA 94143-0422; United States. E-Mail: caro@nanook.ucsf.edu. TI - Dental care access and use among HIV-infected women. SO - American Journal of Public Health Vol 89(6) (pp 834-839), 1999. AB - Objectives. This study sought to identify predictors of dental care use in HIV-infected women. Methods. In a cross-sectional survey of HIV-infected women enrolled in the northern California site of the Women's Interagency HIV Study, dental care use and unmet need were assessed relation to selected variables. Results. Among 213 respondents, who were predominantly Black and younger than 45 years, 43% had not seen a dentist and 53% (among dentate women) reported no dental cleaning in more than a year (although 67% had dental insurance coverage, mainly state Medicaid). Nine percent were edentulous. Among nonusers of dental care, 78% reported that they wanted care but failed to get it. Barriers included fear of and discomfort with dentists, not getting around to making an appointment, and not knowing which dentist to visit. Multivariate analysis showed that lack of past-year dental care was associated mainly with unemployment, a perception of poor oral health, and edentulism. Conclusions. HIV-positive women appear to be underusing dental care services. Fear and lack of information regarding available resources, in addition to unemployment and perception of poor oral health, may be important barriers. [References: 28] <65> UI - 1999202997 AU - Beales PL AU - Elcioglu N AU - Woolf AS AU - Parker D AU - Flinter FA IN - Dr. P.L. Beales, Dept. Medical and Molecular Genetics, 8th Floor Guy's Tower, Guy's Hospital, London SE1 9RT; United Kingdom. TI - New criteria for improved diagnosis of Bardet-Biedl syndrome: Results of a population survey. SO - Journal of Medical Genetics Vol 36(6) (pp 437-446), 1999. AB - Bardet-Biedl syndrome (BBS) is an autosomal recessive condition characterised by rod-cone dystrophy, postaxial polydactyly, central obesity, mental retardation, hypogonadism, and renal dysfunction. BBS expression varies both within and between families and diagnosis is often difficult. We sought to define the condition more clearly by studying 109 BBS patients and their families, the largest population surveyed to date. The average age at diagnosis was 9 years, which is late for such a debilitating condition, but the slow development of the clinical features of BBS probably accounts for this. Postaxial polydactyly had been present in 69% of patients at birth, but obesity had only begun to develop at around 2-3 years, and retinal degeneration had not become apparent until a mean age of 8.5 years. Our study identified some novel clinical features, including neurological, speech, and language deficits, behavioural traits, facial dysmorphism, and dental anomalies. In the light of these features we propose a revision of the diagnostic criteria, which may facilitate earlier diagnosis of this disorder. We present evidence for an overlapping phenotype with the Laurence-Moon syndrome and propose a unifying, descriptive label be adopted (polydactyly-obesity-kidney-eye syndrome). We report an increased prevalence of renal malformations and renal cell carcinoma in the unaffected relatives of BBS patients and suggest that these may be a consequence of heterozygosity for BBS genes. Our findings have important implications for the care of BBS patients and their unaffected relatives. [References: 37] <66> UI - 1999200091 AU - Mjor IA IN - I.A. Mjor, University of Florida, PO Box 100415, Gainesville, FL 32610; United States. E-Mail: imjor@dental.ufl.edu. TI - Biological side effects to materials used in dentistry. SO - Journal of the Royal College of Surgeons of Edinburgh Vol 44(3) (pp 146-149), 1999. AB - This review paper outlines the potential adverse reactions that may occur through the use of dental materials. Reference is made to documented case reports of side effects. Toxic reactions to permanent dental materials are unlikely, because these materials are developed to be inert and stable and the amount of leachable components is very small. Allergic reactions, on the other hand, are feasible because they may be initiated by minute amounts of the allergen in a sensitised individual. The media in some countries have presented individual patients who allegedly have general symptoms that were relieved by the removal of restorations. A characterisation of these self-referred patients shows that many of them have mental disorders. [References: 17] <67> UI - 1999180722 AU - Jovanovic D AU - Vojinovic-Jovanovic S AU - Paravina M AU - Stanojevic M AU - Ljubenovic M AU - Slavkovic-Jovanovic M IN - Dr. D. Jovanovic, Clin. for Skin and Venereal Diseases, Clinical Center Nis B. Taskovic, 18000 Nis; Yugoslavia. TI - Dysplasio ectodermalis hypohidrotica. SO - Acta Dermatovenerologica Alpina, Panonica et Adriatica Vol 8(1) (pp 25-27), 1999. AB - Hypohidrotic ectodermal dysplasia is a rare, congenital disease. We present a child, 10-year-old boy, with dysplasio ectodermalis hypohidrotica. In addition to the characteristic physical appearance, clinical features include total congenital alopecia, reduced sweating, ichthyosis, disturbed permanent dentition, marked mental retardation, and epilepsy. The son of the child's aunt (mother's sister) has the same skin lesions, without epilepsy, the father's brother suffers from epilepsy. [References: 14] <68> UI - 1999174406 AU - Shelley WB AU - Shelley ED IN - Dr. W.B. Shelley, Division of Dermatology, Department of Medicine, Medical College of Ohio, Toledo, OH 43614; United States. TI - Chronic factitial ulcer of chin cured by endodontic (root-canal) surgery for underlying periapical abscess. SO - Journal of the American Academy of Dermatology Vol 40(5 II) (pp 802-804), 1999. AB - In a determined search for the cause of a 'factitial' ulcer of the jaw, consultation with 3 dentists was required before an underlying periapical abscess was discovered. Within 3 months of endodontic surgery, this ulcer of 12 years duration had completely healed and remains healed. Too often dental infection is neither suspected nor detected as a cause of skin disease. [References: 8] <69> UI - 1999162590 AU - Santos RV AU - Coimbra CEA Jr IN - Dr. R.V. Santos, Depto. de Endemias/ENSP/FIOCRUZ, Escola Nacional de Saude Publica, Fundacao Oswaldo Cruz, Rua Leopoldo Bulhoes 1480, Rio de Janeiro, RJ 21041-210; Brazil. E-Mail: santos@ensp.fiocruz.br. TI - Hardships of contact: Enamel hypoplasias in Tupi-Monde amerindians from the Brazilian Amazonia. SO - American Journal of Physical Anthropology Vol 109(1) (pp 111-127), 1999. AB - This paper presents an assessment of enamel defects (hypoplasias) in the permanent anterior teeth of three Tupi-Monde-speaking groups from the Brazilian Amazonia: the Gaviao, Surui, and Zoro. These are native societies that experienced the Onset of permanent contact with Brazilian national society in different periods of the 20th century. Tupi-Monde dentition is highly hypoplastic, which is possibly related to exposure to adverse health and nutritional conditions. Data for the Gaviao, Surui, and Zoro are in agreement with results from other populations that show that certain teeth, the maxillary central incisors and the mandibular canines in particular, tend to be more hypoplastic. Although all types of teeth show hypoplasia concentrations at some enamel zones, there is substantial intertooth variation in the age at which peaks occur. It is argued that hypoplasia concentrations at certain ages are unlikely to be related to postweaning stresses for the Tupi-Monde. Statistically significant associations between presence of enamel defects and deficits in physical growth (height-for-age) were detected in children 7-11 years of age. Diachronic assessment of enamel defects, which rested upon the potential of enamel as 'memory' of past periods of systemic physiological perturbation, allowed us to unravel aspects related to the dynamics of Tupi-Monde life during the 20th century. Frequencies of enamel zones with defects peaked during the contact years of each of the Tupi-Monde groups, attesting to the extreme social and biological hardships that characterized the contact experiences of these native societies with Brazilian national society. [References: 68] <70> UI - 1999158961 AU - Hennequin M AU - Faulks D AU - Veyrune J-L AU - Bourdiol P IN - M. Hennequin, Faculte de Chirurgie Dentaire, Universite d'Auvergne, 11 Boulevard Charles de Gaulle, 63000 Clermont Ferrand; France. E-Mail: martine.hennequin@u-clermont1.fr. TI - Significance of oral health in persons with Down syndrome: A literature review. SO - Developmental Medicine & Child Neurology Vol 41(4) (pp 275-283), 1999. <71> UI - 1999157001 AU - Koch MJ AU - Buhrer R AU - Pioch T AU - Scharer K IN - M.J. Koch, Department of Conservative Dentistry, Im Neuenheimer Feld 400, D-69120 Heidelburg; Germany. TI - Enamel hypoplasia of primary teeth in chronic renal failure. SO - Pediatric Nephrology Vol 13(1) (pp 68-72), 1999. AB - Chronic renal failure (CRF) in the first years of life is associated with developmental defects of enamel in the permanent dentition. We investigated if CRF also affects the primary (deciduous) dentition. Thirty-one children with CRF on conservative treatment (n = 12) or on renal replacement therapy (n = 19) underwent dental inspection. In addition, 18 CRF children provided an exfoliated deciduous tooth for microscopic examination, Enamel defects were detected in a total of 12 children (31%), either clinically or microscopically. Of the 7 children affected clinically, 6 (19% of all examined) presented localized hypoplasia of the primary canines, which was found only in 3% of healthy control children: 1 patient had generalized pitted enamel hypoplasia. By microscopy, 5 of 10 primary canines examined showed enamel hypoplasia localized exclusively in enamel formed after birth. The 'birth line,' a visible structure within the primary enamel, was always present, which excludes a prenatal onset of the defects. Of the 12 patients with an enamel defect, 9 had a documented onset of CRF within the first 7 weeks of life. We conclude that renal disease leading to CRF may affect enamel formation of primary teeth in early postnatal life, resulting in lesions different from those observed in the secondary dentition. [References: 23] <72> UI - 1999146019 AU - Cheesebrough MJ AU - Godwin P AU - Guslandi M AU - Smith AJ AU - Dickson E AU - Roy KM AU - MacKenzie D AU - Jackson MS AU - Bagg J IN - M.J. Cheesebrough, Royal Infirmary, Huddersfield HD3 3EA; United Kingdom. TI - Contributors to antibiotic resistance (multiple letters) [7]. SO - British Medical Journal 06 MAR 1999Vol 318(7184) (pp 669-670), 1999. <73> UI - 1999137495 AU - Atasu M AU - Akesib S AU - Elcioglu N AU - Yatmaz PI AU - Ertas EB IN - Dr. N. Elcioglu, Husrevpasa sk. No-40/11 Hisar ap, Faith 34250, Istanbul; Turkey. E-Mail: elcioglu@istanbul.edu.tr. TI - A Rapp-Hodgkin like syndrome in three sibs: Clinical, dental and dermatoglyphic study. SO - Clinical Dysmorphology Vol 8(2) (pp 101-110), 1999. AB - Rapp-Hodgkin ectodermal dysplasia is an autosomal dominant disorder characterized by distinctive craniofacies, cleft lip or palate, oligodontia or anodontia, hypoplasia of the nails, and a decrease in or absence of the sweat glands and hair follicles. We have identified a family in which three children display clinical features similar to Rapp-Hodgkin syndrome. The father and two other sisters of the patient had normal facial features, but had short stature and had dental anomalies, the latter suggestive of ectodermal dysplasia. The overall clinical, dental, and dermatoglyphic findings of these patients are discussed in relation to reports of families with Rapp-Hodgkin syndrome. [References: 22] <74> UI - 1999130553 AU - Morrison HI AU - Ellison LF AU - Taylor GW IN - Dr. H.I. Morrison, Cancer Bureau, Laboratory Centre for Dis. Control, Tunney's Pasture, Ottawa, Ont. K1A 0L2; Canada. TI - Periodontal disease and risk of fatal coronary heart and cerebrovascular diseases. SO - Journal of Cardiovascular Risk Vol 6(1) (pp 7-11), 1999. AB - Background: Recent reports have implicated periodontal disease as a risk factor for coronary heart disease (CHD). Methods: A retrospective cohort study was conducted using participants in the 1970-1972 Nutrition Canada Survey (NCS). The mortality experience of male and female NCS participants aged 35-84 years without self-reported CHD (n=10 368) or cerebrovascular disease (CVD) (n=11 251) was determined through 1993. The relation between dental health and the risk of fatal CHD and CVD was assessed using Poisson regression modeling. Results: In total, 466 CHD and 210 CVD deaths were observed; missing confounder data reduced these numbers to 416 CHD and 182 CVD deaths. Adjusted for age, sex, diabetes status, serum total cholesterol, smoking, hypertensive status, and province, we found a statistically significant association between periodontal disease and risk of fatal CHD. Rate ratios (RR) of 2.15 [95% confidence interval (CI) 1.25-3.72) and 1.90 (95% CI 1.17-3.10) were observed for severe gingivitis and edentulous status, respectively. Nonstatistically significantly increased RRs of 1.81 and 1.63 were observed for severe gingivitis and edentulous status for CVD. Conclusions: These data indicate that poor dental health is associated with an increased risk of fatal CHD. [References: 19] <75> UI - 1999115875 AU - Giordano PC AU - Harteveld CL AU - Bok LA AU - Van Delft P AU - Batelaan D AU - Beemer FA AU - Bernini LF IN - P.C. Giordano, Department of Human Genetics, Leiden University, Medical Center, Wassenaarseweg 72, 2333 AL Leiden; Netherlands. E-Mail: piero@ruly46.medfac.leidenuniv.nl. TI - A complex haemoglobinopathy diagnosis in a family with both beta [degree] - and alpha( [degree] /+)-thalassaemia homozygosity. SO - European Journal of Human Genetics Vol 7(2) (pp 163-168), 1999. AB - The occurrence of point mutation alpha-thalassaemia and of complex combinations of haemoglobin defects is underestimated. Haemoglobinopathies, the most frequent monogenic recessive autosomal disorder in man, occur predominantly in Mediterranean, African and Asiatic populations. However, countries of immigration with a low incidence in the indigenous population, are now confronted with a highly heterogeneous array of imported defects. Furthermore, the occurrence of severe phenotypes is bound to increase in the near future because of the endogamous growth of the ethnical minorities and the lack of prevention. We describe an Afghan family in which both partners of a consanguineous relationship are carriers of a beta- as well as an alpha-thalassaemia determinant. The combination of defects was revealed by the in vitro measurement of the beta/alpha biosynthetic ratio and was characterised at the DNA level. The molecular defects involved are the Cd5(-CT), a Mediterranean beta [degree] -thalassaemia mutation, and the alpha2( [degree] /+)-thalassaemia AATA(-AA) polyadenylation defect. The alpha-thalassemia defect is a rare RNA-processing mutant described only twice before in heterozygous form in Asian-Indian patients. The mutation suppresses the expression of a alpha2 gene and reduces the expression of the less efficient, 3' located alpha1 gene as well, inducing a near alpha [degree] -thalassaemia phenotype. This defect is now described for the first time in the homozygous condition in one of the children who, in addition to being homozygous for the alpha-thalassaemia point mutation, is also a carrier of the beta [degree] -thalassaemia defect. A previously described homozygous case of the alpha( [degree] /+)-thalassaemia condition, caused by a similar polyadenylation defect, was characterised by a severe HbH disease. However, the patient described here present at 7 years of age with severe caries, like his beta-thalassaemia homozygous brother but without hepatosplenomegaly, haemolysis or severe anaemia. The haematological analysis revealed 9.5 g/dl Hb; 5.4 x 1012/I RBC; 0.33 I/I PCV; 61 fl MCV; 17.6 pg MCH and 6.2% of HbA2. The biosynthetic ratio beta:alpha was 1.6 and no HbH fraction was detectable either on electrophoresis or as inclusion bodies. The parents reported no complications during pregnancy, at birth, or in the neonatal period in rural Afghanistan. We presume therefore that the counterbalancing effect induced by the co-existing beta-thalassaemia defect could have modified a potentially severe perinatal HbH disease into a strongly hypochromic but well compensated 'alpha [degree] -like heterozygous' thalassaemia phenotype. The risk of a severe HbH disease, could have been easily missed in this family which was referred because of a child affected with beta-thalassaemia major. [References: 22] <76> UI - 1999110980 AU - Lo F-S AU - Lee K-S AU - Chen L-S AU - Hung H-Y AU - Hsu C-H AU - Lin S-P AU - Lee Y-J IN - Dr. Y.-J. Lee, Department of Pediatrics, Mackay Memorial Hospital, 92 Chung-San N Road, Taipei 10449; Taiwan. TI - Congenital nasal pyriform aperture stenosis: A diagnostic clue to solitary maxillary central incisor prior to eruption in three infants. SO - Journal of Otolaryngology Vol 28(1) (pp 51-53), 1999. <77> UI - 1999099238 AU - David LR AU - Finlon