Treatment considerations for patient with Amelogenesis Imperfecta: a review.

Amelogenesis imperfecta (AI) is a group of inherited disorders primary affecting the structural of enamel. Patients with AI experience poor esthetic, excessive tooth sensitivity and compromised chewing function that dental treatments are frequently required at early age. This review describes the non-enamel implications, stage-specific management strategies and outcomes of selected restorative materials based on literature evidence.

OBJETIVOS: Amelogêse imperfeita (AI) é um grupo de desordens hereditárias que afetam primariamente a estrutura do esmalte dentário. Pacientes com AI apresentam estética pobre, sensibilidade dentária excessiva e função mastigtória comprometida. Nestes casos, os tratamentos dentários em idades precoces são frequentemente necessários. Esta revisão descreve as implicações não relacionadas com o esmalte dentário, as estratégias de atuação em idades específicas e ainda os resultados de tratamentos restauradores com determinados materiais e baseados em evidência científica.

Assessment of restorative treatment of patients with amelogenesis imperfecta.

PURPOSE: The purpose of this study was to assess restorative treatment outcomes in the mixed dentition of amelogenesis imperfecta (AI) patients and determine the postrehabilitation oral health status and satisfaction of the patients. METHODS: Clinical and radiographic examinations were performed on eight AI patients, who had 74 restorations placed in permanent incisors and molars, to allow evaluation of the integrity of the restorations and periodontal status post-treatment. Subjects completed a survey regarding esthetics, function, and sensitivity. RESULTS: Among the 74 restorations evaluated, seven were lost; of the remaining restorations, 31 were posterior, and 36 were anterior. Ten were rated clinically unacceptable. Teeth with stainless steel crowns had a moderate gingival index (mean=2.3) and plaque index (mean=2.0) scores. Widening of the periodontal ligament and pulp canal obliteration were common radiographic findings. Subject's recall of satisfaction regarding esthetics (P=.002) and sensitivity (brushing-P=.03; eating-P=.01) showed a statically significant difference before and after treatment. CONCLUSIONS: During mixed dentition, teeth with amelogenesis imperfecta may be restored with conventional treatment modalities. Direct restorations should be considered "interim" with multiple repairs anticipated. Post-treatment, gingival inflammation and plaque accumulation were observed. Subjects were satisfied with their appearance and reported a decrease of hypersensitivity.

Retrospective record review: reinforced zinc oxide-eugenol pulpotomy: a retrospective study.

PURPOSE: The purpose of this retrospective study was to evaluate pulpotomies completed without the use of a fixative, preservative, or astringent agent prior to placement of a reinforced zinc oxide-eugenol (ZOE) sub-base in the pulp chamber and subsequent restoration. METHODS: Clinical and radiographic data were collected from a private pediatric dental office in Toronto, Ontario, Canada. The pulpotomy technique used involved: amputation of coronal pulp; radicular hemostasis via pressure with dry cotton pellet; placement of ZOE into the pulp chamber; and restoration with stainless steel crown or amalgam. RESULTS: One-hundred-ninety primary molars in 116 children (follow-up=6-94 months; mean=35.8 months) met the inclusion criteria. The radiographic, clinical, and overall success rates were approximately 95%, 97%, and 94%, respectively. The most frequently observed pathologic pulpal response was furcation radiolucency (N=7, ∼4%). The patient's age at time of pulpotomy, restoration type, tooth type, arch, and location of treatment (in-office vs general anesthesia) were not statistically significant factors influencing the success of the reinforced ZOE pulpotomy technique. CONCLUSION: The success rates indicate that the reinforced zinc oxide-eugenol pulpotomy technique may be an acceptable treatment modality for primary molars requiring vital pulp therapy.

Amelogenesis imperfecta in two families with defined AMELX deletions in ARHGAP6.

Amelogenesis imperfecta (AI) is a group of inherited conditions featuring isolated enamel malformations. About 5% of AI cases show an X-linked pattern of inheritance, which are caused by mutations in AMELX. In humans there are two, non-allelic amelogenin genes: AMELX (Xp22.3) and AMELY (Yp11.2). About 90% of amelogenin expression is from AMELX, which is nested within intron 1 of the gene encoding Rho GTPase activating protein 6 (ARHGAP6). We recruited two AI families and determined that their disease-causing mutations were partial deletions in ARHGAP6 that completely deleted AMELX. Affected males in both families had a distinctive enamel phenotype resembling "snow-capped" teeth. The 96,240 bp deletion in family 1 was confined to intron 1 of ARHGAP6 (g.302534_398773del96240), but removed alternative ARHGAP6 promoters 1c and 1d. Analyses of developing teeth in mice showed that ARHGAP6 is not expressed from these promoters in ameloblasts. The 52,654 bp deletion in family 2 (g.363924_416577del52654insA) removed ARHGAP6 promoter 1d and exon 2, precluding normal expression of ARHGAP6. The male proband of family 2 had slightly thinner enamel with greater surface roughness, but exhibited the same pattern of enamel malformations characteristic of males in family 1, which themselves showed minor variations in their enamel phenotypes. We conclude that the enamel defects in both families were caused by amelogenin insufficiency, that deletion of AMELX results in males with a characteristic snow-capped enamel phenotype, and failed ARHGAP6 expression did not appreciably alter the severity of enamel defects when AMELX was absent.

Treatment considerations for patient with amelogenesis imperfecta: a review / Considerações de tratamento para pacientes com amelogênese imperfeita: revisão de literatura

Objectives: Amelogenesis imperfecta (AI) is a group of inherited disorders primary affecting the structural of enamel. Patients with AI experience poor esthetic, excessive tooth sensitivity and compromised chewing function that dental treatments are frequently required at early age. This review describes the nonenamel implications, stage-specific management strategies and outcomes of selected restorative materials based on literature evidence

Objetivos: Amelogênese imperfeita (AI) é um grupo de desordens hereditárias que afetam primariamente a estrutura do esmalte dentário. Pacientes com AI apresentam estética pobre, sensibilidade dentária excessiva e função mastigatória comprometida. Nestes casos, os tratamentos dentários em idades precoces são frequentemente necessários. Esta revisão descreve as implicações não relacionadas com o esmalte dentário, as estratégias de atuação em idades específicas e ainda os resultados de tratamentos restauradores com determinados materiais e baseados em evidência científica.