Pachyonychia congenita type I with severe oral leukokeratosis

Pachyonychia Congenita (PC) is a rare autosomal dominant keratin disorder that affects a number of ectodermal structures including the nails and palmoplantar skin, and often involves the oral mucosa, tongue, larynx, teeth and hair. Clinical features are usually present at birth or early infancy. There are two main subtypes of PC. Fingernail thickening and oral keratosis are more common and severe in PC-1 and cystic lesions, hair abnormalities, natal teeth and pili torti are more common in PC-2. We report the case of a 6-year-old boy with PC-1 presenting with severe and painful oral leukokeratosis and extensive caries

Hallermann-Streiff Syndrome: a case report from Turkey

Hallermann-Streiff Syndrome (HSS) is a rare disorder characterized primarily by head and face abnormalities, with dental abnormalities also present in 50-80 percent of cases. The first description seems to have been made by Aubry in 1893. HSS was first described completely in 1948 by Hallermann, and then in 1950 by Streiff. We report a 4-year-old girl with HSS, presenting oro-dental characteristics and describe the treatment modalities. An interdisciplinary approach including early preventive-care programmes, detailed oral hygiene instructions, dietary recommendations, counselling of the parents and regular dental visits are the essential procedures

Genç daimi dişlerde kök ucu oluşumunun devamını sağlayan tedavi yöntemi: apeksifikasyon

The process of root development in children is a very critical period that is susceptible to trauma for anterior teeth and to caries for posterior teeth. Apexification should be done for providing continuing root development if the pulp of young permanent teeth became non-vital because of trauma or caries. The purpose of treatment is the completion of root development. The materials used in apexification, their efficiency and administration protocols have been developing with the help of researches in this field. In the light of developing technology, the dentists should pursuit improvements and decide the best treatment plan for their patients. This review focuses on apexification procedure in young permanent teeth, materials and their efficiency. ÖZET Çocuklarn dişlerinde kök gelişimlerinin devam ettiği dönem, ön dişlerinin travmalara ve arka grup dişlerinin de çürüğe karşı hassas olduğu dönemdir. Enfeksiyon veya travma nedeniyle hasara uğramış genç daimi dişte pulpa canlılığını kaybederse, kök gelişiminin devamını sağlamak için “Apeksifikasyon” tedavisi yapılmalıdır. Tedavinin amacı, dişin kök gelişiminin devamını sağlamaktır. Yapılan birçok araştırma ile apeksifikasyon tedavisinde önerilen materyaller, etki mekanizmaları ve uygulama protokolü ile ilgili bilgiler artmaktadır. Diş hekimleri, gelişen teknolojiyle birlikte bu yenilikleri takip etmeli ve bu bilgilerin ışığı altında hastalarına uygun tedavi yöntemini uygulamalıdırlar. Bu derlemede, genç daimi dişlerde apeksifikasyon işlemi, kullanılan materyaller ve etki mekanizmaları sunulacaktır. Anahtar Kelimeler: Apeksifikasyon, MTA, kalsiyum hidroksit, kök hücr

The Relationship of Amelogenesis Imperfecta and Nephrocalcinosis Syndrome

Aim: To analyze the prevalence and associated oral findings of nephrocalcinosis in a group of patients affected with amelogenesis imperfecta (AI). The relationship between types of AI and nephrocalcinosis were also evaluated. Design: This study examines patients who were referred to Pediatric Dentistry Department of SDU between the years of 2002-2007 and who, upon clinical and radiological examination, were diagnosed with AI and treated. Patients were offered information about the possibility of nephrocalcinosis syndrome. Patients who agreed to have tests carried out on their renal system were advised to visit the department of nephrology at the clinic. Results: Suspicious radiopacity was observed during renal ultrasonography of a controlled number of patients with hypoplastic type AI. Laboratory results revealed low Ca values (100-300 mg/days) and normal P values (0.4-1.3 g/days). Delayed eruption, gingival hyperplasia, pulp stones and orthodontic problems were also observed in the same patient groups. Conclusion: Although renal findings were observed in a few patients, pediatric dentists are the doctors who are the first to have early contact with this patient group. Because of the potential risk of nephrocalcinosis, early diagnosis may offer good prognosis

Target gene analyses of 39 amelogenesis imperfecta kindreds

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/90297/1/EOS_857_sm_FigsS1-S3.pdfhttp://deepblue.lib.umich.edu/bitstream/2027.42/90297/2/j.1600-0722.2011.00857.x.pd

Two years survival rate of class II composite resin restorations prepared by ART with and without a chemomechanical caries removal gel in primary molars

The aim was to test the null hypotheses that there is no difference: (1) in carious lesion development at the restoration margin between class II composite resin restorations in primary molars produced through the atraumatic restorative treatment (ART) with and without a chemomechanical caries removal gel and (2) in the survival rate of class II composite resin restorations between two treatment groups after 2 years. Three hundred twenty-seven children with 568 class II cavitated lesions were included in a parallel mouth study design. Four operators placed resin composite (Filtek Z 250) restorations bonded with a self-etch adhesive (Adper prompt L pop). Two independent examiners evaluated the restorations after 0.5, 1, and 2 years using the modified Ryge criteria. The Kaplan–Meier survival method was applied to estimate survival percentages. A high proportion of restorations were lost during the study period. Therefore, the first hypothesis could not be tested. No statistically significant difference was observed between the cumulative survival percentages of restorations produced by the two treatment approaches over the 2-year period (ART, 54.1 ± 3.4%; ART with Carisolv™, 46.0 ± 3.4%). This hypothesis was accepted. ART with chemomechanical gel might not provide an added benefit increasing the survival percentages of ART class II composite resin restorations in primary teeth