نتایج جستجو برای: dentinogenesis

تعداد نتایج: 320  

2016
Najmeh Akhlaghi Ali-Reza Eshghi Mehrnaz Mohamadpour

Dentinogenesis imperfecta (DI) is a hereditary dentin defect caused by an autosomal dominant mutation in dentin sialophosphoprotein gene. Defective dentin development results in discolored teeth that are prone to wear and fracture. Early diagnosis and proper treatment are necessary to achieve better functional and esthetic results and minimize nutritional deficiencies and psychosocial distress....

2014
Ali Al Kaissi Rudolf Ganger Klaus Klaushofer Franz Grill

Swellings over the upper and lower limbs were encountered in a one-year-old child. Skeletal survey showed a constellation of distinctive radiographic abnormalities of osteoporosis, hyperplastic callus and ossification of the interosseous membrane of the forearm, femora, and to lesser extent the tibiae. Neither wormian bones of the skull nor dentinogenesis imperfecta was present. Genetic tests r...

Journal: :Genetics and molecular research : GMR 2009
C Barbirato M G Almeida M Milanez V Sipolatti M R G O Rebouças A N Akel V R R Nunes A M S Perrone M Zatz I D Louro F Paula

Osteogenesis imperfecta is a heterogeneous genetic disorder characterized by bone fragility and deformity, recurrent fractures, blue sclera, short stature, and dentinogenesis imperfecta. Most cases are caused by mutations in COL1A1 and COL1A2 genes. We present a novel splicing mutation in the COL1A1 gene (c.1875+1G>C) in a 16-year-old Brazilian boy diagnosed as a type III osteogenesis imperfect...

2016
Agnès Bloch-Zupan Mathilde Huckert Corinne Stoetzel Julia Meyer Véronique Geoffroy Rabisoa W. Razafindrakoto Saholy N. Ralison Jean-Claude Randrianaivo Georgette Ralison Rija O. Andriamasinoro Rija H. Ramanampamaharana Solofomanantsoa E. Randrianazary Béatrice Richard Philippe Gorry Marie-Cécile Manière Simone Rakoto Alson Hélène Dollfus

A large family from a small village in Madagascar, Antanetilava, is known to present with colored teeth. Through previous collaboration and 4 successive visits in 1994, 2004, 2005, and 2012, we provided dental care to the inhabitants and diagnosed dentinogenesis imperfecta. Recently, using whole exome sequencing we confirmed the clinical diagnosis by identifying a novel single nucleotide deleti...

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