نتایج جستجو برای: robinow syndrome

تعداد نتایج: 621936  

Journal: :Journal of Medical Genetics 1997

2017
Stanisław Warchol Przemyslaw Bombinski Jacek Skiendzielewski Teresa Dudek-Warchol

Congenital posterior urethral diverticulum (PUD) is a rare congenital anomaly. Proper diagnosis and treatment in such cases still is challenging. More common are acquired diverticula of the posterior urethra, which mostly follow surgical repair of imperforate anus. We report a case of a large, congenital PUD which was diagnosed in a 3-month old boy with very rare genetic disorder Robinow syndro...

Journal: :Contemporary Clinical Dentistry 2017

Journal: :international journal of pediatrics 0
shruti saraswat department of paediatrics, hi tech medical college, utkal university, bhubaneswar, odisha, india. satish mohanty department of paediatrics, hi tech medical college, utkal university, bhubaneswar, odisha, india.

background robinow syndrome is a rare congenital disorder with phenotypically heterogeneous abnormalities. two modes of inheritances are known for this syndrome namely autosomal recessive and autosomal dominant. case report we describe here an eighteen-month-old child who had mesomelic short stature, abnormal facial features, clinodactyly, micropenis and vertebral changes which were further sup...

Journal: :Journal of King Abdulaziz University-Medical Sciences 1999

Journal: :American journal of human genetics 2015
Janson White Juliana F Mazzeu Alexander Hoischen Shalini N Jhangiani Tomasz Gambin Michele Calijorne Alcino Samantha Penney Jorge M Saraiva Hanne Hove Flemming Skovby Hülya Kayserili Elicia Estrella Anneke T Vulto-van Silfhout Marloes Steehouwer Donna M Muzny V Reid Sutton Richard A Gibbs James R Lupski Han G Brunner Bregje W M van Bon Claudia M B Carvalho

Robinow syndrome is a genetically heterogeneous disorder characterized by mesomelic limb shortening, genital hypoplasia, and distinctive facial features and for which both autosomal-recessive and autosomal-dominant inheritance patterns have been described. Causative variants in the non-canonical signaling gene WNT5A underlie a subset of autosomal-dominant Robinow syndrome (DRS) cases, but most ...

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