نتایج جستجو برای: Neonatal progeroid syndrome

تعداد نتایج: 695789  

Journal: :international journal of pediatrics 0
imran gattoo government medical college srinagar,india sudesh singh government medical college jammu ,india nucksheeba aziz government medical college srinagar,india

a female one month old with features supporting a diagnosis of neonatal progeroid syndrome(wrs)  presented to our neonatology section of gb pant children hospital srinagar .she had prenatal and post natal growth failure, generalized lipotrophy, triangular face, psedohydrocephalous, sparse scalp hair and eye brows, prominent scalp veins and greatly widened anterior fontenella.

Journal: :Journal of medical genetics 1997
H Arboleda L Quintero E Yunis

Wiedemann-Rautenstrauch (WR) syndrome is known as a neonatal progeroid syndrome, with only few published case reports. We describe three additional patients, two of them sibs, showing the clinical features of WR syndrome. Skeletal abnormalities are reported and assays of hormones and lipids are presented in one patient. Disturbance in bone maturation and lipid and hormone metabolism appear to b...

Journal: :Journal of medical genetics 1997
M B Delatycki M A Cleary A Bankier P N McDougall J S Ahluwalia C W Chow C M Cooke-Yarborough

Twin brothers and their maternal uncle with a previously undescribed neonatal progeroid syndrome are presented. In addition to progeroid features, they had pseudo-obstruction of the urinary and gastrointestinal tracts, severe leucocytosis, liver dysfunction, and low complex III and IV in muscle but not in liver. Previously described neonatal progeroid syndromes and syndromes featuring pseudo-ob...

A female one month old with features supporting a diagnosis of neonatal progeroid syndrome(WRS)  presented to our neonatology section of GB pant children hospital Srinagar .she had prenatal and post natal growth failure, generalized lipotrophy, triangular face, psedohydrocephalous, sparse scalp hair and eye brows, prominent scalp veins and greatly widened anterior fontenella.

Journal: :Indian pediatrics 2011
J P Narayan P Garg G Pareek S Narayan

Wiedemann Rautenstauch (WR) syndrome is a rare autosomal recessive neonatal progeroid syndrome with only few published case reports. We describe a neonate showing clinical features of WR syndrome with peeling of skin, and presented with weak cry and breathing difficulty since birth.

Journal: :Japanese journal of human genetics 1987

2015
Isabelle Schrauwen Szabolcs Szelinger Ashley L. Siniard Ahmet Kurdoglu Jason J. Corneveaux Ivana Malenica Ryan Richholt Guy Van Camp Matt De Both Shanker Swaminathan Mari Turk Keri Ramsey David W. Craig Vinodh Narayanan Matthew J. Huentelman Markus Schuelke

A 3-year-old female patient presenting with an unknown syndrome of a neonatal progeroid appearance, lipodystrophy, pulmonary hypertension, cutis marmorata, feeding disorder and failure to thrive was investigated by whole-genome sequencing. This revealed a de novo, heterozygous, frame-shift mutation in the Caveolin1 gene (CAV1) (p.Phe160X). Mutations in CAV1, encoding the main component of the c...

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