Natural Course of Neonatal Progeroid Syndrome

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Wiedemann-Rautenstrauch neonatal progeroid syndrome: report of three new patients.

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...

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Neonatal progeroid syndrome (Weidman Rautenstrauch syndrome): A case report from Jammu &Kashmir, India.

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neonatal progeroid syndrome (weidman rautenstrauch syndrome): a case report from jammu &kashmir, 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.

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A maternally transmitted lethal neonatal progeroid syndrome with prominent genitourinary and gastrointestinal features.

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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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ژورنال

عنوان ژورنال: Pediatrics & Neonatology

سال: 2009

ISSN: 1875-9572

DOI: 10.1016/s1875-9572(09)60044-9