Related Experiment Video
Updated: Aug 23, 2025

A Strategy to Identify de Novo Mutations in Common Disorders such as Autism and Schizophrenia
Published on: June 15, 2011
Diagnostics and follow-up strategy for Silver–Russell syndrome based on a case report showing familial accumulation
Árpád Ferenc Kovács1, Nikolett Jusztina Beniczky1, Rita Ágnes Bertalan1
11 Semmelweis Egyetem, Általános Orvostudományi Kar, II. Gyermekgyógyászati Klinika Budapest, Tűzoltó utca 7-9., 1094 Magyarország.
Abstract:
"Characterized by both intrauterine and postnatal growth retardation, and consequent small stature, Silver–Russell syndrome is associated with typical minor anomalies (relative macrocephalia, protruding forehead, downturned corners of mouth, micrognathia, low set ears, facial, skeletal and limb asymmetry) and findings involving mainly the endocrine system. The molecular background of the syndrome is complex, but it is most often caused by the involvement of critical regions of chromosome 11 and/or chromosome 7. Beside the molecular diagnosis, the Netchine–Harbison clinical scoring system aims to contribute to the successful diagnosis of Silver–Russell syndrome. Although Silver–Russell syndrome is mostly sporadic, in our case report we present an extremely rare familial accumulation, where three of four siblings are affected by Silver–Russell syndrome. Early diagnosis is important to initiate adequate feeding and nutritional guidance, enhance early development and start growth hormone therapy as soon as possible. We would like to emphasize that management and long-term follow-up is crucial to prevent potential complications and treat specific issues appropriately."
Related Concept Videos
Pedigree Analysis
Incomplete Dominance

