All issues > Volume 32(6); 1989

Original Article

J Korean Pediatr Soc. 1989;32(6):823-833. Published online June 30, 1989.

Bone Age Determination and Hand Radiographic Findings in Children With Russell-Silver Syndrome.

Hun Kyu Lim¹, Sei Won Yang¹, Hyung Ro Moon¹

¹Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea

Received: November 26, 1988;  Accepted: December 13, 1988.

Abstract

Russell.Silver syndrome is a disease of unknown etiology characterized by prenatal onset dwarfism irrespective of full term gestation, growth retardation, body asymmetry, craniofacial dysproportion with a triangular face, short and incurved little finger, etc. Forty.two children were diagnosed as Russell.Silver syndrome by Silver’s criteria at SNU Children' s Hospital from Apr. 1981 till Aug. 1988, and we reviewed clinical data and hand radiographs of the patients Mean birth weight was 2,300 g with term gestation in 38 (91 %). Mean body weight and height were below the 3rd percentile of normal children but growth velocity of the patients was similar to those of the 3rd percentile of normal children (p< 0.05). Bone age was delayed in 39 (95%) of 41 children and normal in 2 (5%), and it could be a good indicator in predicting the body weight and height of children with Russell.Silver syndrome. In hand radiographs, hypoplasia of little finger was noted in 93% of 41 children, ivory epiphysis in 39%, and pseudoepiphysis in 34%.

Keywords :Russell.Silver Syndrome, Growth Velocity, Bone Age, Hypoplasia of little Finger, Ivory Epiphysis, Pseudoepiphysis