All issues > Volume 29(7); 1986

Original Article

J Korean Pediatr Soc. 1986;29(7):715-722. Published online July 31, 1986.

Russell-Silver Syndrome.

Sei Won Yang¹, Hyung Ro Moon¹

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

Abstract

Seventeen cases(12 boys and 4 girls) with Russell-Silver syndrome were evaluated for weight, height and bone age at initial presentation and during follow up. 1) Distribution of patient` s age at initial presentation was between 1 day and 75/12 year of age. 2) In 14 cases, the birth weight was below 3 percentile except for one case(3 percentile). In 15 cases, the birth length was below 3 percentile except for one case(10 percentile). 3) At initial presentation, the percentiles for weight in all cases were below 3 rd. The percentile for height was below 3rd in 14 cases and in 2 cases, at 10 th. The bone age was delayed in 12 cases. In 3 cases, bone age was within normal range. 4) Skeletal asymmetry was observed in 6 cases(37%), incurved and/or short fifth fingers in 15 cases(94%), triangular face in 14 cases(87%) and syndactylism in 5 cases(31%). Other anomalies including downward slanting of mouth angle, frontal bossing, cryptorchidism, cafe-au-lait spot and micrognathia was observed in one case in each. 5) In eight patients, follow-up measurements for height was done and the duration of follow-up ranged from 6 months to 2.5 years. During follow-up, the percentile for height remained below 3 rd and the growth velocity of the patients was steadily depressed compared to the normal subjects of the same age.

Keywords :Growth failure; Russell-Silver syndrome; Short stature.