All issues > Volume 38(12); 1995

Original Article

J Korean Pediatr Soc. 1995;38(12):1677-1685. Published online December 15, 1995.

Cushing Syndrome in Children and Adolescents

Choong Ho CH Shin¹, Sei Won SW Yang¹, Hyung Ro HR Moon²

¹Department of Pediatrics, Seoul National University, College of Medicine, Seoul, Korea
²Department of Pediatrics, Eul ji Hospital, Seoul, Korea

Abstract

Purpose
: Cushing syndrome occurs rarely in children and adolescents. The clinical manifestation is variable and growth is usually adversely affected.
Methods
: We retrospectively analyzed 8 patients with Cushing syndrome between the ages of 1 and 14 year who were admitted to the pediatric department of Seoul National University Children's Hospital during the period from 1978 to 1995.
Results
: 1) Three patients had Cushing disease; two, adrenocortical adenoma and three, adrenocortical carcinoma. 2) Adrenal tumor occurred more frequently in early age, while Cushing disease developed in later childhood. The central obesity was observed in 50 percent of patients. The bone age varied, compared to the chronological age at diagnosis in the children with Cushing syndrome. 5) The height SDS for chronolgical age at diagnosis was -0.7¡¾1.7 and not correlated with plasma cortisol, duration of hypercortisolism, 24 hour urinary 17-hydroxy¡¤ corticosteroid and 17-ketosteroid(P>0.05). There was no significant difference between heigh SDSs for chronological and bone age. After successful treatment, we could not observe any change in height SDS. 6) The high-dose dexamethasone suppression test identified all patients with Cushing disease. 7) The children with Cushing disease had been cured with tumor resection and/or radiation therapy, but one child showed symptoms and signs of hypopituitarism after treatment. All children with adrenocortical adenoma were successfully treated with tumor resection. Two children with adrenocortical carcinoma. recurred and died within one year after operation.
Conclusion
: Weight gain and growth retardation are common clinical characteristics of Cushing syndrome in children and adolescents. The prognosis of final adult height seems to be poor despite successful treatment. The prognosis of adrenocortical carcinoma is gaurded due to high rate of recurrence.

Keywords :Cushing syndrome