Abstract
Case report: Child prematurely born after suspected placental insufficiency (low estriol output). 2 1/2 weeks of age diarrhoea and vomi ting. Increased pigmentation. Na 110 meq/1, K 8,5 meq/1. Treatment with DOCA and Hydrocortisone for suspected CAH-syndrome. Biochemical work-up: Urinary steroid analysis shows no evidence of any known adrenal enzyme deficiency. Salt losing syndrome with low cortisol and high ACTH-plasma-concentrations. No stimulation of plasma-cortisol after prolonged (3 weeks) ACTH-stitnulation. Urinary steroid output (gaschromatography): No increased excretion of any metabolite after prolonged ACTH-stimulation. Normal renin and low aldosterone-plasma-concentrations. Diagnosis: Congenital adrenal hypoplasia. At 12 months pubarche, increased penis-size, testes>2 ml. Bone age more than 4 SD advanced. Supine length crossing from P 25 over P 97: Height velocity well over P 97 for age. Plasma and urinary testosterone in the adult male range, non-suppressable with Dexamethason. A combined insulin-TRH LH-RH-stimulation reveals a complex hypothalamo-pituitary disorder. Hypothesis: Congenital adrenal hypoplasia with true precocious puberty due to congenital hypothalamic defect.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Girard, J., Zachmann, M. PRIMARY ADRENAL INSUFFICIENCY AND PRECOCIOUS PUBERTY IN A BOY 12 MONTHS OF AGE. Pediatr Res 9, 669 (1975). https://doi.org/10.1203/00006450-197508000-00030
Issue Date:
DOI: https://doi.org/10.1203/00006450-197508000-00030