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Two cases of salt losing form of congenital adrenal hyperplasia.

Journal of the Korean Pediatric Society 1978;21(9):614-621.
Published online September 30, 1978.
Two cases of salt losing form of congenital adrenal hyperplasia.
K C Park, H J Chun, M J Shin, S C Kang
Department of Pediatrics, Seoul Red Cross Hospital, Korea.
Salt Losing 型의 Congenital Adrenal Hyperplasia 2 례
박규정, 전행조, 신미자, 강석철
서울적십자병원 소아과
Abstract
We experienced 2 case of losing form of congenital adrenal hyperplasia which were regarded to be sucessfully controlled with hormonal replacement, DOCA and hydrocortisone. Case 1 was a 40 days-old male infant who had hyperpigmented phallus, scrotal rugae and nipples but whose phallus seemed to be normal in size and shape. He was admitted at the age of 40 days because of diarrhea, when he revealed in severely dehydrated stated with hyponatrema but responded well to fluid replacement over 2 days. On second admission, he was presented in lethargic state with severe dehydration because of vomiting and diarrhea for 9 days, Which was impressed chlincally as pyloric stenosis. Serum electrolytes revealed hyponatremai an dhyperkalemia. Urinary 17-KS level was increased up to 7.5mg/24 hrs urine. Five days after the replacement of DOCA and hydrocortisone, urinary 17-KS level was markedly diminished to 0.4mg/24hrs and serum electroytes because normal in range. Family history revealed his elder brother(male) expired at the age of one month because of severe diarrhea and dehydration, but had normal external genitalia. Case 2 was a 2 months old male infant whose phallus was unusually large for his age up to 4 cm in length, whose genitalia as well as his nipples were also hyperpigmented. This case was brought to Emergency Room in moderately dehydrated state because of diarrhea and vomiting. The family history of 2 siblings who were expired at 20 days and 2 months of age respectively hand malformed ambiguous genitalia with downward urethral openings and regarded as male at birth. Serum sodium was 104 mEq/L and serum potassium was 6. 2mEq/L. Urinary 17-KS was 8.4 mg/24hrs urine. During the correction of fluid and electrolytes as well as hormonal replacement with DOCA and hydrocortisone, Case I had adrenal crisis which was precipitated while withholding the hormonal replacement because of phlebitis complcated with sepsis. This crisis was well corrected with the emergency replacement with Soluco tef, fluid and electroly in 2 days. Both cases were maintained in good general condition for 1-2 months with DOCA, and hydrocortisone replacement therapy.


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