The authors report two cases of congenital adrenogenital syndome. The patient in case I was a girl born with ambiguious sex, and manifested virilization and accelerated growth. The height and bone age on admission at age of 3 years and 10 mounths was 103.5 cm. and 6 years and 9 months, respectively. The vaginogram revealed normal internal genital organs. The blood pressure and electrolytes studies were normal. The 24 hours urinary 17-Ketosteroids was 8.72 mg. prior to treatment. She was discharged on cortisone acetate 25 mg. daily after cliteridectomy. She was missed during the second years of follow-up. When she was again seen at the outpatient department two years after the discharge, physical examination revealed acne and pubic hair. Her bone age and urinary 17-Ketosteroids was 13 years and 14.4 mg/24 hrs, respectively.
The patient in case II, a 50 day-old male infant was admitted for the second time to the hospital in salt-losing crisis. His phalus was unusually large and his nipples as well as phalus showed increased pigmentation. He responded poorly to fluid therapy and showed signs of hemorrhagic renal infarction: during the first hospitalization. On each admission, the urinary 17-Ketosteroids determination revealed 2.7 mg/24 hrs and 4.7 mg/24 hrs. He expired at home 3 days after the second discharge.
Both patients were considered to have 21-hydroxylation defect, the latter `salt-loser" and former `non salt-loser" from the clinical evidence.
|