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KMID : 0367419620030010045
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Journal of Korean Pediatric Society
1962 Volume.3 No. 1 p.45 ~ p.51
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THREE CASES OF ADRENOGENITAL SYNDROME DUE TO CONGENITAL VIRILIZING ADRENAL HYPERPLASIA
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文永殷/Moon, Yong Eun
高克勳/林宜善/Ko, Kook Hoon/Lim, Eui Sun
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Abstract
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Adrenogenital syndrome of the congenital type is a familial condition in which excessive androgen secretion occurs in homozygous offspring as a result of an inborn error in metabolism. The excessive secretion of androgen originates during the gestational period and continues throughout
life if not treated. Accurate clinical and anatomical descriptions of the disease have been availaule since the early years of this century. However, it is only since the discovery by Wilkins and associates e`´ the efficacy of cortisone in controlling this. abnormal secretion, thus enabling these individuals to lead a .ear-normal life, that interest in this condition has became widespread.
The abnormal secretion of androgen with its virilizing effects exerts a profound influence on sexual development and somatic growth. In the female there occurs the syndrome of female pseudohermaphroditism, and, in the male, macrogenitosomia precox.
This report is a presentation of three cases of female pseudohermaphroditism due to congeni tal virilizing adrenal hyperplasia observed is the last year. Case 1 and 2 are sibling. The diagnosis and management are discussed.
Report of Cases
Case I. J.O. Koo, a twelve year old Korean girl was admitted to Severance Hospital on April 4, 1959, with evidences of marked viriliz.ation due to prolonged influence of excessive androgens, The patient had virilizing manifestation include the increase in somatic growth facial hirsutis-n, acnei form rash enlarged clitoris and precocious growth of pubic hair. Also, she had deepening of voice. Secondary female sexual maturation is not developed. Diagnosis is made by an excessive androgen secretion which results in an abnormally high urinary 17-ketosteroids output and cytological determination of sex, and the demonstration of urogenital sinus and vagina on the urethrogram. Advanced bone age with early closure of the epiphyses was demonstrated by roentgenogram. The patient was treated with cortisone and was given surgical treatment including clitoridectomy and plastic remodeling of the urogenital sinus. The patient was discharged after 108 days of hospitalization. The urinary 17-ketosteroid ranged 50 mg. per 24 hours and cortisone decreases the 17-ketosteroid excretion as this is illustrated in Fig. 1.
Case 2. O. S. Koo, a sixteen year old Korean girl who is sister of case 1. She was also admitted to this hospital on July 22, 1959, with marked virilizing manifestation include the increase in somatic growth, facial hirsutism, acnei form rash and precocious growth of pubic hair. The external genitalia presents also same picture of case 1 as described on above. Diagnosis is made by same manner in case 1, and exploratory laparotomy and ovarian biopsy. The patient was treated with cortisone intramuscular routes and was given clitoridectomy and plastic remodeling of the urogenital sinus. The patient was discharged 56 days without any improvement on virilizing manifestation except for excessive androgen secretion. The initial 17-ketosteroid levels and the suppression by steroid theraphy in this case is illustrated in Fig. II.
Case 3. S.G. Son, a three month old infant who was erroneously assigned to the male sex. The patient was consulted to out patient clinic of this hospital on August 19, 1959, with complains of hypospadias and undescended testes. But cytological sex pattern was u. _ques tio. ably female. There were a phallus-like clitoris and coalescent labia. No introitus nor external urinary orifice was present. There was no evidence of salt losing syndrome or virilizing manifestation and 17-ketosteroids output was 4 mg. per 24 Hours. drethrogram showed urogenital sinus. The decision was to withhold cortisone and wait for signs of progressing virilism in the form of increasing 17-ketosteroid output and bone maturation.
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