Classic congenital adrenal hyperplasia: A delayed presentation

被引:3
|
作者
Siddiqui, Saima Aziz [1 ,3 ]
Soomro, Nargis [1 ,3 ]
Ganatra, Ashraf [2 ,3 ]
机构
[1] Dow Univ Hlth Sci, Dept Obstet & Gynaecol, Karachi, Pakistan
[2] Dow Univ Hlth Sci, Dept Plast Surg, Karachi, Pakistan
[3] Civil Hosp Karachi, Karachi, Pakistan
关键词
Congenital adrenal hyperplasia; Clitoromegaly; Virilization; Clitoroplasty; Ambiguous genitalia; AMBIGUOUS GENITALIA; EXPERIENCE;
D O I
10.12669/pjms.291.2830
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Congenital adrenal hyperplasia (CAH) is a rare congenital disorder, which in cases of female genotype may result in virilization. Specific enzyme deficiencies in adrenocorticoid hormones biosynthetic pathway lead to excess androgen production causing virilization. Classic type presents early in infant life as salt losing or simple virilizing type, whereas non classic form presents late at puberty or in adult life. Depending on the type of classic CAH, type of adrenocorticoid deficiency, extent of virilization & genotype, surgical corrective procedures, glucocorticoid & mineralocorticoid replacement therapy are the mainstay of management. We present here a case of classic congenital adrenal hyperplasia of simple virilizing type, which presented later in childhood.
引用
收藏
页码:220 / 223
页数:4
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