Lipoid Congenital Adrenal Hyperplasia: Pathologic Features of the Testis

被引：7

作者：

Kim, Sun-Ouck

Choi, Chan

Kim, Chan Jong

Kim, Jun Seok

Kwon, Dongdeuk

Kang, Taek Won

Park, Kwangsung

Ryu, Soo Bang

Jung, Seung Il

Oh, Kyung Jin

Im, Chang Min

机构：

[1] Chonnam Natl Univ, Sch Med, Dept Urol, Kwangju 501757, South Korea

[2] Chonnam Natl Univ, Sch Med, Dept Pathol, Kwangju 501757, South Korea

[3] Chonnam Natl Univ, Sch Med, Dept Pediat, Kwangju 501757, South Korea

来源：

UROLOGY | 2010年 / 75卷 / 01期

关键词：

ACUTE REGULATORY PROTEIN; PATIENT; FEMALE;

D O I：

10.1016/j.urology.2009.04.073

中图分类号：

R5 [内科学]; R69 [泌尿科学（泌尿生殖系疾病）];

学科分类号：

1002 ; 100201 ;

摘要：

Lipoid congenital adrenal hyperplasia (lipoid CAH) is a rare disorder of steroid biosynthesis that is caused by a molecular defect in the steroidogenic acute regulatory protein. Patients with lipoid CAH usually experience life-threatening adrenal insufficiency and develop female external genitalia in both genetic sexes because of deficient gonadal steroid synthesis. Little is known regarding testicular histology in patients with lipoid CAH, and less is known about the changes in testicular histology with growth. This article describes testicular histopathology in a patient with 46, XY lipoid CAH who underwent orchiectomy at the age of 8 years. UROLOGY 75: 176-178, 2010. (C) 2010 Elsevier Inc.

引用

页码：176 / 178

页数：3

共 9 条

[1] Testicular histopathology in congenital lipoid adrenal hyperplasia: A light and electron microscopic study [J].