title: Lipoid congenital adrenal hyperplasia: pathologic features of the testis.
abstract: 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.
authors.affiliation: Department of Urology, Chonnam National University Medical School, Gwangju, Korea.
authors.forename: Sun-Ouck
authors.initials: SO
authors.lastname: Kim
authors.forename: Chan
authors.initials: C
authors.lastname: Choi
authors.forename: Chan Jong
authors.initials: CJ
authors.lastname: Kim
authors.forename: Jun Seok
authors.initials: JS
authors.lastname: Kim
authors.forename: Dongdeuk
authors.initials: D
authors.lastname: Kwon
authors.forename: Taek Won
authors.initials: TW
authors.lastname: Kang
authors.forename: Kwangsung
authors.initials: K
authors.lastname: Park
authors.forename: Soo Bang
authors.initials: SB
authors.lastname: Ryu
authors.forename: Seung Il
authors.initials: SI
authors.lastname: Jung
authors.forename: Kyung Jin
authors.initials: KJ
authors.lastname: Oh
authors.forename: Chang Min
authors.initials: CM
authors.lastname: Im
mesh_terms: D000312:Adrenal Hyperplasia, Congenital / Q000473:pathology*; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D013737:Testis / Q000473:pathology*