Virilizing adrenal tumour mimicking congenital adrenal hyperplasia with P450c11 (11β-hydroxylase) deficiency

Virilizing adrenal tumour mimicking congenital adrenal hyperplasia with P450c11 (11β-hydroxylase) deficiency

In a girl aged 5 years with a virilizing adrenal adenoma the urinary and plasma steroid findings suggested the diagnosis of congenital adrenal hyperplasia due to P450c11 (11 beta-hydroxylase) deficiency. After removal of the tumour clinical signs receded and the hormonal values normalized. RNA analy...

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Veröffentlicht in:European journal of pediatrics 1994-06, Vol.153 (6), p.411-415
Hauptverfasser: WERDER, E. A, VOUTILAINEN, R, ZACHMANN, M
Format: Artikel
Sprache:eng
Schlagworte:
Adenoma - diagnosis
Adenoma - enzymology
Adrenal Gland Neoplasms - diagnosis
Adrenal Gland Neoplasms - enzymology
Adrenal Hyperplasia, Congenital - diagnosis
Adrenal Hyperplasia, Congenital - metabolism
Adrenals. Adrenal axis. Renin-angiotensin system (diseases)
Biological and medical sciences
Child, Preschool
Endocrinopathies
Female
Humans
Medical sciences
Non tumoral diseases. Target tissue resistance. Benign neoplasms
Steroids - metabolism
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Zusammenfassung:In a girl aged 5 years with a virilizing adrenal adenoma the urinary and plasma steroid findings suggested the diagnosis of congenital adrenal hyperplasia due to P450c11 (11 beta-hydroxylase) deficiency. After removal of the tumour clinical signs receded and the hormonal values normalized. RNA analysis of the tumour tissue revealed low amounts of P450c11 mRNA which indicates that P450c11 deficiency of the adenoma caused the steroid abnormalities in this girl.
ISSN:0340-6199
1432-1076
DOI:10.1007/BF01983403