Glucocorticoid Activity of Adrenal Steroid Precursors in Untreated PatientsWith Congenital AdrenalHyperplasia

Glucocorticoid Activity of Adrenal Steroid Precursors in Untreated PatientsWith Congenital AdrenalHyperplasia

Context and Objective: We describe the clinical features and biochemical characteristics of a unique population of severely affected untreated patients with congenital adrenal hyperplasia (CAH) from an Indonesian population with proven cortisol deficiency but without clinical signs of cortisol defic...

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Veröffentlicht in:The journal of clinical endocrinology and metabolism 2019-11, Vol.104 (11), p.5065
Hauptverfasser: Engels, Manon, J. Pijnenburg-Kleizen, Karijn, Utari, Agustini, Faradz, Sultana M.H, Oude-Alink, Sandra, Herwaarden, Antonius E. van, Span, Paul N, Sweep, Fred C, Hedi L. Claahsen-Grinten, van der
Format: Artikel
Sprache:eng
Schlagworte:
ACTH
Aldosterone
Corticosterone
Genetic disorders
Hydroxyprogesterone
Liquid chromatography
Mass spectrometry
Medical research
Medicine, Experimental
Progesterone
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Zusammenfassung:Context and Objective: We describe the clinical features and biochemical characteristics of a unique population of severely affected untreated patients with congenital adrenal hyperplasia (CAH) from an Indonesian population with proven cortisol deficiency but without clinical signs of cortisol deficiency. We evaluated the in vitro glucocorticoid activity of all relevant adrenal steroid precursors occurring in patients with CAH. Design: Cross-sectional cohort study and translational research. Intervention/Main Outcome Measures: Adrenal steroid precursor concentrations before and 60 minutes after ACTH administration to 24 untreated patients with CAH (3 to 46 years) with proven cortisol deficiency (
ISSN:0021-972X
DOI:10.1210/jc.2019-00547