Titlebook: Congenital Adrenal Hyperplasia; Maria I. New,Lenore S. Levine Book 1984 Springer-Verlag Berlin, Heidelberg 1984 Adrenogenitales Syndrom.gl

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Recent Advances: The Fasciculata and Glomerulosa as Two Distinct Glands, suppressed by treatment, natriuresis occurs and renin-angiotensin levels consequently rise, stimulating the glomerulosa to synthesize aldosterone (Fig. 9). Because the glomerulosa, unlike the fasciculata, is spared the 11 .-hydroxylase defect, it can respond to this stimulation with an appropriate

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Congenital Adrenal Hyperplasia978-3-642-82011-3Series ISSN 0077-1015

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0077-1015 Overview: 978-3-642-82013-7978-3-642-82011-3Series ISSN 0077-1015

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,Electron–Specimen Interactions., A simplified scheme of adrenal steroidogenesis appears in Fig. 1. Each hydroxylation step is indicated and the newly added hydroxyl group is circled. The adrenocorticotropic hormone (ACTH) acts on the adrenals to increase the conversion of cholesterol to pregnenolone, the principal substrate for al

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,Electron–Specimen Interactions., (1970) suggested that an 11 .-hydroxylase defect might occur only in the fasciculata, sparing the glomerulosa from an 11 .-hydroxylase deficiency. The hypothesis accounts for the rise in aldosterone secretion observed in a patient with 11 .-hydroxylase deficiency after excessive DOC levels were sup