Pheochromocytomas and Paragangliomas: Genetics and Pathophysiology

Sep 1, 2019 by in ENDOCRINOLOGY Comments Off on Pheochromocytomas and Paragangliomas: Genetics and Pathophysiology

Fig. 8.1 Catecholamine and metanephrine synthesis pathway . PNMT phenylethanolamine N-methyltransferase, COMT catechol-O-methyltransferase, MAO monoamine oxidase, VMA vanillylmandelic acid Consequences of Catecholamine Hypersecretion Catecholamines and metanephrines bind to the seven…

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Regulation of Adrenal Steroidogenesis

Sep 1, 2019 by in ENDOCRINOLOGY Comments Off on Regulation of Adrenal Steroidogenesis

Fig. 2.1 Steroid biosynthetic pathways in the human adrenal cortex. Shown are enzymes (underlined) and intermediates in the biosynthesis of adrenal steroid hormones. 17α-Hydroxypregnenolone is the preferred substrate for the…

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Diagnosis and Management of Primary Aldosteronism

Sep 1, 2019 by in ENDOCRINOLOGY Comments Off on Diagnosis and Management of Primary Aldosteronism

Aldosterone-producing adenoma (APA)—30% of cases Bilateral idiopathic hyperplasia (IHA)—60% of cases Primary (unilateral) adrenal hyperplasia—2% of cases Aldosterone-producing adrenocortical carcinoma—<1% of cases Familial hyperaldosteronism (FH)  Glucocorticoid-remediable aldosteronism (FH type 1)—<1%…

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Diagnosis and Management of Adrenal Insufficiency

Sep 1, 2019 by in ENDOCRINOLOGY Comments Off on Diagnosis and Management of Adrenal Insufficiency

Primary Genetic disorders  Congenital adrenal hyperplasia Adrenoleukodystrophy ACTH resistance syndromes ACTH insensitivity: familial glucocorticoid deficiency, triple-A syndrome Adrenal hypoplasia congenita Other transcription factors: SF1 (NR5A1) Drug induced  Adrenostatic/adrenolytic: etomidate, ketoconazole,…

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Adrenal Zonation and Development

Sep 1, 2019 by in ENDOCRINOLOGY Comments Off on Adrenal Zonation and Development

Fig. 1.1 Concentric layers of the mouse adrenal gland. (a) Schematic of the various regions of the adrenal. (b) A representative H&E longitudinal mouse adrenal section. Zones are identified by…

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