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This patient is presenting with ambiguous genitalia and hypotension for which the most common cause is 21-hydroxylase (21-OH) deficiency. 21-OH deficiency is associated with decreased aldosterone and cortisol and increased androgens and potassium.21-OH deficiency is the most common cause of congenital adrenal hyperplasia. The absence of the enzyme 21-OH blocks the production of mineralocorticoids (aldosterone) and cortisol. As a result, patients will lose sodium and water in the urine causing hypotension. In addition, aldosterone typically causes the principal cells of the kidney to waste potassium. Without aldosterone hyperkalemia is often present. Finally, the decreased activity of 21-OH causes a shunt of the substrate toward 17-hydroxyprogesterone production and increases the androgen level. Treatment is with metabolite repletion and hormone supplementation.Parsa and New review the evidence regarding the diagnosis and treatment of 21-OH deficiency. They discuss how this disease is inherited in an autosomal recessive manner. They recommend treating patients with metabolite repletion and hormone supplementation.Figure A is a table demonstrating the changes that would occur in 21-OH deficiency (E), 11-OH deficiency (D), and 17-OH deficiency (A).Incorrect Answers:Answer 1: A in the table represents 17-OH deficiency with an increased aldosterone level and a decreased cortisol, androgen, and potassium levels. Treatment is with metabolite repletion and hormone supplementation.Answer 2: B in the table demonstrates an aldosterone-producing adenoma with an increased aldosterone level and a decreased potassium. Treatment is with resection of the adenoma.Answer 3: C in the table does not correspond to any common pathology.Answer 4: D in the table corresponds to 11-OH deficiency with a decreased aldosterone, cortisol, and potassium and increased androgen level. The decreased aldosterone level is secondary to hypertension from increased 11-deoxycorticosterone. Treatment is with metabolite repletion and hormone supplementation.Bullet Summary:21-OH deficiency causes decreased aldosterone and cortisol levels and increased androgen and potassium levels.
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