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Old 03-27-2011
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Kids Hypotensive, Hypovolemic, Hyperkalemic, baby with ambiguous genitalia

One hour after the delivery of a child with APGAR scores of 8 and 9 for the 1 minute and 5 minute marks respectively, a pediatrician notices that the child is hypotensive, hypovolemic, hyperkalemic and has ambiguous genitalia. Cytology reveals a 46XX genotype. What disorder does this child most likely have?

A - 11-Hydroxylase Deficiency
B - 21-Hydroxylase Deficiency
C - 17-Hydroxylase Deficiency
D - Desmolase Deficiency
E - 5 Alpha Reductase Deficiency
F - Conn's Syndrome
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Old 03-27-2011
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This is a classisc presentation of Congenital Adrenal Hyperplasia caused by 21 hydroxylase deficiency. Lack of this enzyme will cause deficiency of mineralocorticoid and glucocorticoid responsible for hypotension and electrolyte abnormality in this patient (salt wasting) and overproduction of adrenal androgen responsible for virilization of this baby girl.

The answer is : B
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Old 03-27-2011
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Default Answer BBBBBB

Answer is B. There is a wonderful flow chart made by Wazeer the kaplan lecturer of Physiology in Endocrine section. this flow chart makes it all easy.
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