humbty
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7 yo boy. PMH insignificant.
severe cystic acne, enlarged testicles and penis, coarse pubic and axillary hair. 98th percentile in lenght and 80th percentile in weight.
Most likely diagnosis:
A)Hypothalamic dysfunction
B) 21 Hydroxylase Deficiency
C) severe hypothyroidism
D) 47 XXY
E) 47 XYY
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| GDS2008
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B) late onset congenital adrenal hyperplasia i.e 21-OHlase deficiency
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| humbty
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Can you explain why?
Thats UWs answer too. But enlarged testicles are not a sign of hypogonadotropic precocious puberty like CAH. CAH has small testicles because the high androgens supress the gonadotropins.
Enlarged testicles are a sign of hypergonadotropic precocious puberty like hypothalamic dysfunction.
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| GDS2008
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Enlarged testicles is the sign of puberty. In fact, testicular enlargement is one of the secondary sexual characteristics (included in Tanner staging). The way I think about it is that secondary sexual characterisitcs depend on testosterone (regardless of where it is coming from). In this case it is mentioned to point out that this boy indeed has precoccious puberty...
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| humbty
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NO thats not correct. The testicles grow in response to gonadotropins not in response to Androgens. They then produce the Androgens in true precocious puberty after being stimulated by gonadotropins.
In Precocious Pseudopuberty like CAH the Androgens are produced elsewhere, gonadotropins are low and the testicles remain small. Harrisons 16th edition page 2189 writes: Congenital adrenal hyperplasia: LH is low and testes are small.
I would say that question is not very well written. With that description of enlarged testicles I would say clues point towards true precocious puberty that means a HypERgonadotropic cause like hypothalamic dysfunction.
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| msyamp
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salaam namasthe humpty. great points.
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