som
Forum Guru
Topics: 59
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6 yr old boy comes to your clinic. on/e you find coarse dark pubic hair, enlarged testis, acne on face and upper back. His mom says he has a body odor simililar to his teenage brotjer after playing sports. child is 99 percentile of ht for his age gp.likely diaognsis:
cong adrenal hyperplasia
hypothalamic tumor
klinefelter syndrome
male pseudohermaphroditism
xyy syndr
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| frank100
Forum Guru
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hypothalamic tumor
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| mazinger
Forum Guru
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I go for XYY syndrome... Hey Dr Frank how's it going?
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| cbenitesch
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Letīs start the elimination round!... Cong adrenal hyperplasia, has most of the described above but testis are small, and large peanut!... XYY syndrome, course with long height, acne problems and cognoscitive abnormalities, Klinefelter Syndrome and male pseudohermaphroditism if course you what are all about so... the answer is XYY syndrome!!!!!, but my doubt is hipotalamic gliomas has restrictions to thrive!... but this is on 99th percentile
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| frank100
Forum Guru
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Yes it is XYY Ssyndrome
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| GDS2008
Forum Elite
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XYY syndrome typically presents as severe acne without precoccious puberty -- so i do not think that can be the answer
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| GDS2008
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I believe the answer is Congenital adrenal hyperplasia (late onset). Although, enlarged testes is not a typical feature of CAH, it may be present in a variant of CAH, when there are adrenal rests in the testis. The reason why I did not select any other is beacuse they do not fit into the picture...
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| som
Forum Guru
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cud someone pls clarify me why testes are small in CAH?
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| GDS2008
Forum Elite
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Small testes is not due to CAH. Normally, when HPA is activated and GnRH is secreted, pituitary releases FSH and LH which then acts on the sertoli-lyedig cells (in the testes) to get activated and start to make androgens. This activates the pre-pubertal (small) testes whihc then become post-pubertal (large). Thsi is a normal physiological process and can also be seen in precocious puberty due to premature activation of HPA. The main stimulus for testes to enlarge is FSH and LH which inturn are released due to GnRH.
However, in CAH the androgens come from the adrenal gland, which if anything suppreses the release of GnRH and FSH and LH. Therefore the testes remain small in CAH. However, in about 20% of CAH testes have adrenal rests whihc become enlarged and may resutl in enalrged testes.
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| som
Forum Guru
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Posts: 308
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thanks GDS.
this is a case of hypothalamic tumor
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| robin082006
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http://www.prep4usmle.com/forum/templates/default...
nod
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