Kazem
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A malnourished 10 year old boy, with pointed mandible, broad forehead,visible Abdominal veins,ascites,and jaundice.
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| chemamr
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donīt you have more information about that poor boy??? 
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| Doc2378
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yeah...some more info would be good..
..but if its just a case of malnourisment...how about Kwashiorkar? ...that can cause a fatty liver and would explain the jaundice, ascites, distended veins etc..
(but how to reconcile a pointed mandible, and broad forehead with Kwashiorkar????..... by the sounds of it looks like its somehow secondary to a GH elevation???
..yeah, definitely would appretiate some more pointers here...
GL!
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| merrk
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umm?? what about thalassemia... broad forehead and liver failure d/t extramedullary hematopoisis? child would be malnourished... is the pointed mandible part of malnourishment? what does it mean? the edges are sharp or the chin?
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| Kazem
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more hints:
its an autosomal dominant disorder, more prevalent in boys, there is congenital atrophy of the bile ducts and thence a reduced bile flow. pruritis and xanthomas are usually the presenting features. heart defects with pulmonary stenosis can be seen in these patients. there is a reduced sensitivity to GH.
Edited by Kazem on 09/06/05 - 03:56 PM
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| drkpp
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alagille syndrome?
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| chemamr
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itīs similar, yes. Wow drkpp!! 
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| drkpp
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hey no big deal!!!! was just too easy although had too google a bit.
hey kazem if thats wrong,give the right answer before alagille goes into everybodies head
thanx
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| chemamr
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was just too easy although had too google a bit
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| Kazem
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Answer correct well done
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| chemamr
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well done drkpp !!!
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