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Background: Shwachman-Diamond syndrome (SDS) is a rare congenital disorder characterized by pancreatic insufficiency, bone marrow dysfunction, and short stature. In 1964, Shwachman, Diamond, Oski, and Knaw first reported the syndrome in a group of 5 children participating in a cystic fibrosis (CF) clinic at Harvard Medical School. SDS is the second most common cause of inherited pancreatic insufficiency.


Pathophysiology: All patients with SDS have varying degrees of pancreatic insufficiency, which is defined as the loss of exocrine function resulting in the inability to digest and therefore assimilate nutrition normally. Symptoms of malnutrition typically develop when greater than 98% of pancreatic reserve is lost. In individuals with this condition, pancreatic acinar cells do not develop in utero and are replaced by fatty tissue.

http://www.emedicine.com/ped/topic2060.htm

http://www.medscape.com/medline/abstract/9691213?src=emed_ckb_ref_0

http://books.dreambook.com/ceejohns/sdfguests.html

2006-07-16 05:47:59 · answer #1 · answered by Jen 5 · 0 0

It's shwachman-diamond disease, and it's pretty rare.

2006-07-16 03:55:17 · answer #2 · answered by Nosy Parker 6 · 0 0

no, and when i searched it nothing came up

2006-07-16 00:57:41 · answer #3 · answered by Pussycat 4 · 0 0

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