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일과성 골수증식장애, 간섬유증 및 혈색소증이 동반된 다운증후군 신생아 1례

Other Titles
 Down Syndrome with Transient Myeloproliferative Disorder, Hepatic Fibrosis, and Hemochromatosis 
Authors
 이지연  ;  임휘규  ;  박찬일  ;  어영  ;  임백근  ;  김황민 
Citation
 Journal of the Korean Society of Neonatology (대한신생아학회지), Vol.10(1) : 83-87, 2003 
Journal Title
 Journal of the Korean Society of Neonatology (대한신생아학회지) 
ISSN
 1226-1513 
Issue Date
2003
MeSH
Down syndrome ; Transient myeloproliferative disorder ; Hepatic fibrosis ; Hemochromatosis
Keywords
Down syndrome ; Transient myeloproliferative disorder ; Hepatic fibrosis ; Hemochromatosis
Abstract
Transient myeloproliferative disorder (TMD), which may mimic acute leukemia, occurs in neonates with Down syndrome along with hepatic fibrosis. TMD is recognized shortly after birth or in the neonatal period and is characterized by leukocytosis and thrombocytopenia, which resolve spontaneously in four to six weeks. And hepatic fibrosis is characterized by diffuse intralobular sinusoidal fibrosis, extramedullary hematopoiesis and hemochromatosis. A newborn male infant with Down syndrome, atrial septal defect and ventricular septal defect is reported. He showed abnormal myelopoiesis accompanying characteristic hepatic sinusoidal fibrosis. Knowing the cellular mechanism of hepatic fibrosis and its modulation by growth factors, a pathogenetic link between transient myeloproliferative disorder and the development of liver fibrosis in Down syndrome neonates, association of this triad no longer appears to be accidental.
Files in This Item:
T200302802.pdf Download
DOI
OAK-2003-00219
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
Yonsei Authors
Park, Chan Il(박찬일)
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/113379
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