Fatal congenital systemic juvenile xanthogranuloma with liver failure

This is the second reported patient with systemic juvenile xanthogranuloma (JXG) to die with liver failure. The infant was born with multiple skin lesions and mild hepatomegaly. Direct hyperbilirubinemia was noted on the 2nd day of life, followed by progressive hepatomegaly, cholestasis, and death a...

Full description

Saved in:
Bibliographic Details
Published inPediatric and developmental pathology Vol. 7; no. 1; pp. 71 - 76
Main Authors Hu, Weimin K, Gilliam, Anita C, Wiersma, Susan R, Dahms, Beverly B
Format Journal Article
LanguageEnglish
Published United States 01.01.2004
Subjects
Abdomen
Fatal Outcome
Female
Humans
Infant, Newborn
Liver Failure - complications
Liver Failure - diagnostic imaging
Liver Failure - pathology
Lymph Nodes - pathology
Pancreas - pathology
Skin - pathology
Spleen - pathology
Tomography, X-Ray Computed
Xanthogranuloma, Juvenile - complications
Xanthogranuloma, Juvenile - congenital
Xanthogranuloma, Juvenile - pathology
Online AccessGet full text

Cover

More Information
Summary:This is the second reported patient with systemic juvenile xanthogranuloma (JXG) to die with liver failure. The infant was born with multiple skin lesions and mild hepatomegaly. Direct hyperbilirubinemia was noted on the 2nd day of life, followed by progressive hepatomegaly, cholestasis, and death at 29 days of age. At autopsy, nodular tumor infiltrates of JXG were present throughout the liver, as well as in skin, abdominal lymph nodes, spleen, and pancreas.
Bibliography:ObjectType-Case Study-2
SourceType-Scholarly Journals-1
ObjectType-Feature-4
content type line 23
ObjectType-Report-1
ObjectType-Article-3
ISSN:1093-5266
1615-5742
DOI:10.1007/s10024-003-4040-3