Open AccessHISTOCHEMICAL STUDIES IN WOLMAN'S DISEASE – REPORT OF AN AUTOPSY CASE ACCOMPANIED WITH A LARGE AMOUNT OF MILKY ASCITES –
Author(s) -
Uno Yutaka,
Taniguchi Aki,
Tanaka Eiichi
Publication year - 1973
Publication title -
acta patholigica japonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
ISSN - 0001-6632
DOI - 10.1111/j.1440-1827.1973.tb02775.x
Subject(s) - ascites , pathology , autopsy , disease , medicine , biology , gastroenterology
This is a case of a male infant who suffered from “Primary familial xanthomatosis with involvement and calcification of adrenals” (Wolman's disease). The clinical symptoms were characteristic hepatosplenomega‐ly, abdominal distention and a large amount of milky ascites. The patient died at the age of 2 1/2 months. X‐ray revealed enlarged, well‐shaped, calcified adrenal glands. Foamy cells in different organs which were observed at autopsy confirmed the diagnosis. On histochemical examination, deposition of a triglyceride‐cholesterol mixture was found in mesenchymal and epithelial cells of the liver, adrenals, spleen, lymph nodes and mucosa of the small intestine.