Presentation
No abnormally accumulated lipid was present, however, within the myenteric ganglion cells available for examination. [amjmed.com]
Summary A case of xanthomatous biliary cirrhosis has been presented. [gastrojournal.org]
[…] phagocytes; the most common subtype is the non-neuronopathic form, a slowly progressive condition characterized by hepatosplenomegaly and skeletal deformities; the neuronopathic forms are divided into infantile and juvenile forms; the infantile form presents [icd9data.com]
Entire Body System
- Whipple Disease
Some morphological characteristics of Whipple's disease. Am. J. Path. 1963; 42: 415 PubMed Google Scholar Rowlands Jr., D.T. Landing B.H. Colonic histiocytosis in children: report of a form resembling that seen in Whipple's disease. Am. J. [amjmed.com]
- Anemia
[…] is the non-neuronopathic form, a slowly progressive condition characterized by hepatosplenomegaly and skeletal deformities; the neuronopathic forms are divided into infantile and juvenile forms; the infantile form presents at 4-5 months of age with anemia [icd9data.com]
Liver, Gall & Pancreas
- Jaundice
Simple Stricture of Hepatic Duct, Causing Chronic Jaundice And Xanthelasma. Tr. Path. Soc., London. 1873; 24: 129 Google Scholar Murchison C. [gastrojournal.org]
- Hepatomegaly
272.7 splenomegaly (cerebroside lipidosis) 272.7 Hepatomegaly (see also Hypertrophy, liver) 789.1 Gaucher's 272.7 Histiocytosis (acute) (chronic) (subacute) 277.89 lipid, lipoid (essential) 272.7 Hyperlipidosis 272.7 hereditary 272.7 Lipidosis 272.7 [icd9data.com]
Skin
- Angiokeratoma
[…] corporis diffusum) 272.7 Syndrome - see also Disease cardiovasorenal 272.7 Fabry (-Anderson) (angiokeratoma corporis diffusum) 272.7 Niemann-Pick (lipid histiocytosis) 272.7 Ruiter-Pompen (-Wyers) (angiokeratoma corporis diffusum) 272.7 sea-blue histiocyte [icd9data.com]
Angiokeratoma corporis diffusum universale (Fabry): first American report of a rare disorder. Arch. Int. Med. 1955; 95: 469 Scopus (20) Crossref Google Scholar Hermansky F. Pudlak P. [amjmed.com]
- Purpura
Thrombocytopenic purpura with histiocytosis of the spleen. New England J. Med. 1961; 265: 572 Scopus (23) PubMed Crossref Google Scholar Fessas P. Wintrobe M.M. Cartwright G.E. [amjmed.com]
272.7 Lipoidosis (see also Lipidosis) 272.7 Mucolipidosis I, II, III 272.7 Niemann-Pick disease (lipid histiocytosis) (splenomegaly) 272.7 Pick's disease Pick-Niemann disease (lipid histiocytosis) 272.7 Pseudo-Hurler's disease (mucolipidosis III) 272.7 Purpura [icd9data.com]
Workup
Biopsy
- Foam Cell
Observations on foam cells in thalassemia. Blood. 1960; 16: 1039 PubMed Google Scholar Dooren L.J. Von Gelderen H.H. Vink C.L.J. Exudative enteropathy. Acta paediat. 1961; 50: 249 Scopus (1) PubMed Crossref Google Scholar Crocker A.C. Farber S. [amjmed.com]
Treatment
Treatment with a low-fat, cholesterol-free and low caloric diet combined with lipotropic agents resulted in a moderate decrease of the serum cholesterol levels and in disappearance of some of the xanthomatous lesions. [gastrojournal.org]
Pathophysiology
Distribution of glycerolipids, metabolism, and pathophysiological correlations. [link.springer.com]
Prevention
This treatment, however, did not prevent increased endogenous production of cholesterol; it did not interfere with production of pericholangiolar fibrosis, and it did not alter the course of the disease sufficiently to prevent a fatal outcome. [gastrojournal.org]