Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The combination of bilateral lytic lesions in the bones of the lower and upper extremities and presenile dementia is characteristic of polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy, also known as Nasu-Hakola disease. The clinical course of this rare and fatal disorder is characterized by pathologic fractures of these often painful lesions, rapid progression of dementia, and death in the fifth decade of life. The radiographic changes may be confused with cystic angiomatosis, focal metastasizing hemangioendothelioma, or Langerhans' cell histiocytosis. We report five patients to illustrate the clinical presentation, radiographic images, psychiatric abnormalities, and new genetic findings. Three of the patients were siblings. A biopsy is not needed to confirm the diagnosis of polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy because of the unique combination of radiographic and neurologic features.
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PMID:Nasu-Hakola disease (PLOSL): report of five cases and review of the literature. 1690 6

At the close of the 20th century, a selection of articles published in 1999 with relevance to liver pathology reflects the wealth of technological and intellectual progress made during the span of the century. Immunohistochemical staining for hepatitis B virus antigens focused attention on a correlation between cytoplasmic expression of core antigen in individuals with precore mutants and higher activity of hepatitis. Infection of ducklings with a presurface mutant strain of duck hepatitis B virus produced cytopathic liver cell damage. Fibrosing cholestatic hepatitis, originally described as an unusual form of recurrent hepatitis B after liver transplantation, has now been described in hepatitis C virus-positive patients with renal transplants. It may be related to the emergence or selection of hepatitis C virus quasispecies. In biliary tract disease, researchers investigated the canal of Hering as a possible source of hepatic stem cells, sporadic mutations in the JAGGED1 gene (involved in cell differentiation) in Alagille syndrome, and several models of nonsuppurative destructive cholangitis. Further work was accomplished on nonalcoholic steatohepatitis, including a proposal of a grading and staging system as well as its detection in workers exposed to volatile petrochemicals. Among hepatic neoplasms and proliferative disorders, epithelioid hemangioendothelioma, angiomyolipoma and Langerhans' cell histiocytosis received coverage in articles describing the diagnostic pathology in collected series of patients.
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PMID:Pathology of the liver. 1702 77