Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0020639 (hypoproteinemia)
 1,134 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A simian acquired immunodeficiency syndrome (SAIDS) associated with retroperitoneal fibromatosis (RF) has been observed in several species of macaque at the Washington Regional Primate Research Center. Clinical signs were recurrent diarrhea, weight loss, mesenteric lymphadenopathy, and opportunistic infections. Most affected macaques in the later stages of illness showed marked immunodeficiency. Response of peripheral blood mononuclear cells to mitogens was impaired significantly. There was sharply depressed primary and secondary antibody response to the T-cell dependent antigen, bacteriophage phi X174. Affected monkeys did not switch from IgM to IgG antibody following a secondary immunization, as did normal macaques. Twenty-four (67%) of 36 affected animals with progressive RF or deteriorated stages of illness had hypoproteinemia and hypoalbuminemia. Quantitative serum immunoglobulins of 23 cases showed that eight (35%) had hypogammaglobulinemia, six (26%) had hypergammaglobulinemia, and the remainder (39%) were within the normal range. Opportunistic infections were predominantly bacterial pathogens. Type D retrovirus appeared to be closely associated with RF-affected macaques (12/12 or 100%). The case fatality rate (including animals sacrificed after prolonged illness) was 98%. The leading cause of death was due directly to RF lesions in 43%, to enterocolitis in 36%, septicemia in 12%, amyloidosis in 5%, and malignant lymphoma (2%). Clinical, immunologic and pathologic changes reveal an acquired immunodeficiency syndrome that has many similarities to human AIDS. SAIDS and RF may be a useful model for studying human AIDS.
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PMID:Retroperitoneal fibromatosis and acquired immunodeficiency syndrome in macaques: clinical and immunologic studies. 348 18

Thirty-five cases of histiocytosis X in the National Children's Hospital were clinicopathologically studied. Fourteen cases were categorized in diffuse histiocytosis X, Letterer-Siwe type (DHX), 19 cases in multifocal eosinophilic granuloma (MEG) and 2 cases in unifocal eosinophilic granuloma (UEG). Nine of 14 DHX died, of which 6 died of opportunistic infection due to hypoproteinemia and pancytopenia, and 3 died of pulmonary fibrosis probably due to histiocytic infiltration and resultant lymphedema. Infiltration of histiocytes in the bone marrow, thymus and lungs, in addition to the lymphoreticular organs, was conspicuous in autopsy cases of DHX. Skin biopsy was valuable for diagnosis and the immunostaining with anti-S100 antibody was a good marker to characterize infiltrating histiocytes. Prognostic factors and effects of treatments were also evaluated. Only one of 19 MEG died of opportunistic viral infection, but a longer duration for treatment was usually necessary compared to that for DHX. Pathogenesis of histiocytosis X was discussed in relation to T-zone histiocytes.
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PMID:A clinicopathological study of histiocytosis X. 633 90