Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 32-yr-old female who suffered from typical Weber-Christian disease developed hepatosplenomegaly and hepatic dysfunction. A liver biopsy specimen displayed many fat droplets and alcoholic hyalins (Mallory bodies). Electron microscopic hepatic injury was indicated by many fat droplets, alcoholic hyalins, marked deformities of the rough endoplasmic reticulum, and by some deformed nuclei and nuclear bodies. There has been no other report describing alcoholic hyalins in the hepatic changes of Weber-Christian disease. Additionally, based on electron microscopic observation, we assume that fatty changes were due to a relative reduction in lipoprotein synthesis in the deformed rough endoplasmic reticulum.
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PMID:Alcoholic hyalins (Mallory bodies) in a case of Weber-Christian disease: electron microscopic observations of liver involvement. 615 73

Five patients had a histiocytic, cytophagic panniculitis and a febrile, progressive course, which included hepatosplenomegaly, pancytopenia, liver function abnormalities, and a hemorrhagic death. The terminal hemorrhage was characterized by features of intravascular coagulation and liver failure. The histiocytosis could be found at times also in bone marrow, lymph nodes, liver and spleen, and serosal tissues, as well as in skin and subcutaneous tissue. Adequate biopsy diagnosis will prevent such cases from being labeled as Weber-Christian disease with bleeding, and offer an opportunity for appropriate treatment of this new syndrome of histiocytic, cytophagic panniculitis.
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PMID:Hemorrhagic diathesis associated with benign histiocytic, cytophagic panniculitis and systemic histiocytosis. 743 42

We report a 35-year-old man, who had been diagnosed with Weber-Christian disease, presented with acute onset of high fever, malaise, jaundice and hepatosplenomegaly with subcutaneous nodules. Laboratory tests showed elevated serum ferritin and liver enzymes, especially lactate dehydrogenase (LDH), with pancytopenia and coagulation abnormalities. Peripheral blood and bone marrow examinations showed erythro-, leuko- and thrombo-phagocytic histiocytes and macrophages. The patient developed the same clinical features seven years ago. Based on diagnosis of cytophagic histiocytic panniculitis, the patient was treated with steroid pulse therapy and oral cyclosporin A. The combination therapy caused a marked improvement in the clinical condition.
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PMID:A case of cytophagic histiocytic panniculitis: successful treatment of recurrent attacks with steroid pulse therapy and oral cyclosporin A. 925 59