Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This report deals with a case of double gammopathy (IgM-kappa, IgG-lambda) with Crow-Fukase syndrome, which developed into primary macroglobulinemia four years after the diagnosis. In May 1980, a 74-year-old woman was admitted to the hospital because of a rapid progression of peripheral neuropathy. The patient was diagnosed as having Crow-Fukase syndrome from the following data: albumin-cytologic dissociation of cerebrospinal fluid, peripheral edema, diffuse hyperpigmentation of the skin, diabetic glucose intolerance, serum double gammopathy (IgM-kappa, IgG-lambda) and hepatomegaly. The administration of prednisolone yielded the improvement of neuropathy. In December 1984, serum IgM level was increased from 104 mg/dl to 3,025 mg/dl. Plasma cells in the bone marrow increased in the percentage from 5.6% to 18.4%, and then Bence Jones protein (kappa type) was excreted in the urine. No antibody activity to myelin antigens was detected in the serum. The patient died of cerebral infarction in 1985. At postmortem examination, lymphomatous involvement was found in the jejunum. At the immunohistological examination of the tumor specimens, the morphology and the distribution of IgM- and IgG-positive cells corresponded to that of kappa- and lambda-positive cells, respectively. A small number of cells containing both kappa and lambda light chains were also demonstrated. It seems likely that IgM (kappa)- and IgG (lambda)-positive cells were derived from the common precursor cells.
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PMID:[Progression from Crow-Fukase syndrome with double gammopathy (IgM-kappa, IgG-lambda) to primary macroglobulinemia]. 194 31

Case records of HIV infected patients were analyzed for identifying neurological manifestations. Eight patients (7 males) were identified to have probable HIV encephalopathy (in a period of 24 months) as per the CDC revised classification system. Their ages ranged from one year to ten years. The neurological manifestations noted included-developmental delay (2 cases), seizures (6 cases), acute onset alteration of sensorium (4 cases), aphasia (2 cases), loss of vision (2 cases), focal neurological deficits (6 cases), brisk deep tendon reflexes (7 cases), extensor plantar responses (5 cases) and signs of cerebellar dysfunction (2 cases). Other clinical features included growth failure, microcephaly, fever, lymphadenopathy, hepatomegaly, splenomegaly, pneumonia, otorrhea and oral candidiasis. Cerebrospinal fluid studies were normal. The neuroimaging features included cerebral atrophy and ventricular dilatation, cerebral infarction, basal ganglia calcification and cerebellar atrophy. Childhood HIV infection may have a variety of neurological abnormalities. HIV infection should be suspected in children presenting with unexplained neurological manifestations and growth failure.
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PMID:Neurological manifestations of HIV infection. 1265 56