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 23-year-old man with persisting high fever developed hepatosplenomegaly, lymphadenopathy and massive pericardial effusion. Immunological examination revealed a marked elevation of anti-Epstein-Barr virus antibodies (anti-viral capsid antigens IgG-antibody 1:10,240, anti-early antigens-DR IgG-antibody 1:5,120), decreased activities of Epstein-Barr virus specific cytotoxic T lymphocytes, natural killer cells and lymphokine activated killer cells. A liver biopsy showed moderate sinusoidal lymphocytosis with punched-out lesions. These findings suggested severe chronic active Epstein-Barr virus infection syndrome. The patient was treated with recombinant human interleukin-2, but it was discontinued because of an adverse reaction. Twelve months later, he died of suspected pulmonary infection.
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PMID:An adult case of severe chronic active Epstein-Barr virus infection syndrome. 133 20

Adult T-cell leukemia/lymphoma (ATLL) is an HTLV-I associated lymphoid malignancy frequently seen in Japan. Abdominal involvement in 40 patients with ATLL were assessed by ultrasonography and the findings seen in four clinical types, acute, chronic, lymphoma and smoldering, were compared. Splenomegaly was frequently found in the cases of acute and lymphoma types, and the sizes of the spleens measured by ultrasonography correlated well with the disease activity. Hepatomegaly was also found more frequently in acute and lymphoma types, and hepatosplenomegaly was proved to be due to the infiltration by ATL cells. Nodular lesions in spleen and liver and abdominal lymph node swelling were also found frequently in the lymphoma type but rarely in the other types. Ascites, pleural effusion, and pericardial effusion were found in the active stage of acute and lymphoma types. Ultransonography also could detect findings associated with therapies. Thus, ultrasonography studies were found to be very useful for assessing the clinical classification, examining various pathological conditions associated with ATLL, and monitoring the disease activity.
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PMID:Assessment of abdominal involvement of adult T-cell leukemia/lymphoma by ultrasonography: comparison among four clinical types. 165 79

A 33-year-old man was admitted to Kushiro City General Hospital on February 27, 1989, because of palpitation, shortness of breath and anasarca. Eight months previously he had noted the onset of pretibial edema, which had progressed to anasarca. He had had a meal only once a day for nine months. Physical examination revealed a blood pressure of 114/46 mmHg and pulse rate of 80/min. The 3rd sound was audible. No rales in the chest and no hepatosplenomegaly were noted. Ascites, pretibial edema and anasarca were present. Vibration sensation was diminished, and the deep tendon reflexes were absent in the legs. The blood thiamine level on the 4th day of hospitalization decreased to 2.9 micrograms/dl. The red cell transketolase activity and TPP effect on the 10th hospital day were 0.76 IU/gHb and 11%, respectively. A chest roentogenogram showed pulmonary congestion and cardiomegaly (CTR 61.3%). The electrocardiogram showed non-specific T wave changes. On the echocardiogram, remarkable pericardial effusion and diffuse hypertrophy of the left ventricular wall were observed. In addition, the left ventricular wall motion showed a hyperkinetic state. On the basis of these findings, the diagnosis of beriberi heart was made. The hemodynamic study performed on the 10th hospital day showed a remarkable high cardiac output (CO) of 10.7 l/min and an extremely reduced total peripheral resistance (TPR) of 352 dynes.sec.cm-5. 15 min after intravenous administration of Fursultiamine 100 mg, CO decreased to 7.24 l/min and TPR increased to 848 dynes.sec.cm-5. Following the administration of Fursultiamine 75 mg, po/day, his symptoms and abnormal findings of clinical examination data rapidly improved.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of beriberi heart--with special reference to the rapid effect of fursultiamine on hemodynamics]. 202 80

We describe an 18-year-old white male who developed lower extremity ischemia requiring amputation. He presented at 14 with pulmonary infiltrates, hepatosplenomegaly, fever, rash, adenopathy, uveitis, and arthralgias; clinical and laboratory findings were consistent with Mycoplasma pneumoniae infection. Despite adequate treatment with antibiotics, he developed chronic arthralgias and fevers, with rash and pericardial effusion. Criteria for the diagnosis of systemic lupus erythematosus were not met; juvenile rheumatoid arthritis was diagnosed presumptively. Over the subsequent 4 years he developed lymphadenopathy with biopsy-proven nonnecrotizing granulomas, chronic leg ulceration with granulomatous histology, and acute-onset impending gangrene of the left foot. A biopsy of the posterior tibial artery demonstrated giant cell arteritis. Although the histologic features were consistent with Takayasu's arteritis, complete aortic arteriography was normal. Examination of the amputated leg showed multifocal segmental giant cell arteritis. Clinicopathologic features suggested, but were not fully consistent with, juvenile systemic granulomatosis. His disease may represent a separate sarcoid-like entity in the broad spectrum of vasculitis.
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PMID:Giant cell vasculitis with extravascular granulomas in an adolescent. 205 10

Natural disease being mistaken for child abuse is rare. A two-year-old child was found unresponsive at home and transported to a local hospital, where she expired in the emergency room. Several cutaneous contusions were observed. Prior to the autopsy it was learned that an anonymous report of "child abuse" had been previously filed concerning this child. At autopsy there were multiple metasynchronous cutaneous contusions, but no radiologic or gross evidence of other injuries. A pericardial effusion, massive hepatosplenomegaly and generalized lymphadenopathy were apparent. The bone marrow, liver, spleen, lymph nodes, kidneys, pancreas, heart, stomach, and dura mater showed a monotonous lymphocytic infiltrate. Immunocytochemical studies confirmed the diagnosis of acute lymphoblastic leukemia of childhood. This case reaffirms the need for an objective examination of all cases by a forensic pathologist.
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PMID:Undiagnosed, untreated acute lymphoblastic leukemia presenting as suspected child abuse. 218 46

A case of primary myelofibrosis complicated with pericardial effusion and proteinuria is described. A 66-year-old female was admitted to our hospital because of abdominal fullness and shortness of breath. On admission, hepatosplenomegaly and pericardial effusion were observed. Blood examination revealed leukoerythroblastic anemia and thrombocytosis with tear drop cells and giant platelets. Bone marrow aspiration was dry tap and its biopsy showed remarkable myelofibrosis. Urinalysis indicated severe proteinuria. Although neutrophilic alkaline phosphatase score was low, no signs of acute blastic crisis of chronic myelogenous leukemia was found. The diagnosis of an atypical type of primary myelofibrosis was obtained. Administration of MCNU was started in August 1987. Hepatosplenomegaly, pericardial effusion and proteinuria were gradually improved after the administration. The etiology of the pericardial effusion and proteinuria were not obvious, however, these facts suggest that these abnormal findings might be related to PMF itself and MCNU was effective to PNF.
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PMID:[The use of MCNU to a patient of primary myelofibrosis complicated with pericardial effusion and proteinuria]. 276 70

An infant boy is described whose clinical findings include congenital ascites, hepatosplenomegaly, postnatal growth failure, dysostosis multiplex, delayed development, pericardial effusion, and the nephrotic syndrome. Death occurred before he reached 2 years of age. Evidence indicates that these abnormalities resulted from an autosomal recessive inherited deficiency of neuraminidase.
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PMID:A severe infantile sialidosis: clinical, biochemical, and microscopic features. 735 70

A 23-year old woman was admitted to our hospital because of fever, leukocytopenia, thrombocytopenia and cervical lymphadenopathy. From pathological findings of the cervical lymphnode she was diagnosed as subacute necrotizing lymphadenitis (SNL). Bone marrow aspiration was hypocellular with proliferation of mature histiocytes with hemophagocytosis. Lactate dehydrogenase was elevated to 1863 IU/l and mild liver dysfunction was observed. Hepatosplenomegaly, lymphnode swelling in abdominal cavity, pleural effusion and pericardial effusion were detected. After glucocorticoid therapy all such disorders were normalized. In this course, Epstein-Barr (EB) virus genome was detected by a polymerase chain reaction method from peripheral mononuclear cells. After normalization, EB virus genome became not to be detected. From present case we concluded that EB virus was expanded in clinical course and was associated with pathogenesis of SNL.
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PMID:[Detected Epstein-Barr virus genome using polymerase chain reaction in peripheral mononuclear cells--subacute necrotizing lymphadenitis]. 769 34

Five patients with proven intra-abdominal tuberculosis were examined with computed tomography (CT) over a period of one year. This revealed hepatosplenomegaly in all five cases. Macroscopic nodules were noted in the liver and spleen in three cases. All three cases showed more severe involvement of the spleen over the liver. Other features were enlarged (para-aortic, para-caval, peripancreatic and mesenteric) nodes, ascites, stellate mesentery and thickened bowel wall. Incidental extra-abdominal findings included intrathoracic nodules, pleural effusion, pericardial effusion with constrictive pericarditis, bony osteomyelitis and psoas abscess. Follow-up CT was performed on four of the five cases and showed significant resolution of the above findings. In addition, one case showed calcification within a healing splenic nodule. The presence of macroscopic nodules detected on CT, especially in the spleen, is an unusual finding in prior reports. This paper attempts to account for its high local incidence and discusses the significance of other findings in the diagnosis of abdominal tuberculosis.
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PMID:Computed tomographic findings of abdominal tuberculosis--report of five cases. 826 65

We reported a case of Crow-Fukase syndrome and examined the mechanism of hepatomegaly in Crow-Fukase syndrome. A 67-year-old woman was presented with polyneruopathy, hepatosplenomegaly, pericardial effusion and M proteinemia. On examination, all laboratory data and clinical symptoms were compatible with Crow-Fukase syndrome. The patient was treated with prednisolone and immunoadsorption plasmaphresis therapy. All the clinical manifestations including hepatomegaly gradually improved. In order to examine whether some factors that promote the prolifelation of hepatocyte may exist in the patient's serum, we cultured mouse hepatocyte in the presence of patient's or control serum together. Though the number of hepatocytes decreased after 2 days culture, the number of hepatocytes cultured with patient's serum in active disease stage remained significantly greater than of hepatocytes cultured with either control serum or patient's serum in healing stage. There were no abnormal pathological findings in biopsied liver. Liver suggesting that hepatomegaly was the results of normal hepatocytes proliferation. Taken together, these findings suggest there were some factors that may promote the proliferation of hepatocytes or may have protective effect of hepatocyte in patient's serum. Though the level of human hepatocyte growth factor (h-HGF) in patient's serum in active disease stage was slightly increased, hepatomegaly cannot be attributable solely to h-HGF. Organomegaly is one of the important symptoms of the Crow-Fukase syndrome, however, in so far as we are aware its mechanism is not examined. In this report it is suggested that several unknown factors other than h-HGF may contribute the hepatomegaly of the Crow-Fukase syndrome.
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PMID:[The detection of factors that may promote the hepatocyte proliferation in the serum of a patient with crow-fukase syndrome]. 885 11


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