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)

We report the findings in 21 Belgian patients (12 males and 9 females, median age 61 years) with LGLPD. Symptoms at presentation included infection (n = 9), weight loss (n = 5), asthenia (n = 9), pruritus (n = 2) and arthralgia (n = 7). Four patients were asymptomatic. The main clinical findings were hepatomegaly (n = 5), splenomegaly (n = 8), lymph node enlargement (n = 3) and arthritis (n = 5). All patients had an increased LGL count associated with anemia (n = 12), neutropenia (n = 17), often less than 0.5.10(9)/L (n = 10) and thrombocytopenia (n = 6). Three patterns of lymphocyte surface markers were observed: CD3+CD4-8+ (14 patients), CD3+CD4-8+ (5 patients) and CD3+CD4+8- (1 patient). An abnormal karyotype was found in 2 patients. T-cell receptor gene was rearranged in all cases tested (9/9).
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PMID:Large granular lymphocyte proliferative disease: 21 Belgian cases and review of the literature. 131 80

We present a patient complaining of abdominal pain during eighteen months, localized in left subcostal region, weight loss, asthenia and anorexia for the previous 5 months and fever one month before his admission. He presented painful hepatomegaly, leukocytosis, with an increased percentage of mature and inmature neutrophils, anemia, elevated erythrocyte sedimentation, thrombocytosis, hypergammaglobublinemia and dissociated cholestasis. Complementary exams demonstrated the presence of a poorly vascularized occupying process, localized in the left hepatic lobe. Following laparotomy and biopsy-resection of the process in hepatic left lobe, staining revealed isolated, Gram + filaments and histopathological study showed multiple micro-abcesses displaying actinomyces-like characteristics. There was no evidence of any other pathological process except for previous history of dental abscesses and extractions, which could represent the primary focus for hematogenous dissemination. There was satisfactory response to erythromycin.
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PMID:[Primary hepatic actinomycosis]. 361 61

The authors describe five white patients with peripheral T-cell lymphoma. Four patients were older than 65 years. All cases presented with a short clinical course and advanced stage at the time of diagnosis. Clinical manifestations included asthenia, weight loss, peripheral and abdominal lymphadenopathy. One case showed tonsillar involvement and subcutaneous lymph node enlargement; hepatomegaly was present in four cases, two of them with splenomegaly. Only one case presented peripheral lymphocytosis and antibodies to human T-leukemia virus. Although three cases were classified as diffuse mixed lymphomas and two as poorly differentiated lymphocytic lymphomas, there were some common characteristics: diffuse infiltration by different proportions of small lymphoid cells and large immunoblasts, some of them multinucleated and similar to Reed-Sternberg cells; accumulation of histiocytes, plasmacytosis, eosinophilia, venular proliferation and compartmentalization were also found. Bone marrow infiltration was observed in two patients. Results of monoclonal markers showed four cases to be OKT4+ and the other OKT8+. The morphologic and immunologic characteristics of these patients were typical and similar to those reported from other geographical areas.
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PMID:Peripheral T-cell lymphoma. A clinical, histologic, and immunologic study of five cases. 387 77

This report describes a third mucopolysaccharidosis in animals: canine mucopolysaccharidosis VII. The affected dog was the offspring of a father-daughter mating. Weakness in the rear legs was evident at 8 weeks of age and became progressively worse. He had a large head, a shortened maxilla, and corneal granularities. Most joints were extremely lax, easily subluxated, with joint capsules that were swollen and fluctuant. The dog was alert and had apparently normal pain perception. At 13 months of age, there was radiographic evidence of extensive skeletal disease including bilateral femoral head luxation, abnormalities in the shape and density of the carpal and tarsal bones, radiolucent lesions of the epiphyseal regions of most long bones, and cervical vertebral dysplasia and platyspondylia. The electrophoretic pattern of precipitated glycosaminoglycans indicated a predominance of chondroitin sulfate. The animal died suddenly from gastric dilatation. There was generalized hepatomegaly, thickening of the atrioventricular heart valves, and generalized polyarthropathy. Vacuolated cytoplasm was observed in hepatocytes, keratocytes, fibroblasts, chondrocytes and cells of the synovial membrane, retinal pigment epithelium, and cardiac valves. Neurons had cytoplasmic vacuoles. Electron microscopy demonstrated membrane-bound cytoplasmic inclusions in polymorphonuclear leukocytes, hepatocytes, synovium, heart valves and spleen. The activities of 12 lysosomal hydrolases were determined in liver from the affected and control dogs: beta-glucuronidase (EC 3.2.1.31), beta-hexosaminidases A and B (EC 3.2.1.30), alpha-hexosaminidase (EC 3.2.1.-), alpha-L-iduronidase (EC 3.2.1.76), alpha-galactosidase A (EC 3.2.1.22), beta-galactosidase (EC 3.2.1.23), arylsulfatases A and B (EC 3.1.6.1), acid alpha-mannosidase (EC 3.2.1.24), acid beta-mannosidase (EC 3.2.1.25), and N-acetyl-D-galactosamine-6-sulfate sulfatase (EC 3.1.6.-).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Beta-glucuronidase deficiency in a dog: a model of human mucopolysaccharidosis VII. 643 80

The authors describe a case of lymphoblastic lymphoma of the renal lodge complicated by hemorrhagic pericarditis, cardiac tamponade and quickly evolved in an unfavourable way. Lymphoblastic lymphoma is classified into lymphocytic lymphomas (or non-Hodgkin) with a higher grade of malignancy. These lymphomas have, in 40% of cases, an initially extra-lymph node localization: among these, the renal or perirenal localization is not very frequent. The concomitant presence of a pericarditis of hemorrhagic type is also less frequent. Unlike Hodgkin's lymphomas, lymphocytic lymphomas are frequently in an advanced stage at the moment of diagnosis, and their evolution is of acute-subacute, often fatal type. The case report is about a 70 year old male, who arrived at the clinical investigation for the subjective presence of asthenia, dyspnea, tachycardia and the objective evidence of jugular turgor, hepatomegaly and distal edemas, hypophonesis of right lung basis, according to a clinical picture of systemic venous congestion which instrumental examinations confirm to be of pericardial origin. The abdominal echographic examination showed an occupation of the perirenal space, so the CAT characterized as a 25 cm long formation (from the renal cavity to pelvis), with a dislocation of close structures. Unfortunately, the clinical picture had a quick evolution towards the exitus for cardiac tamponade, only permitting us a bioptic examination for the diagnosis of the case.
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PMID:[Lymphoblastic lymphoma of the renal cavity associated with hemorrhagic pericarditis. Description of a case]. 785 60

A 78-year-old man admitted to our hospital with signs and symptoms of right ventricular failure, consisting of severe edema of the scrotum and the penis, ankle edema, hepatomegaly, and a history of asthenia associated with a recent weight loss. Two-dimensional echocardiography showed an intracavitary mass in the right atrium and a moderate pericardial effusion; the remaining structures were normal. To better define the origin of this mass, transesophageal echocardiography was performed. The mass extended from the inferior vena cava with no sites of attachment to the atrial wall. The mass was elongated, mobile, with a triangular termination near the tricuspid valve, without signs of right ventricular obstruction. An abdominal-pelvic CT scan demonstrated the origin of the mass at the superior pole of the left kidney extending through the renal vein and the inferior vena cava into the right atrium. The mass was surgically removed and the pathological examination revealed a renal cell carcinoma (hypernephroma) of the clear cells subtype. After surgery, the patient did well with the resolution of the picture of right ventricular failure.
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PMID:[Multiplanar transesophageal echocardiography in a case of hypernephroma]. 785 33

Epidemiological, clinical, biochemical and topographic features of primary hepatic cancer (PHC) were reviewed retrospective and prospectively in this study. This review consisted of 76 patients from 1971 to 1990. Forty nine males and 27 females. The mean age was 66.1 +/- 11.7 years. Hepatocellular carcinoma (HC) was the most frequent histological type (84.1%), followed by cholangiocarcinoma (87.7%). Mixed carcinoma and hepatoblastoma were 4.3 and 2.9% respectively. The prevalence af PHC among 1485 autopsies was 0.74%. The most frequent sites af metastasis were the lungs (66%) and portal vein (50%). Hepatocellular carcinoma was associated to cirrhosis in 80% of the cases. A syndrome including asthenia, weight loss, hepatomegaly and cholestasis was identified in most of the patients, and alkaline phosphatase was the most frequently disturbed laboratory test. 60% of tumors were bilateral and none of the solitary tumors had less than 5 cms in diameter. 20% of HC showed normal serum levels of AFP (< 20 ng/ml). 40% had at least one of the markers of B virus hepatitis in serum.
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PMID:[Primary liver cancer. Its epidemiological, clinical and biochemical characteristics]. 820 48

We report a case of Zieve's Syndrome that developed after an important alcohol consumption in a 32-yr-old female patient. She was admitted to the hospital with anorexia, asthenia and jaundice. Physical examination showed liver stigmata and hepatomegaly. Laboratory tests demonstrated increased aminotransferase levels, hyperbilirubinemia, hyperlipidemia and normocytic and normochromic anemia with dianocytes in peripheral blood smear. Ultrasonography showed a hyperechoic liver and a liver biopsy showed acute and chronic alcoholic liver disease. Clinical evolution was satisfactory and the therapy consisted of blood transfusion, parenteral fluids, B-complex vitamin and a fatty free diet. Jaundice, hyperlipidemia and haemolytic anemia define Zieve's Syndrome (Z.S.) There is a pathogenetic relationship among the clinical and biological phenomena in this syndrome, whose starter is an acute alcohol intake. Haemolysis is the distinctive feature with respect to the classical acute alcoholic hepatitis, and it is due to erythrocyte's metabolic and osmotic instability in relation to lipids abnormalities. Its clinical resolution precedes the normalization of serum lipids levels. Therapy is similar to that for acute alcoholic hepatitis although sometimes the anemia requires blood transfusion.
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PMID:[Zieve's syndrome. A case report]. 864 20

Non-Hodgkin's lymphomas presenting exclusively in the liver are rather uncommon in adults and extremely rare in children. We describe a six-year-old white boy with jaundice, abdominal pain, and weight loss of two weeks duration. Physical examination disclosed asthenia, jaundice, abdominal swelling, large hepatomegaly, and ascitis. Aminotransferases bilirubin, and alkaline phosphatase were significantly elevated. Bone marrow aspiration, cerebrospinal fluid, chest x-ray, renal function tests, and uric acid were normal. Abdominal ultrasound showed liver enlargement with irregular regular borders, many parenchymal nodules in both liver lobes, a large hypoechogenic mass in the inferior segment of the liver, normal biliary ducts and two pancreatic nodules resembling those in the liver. Liver needle biopsy disclosed diffuse lymphomatous infiltration. Blast cells were positive for leukocyte common antigen (CD 45). Immunohistochemistry study for T or B cell lineage differentiation was not done. The child showed an excellent response to chemotherapy based on the BFM-83 protocol for B cell non-Hodgkin's lymphomas. The patient had his therapy discontinued in June 1995 and remains in first complete remission as of May 20th, 1996.
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PMID:Primary hepatic non-Hodgkin's lymphoma in children: a case report and review of the literature. 912 4

Primary plasma cell leukaemia (P-PCL) is a variant of multiple myeloma (MM) first diagnosed in the leukemic phase, with >2000/mm(3) circulating plasma cells (PCs) and plasmacytosis >20% of the white cell count. We investigated the clinical characteristics, therapy, immunophenotype and prognosis factors of 18 patients. Common features at diagnosis were asthenia (seven patients), renal insufficiency (ten patients), bone pain (seven patients), splenomegaly or hepatomegaly (five patients). Hypercalcemia was present at diagnosis in seven patients and was the most potent poor prognosis factor (P<0.05). Most patients (16 out of 18) were treated with an anthracyclin containing regiment; complete remission was attained in one patient and partial remission in 11 patients while six patients had no response. The median survival time from diagnosis was 7 months (2--12, 95% confidence interval), but response to treatment had favorable predictive value (P<0.05). The PCs were usually positive for mature B-cell markers (PCA-1, CD38). They expressed integrins which may increase their binding to endothelial cells and thus participate in PCL physiopathology by favoring plasmocyte extramedullary spread.
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PMID:Primary plasma cell leukaemia: a report of 18 cases. 1116 24


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