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)

Sixty-one patients with essential thrombocythemia (ET) were followed from 1974 through 1987 at the Medizinische Poliklinik. Fifty-one patients (84%) presented with thromboembolic complications, and eight patients (13%) with hemorrhages. In seven patients (12%), a thrombocytosis was detected accidentally. Disturbances of the microcirculation (67%), mainly of the fingers and toes (53%), were the most frequent thromboembolic symptoms. The mean age of all patients was 58 years (male patients, 61 years; female patients, 56 years). The average platelet count at diagnosis was 897,000/microliter. The average maximal platelet count was 1.231 X 10(6)/microliter (range, 500,000/microliter to 4 X 10(6)/microliter). Seventy-two percent had a moderate leukocytosis (average, 12,400/microliter), 34% a splenomegaly, 29% a hepatomegaly. Signs of hypermetabolism were infrequent, lactate dehydrogenase (LDH) and uric acid elevations, if present, were moderate. Bleeding time and viscosity were normal in most patients. Spontaneous platelet aggregation was increased in 81% of patients (n = 40). Platelet aggregation studies with the aggregation inducing substances adenosine diphosphonate (ADP), platelet activating factor (PAF), thrombin, collagen, and adrenalin showed hypoaggregation in most patients. Adrenalin-induced aggregation distinguished best between ET-patients and reactive thrombocytosis showing hypoaggregation in all ET-patients tested (n = 16) and in none of 22 controls. Bone marrow studies were performed in 57 patients. The histologic studies (done in 49 patients) were consistent with a chronic myeloproliferative disorder in all cases. In 41 cases (84%) the picture of a megakaryocytic myelosis was found, in 12 of these a granulocyte-rich form of megakaryocytic myelosis. Cytologic studies only (eight patients) did not differentiate ET well from reactive thrombocytosis. Platelet aggregation studies and bone marrow histology may be of help in the diagnosis of difficult cases of thrombocytosis. The Philadelphia status was negative in all cases studied (14 patients). Fourteen patients died. The causes of death were thromboembolic complications in probably 11 and acute leukemia in two patients. The probability of 10-year survival is 64% after a mean follow-up time of approximately 5 years. It appears that considering the average age of ET patients at diagnosis, life expectancy is close to normal.
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PMID:Essential thrombocythemia. Clinical characteristics and course of 61 cases. 336 70

A 50-year-old black female developed hepatic dysfunction secondary to captopril therapy, 25 mg tid for one month. Liver biopsy showed primarily cholestasis, with secondary hepatocellular elements. Symptoms consisted of jaundice, pruritus, anorexia and weight loss, hepatomegaly, and abdominal tenderness. Total bilirubin, alkaline phosphatase, lactate dehydrogenase (LDH), and serum glutamic-oxaloacetic transaminase (SGOT), reached highs of 506 mumol/L, 737 U/L, 319 U/L, and 100 U/L, respectively. Recovery was slow, but complete, after discontinuation of the drug.
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PMID:Captopril-induced cholestatic jaundice. 636 96

The serum levels of soluble intercellular adhesion molecule 1 (ICAM-1) were significantly elevated (P < .001) in patients with chronic B-lymphocytic leukemia (B-CLL, n = 113) compared with healthy controls (n = 31). sICAM-1 levels in B-CLL were positively correlated to the tumor mass as reflected by the modified Rai and the Binet staging systems, lymphocyte counts, and isolated spleno/hepatomegaly. During disease progression or regression on cytoreductive therapy, the circulating sICAM-1 levels changed accordingly. sICAM-1 was also correlated to a kinetic parameter such as the lymphocyte doubling time. Furthermore, the serum sICAM-1 levels were inversely correlated to hemoglobin levels in patients with early clinical stage, and this may turn out to be of prognostic value. sICAM-1 was compared with other serum markers said to reflect disease activity in B-CLL, ie, soluble CD23, thymidine kinase, lactate dehydrogenase (LDH), and beta 2-microglobulin. sICAM-1 was equally well or better correlated to clinical stage and lymphocyte doubling time. In univariate regression analysis, all serum markers but LDH correlated with survival, and in multivariate analysis, sICAM-1 was the only marker approaching significance for additional prognostic information when included after clinical stage and lymphocyte doubling time. Based on the present observations, it appears that prospective studies repeatedly monitoring serum sICAM-1 in B-CLL are justified.
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PMID:Serum levels of soluble intercellular adhesion molecule 1 are increased in chronic B-lymphocytic leukemia and correlate with clinical stage and prognostic markers. 777 31

A case of primary lymphoma of the liver in a patient with acquired immunodeficiency syndrome (AIDS) is presented. Persistent fever, tender hepatomegaly, and mildly abnormal liver function tests combined with an elevated lactate dehydrogenase level gave the clue for the diagnosis. High-grade, non-Hodgkin's lymphoma of B-cell phenotype was demonstrated by percutaneous liver biopsy. Primary hepatic lymphoma is a rare event among patients with AIDS. We reviewed the cases in the literature and noted that a preponderance of these patients presented with multiple lesions (a rather rare occurrence in primary lymphoma of the liver in patients not infected with the human immunodeficiency virus).
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PMID:Primary lymphoma of the liver in AIDS. Report of one new case and review of the literature. 877 98

This report presents new data on mammalian peroxisomes by studying an unusual rodent: the jerboa (Jaculus orientalis). This animal exhibits some unique peroxisomal properties compared to the rat, such as higher cyanide-insensitive palmitoyl-CoA oxidase specific activity, pattern differences in SDS-PAGE peroxisomal proteins as well as in acyl-CoA oxidase immunoblotting. There is also a peculiar response to a peroxisome proliferator, ciprofibrate. With 250 ppm of ciprofibrate in the diet for 2 weeks, we observed a limited liver peroxisome proliferation as well as a palmitoyl-CoA oxidase activity, enzyme content and mRNA increase. However, there was no increase in catalase activity, nor hepatomegaly which are prominent features of peroxisome proliferation in rats treated under the same conditions. The palmitoyl-CoA oxidase activity increase was weak in the kidney and not observed in the heart. Other subcellular organelle marker enzyme activities did not significantly change, especially the mitochondrial D-3-hydroxybutyrate and succinate dehydrogenases, lysosomal acid phosphatase, cytosolic lactate dehydrogenase and the endoplasmic reticulum NADPH-cytochrome c reductase. However, the activity of the liver membrane endoplasmic reticulum linked omega-lauryl hydroxylase (cytochrome P450 IV A1) increases after ciprofibrate treatment. Jerboa also behaves differently compared to the guinea pig after ciprofibrate treatment since the guinea pig has a weak response towards peroxisome proliferators. In conclusion, this first peroxisome study utilizing a different type of rodent as a laboratory animal, reveals that the jerboa shows unique peroxisome properties and responds in a moderate manner to a peroxisome proliferator, ciprofibrate, without leading to any increase in liver mass. This supports the fact that fibrate molecules may have different targets depending upon the species.
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PMID:Properties of peroxisomes from jerboa (Jaculus orientalis). 879 87

It has been reported that the administration of interferon alpha-2b is of potential benefit in the treatment of mastocytosis based on a single patient study (NEJM, Feb 27, 1992, 326(9):619-623). Following this report, we administered interferon alpha-2b at a dose of 4 to 5 million units per square meter of body surface area for at least 12 months to one patient with mastocytosis with an associated hematologic disorder (patient 1), one patient with aggressive systemic mastocytosis (patient 2), and one patient with indolent mastocytosis (patient 3). Patients were monitored with the following clinical and laboratory parameters: serial bone marrow biopsies and aspirates, patient log of histamine release attacks, medication dependency, plasma tryptase levels, serum lactate dehydrogenase (LDH) levels, white blood cell counts and differentials, extent of urticaria pigmentosa lesions, bony involvement, and extent of gastrointestinal involvement and hepatomegaly. We also examined the ability of interferon alpha-2b to inhibit recombinant human stem cell factor (rhSCF)-dependent mast cell proliferation from CD34+ bone marrow-derived cells. All patients demonstrated continued progression of disease in one or more clinical criteria at one year of therapy. Similarly, interferon alpha-2b did not inhibit the culture of mast cells from CD34+ bone marrow-derived cells in the presence of SCF. Thus, in our study of three patients with systemic mastocytosis, treatment with interferon alpha-2b was found to be ineffective in controlling progression of disease.
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PMID:Treatment of three patients with systemic mastocytosis with interferon alpha-2b. 888 64

In this report, we describe four cases of small-cell carcinoma of the lung manifesting as acute hepatic failure. These cases were noteworthy for the presence of hepatomegaly and substantially increased serum lactate dehydrogenase and uric acid levels. The ratio of normalized serum lactate dehydrogenase to normalized serum alanine aminotransferase from the 4 cases reported herein (mean +/- SE, 3.63 +/- 1.10) was significantly greater than the ratio obtained from the 12 cases of nonmalignant fulminant hepatic failure (mean +/- SE, 0.46 +/- 0.18; P < 0.001). Chest radiographs and abdominal imaging studies showed no neoplastic process in three of the four cases. Postmortem examinations disclosed extensive infiltration of the liver by metastatic small-cell carcinoma of the lung. A review of the literature revealed 13 additional similar cases. We conclude that metastatic small-cell carcinoma of the lung should be considered in cases of acute hepatic failure associated with hepatomegaly, substantially increased lactate dehydrogenase levels in comparison with alanine aminotransferase values, and increased uric acid levels even if imaging studies show no lesion. A liver biopsy done early during the hospital course is appropriate for diagnosis and for prevention of inappropriate transfer of the patient to a liver transplant center.
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PMID:Small-cell carcinoma of the lung manifesting as acute hepatic failure. 927 11

We examined 111 patients with acute type- or lymphoma type-adult T-cell leukemia (ATL) and compared them with 106 patients with non-Hodgkin's lymphoma (NHL). In addition to skin involvement and hypercalcemia which are already known to be frequent in ATL, ATL patients showed an higher incidence of hepatic involvement. There was more frequent palpable hepatomegaly, higher total bilirubin, GOT, GPT, lactate dehydrogenase (LDH), and alkaline phosphatase values in ATL than in NHL patients (p < 0.0001). Among 36 autopsied liver samples, invasion of ATL cells was confirmed in 22 cases. ATL patients with impaired hepatic function showed shorter survival times than patients without hepatic dysfunction. Moreover, ATL patients showed a worse performance status (PS), a higher incidence of lytic bone lesions, lower total protein (TP) and serum albumin levels than NHL patients. This invasive characters of ATL cells and consequent impaired general condition seemed to be factors affecting the poor prognosis recorded in ATL.
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PMID:Frequent hepatic involvement in adult T cell leukemia: comparison with non-Hodgkin's lymphoma. 932 95

Myotonic dystrophy (DM) is an autosomal dominant multisystem disorder. Little evidence suggests the existence of liver damage in a small number of patients. We have prospectively evaluated liver and gallbladder function in 53 patients with DM in relation to clinical and genetic parameters. None of the patients had an enlarged liver, signs of cirrhosis, or portal hypertension. All were free of medication, and none were pregnant or had a history of alcohol abuse. In 35 (66%) patients, serum activity of at least one of six liver enzymes assayed was abnormal. An elevated level of alkaline phosphatase was found in 50.9%, of gamma-glutamyltransferase in 52.8%, of 5' nucleotidase in 43.4%, of serum aspartate aminotransferase in 35.8%, of serum alanine aminotransferase in 33.9%, and of lactate dehydrogenase in 37.7%. Liver function test results did not correlate with severity of muscle weakness, disease duration, or serum levels of creatine kinase, glucose, or lipids. Motility of gallbladder and abdominal ultrasonography were normal. Cytosine-thymidine-guanine repeat expansion by southern blot did not correlate with liver enzyme abnormalities. We conclude that elevation of liver enzymes is frequent in DM and should be included as an additional laboratory finding of the disease.
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PMID:Abnormal liver test results in myotonic dystrophy. 964 14

A high frequency of disseminated intravascular coagulation (DIC) in adult acute lymphoblastic leukemia (ALL) has been reported; however, its clinical relevance and characteristics have not been fully determined. We studied 67 adults with newly diagnosed ALL between 1982 and 1996 to clarify these questions. DIC was diagnosed in ten of 64 patients (16%) who underwent coagulation study at presentation and in 14 of 40 patients (35%) screened for DIC within 7 days after starting remission induction therapy. Overall, 24 of 67 patients (36%) had DIC during this period. Hemorrhagic symptoms were generally mild, while two patients required red blood cell transfusions. Patients who developed DIC had higher white blood cell counts and more frequently a palpable spleen than those who did not. There was no difference in age, French-American-British subtype, karyotype, immunophenotype, lactate dehydrogenase level, percentage of blasts in bone marrow, or frequency of lymphadenopathy or hepatomegaly between patients who had DIC and those who did not. Fibrinolysis tended to be milder in DIC complicating ALL than in that complicating acute promyelocytic leukemia; however, there was no difference in other coagulation parameters between these two subtypes. An etiological link between CD34 expression in common ALL patients and DIC was suggested.
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PMID:Disseminated intravascular coagulation in acute lymphoblastic leukemia at presentation and in early phase of remission induction therapy. 969 14


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