Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the clinical outcome of 105 essential mixed cryoglobulinemia (EMC) patients with renal involvement collected throughout 25 years in three renal Units of Milan. The median follow-up was 72 months since renal biopsy and 131 months since the clinical onset of EMC. Patient survival was 49% at 10 years after renal biopsy. Forty-two patients died primarily from cardiovascular and liver disease or infection, whereas 15 patients developed chronic renal failure. Two patients had a complete remission of the disease while 15 had a remission only of renal signs. Thirty-one patients are alive with persistent renal and extrarenal manifestations. Anti-HCV antibodies were retrospectively detected in 34 patients and were present in 85% of them. This variable was not included in the statistical evaluation. At multivariate analysis, age older than 50 years, purpura, splenomegaly, cryocrit levels higher than 10%, C3 plasma levels lower than 54 mg/dl, and serum creatinine higher than 1.5 mg/dl were independent risk factors for death or dialysis. In conclusion, several factors may influence the outcome of patients with EMC nephritis. Markers of disease activity and an impaired renal function can herald a bad prognosis. It should be stressed, however, that only a minority of patients eventually develop renal failure, probably because in the most severe cases patients die earlier.
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PMID:Long-term predictors of survival in essential mixed cryoglobulinemic glomerulonephritis. 772 49

Induction of a graft-vs-host reaction in irradiated (BALB/c X C57BL/6)F1 mice (CBF1 mice) with bone marrow cells (BMC) plus spleen cells of BALB/c mice leads to bone marrow transplantation--GVHD (BMT-GVHD). BMT-GVHD is characterized by liver disease, splenomegaly, and hypergammopathy. In addition, we found that increased serum IgE and IgG1 levels were correlated with BMT-GVHD such as liver disease and splenomegaly. The allotype of increased IgE levels in BMT-GVHD was IgEa of donor origin, not IgEb of host origin. We also found that in the thymus of murine BMT-GVHD, the CD4+ CD8+ double-positive T cells were decreased, but the CD4+ CD8- or CD4- CD8+ single-positive T cells were increased. Interestingly, double-positive T cells appeared in the spleen, suggesting that abnormal T cell differentiation existed in murine BMT-GVHD. When the recipients were treated with anti-IL-4 Ab (11B11), the increase of IgE and IgG1 was markedly reduced and liver disease and splenomegaly were also prevented. Moreover, abnormal T cell differentiation and maturation were suppressed. These observations suggest that IL-4 plays an important role in immunoregulation or pathogenesis of allogeneic effects, and 11B11 prevents immunodysfunction including T cell differentiation in the thymus or the spleen and autoimmune symptoms in murine BMT-GVHD.
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PMID:Anti-IL-4 antibody prevents graft-versus-host disease in mice after bone marrow transplantation. The IgE allotype is an important marker of graft-versus-host disease. 787 41

The serum bilirubin concentration is a specific marker of liver disease but the sensitivity is low for detecting liver damage and remains within normal limits in many patients with compensated liver cirrhosis. In liver cirrhosis, portal blood flow is distorted accompanied by a decrease in hepatic clearance of bilirubin. In addition, portosystemic shunting as well as splenomegaly results in an increase in hemolysis and production of bilirubin. These together result in an increase in the concentration of unconjugated bilirubin in serum. In advanced cirrhosis, glucuronyl conjugation of bilirubin and biliary excretion of conjugated bilirubin are markedly impaired and jaundice appears. The concentration of bilirubin in serum thus become a good prognostic marker for patients with incompensated liver cirrhosis.
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PMID:[Bilirubin metabolism in liver cirrhosis]. 811 83

Wilson's disease is an autosomal recessive disease of copper metabolism which is widely recognized as a disease occurring clinically in children, adolescents, and young adults. Unrecognized and therefore untreated Wilson's disease in patients over age 40 is thought to occur either rarely or not at all. Two cases of Wilson's disease presenting at an age greater than 40 years are presented. The first is a 42-year-old Israeli women who presented with fulminant hepatic failure. The serologic and biochemical investigations obtained at the time of her fulminant hepatic failure included copper studies which suggested the diagnosis of Wilson's disease, which was confirmed by an examination of the native liver following successful orthotopic liver transplantation. The second case is that of a 56-year-old white male who presented to the hospital with a three-year history of neurological dysfunction, pancytopenia, and mild splenomegaly. A battery of serologic and biochemical investigations suggested a diagnosis of Wilson's disease. The diagnosis was confirmed by quantitative hepatic copper estimation and the demonstration of Wilson's disease in three of his siblings, all of whom were diagnosed after the proband case had been identified. This man and his siblings have been treated with d-penicillamine, with remarkable improvement in their neurologic and hepatic function. The proband is currently well 11 years after his diagnosis was established. These two cases demonstrate that a diagnosis of Wilson's disease should be considered as part of the differential diagnosis of individuals in the fourth and fifth decades of life who present with unexplained liver disease.
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PMID:Wilson's disease: a diagnosis made in two individuals greater than 40 years of age. 822 72

Mycoplasma-like organisms (MLO) are non-cultivated intracellular cell-wall deficient pathogenic bacteria with a distinctive ultrastructural appearance. Diagnosis of MLO disease depends on finding the organisms in parasitized cells using a transmission electron microscope. MLO are a well studied cause of transmissible chronic plant disease responsive to antibiotics. MLO have recently been found to cause human chronic uveitis, orbital, and retinal disease with autoimmune features. Ophthalmic leucocytes in these patients display MLO parasitization. Inoculation of human uveitis MLO into mouse eyelids produced chronic uveitis. MLO also disseminated to produce randomly distributed lethal systemic disease including chronic hepatitis. MLO parasitized leucocytes were present in all disease sites. Direct intrahepatic inoculation of human hepatic pathogens is a simple and efficient technique to produce murine hepatitis. This report describes the delayed onset widespread inflammatory liver disease produced by direct intrahepatic inoculation of human chronic uveitis MLO in 12 of 20 mice versus 0 in 40 controls (P < 0.05). The liver disease was accompanied by elevated serum SGOT levels, splenomegaly, and accelerated mortality. All 12 inflamed livers displayed MLO parasitized leucocytes versus 0 of 10 control livers. The resemblance of human chronic active hepatitis, massive hepatic necrosis, and post-necrotic cirrhosis to the MLO induced murine liver disease, the role of molecular biologic techniques in the detection and classification of those bacteria, and in therapy of MLO disease are discussed.
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PMID:Experimental murine chronic hepatitis: results following intrahepatic inoculation of human uveitis mycoplasma-like organisms. 839 4

The clinical, biochemical and histological features of 102 consecutively referred patients with chronic hepatitis C virus infection were analysed. Demographic, epidemiological, biochemical, haematological and histological details were catalogued for each patient. The mean follow-up was 49 +/- 6 months. Liver biopsies were obtained from 92 patients; a second biopsy was obtained from 35 patients. The average known duration of infection was 8.6 +/- 0.7 years. The most common risk factors that could be identified were past blood transfusion, surgery or intravenous drug abuse. Twenty-four of the 27 patients (85%) with past blood transfusion had received blood in countries outside of northern Europe. In contrast, 12 of the 16 former drug users were northern European. Patients were frequently diagnosed incidentally; one-quarter had no symptoms of liver disease and were generally asymptomatic or had presented with non-specific complaints and were found to have abnormal serum aminotransferase levels after routine screening. The mean serum aminotransferase levels were not significantly different in those presenting with fatigue compared to those diagnosed incidentally. The most common physical sign in these patients was a palpable liver, which was present in 52%. The mean serum albumin concentration in patients older than 40 years was significantly lower than that in younger patients. Splenomegaly and endoscopic evidence of varices was also more common in older patients. Cirrhosis was present in 37% of patients at presentation: 20% showed progression on rebiopsy, and 5% developed cirrhosis within 4 years of initial presentation. Of those treated, 27% showed histological improvement. Histological severity did not correlate with duration of disease, but did correlate with age.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Clinical, biochemical and histological features in 102 patients with chronic hepatitis C virus infection. 846 87

This project was initiated with the introduction of magnetic resonance (MR) in Denmark in order to evaluate the possibilities of this technique as a diagnostic aid in non-focal liver and splenic diseases. The signal intensities in the MR image are sensitive to the longitudinal relaxation (T1), the transverse relaxation (T2), flow and chemical shift. All these parameters may be quantified by developing specific pulse sequences sensitive to the parameter in question. Previous studies had indicated that relaxation time measurements might be of value in the diagnosis of liver cirrhosis and haemochromatosis. Measuring relaxation times in these 2 groups of patients posed different challenges. In patients with liver cirrhosis a method had to be developed for simultaneous T1 and T2 relaxation time measurements, which was robust to the respiratory motion of the liver. A combination of multi-echo pulse sequences with different repetition times was chosen, because motion effects were partly refocused. Multi-acquisition was used to improve the signal-to-noise ratio in the heavily saturated experiments with short repetition times, to further reduce the sensitivity to motion. To test the quality of this pulse sequence, phantom experiments were performed, and sensitivity to motion was tested by measuring with and without respiratory synchronization. Respiratory synchronization gave a marked improvement in focal liver diseases, whereas no difference was found in non-focal diseases. Standard imaging sequences with a minimum echo time of 30 ms could not be used to measure the short T2 relaxation times found in patients with increased liver iron. A volume-selective multi-echo spectroscopic pulse was developed with a minimum echo time of 4 ms. Biexponential signal decay could be shown in patients with increased liver iron by using this sequence. Patients with liver cirrhosis, as a group, had increased T1 relaxation times compared to normal volunteers, but an overlap in T1 values was found. No correlation between the degree of fibrosis and the T1 relaxation time was found. Liver iron concentration could be quantified either by using the fast component of the T2 signal decay or by using the decreased signal in spin-echo and gradient echo images. Patients with leukemias and myeloproliferative disorders had prolonged T1 relaxation times in the spleen, but a considerable overlap was found between this group and a group of patients with benign hyperplasia and patients with splenomegaly secondary to portal hypertension. Volume-selective proton spectroscopy was developed and used to quantify the liver fat concentration. The accuracy of the method was about 3 g/100 g. With the implementation of a second generation scanner system it became possible to develop a pulse sequence, using the phase information in the MR signal, to measure portal vein flow during breath-holding. This method made it possible to estimate the portal vein flow during fasting, and the flow increase after eating. Quantitative MR methods may contribute to the diagnosis of non-focal liver diseases by estimation of liver fat and liver iron and by assessment of portal vein blood flow. Increased T1 relaxation time is a sign of a disease process in the liver rather than specific for any liver disease.
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PMID:Quantitative magnetic resonance methods for in vivo investigation of the human liver and spleen. Technical aspects and preliminary clinical results. 860 19

Ultrasound examination should be carried out as the first investigation in all patients with suspected hepatobiliary disease. It has a high accuracy in detecting signs of portal hypertension such as ascites, splenomegaly and collateral veins. The most common cause of portal hypertension in the Western world is cirrhosis. Although there is an overlap of ultrasound findings in normal liver, fatty liver, chronic liver disease with and without fibrosis, and cirrhosis, a correct diagnosis of cirrhosis by judgment of ultrasound findings is reported to be achieved in up to 80%. Diagnostic parameters used are size and outline of the liver, its reflectivity and attenuation of the echo-pattern. If used in combination with the Doppler duplex system, the information gained vastly increases. Areas where this technique is able to provide useful and reliable data have been defined in a consensus conference held in Bologna. Doppler flowmetry of hepatic vessels allows detection of presence, direction and characteristics of blood flow. The reliability of the quantitative measurements of blood flow in abdominal vessels is still questioned. When using the same equipment by skilled operators, however, duplex-Doppler data of the portal vein are of value in the assessment of the risk of variceal bleeding, in the evaluation of the progression of liver disease and in the evaluation of medical therapy for portal hypertension. Furthermore, the technique is of practical value in pre-and postoperative examinations for shunt surgery as well as in the pre-procedural work-up of transjugular intrahepatic portosystemic stent shunt (TIPS), during the TIPS procedure and in the post-TIPS followup.
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PMID:Ultrasound and duplex-Doppler in the diagnosis and follow-up of portal hypertension. 877 98

Splenomegaly was studied at a municipal teaching hospital in an 11-year retrospective review. The 170 patients were classified into six diagnostic groups. The associated clinical and laboratory features were tested for statistical association (X2), to determine predictive values. Hepatic diseases caused 36% of the splenomegaly; hematologic, 35%; infectious diseases (ID), 16%; inflammatory, 5%; primary splenic, 4%; and other, 3%. The acquired immunodeficiency syndrome (AIDS) occurred in 54% of patients with ID. Hematologic diseases were significantly associated (P < 0.01) with massive splenomegaly, left upper quadrant (LUQ) abdominal tenderness, and all blood "cytoses." The most common disease with massive splenomegaly was myelofibrosis. Surprisingly, hepatic diseases caused 29% of massive splenomegaly. Hepatic diseases were significantly associated (P < 0.01) with hepatomegaly, abnormal liver-function tests (LFT), and blood "cytopenias." Compared with previous reports, both congestive heart failure and endocarditis now rarely cause splenomegaly. All blood "cytopenias" had highly significant associations (P < 0.01) only with hepatic diseases, which suggests that hypersplenism remains a useful concept for the splenomegaly of liver disease.
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PMID:Splenomegaly at a United States County Hospital: diagnostic evaluation of 170 patients. 885 64

Apart from viruses, hepatotoxins, hereditary metabolic disorders, immunological factors and cholestasis may cause chronic hepatitis both clinically and histologically. As far as the etiology is concerned, a complete history can be very helpful. The clinical examination, however, is rarely diagnostic. Nevertheless, some clinical signs (e.g. ascites, splenomegaly, spider naevi) are suggestive of cirrhosis. The activities of gammaglutamyl transferase and ALT in the serum are augmented in most of the patients with chronic hepatitis independent of its etiology. Electrophoresis reveals disturbance of serum albumin and globulin ratios. "Basic' laboratory tests are supplemented by carefully selected additional investigations (e.g. immunological tests) according to the history and clinical data of the individual patient. Retrograde cholangiography is diagnostic in the majority of patients suffering from primary-sclerosing cholangitis. Liver histology, best obtained during laparoscopy, allows classification (and prognosis) of the underlying liver disease in many patients. Results of iron and copper determination in liver tissue are diagnostic in cases of congenital liver disease (hemochromatosis, M. Wilson).
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PMID:[Current diagnosis of chronic nonviral hepatitis]. 898 77


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