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)

Four children, ages seven to ten years, with direct antiglobulin test (DAT)-positive chronic hemolytic anemia are presented. The patients were followed for 3 to 10 years. Autoantibody against red cell antigens was nonspecific IgG type in all of the patients. In one of the four patients, anemia was associated with splenomegaly and jaundice. In this patient, the third component of the complement was also detected on the red cell surface. In one patient, serum IgA deficiency and frequent pulmonary infections were associated with the disease. This patient developed rheumatoid arthritis five years after diagnosis of hemolytic anemia. The third patient initially had thrombocytopenia subsequently developed DAT-positive hemolytic anemia, vitiligo and alopecia without any evidence of serologic changes suggestive of collagen vascular disorders. In these three patients, partial response was obtained with steroid therapy. The fourth patient developed DAT-positive hemolytic anemia twice during the five year follow-up period. Anemia resolved completely with steroid therapy in two months during the first episode, and in five months in the second. Generalized and peripheral lymphadenopathies which developed at the time of the second hemolytic anemia episode have persisted for the last three years. Administration of cyclosporine in two of the four patients did not result in any amelioration of the symptoms.
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PMID:Chronic autoimmune hemolytic anemia in children: a report of four patients. 799 66

Twenty-six cases of adult T-cell leukemia/lymphoma (ATLL) were identified between 1983 and 1991 in Martinique (French West Indies). There were 14 men and 12 women, all of mixed racial descent and born in Martinique. Their ages ranged from 23 to 95 years. The main clinical and laboratory features at initial presentation were peripheral lymphadenopathy (22 cases), hepatomegaly (11 cases), splenomegaly (10 cases), cutaneous lesions (12 cases), hypercalcemia (16 cases), refractory infection by Strongyloides stercoralis (12 cases), and pre-existing autoimmune disorders (4 cases). All patients had absolute lymphocytosis with circulating pleomorphic abnormal lymphocytes. The prognosis was poor, with most patients (20 cases) surviving for less than 6 months. Although the overall clinicopathologic features of ATLL in this series are similar to those described in previous reports, we observed three additional points of interest: a high association with Strongyloides infection, an increased incidence of tropical spastic paresis/HTLV-1 associated myelopathy (TSP/HAM) among the relatives of the patients (5 cases), and the presence of prior collagen vascular diseases.
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PMID:Adult T-cell leukemia-lymphoma: a clinico-pathologic study of twenty-six patients from Martinique. 811 52

Thrombocytopenia and splenomegaly improved in one of the four patients receiving 1 alpha-hydroxyvitamin D3 (1 alpha(OH)D3) for treatment of primary myelofibrosis (PMF). We compared the clinical results with the in vitro effects of vitamin D3 metabolites on the growth and collagen synthesis of bone marrow fibroblasts. The effects of vitamin D3 metabolites on control human bone marrow fibroblasts were first studied in vitro. On the growth, 1,25-dihydroxyvitamin D3 (1,25(OH)2D3) and 24,25-dihydroxyvitamin D3 (24,25(OH)2D3) inhibited the platelet derived growth factor (PDGF) or human serum-induced proliferation at the concentrations of more than 10(-10) M, while in the presence of transforming growth factor-beta (TGF-beta) the inhibitory effects were mild or non-inhibitory. Both vitamin D3 metabolites inhibited procollagen synthesis at a concentration of more than 10(-8) M. The effect of 1,25(OH)2D3 on the PMF patients was examined. In two of the four patients, the human serum-induced growth inhibitory effect was observed at the concentration of 10(-8) M, in one patient no inhibition was observed and in one patient inhibition was observed at 10(-10) M, as was observed in the control donors. During the treatment with 1 alpha(OH)D3 the serum level of 1,25(OH)2D3 was 1-2 x 10(-10) M. These findings suggest that a minority of PMF patients have marrow fibroblasts with growth sensitivity to a pharmacological level of vitamin D3 and could be treated with 1 alpha(OH)D3 with some clinical improvements.
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PMID:1 alpha-Hydroxyvitamin D3 in the treatment of primary myelofibrosis: in vitro effect of vitamin D3 metabolites on the bone marrow fibroblasts. 838 71

Twenty-eight patients (aged: 44-76 years) with liver cirrhosis secondary to chronic hepatitis were included in this study. None of the patients had a history of gastric surgery, diabetes, hypothyroidism, or neurological or collagen vascular disease. The patients were divided into groups based on the presence or absence of the following conditions: (1) ascites, (2) splenomegaly, (3) esophageal varices. Radionuclide labelled solid meals were used to evaluate gastric emptying (GE). GE was represented by the gastric retention ratio of the solid meal at 90 min (RR90), and calculated by the following formula: RR90 = residual radioactivity within the region of interest (ROI) covered the whole stomach at 90 min divided by the initial radioactivity within the ROI at 0 min. The RR90 values of the patients were compared with the RR90 values of 25 normal control volunteers. If the RR90 was more than two standard deviations (SD) above the mean of the control group (> 0.687), GE was defined as abnormal. The results showed 71% (20/ 28) of the cirrhotic patients had abnormal GE. Patients with ascites, splenomegaly, or esophageal varices had higher RR90 values than patients without ascites, splenomegaly or esophageal varices. The differences in RR90 among patients with and without ascites, and patients with and without splenomegaly, were not significant (P values > 0.05). However, there were significant differences among the 2 patient groups separated according to the presence or absence of esophageal varices (P values < 0.05). In addition, poor correlations (R square values < 0.01) were found for RR90 and serum values of bilirubin and albumin.
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PMID:Abnormal gastric emptying in patients with liver cirrhosis. 867 34

Heparin effects were studied on Lewis rats with alpha 1 antitrypsin (AT) defect. Among 8 rats that were born at the same birth, three rats were shown to have mild defect of alpha 1 AT. Heparin was injected repeatedly into all the 8 rats. Interstitial pneumonia and localized periodic acid-Schiff (PAS) stain of hepatocytes were found in alpha 1 AT defective male. One of the three alpha 1 AT defective rats had about a half of normal alpha 1 AT level. Antithrombin (AT) III level was slightly low in the alpha 1 AT defective female with splenomegaly. Lung electron micrograph of the other alpha 1 AT defective female showed edematous changes of capillaries and alveolar basement membranes and also proliferated collagen fibers. In the lung of alpha 1 AT defective male, many thrombocytes adhered to alveolar degenerated smooth muscles that were recognized as Masson bodies. Extracted platelet-activating factor (PAF) in the plasma of the alpha 1 AT defective male was shown to trigger T lymphocyte chemotaxis. Five normal Lewis rats were immunized with bovine serum albumin (BSA). IgG1 antibody to BSA was produced in all the rats. The rats with high titers of IgG1 anti BSA antibody showed more strongly atrophic changes of glomerulus than those of the mild alpha 1 AT defective rats treated with heparin.
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PMID:Alpha 1 antitrypsin defective Lewis rats injected with heparin: comparison of the glomerular changes with those of Lewis rats produced anti BSA antibody. 868

This study documents the hepatic morphology and the ultrastructure of a model of hepatic fibrosis in rabbits. Rabbits were given a cholesterol-supplemented diet (1%), a stilbestrol diet (10 mg subcutaneously twice a week), or both treatments simultaneously for 7 weeks. Rabbits given the combined treatment developed sinusoidal and portal fibrosis with only a mild disturbance of acinar vascular relationships. Ultrastructurally, there was marked widening of the spaces of Disse by collagen fibers, basement membrane material adjacent to endothelial cells and hepatocytes, blunted hepatocellular microvilli, activated stellate cells, lipid droplets in endothelial cells and hepatocytes, and degranulated platelets in sinusoids. The hepatic hydroxyproline content was markedly increased (12.0 +/- 5.2 vs. 4.8 +/- 1.5 mmol/g of liver dry weight; P < .001). Plasma bile acids were markedly increased (222 +/- 180 vs. 12 +/- 5 in controls; P < .001). Dipyridamole (25 mg every 12 hours) that was given in addition to cholesterol and stilbestrol decreased the hepatic collagen content (-49% and -48%, in two experiments; P < .05 in both) and splenomegaly. This model provides a reliable method for the production of extensive sinusoidal fibrosis with capillarization of sinusoids. Hepatocellular degeneration is only mild to moderate, and fibrosis occurs slowly without the sudden pathological changes that occur with other models of hepatic fibrosis, such as with the administration of CCl4 or galactosamine. The mechanism of injury may involve the accumulation of bile salts or the generation of free radicals from cholesterol oxidation products. The possibility that the sinusoidal release of platelet-derived factors may have a role in the activation of stellate cells (lipocytes) is supported by the suppression of fibrogenesis by dipyridamole.
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PMID:Hepatic sinusoidal fibrosis induced by cholesterol and stilbestrol in the rabbit: 1. Morphology and inhibition of fibrogenesis by dipyridamole. 885 88

Chronic (20-week) Schistosoma mansoni infections in male CBA/J mice present as one of two pathophysiologic forms: severe hypersplenomegaly syndrome (HSS) or a less severe, moderate splenomegaly syndrome (MSS). HSS mice are cachectic (including anemia and hypertriglyceridemia) and exhibit high levels of periportal and perioval fibrosis. Because tumor necrosis factor-alpha (TNF-alpha) is associated with the symptoms of cachexia, we measured TNF-alpha protein and mRNA levels in the livers of infected and uninfected animals. TNF-alpha levels in liver homogenates from mice with acute infections (8-week) were high (mean +/- SEM; 41.0 +/- 1.6 ng/g tissue) and remained high in livers of HSS mice (41.8 +/- 3.0 ng/g tissue) while TNF-alpha levels in liver homogenates of MSS mice were significantly lower (27.9 +/- 2.0 ng/g tissue). Similarly, hepatic TNF-alpha mRNA levels from HSS mice were two- to threefold higher than those from MSS mice. Hydroxyproline levels in these animals were determined as a measure of collagen deposition and fibrosis and showed increased overall levels in the livers of HSS animals. To investigate the progression of HSS development, hematocrit and serum triglyceride levels were followed over a 20-week period after infection. In mice that developed HSS, hematocrit levels decreased significantly and progressively from Weeks 10 through 20. These same animals showed significant increases in serum triglycerides compared to 8-week-infected mice or the mice which developed MSS over the same time period. These results suggest that failure to downregulate hepatic production of TNF-alpha correlates with, and may contribute to, the development of liver fibrosis and HSS in experimental schistosomiasis.
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PMID:Schistosoma mansoni: relationship of tumor necrosis factor-alpha to morbidity and collagen deposition in chronic experimental infection. 893 61

Among the chronic myeloproliferative disorders essential thrombocythemia (ET) is known to be a distinct clinical entity in which an excessive number of morphologically and functionally abnormal platelets are produced. The clonal nature of the disease is well established. Based on a review of the literature the present authors propose the following novel criteria for the diagnosis of ET: A1. Platelet count in excess of 600 x 10(9)/L. A2. No increase in red-cell mass (RCM) in the presence of stainable iron in the bone marrow or failure of iron trial (RCM < 36 mL/kg in males and < 32 mL/kg in females; or RCM < 25% above mean normal predicted value*). A3. No Philadelphia chromosome. A4. Megakaryocytic hyperplasia (= increased megakaryocyte number and size) in histological sections of bone marrow and/or increased megakaryocytic ploidy (two-color flow cytometry); no collagen fibrosis. B1. Splenomegaly on isotopic scan or echogram. B2. Unstimulated growth of BFU-E and/or CFU-Meg present. B3. Normal ESR/fibrinogen. The diagnosis of ET is considered to be established if A1 + A2 + A3 + A4 or A1 + A2 + A3 + two B-criteria are fulfilled. (* Br J Haematol 1995; 89:748-756.)
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PMID:Diagnostic and differential criteria of essential thrombocythemia and reactive thrombocytosis. 895 71

Transgenic mice overexpressing a constitutively active human TGF-beta1 under control of the rat phosphoenolpyruvate carboxykinase regulatory sequences developed fibrosis of the liver, kidney, and adipose tissue, and exhibited a severe reduction in body fat. Expression of the transgene in hepatocytes resulted in increased collagen deposition, altered lobular organization, increased hepatocyte turnover, and in extreme cases, hemorrhage and thrombosis. Renal expression of the transgene was localized to the proximal tubule epithelium, and was associated with tubulointerstitial fibrosis, characterized by excessive collagen deposition and increased fibronectin and plasminogen activator inhibitor-1 immunoreactivity. Pronounced glomerulosclerosis was evident, and hydronephrosis developed with low penetrance. Expression of TGF-beta1 in white and brown adipose tissue resulted in a lipodystrophy-like syndrome. All white fat depots and brown fat pads were severely reduced in size, and exhibited prominent fibroplasia. This reduction in WAT was due to impaired adipose accretion. Introduction of the transgene into the ob/ob background suppressed the obesity characteristic of this mutation; however, transgenic mutant mice developed severe hepato- and splenomegaly. These studies strengthen the link between TGF-beta1 expression and fibrotic disease, and demonstrate the potency of TGF-beta1 in modulating mesenchymal cell differentiation in vivo.
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PMID:Hepatic fibrosis, glomerulosclerosis, and a lipodystrophy-like syndrome in PEPCK-TGF-beta1 transgenic mice. 938 33

Chronic liver disease is often accompanied by hypoxaemia. We investigated the clinical factors that were related to the arterial oxygen tension (PaO2) in 40 women, all non-smokers with chronic liver disease. They were positive for hepatitis C virus (HCV) antibody and had no evidence of cardiopulmonary disease. Arterial blood was collected from patients at rest (> 15 min) for analysis of blood gases. We determined the correlation between blood gas tension and the clinical variables, i.e. the presence or absence of skin manifestations such as cutaneous spider nevi and palmar erythema, the presence or absence of splenomegaly, vital capacity, forced expiratory volume in one second, V25/body height, serum alanine aminotransferase (AST), serum asparate aminotransferase (ALT), serum cholinesterase, serum gamma-globulin/total protein, excretion of indocyanine green at 15 min (15-min retention rate, ICG level), blood level of ammonia, blood level of endotoxin, plasma level of glucagon and the serum level of type IV collagen-7S. The mean level of PaO2 was 78 +/- 11 (range: 43-95) torr. The mean alveolar-arterial oxygen tension gradient (A-aDO2) was 19 +/- 13 (range: 2-60) torr. Multiple regression analysis used PaO2 and A-aDO2 as objective variables, and the clinical findings as explanatory variables. The explanatory variables that were significantly correlated with blood gas values were ICG level, blood level of endotoxin and presence of skin manifestations. The ICG level showed a high correlation with blood gas values; the ICG level increased, the PaO2 decreased (r = -0.69), while the A-aDO2 showed a high positive correlation (r = +0.78, P < 0.001). Findings suggest that a reduction in hepatic blood flow and hepatocellular function interfere with the inactivation of vasoactive substances such as endotoxin by the liver, leading to the development of skin manifestations, the dilatation of intrapulmonary capillaries and the induction of hypoxaemia.
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PMID:Clinical factors that affect blood gases in non-smoking women with chronic liver disease. 951 26


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