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)

Blood volume was measured using 125iodinated human serum albumin in 27 children with acute lymphoblastic leukemia, and in 7 children with various types of leukemia. Total blood volume was normal in patients without marked enlargement of spleen and liver, and increased progressively as spleen and liver size increased. The hypervolemia was entirely due to expansion of plasma volume. In the children with marked hepatosplenomegaly, only hematocrit (but not red cell mass) was below the normal range in most cases. Both hematocrit and red cell mass were subnormal in the majority of patients without considerably enlarged spleen and liver. Therefore, anemia in children with marked hepatosplenomegaly may be partly caused by hemodilution of red blood cells in expanded plasma volume.
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PMID:Blood volume of children with leukemia. 27 98

Hypocalcemia is seen in patients with leukemia and is usually due to renal impairment or to low serum albumin concentrations. Four patients are reported who had hypocalcemia but without these usual explanations. One patient had chronic lymphatic leukemia and overwhelming infections which led to death. The other three patients had chronic myelogenous leukemia in an accelerated phase of the disease characterized by increasing blast cells in circulation, massive hepatosplenomegaly, and myelofibrosis. The cause of the hypocclcemia is unknown.
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PMID:Hypocalcemia in leukemia. 105 54

We retrospectively reviewed the charts and radiographs of 38 patients with systemic-onset juvenile rheumatoid arthritis, attempting to identify early in the disease course the clinical and laboratory observations most predictive of the later development of destructive arthritis. In 12 of the patients, destructive arthritis developed within 2 years of disease onset. When first examined, these patients could not readily be differentiated from those in whom joint destruction did not develop, but they more commonly had hepatosplenomegaly (p less than 0.04), serositis (p less than 0.01), and a lower mean serum albumin concentration (26.7 vs 31.3 gm/L; p less than 0.02). However, by 6 months after onset, patients with destructive arthritis more frequently had persistent systemic symptoms (92% vs 12%; p less than 0.0001), polyarthritis (67% vs 19%; p less than 0.0005), a lower mean hemoglobin level (95 vs 114 gm/L; p less than 0.001), a higher mean leukocyte count (21.2 vs 10 x 10(9)/L; p less than 0.0003), a higher mean platelet count (794 vs 400 x 10(9)/L; p less than 0.0001), and a higher mean erythrocyte sedimentation rate (43 vs 24 mm/hr; p less than 0.05). Multivariate analysis of the results at 6 months revealed that persistent systemic symptoms and a platelet count greater than or equal to 600 x 10(9)/L were the variables most highly predictive of the later development of joint destruction. We conclude that patients at high risk for the development of destructive arthritis may be identified within 6 months of disease onset, thereby indicating the need for more aggressive early therapy.
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PMID:Prognostic indicators of joint destruction in systemic-onset juvenile rheumatoid arthritis. 173 15

Since 1978, 187 patients (age range, 15 to 59, median 44 years) have received short-term chemotherapy as part of three sequential open studies (B-IX, X, Xb) or a randomized clinical trial (B-XI). An intended six cycles of Adriamycin (ADR) (doxorubicin; Adria Laboratories, Columbus, OH), cytarabine (ara-C), and thioguanine (TG) were administered with as short an intercycle time as possible. No further therapy was administered. Complete remission (CR) was achieved in 118 of 187 patients (63%). On univariate and multivariate analyses achievement of CR correlated adversely with a low serum albumin at presentation and an antecedent marrow disorder. Forty-five patients continue in first remission between 15 months and 8 1/2 years, no relapses being seen after 3 1/2 years (median follow-up, 3 1/2 years). The median duration of remission was 1 year. M3 morphology, a blast count less than 100 x 10(9)/L, and absence of hepatosplenomegaly correlated favorably with remission duration. There was no difference in duration of remission between patients receiving 3, 4, 5, or 6 cycles. The best results overall were achieved in patients under the age of 40, with 43% projected to remain free of disease at 5 years. Fifty patients remain alive between 17 months and 9 years, the predicted actuarial survival being 25% at 5 years.
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PMID:Short-term therapy for acute myelogenous leukemia. 342 61

In an isolated rural community in Papua New Guinea, 88 children were found to be infected with Strongyloides cf. fuelleborni; 50 of these also had hookworm infections. Their ages ranged from one to 124 months and all were treated with thiabendazole suspension, 25 mg/kg twice daily for 3 days. 2 patients with Strongyloides and 5 with hookworm were still passing ova 2 weeks after treatment although their egg counts were lower or the same as the pre-treatment counts. 26 children experienced some side effects from the drug, vomiting being the most common; in none did side effects prevent completion of therapy. No association between Strongyloides egg load and nutritional status, hepatosplenomegaly, respiratory ailments, oedema or serum albumin levels was found.
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PMID:An intervention study using thiabendazole suspension against Strongyloides fuelleborni-like infections in Papua New Guinea. 344 23

Three hundred twenty-five previously untreated patients with chronic lymphocytic leukemia were analyzed to identify significant prognostic factors for survival. Univariate analysis identified the following characteristics associated with survival: (1) clinical characteristics: age, race, sex, performance status, lymphadenopathy, and hepatosplenomegaly; (2) hematologic parameters: WBC count, absolute lymphocyte and granulocyte counts, hemoglobin level, and platelet count; and (3) biochemical parameters: serum albumin, calcium, uric acid, lactate dehydrogenase, alkaline phosphatase, BUN, and creatinine. Multivariate regression analysis in a randomly selected training subset of 217 patients demonstrated that the combination of uric acid, alkaline phosphatase, lactate dehydrogenase, external lymphadenopathy, and age had the strongest predictive relation to survival time. The resulting model was validated in the remaining independent subset of 108 patients and led to classification of patients into low, intermediate, and high-risk groups with five-year survival rates of 75%, 59%, and 14%, respectively, and with distinctively different annual mortality rates (P less than .01). Both the regression model and Rai staging were highly effective in identifying risk groups among the entire patient population (P less than 0.001). Overall the regression model was superior to Rai staging in defining prognostic risk groups. In addition, it was able to separate patients into significantly different risk categories within each Rai stage, thus improving on the prognostic prediction of individual patients with chronic lymphocytic leukemia.
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PMID:Prognosis of chronic lymphocytic leukemia: a multivariate regression analysis of 325 untreated patients. 381 21

17 cases of Budd-Chiari syndrome with polycythemia diagnosed by the authors are reviewed, and the primary or secondary etiology of this disorder in these cases, in the other 36 reported in the literature, and in the 8 users of oral contraceptives who have suffered the combined syndrome since 1966, is discussed. The Budd-Chiari syndrome is a primitive thrombosis of all or part of the subhepatic veins, and among 350 known cases, only 36 have also had polycythemia. Contrary to the mostly older patients with polycythemia vera, these 17 cases averaged 36.8 years, and were more often female (10 of 17), 4 had gynecologic antecedents 1 (oral contraceptives, 1 pregnancy, 1 abortion, 1 myomectomy). The clinical findings commonly seen were ascites (82%), hepatosplenomegaly (94%), bromosulphthalein retention (81%), low serum lipids (59%), high serum albumin (81%), low serum iron (75%), and other extensive thrombosis. 2 of these patients died within 2 months, 5 died within 2 years, and 5 are still living. The patient who took oral contraceptives had had 3 induced abortions and 1 pregnancy; took Ovariostat for 5 days, 1 month after first contracting progressive jaundice; was hospitalized 9 months later; and was successfully treated. The authors commented that polycythemia was documented in all cases of Budd-Chiari syndrome in pill users in which tests were made. Among these 17 cases, 2 had true polycythemia, and 8 has secondary polycythemia.
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PMID:[Polycythemia and Budd-Chiari syndrome. Apropos of 17 cases]. 515 23

The pharmacokinetics and therapeutic efficacy of praziquantel (Distocide; Epico, El-Asher-Men-Ramadan City, Egypt) were studied in 40 patients with schistosomiasis mansoni and various degrees of hepatic dysfunction. The patients were allocated into four groups: the first included 10 patients with simple active schistosomiasis while the other three were made up of patients with schistosomiasis associated with liver cirrhosis and splenomegaly according to Child's classification of hepatocellular function. Every patient was treated with 40 mg/kg of praziquantel as a single oral dose. The efficacy of the drug was evaluated after two months by rectal snip examination. The pharmacokinetic parameters did not differ significantly between patients with simple active schistosomiasis (group 1) and those with hepatosplenomegaly with liver involvement but without ascites and jaundice (group 2). However, as liver cell dysfunction became more evident (groups 3 and 4), pharmacokinetic parameters of praziquantel such as the half-life of elimination, the half-life of absorption, the maximum concentration, the time to maximum concentration, and the area under the concentration-time curve increased proportional to the degree of hepatic insufficiency. Linear correlations were found between each of the these parameters on the one hand and hepatic function test results (total bilirubin, direct bilirubin, and serum albumin) on the other. In spite of these pharmacokinetic differences, the cure rates were 70%, 80%, 90%, and 90% in the four groups, respectively. Although the incidence of side effects was high (53%), such effects were transient and mild.
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PMID:Clinical and pharmacokinetic study of praziquantel in Egyptian schistosomiasis patients with and without liver cell failure. 781 Aug 16

Gaucher disease type 1, a non-neuronopathic lysosomal storage disease, is caused by mutations at the acid beta-glucosidase locus. Periodic infusions of macrophage-targeted acid beta-glucosidase reverse hepatosplenomegaly, hematologic, and bony findings in many patients. Two patients receiving enzyme therapy developed neutralizing antibodies to acid beta-glucosidase that were associated with a lack of improvement or progressive disease. After initial improvement, case 1 had no additional response to 2 years of high-dose (50 U/kg every 2 weeks) enzyme therapy. Similarly, case 2 initially showed a favorable response to enzyme therapy that plateaued after 1 year of treatment. Both patients developed minor allergic reactions and antibodies to acid beta-glucosidase within the first 6 months of treatment. Enzyme therapy was discontinued in case 1, with resultant disease progression and need for splenectomy. An immunosuppression/tolerization protocol was initiated in case 2 because of disease progression and stable neutralizing antibody titers. The IgG neutralizing antibodies rapidly and completely inactivated the wild-type, but not the N370S, acid beta-glucosidase in vitro. Antibodies to human serum albumin and chorionic gonadotropin also developed. The finding of neutralizing antibodies to acid beta-glucosidase during enzyme therapy for Gaucher disease has significant implications for monitoring the therapeutic responses and for potential alternative future therapies for Gaucher disease.
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PMID:Enzyme therapy in Gaucher disease type 1: effect of neutralizing antibodies to acid beta-glucosidase. 920 36

Cell mediated immune response (CMIR) was studies in 120 patients having chronic liver diseases. Patients were divided into 6 groups, (20 each). (1) Early hepatosplenic Schistosomiasis. (EHSS), (2) Late hepatosplenic Schistosomiasis. (LHSS), (3) Hepatosplenic Schistosomiasis with hepatitis B and/or C infections, (4) Hepatitis B virus cases. (HBV), (5) Hepatitis C virus cases (HCV), (6) Hepatocellular carcinoma cases. (HCC). Twenty within normal subjects taken as controls. Laboratory investigations revealed significant esinophilia in patients of group (1), haemoglobin level was significantly reduced in patients of group (1, 2, 3, & 6), serum albumin was significantly reduced in group (2). The percentage of positivity of skin testing using purified protein derivative, ranged between 10% of patients with LHSS, HBV, HCC and HSS with HBV and/or HCV, 20% of patients with HCV and 25% of patients with EHSS. Percentage of positivity in control group was 100%. The mean diameter of delayed intradermal reaction (2.2 +/- 0.5-6.1 +/- 2.1 mms.) was significantly lower in patients than controls. The response of lymphocyte transformation test to phytohaemmagglutinin was significantly lower in patients when compared to controls. The association of HBV and/or HCV with hepatosplenomegaly was accompanied with a marked depression in cell mediated immune response. Anaemia, hypoalbuminemia and nutritional status of the patients with chronic liver diseases play a major role in the suppression of cell mediated immune response.
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PMID:Cell mediated immune response in chronic liver diseases: schistosomal, viral and neoplastic. 991 13


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