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)

To study the pathogenesis of tropical splenomegaly syndrome, we compared immunologic findings in patients from Flores, Indonesia, with those obtained in local residents without splenomegaly and in controls. Villagers with tropical splenomegaly syndrome had markedly elevated levels of total IgM, higher titers of IgM antibodies to Plasmodium vivax, and reduced levels of circulating T lymphocytes. The latter were caused by a decrease in the total number of T cells with the suppressor/cytotoxic phenotype (T8+). Levels of B lymphocytes were similar in all groups. All immunologic abnormalities reverted toward normal in patients treated weekly for 9 to 26 months with chloroquine phosphate. These findings suggest that overproduction of immunoglobulins in patients with tropical splenomegaly syndrome is caused by an imbalance in the normal ratio of helper: suppressor T cells that regulate B-lymphocyte function, and that this imbalance is due to a decrease in suppressor T lymphocytes.
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PMID:Reduction of suppressor T lymphocytes in the tropical splenomegaly syndrome. 622 39

A 72-year-old patient with marked splenomegaly and low sphingomyelinase (6% of lowest control value) in peripheral blood leukocytes is described. Much higher but variable residual sphingomyelinase activity was observed in cultured skin fibroblasts (40-67% of lowest control value); reduced activity was also found in a liver biopsy sample. Excess storage of sphingomyelin was not observed in a liver biopsy; instead, a lipid tentatively identified as bis(monoacylglycerol) phosphate was present in amounts at least 20 times greater than in age-matched control livers. The biochemical relationship of this patient to patients with sphingomyelin storage disease (Niemann-Pick disease) and phospholipidosis Type II is discussed.
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PMID:Hepatic storage of bis(monoacylglycerol) phosphate without concomitant storage of sphingomyelin in a 72-year-old patient with a partial deficiency of sphingomyelinase. 629 65

A 43-year-old man presented with splenomegaly and a 20-year history of a neurologic disorder that included vertical supranuclear ophthalmoplegia, mild dementia, and a movement disorder. Adult dystonic lipidosis was diagnosed from the clinical picture and demonstration of foamy and sea-blue histiocytes in bone marrow. Ultrastructural patterns in cytolysosomes suggested accumulation of neutral fat and phospholipids. Liver content of bis-(monoacylglycerol) phosphate was increased, probably because the number of lysosomes had increased. Sphingomyelinase activity was normal in cultured skin fibroblasts. Juvenile and adult dystonic lipidosis form a clinically, histologically, and biochemically distinct neurovisceral storage disease that differs from Niemann-Pick disease.
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PMID:Adult dystonic lipidosis: clinical, histologic, and biochemical findings of a neurovisceral storage disease. 689 Jan 67

The surface of Propionibacterium acnes, VPI 0009, was studied using microelectrophoresis following chemical treatments intended to modify specific charged groups. The effect of specific modifications on ability of cells to induce splenomegaly, an indicator of stimulation of the reticuloendothelial system, was also determined. There was little difference between pH mobility curves of P. acnes VPI 0009 and other strains of propionibacteria which were not able to stimulate the reticuloendothelial system. Amino and carboxyl groups were found to be the sole ionizable groups at the cell surface and modification of these groups caused a substantial decrease in the ability of cells to stimulate the reticuloendothelial system. No phosphate groups were detected. Evidence for two types of amino groups was found: one type was present on protein moieties and their modification did not affect ability to stimulate the reticuloendothelial system, whereas modification of the other type, which was present on carbohydrate moieties, caused a loss of ability to stimulate the reticuloendothelial system. Mild oxidation with sodium metaperiodate caused abolition of reticuloendothelial system stimulation, but had no effect on surface charged groups, indicating it was acting on the unsubstituted linkages of sugar residues. Treatments with strong acids caused abolition of ability to stimulate the reticuloendothelial system and this was accompanied by release of polysaccharide material.
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PMID:The cell surface of Propionibacterium acnes: effect of specific chemical modifications on the ability of vaccines to produce splenomegaly in mice. 709 18

It was recently shown in this laboratory that treatment of newborn animals with certain enzymic inducers causes stable changes in the activities of the inducible enzymes at a later adult stage. Cataracts, hepato-splenomegaly and other galactosemia symptoms in galactosemic W/SSM rats develop spontaneously. The increased uptake of galactose by erythrocytes, but not the decreased level of galactose-1-phosphate uridyl transferase (Gal-1-PUT) activity was assumed to be the major cause of the disease. The administration of galactose to the newborn W/SSM rats (2 mg/g of body weight for 14 days) resulted in a sustained decline in the uptake of 14C-galactose by erythrocytes at least for five months, in an increase of glucoso-6-phosphate dehydrogenase activity and in a continuous fall of Gal-1-PUT activity. The neonatal treatment of the galactosemic rats with galactose abolished the main symptoms of galactosemia (cararacts, hepato-splenomegally) in adult animals, perhaps ar a consequence of the stable changes in the galactose metabolism.
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PMID:[Correction of the symptoms of hereditary galactosemia in W/SSM strain rats by enzymatic imprinting]. 720 Apr 39

A randomized controlled trial of the effect of chloroquine prophylaxis versus placebo on the occurrence of clinical malaria was carried out in 1988 among children aged 1-14 in the Awash Rift Valley of central Ethiopia. At the time of the study, chloroquine resistance had not been reported from this area. Two thousand children were randomly allocated to either chloroquine phosphate (5 mg base kg-1) or a multivitamin tablet. Treatment and weekly follow-up were carried out for 10 weeks during the peak malaria transmission season. There was no difference between chloroquine and placebo groups in the occurrence of at least one episode of clinical malaria, in smear positivity in those who remained free of attacks until the end of the study period, or in the prevalence of splenomegaly at the end of the study period. It is concluded that chloroquine prophylaxis is ineffective in preventing at least one clinical attack of malaria in children in this area.
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PMID:Chloroquine chemoprophylaxis in children during peak transmission period in Ethiopia. 806 43

As it is not known what changes to leucocyte homeostasis are mandatory for effective adjuvant action, the biological relevance of systemic changes elicited by different vaccine formulations can only be interpreted in the context of the immunological outcomes. We used flow cytometry to quantify the changes in leucocyte subsets induced in mice intradermally immunized with SAMA4 (adjuvant group), outer membrane proteins (OMP) purified from Actinobacillus pleuropneumoniae (OMP antigen group), SAMA4 adjuvanted OMP (OMP vaccine group), or phosphate-buffered saline (PBS: control group). This approach allowed direct comparisons to be made between the effects of antigen, adjuvant or antigen-adjuvant complexes on immune effector cell populations. Antigens complexed with the liposome-iscom hybrid adjuvant, SAMA4, generated strong antibody responses and cytotoxic T-cell activity in animals immunized intradermally, reflecting remobilization and recruitment of specific cell populations. Splenomegaly, due to granulocytosis, monocytosis and megakaryocytosis, was most prominent in the OMP vaccine group. Histological examination of spleen sections confirmed that these changes were due primarily to splenic haematopoiesis. Circulating numbers of granulocytes and monocytes increased significantly (P < 0.05) in the blood of the OMP vaccine group, as did granulocyte numbers in the lungs (P < 0.05). No changes in T- and B-cell numbers were detected by flow cytometry in the spleens, lungs or blood over the 28-day period in any treatment group. Thymocyte numbers (predominantly CD4+CD8+ cells) in the OMP vaccine group fell by 95% within 3 days of immunization. Identical cellular responses were obtained when an innocuous antigen, ovalbumin, was complexed with SAMA4 instead of OMP, thus demonstrating that the adjuvant effects of SAMA4 were due to synergistic interaction between antigen and adjuvant and not due to the presence of toxic components. The association of strong adaptive immune responses with such complex changes in leucocyte homeostasis induced by complexing adjuvant and antigen suggested that the changes were important for effective vaccination and were not purely circumstantial.
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PMID:Flow cytometric analysis of cellular changes in mice after intradermal inoculation with a liposome-iscom adjuvanted vaccine. 951 63

The present study was carried out to establish a human chronic lymphocytic leukemia (CLL) mouse model by transplantation of a JOK-1 human CLL cell line into SCID (severe combined immunodeficient) mice and to examine anti-leukemic effects of fludarabine phosphate, a prodrug of 9-beta-D-arabinofuranosyl-2-fluoroadenine (2F-ara-A). In vitro cytotoxic screening pattern of 2F-ara-A differed from those of other anticancer agents. Intraperitoneal inoculation with JOK-1 cells in SCID mice allowed the cells to infiltrate into a variety of organs including the liver and thymus, and resulted in the death of the mice with a median survival time of 29.5 days, associated with hepatomegaly, splenomegaly and enlarged lymph nodes. The ascitic cells expressing the human B-lymphocytic cell surface antigen CD19 actually grew after a latent period of 15 days. In this model, twice daily administration of fludarabine phosphate at a dose of 135 mg/kg for 5 days prolonged the survival time of the mice for considerably longer period than once-a-day treatment. Fludarabine phosphate in the doubled course of twice daily increased life span of 32.9%, which was in a similar range to that of doxorubicin. Thus, intraperitoneal inoculation of the human JOK-1 CLL cells into SCID mice seems to serve as an animal model potentially for human leukemia, suggesting that transplantation and subsequent infiltration processes of human CLL cells is useful measures to explore mechanistic aspects for drug-induced modulation of the tumor progression.
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PMID:A human B-cell CLL model established by transplantation of JOK-1 cells into SCID mice and an anti-leukemia efficacy of fludarabine phosphate. 1060 87

Avian hepatitis E virus (HEV), a novel virus identified from chickens with hepatitis-splenomegaly syndrome in the United States, is genetically and antigenically related to human HEV. In order to further characterize avian HEV, an infectious viral stock with a known infectious titer must be generated, as HEV cannot be propagated in vitro. Bile and feces collected from specific-pathogen-free (SPF) chickens experimentally infected with avian HEV were used to prepare an avian HEV infectious stock as a 10% suspension of positive fecal and bile samples in phosphate-buffered saline. The infectivity titer of this infectious stock was determined by inoculating 1-week-old SPF chickens intravenously with 200 microl of each of serial 10-fold dilutions (10(-2) to 10(-6)) of the avian HEV stock (two chickens were inoculated with each dilution). All chickens inoculated with the 10(-2) to 10(-4) dilutions of the infectious stock and one of the two chickens inoculated with the 10(-5) dilution, but neither of the chickens inoculated with the 10(-6) dilution, became seropositive for anti-avian HEV antibody at 4 weeks postinoculation (wpi). Two serologically negative contact control chickens housed together with chickens inoculated with the 10(-2) dilution also seroconverted at 8 wpi. Viremia and shedding of virus in feces were variable in chickens inoculated with the 10(-2) to 10(-5) dilutions but were not detectable in those inoculated with the 10(-6) dilution. The infectivity titer of the infectious avian HEV stock was determined to be 5 x 10(5) 50% chicken infectious doses (CID(50)) per ml. Eight 1-week-old turkeys were intravenously inoculated with 10(5) CID(50) of avian HEV, and another group of nine turkeys were not inoculated and were used as controls. The inoculated turkeys seroconverted at 4 to 8 wpi. In the inoculated turkeys, viremia was detected at 2 to 6 wpi and shedding of virus in feces was detected at 4 to 7 wpi. A serologically negative contact control turkey housed together with the inoculated ones also became infected through direct contact. This is the first demonstration of cross-species infection by avian HEV.
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PMID:Generation and infectivity titration of an infectious stock of avian hepatitis E virus (HEV) in chickens and cross-species infection of turkeys with avian HEV. 1518 48

This study describes experimental infections in 4-week-old chickens inoculated intravenously with approximately 10(8) colony-forming units Streptococcus gallinaceus strain CCUG 42692T (C13156) or Enterococcus hirae strain DSM 20160 (C17410). Birds were necropsied following death and obvious clinical signs of disease or were euthanized weekly after infection for up to 4 weeks. At necropsy, lesions included splenomegaly, hepatomegaly, valvular and/or mural endocarditis. Cardiac lesions included focal necrotizing myocarditis and/or yellow-white vegetative valvular endocarditis or greyish proliferations associated with the mitral valves in 35% (6/20) and 79% (19/24) of birds infected with S. gallinaceus and in 20% (4/20) and 55% (12/22) of birds infected with E. hirae via the brachial and jugular veins, respectively. S. gallinaceus was reisolated from heart valves in 45% (9/20) and 75% (18/24) and E. hirae in 35% (7/20) and 73% (16/22) after inoculation via brachial and jugular veins, respectively. Both challenge strains were also isolated from liver, spleen, bone marrow and hock joints. A significant difference between the infections with the two strains was seen only with reisolation of E. hirae from hock joints (P < 0.007). Significant differences were apparent between the two inoculation routes only with E. hirae, where infection via the jugular vein was associated with higher culture positive isolations from the heart (P = 0.029), bone marrow (P = 0.002) and hock joints (P < 0.001) compared with the brachial vein. Birds injected with sterile phosphate-buffered saline were negative for culture of the challenge strains and no lesions were observed in these controls. The results confirm that both S. gallinaceus and E. hirae can cause endocarditis in experimentally infected chickens.
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PMID:Reproduction of sepsis and endocarditis by experimental infection of chickens with Streptococcus gallinaceus and Enterococcus hirae. 1619 8


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