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)

The effect on plasma fatty acid composition of 3-6 weeks feeding of standard diets supplemented with various omega 6 polyenoic fatty acids (18:2, 18:3, 20:3 or 20:4) was studied in two young brothers with multineuronal degeneration plus. These boys had mental retardation or maldevelopment, neurosensory hearing loss, retinitis pigmentosa, progressive muscular atrophy, hepatosplenomegaly and adrenal failure. The study objectives were to localize the site of metabolic block and to assess the safety and short-term clinical effect of dietary treatment. Our studies have shown that the low plasma levels of 20:4 omega 6 can be corrected by feeding ethyl arachidonate and that no adverse effects were experienced. A diet enriched in ethyl linoleate produced no obvious increases of 18:2 omega 6 metabolites, indicating that these patients do not have a linoleate deficiency in their omega 6 polyenoic fatty acid pathway. Lack of incorporation of 20:4 omega 6 and a retroconversion of 20:3 omega 6 to 18:2 omega 6 after a dihomo-gamma-linolenate-enriched diet suggest that a defect of delta 5 desaturase may be involved.
J Neurol Sci 1983 Dec
PMID:Effects of polyunsaturated fatty acid diets on plasma lipids of patients with adrenomultineuronal degeneration, hepatosplenomegaly and fatty acid derangement. 666 79

A toxin associated with Toxoplasma gondii infection was obtained from the trophozoites and culture medium used to propagate the parasite in cell cultures. The toxin, named Toxofactor (TF), administered parenterally or nonparenterally in adult mice, produces transient symptoms of lethargy, ruffled fur, and body weight loss. Organ changes which accompanied the outward symptoms included hepatosplenomegaly and involuted thymus. TF activity was detected in extracts of the blood, peritoneal fluid, liver, and spleen of infected mice. Severe damage to embryonal and fetal development was induced when TF was administered during pregnancy. Resorption, abortion, and congenital abnormalities were produced, dependent upon the stage of development at the time of exposure. Adult mice which had reacted to and recovered from an initial intraperitoneal injection to TF were protected against a secondary challenge from TF. Fetal development was also protected from damage when TF was used to challenge adults previously exposed to TF. Mouse and rabbit anti-TF sera neutralized TF activity in the adult. In no instance did control mice show any deleterious effect when exposed to soluble cell lysate from the uninfected cell line (BHK-21) used to propagate the organism plus the used medium from these same uninfected cells. TF activity was not attributed to bacterial, myocoplasmal, or viral contamination. TF toxic activity is labile to elevated temperature and high or low pH, which also destroy its protective properties. TF activity was sensitive to trypsin and was obtained in the elution fraction (alpha-methyl-D-mannoside) from affinity chromatography (concanavalin A-Sepharose 4B). Ultrafiltration indicated the molecular weight to be between 50,000 and 100,000. TF, apparently a glycoprotein, was quantitated for activity by a weight loss assay. A unit of activity was defined as the minimum quantity of TF (highest dilution) which produced at least a 10% average body weight loss in adult Nya:NYLAR female mice between days 7 and 12 post-intraperitoneal injection.
Infect Immun 1983 Dec
PMID:Toxofactor associated with Toxoplasma gondii infection is toxic and teratogenic to mice. 668

Lysinuric protein intolerance is an autosomal recessive disease, due to a defect in intestinal, renal and hepatic dibasic amino acid transport. Two new cases in the same family are reported. The disease appears progressively during the first months of life with failure to thrive, anorexia, vomiting, diarrhea, hepatosplenomegaly, muscular weakness, osteoporosis, anemia, leukothrombocytopenia, hyperammonemia and orotic aciduria after a high-protein intake. Hyperdibasicamino-aciduria was associated with subnormal plasma concentrations of the same aminoacids. Oral l-arginine, l-ornithine, l-lysine, and lysyl-glycine loads confirmed the diagnosis. The supplementation of the diet with l-citrulline resulted in normal levels of blood ammonia. However, hepatosplenomegaly, muscular weakness, osteoporosis remained unchanged and growth was not improved. These may be due to lysine deficiency.
Arch Fr Pediatr 1981 Dec
PMID:[Lysinuric protein intolerance: a severe hyperammonemia secondary to l-arginine deficiency (author's transl)]]. 680 Mar 34

A 29 year old pregnant woman presented at 28 weeks with severe upper abdominal pain. Features of pre-eclamptic toxaemia became apparent and intrauterine death occurred abruptly. The development of shock, a reduction in haemoglobin from 124 to 88 g/l without evidence of external blood loss and tender hepatosplenomegaly suggested major intrahepatic and intrasplenic haemorrhage. This suggestion was supported by ultrasound and CT scans. The patient made a complete recovery over a period of weeks. She was shown to have a circulating LE-inhibitor and had major thrombotic episodes involving her leg veins.
N Z Med J 1982 Dec 08
PMID:Hepatic and splenic haemorrhage as a complication of toxaemia of pregnancy in a patient with circulating lupus anticoagulant. 681 95

Twenty-two patients with chronic lymphocytic leukemia, score 2 according to Rai et al. (10), who received only a course of splenic irradiation are reviewed. Splenic doses ranged from 420 to 1080 rad. Response to splenic irradiation was rated by evaluating peripheral lymphocytosis, hepatosplenomegaly, adenomegaly and disease-related symptoms. Following splenic irradiation, 8 patients showed a significant reduction in splenomegaly; 7 patients showed a significant reduction in peripheral lymphocytosis (less than 10,000/mm3), which has lasted from 15-42 months without any other treatment. In 14 patients, response to splenic irradiation was partial, and it has successively been necessary to treat 12 patients with chemotherapy. Methods of splenic irradiation, survival, clinical and hematologic behavior are discussed in detail.
Tumori 1982 Dec 31
PMID:Treatment by splenic irradiation in 22 chronic lymphocytic leukemia patients. 682 Feb 5

Malaria manifested during the first few months of life may be result of acquisition during pregnancy, at the time of delivery, or by mosquito bite after birth. Both congenital and perinatal malaria are acquired by the transmission of parasitized maternal erythrocytes across the placenta. An infant is described whose mother was diagnosed to have malaria at six months of gestation. The infant developed intermittent fever at 5 weeks of age and presented with anemia and hepatosplenomegaly at 3 months of age at which time Plasmodium falciparum parasites were found on examination of thick smears of the infant's blood. IgG and IgM antimalarial antibodies were detected in maternal blood, but only IgG antibodies were found in the infant's blood at delivery and at the time of diagnosis. These transplacentally transmitted antibodies may afford transient protection for the infant and thus delay the onset of clinical manifestations. Due to the absence of an exoerythrocytic life cycle in congenitally acquired malaria, chloroquine is the drug of choice for treatment. Infections with chloroquine-resistant strains require multiple drug therapy.
Pediatrics 1980 Dec
PMID:Congenital malaria due to Plasmodium falciparum. 700 57

The host-parasite relationship in human visceral leishmaniasis remains poorly understood. In the present study, pathophysiological and immunological changes were examined in BALB/c mice infected with Leishmania donovani. These animals developed chronic infection with massive hepatosplenomegaly and hypergammaglobulinemia. In contrast to mice inoculated with 0.8 X 10(6) or 4 X 10(6) amastigotes, mice infected with 20 X 10(6) amastigotes failed to reduce liver parasite loads during 2 to 8 weeks of observation. At 8 weeks, liver size was increased by 26, 63, and 94%, respectively, in groups infected with 0.8 X 10(6), 4 X 10(6), or 20 X 10(6) amastigotes. Serum immunoglobulin G and M levels at 8 weeks in animals with the heaviest infection were increased by 53 and 80%, respectively, compared with controls. Specific antileishmanial antibodies were detected in the absence of antigen-specific delayed-type hypersensitivity or in vitro lymphocyte responses. Infection did not suppress the in vivo responses of mice to the non-parasite-related antigens sperm whale myoglobin or pneumococcal polysaccharide. Splenic mononuclear cell responses to phytohemagglutinin were suppressed as early as 2 weeks, and by 8 weeks, mice infected with 0.8 X 10(6), 4 X 10(6), or 20 X 10(6) amastigotes had phytohemagglutinin responses which were, respectively, 27.7, 13.9, and 15.8% of controls. Decreased phytohemagglutinin responses could not be related to reductions in splenic T cells; however, splenic B cells and macrophages were increased at 8 weeks of infection. The course of L. donovani infection and disease in BALB/c mice resembles events occurring in humans and should prove useful in defining mechanisms of immune alterations in visceral leishmaniasis.
Infect Immun 1982 Dec
PMID:Host-parasite relationship in murine leishmaniasis: pathophysiological and immunological changes. 715 67

Identical twin Caucasian boys, age 3 months, were seen with fever of unknown origin, hepatosplenomegaly, and pancytopenia. The diagnosis of familial erythrophagocytic lymphohistiocytosis (FEL) was suspected after examination of Twin A's bone marrow and confirmed by an open liver biopsy of Twin B. Twin A died shortly after diagnosis despite treatment with vincristine and prednisone. At autopsy, the diagnosis was confirmed. Twin B responded initially to a three-week course of weekly vincristine and daily prednisone, but symptoms soon recurred. In an effort to enhance delivery of chemotherapy to the active macrophage target, platelets were loaded with vinblastine and then administered intravenously to th patient every 7-10 days. There was an encouraging response reflected by the disappearance of symptoms and the return of peripheral blood count to the normal range, although increased number of histiocytes was still demonstrable in his bone marrow. After nine weeks, he lapsed completely and became refractory to treatment. He died of pseudomonas sepsis four months after diagnosis. This is the first known attempt to deliver a chemotherapeutic agent directly to the macrophages in treating this disease and represents an interesting concept that merits further exploration.
Cancer 1980 Dec 15
PMID:Familial erythrophagocytic lymphohistiocytosis: treatment with vinblastine-loaded platelets. 719 95

A patient with eosinophilic gastroenteritis of 30 years' duration is presented. During the course of the illness, anemia, hepatosplenomegaly, diarrhea and hepatic giant cell granulomas with surrounding eosinophilia were noted. The differential diagnosis of the granulomatous lesion is discussed. The patient's initially serious but subsequently benign illness illustrates the diverse manifestations of eosinophilic gastroenteritis. The need for a cautious therapeutic approach to the disorders of eosinophilia and organ infiltration is emphasized.
Am J Gastroenterol 1980 Dec
PMID:Eosinophilic gastroenteritis with hepatic eosinophilic granulomas. Report of a case with 30-year follow-up. 721 15

Combined application of clinical, genetic and histological criteria in general allows a definite diagnosis of autosomal dominant ichthyosis vulgaris and of X-linked recessive ichthyosis. For differential diagnosis, the following rare syndromes should be considered: ichthyosis bullosa: Refsum syndrome; Jung-Vogel syndrome; ichthyosis with corneal opacity, pili torti and alopecia; ichthyosis with deafness, pili torti and dental anomalies; and ichthyosis with hepatosplenomegaly and cerebellar degeneration.
Fortschr Med 1980 Dec 11
PMID:[Clinical features and genetics of the ichthyosis vulgaris group]. 727 18


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