Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
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Systemic mastocytosis is a rare disease of mast cell proliferation with cutaneous and multi-visceral involvement. Portal hypertension and ascites are rare manifestations of systemic mastocytosis. We report a case of systemic mastocytosis presenting with extensive nodular cutaneous lesions and hepatic dysfunction, manifested by portal hypertension (ascites, splenomegaly) and derangement of metabolic function (hyperammonemia, hypoalbuminemia, hypocholesterolemia), a picture resembling that of a common cirrhotic form. The correct diagnosis was established only after tissue sections were appropriately stained for mast cells. On the basis of our and other observations we suggest that systemic mastocytosis be added to the list of infiltrative diseases of the liver with potential evolution to portal hypertension and compromise of biochemical functions.
Minerva Med 1992 Sep
PMID:[Systemic mastocytosis with portal hypertension and hepatocellular failure]. 143 8

(B10.A x A/WySn)F1 mice, infected with the Friend virus (FV) complex, were used as a predictive therapeutic model for AIDS. These infected mice exhibit many of the viral and immunologic manifestations of AIDS. Bropirimine (2-amino-5-bromo-6-phenyl-4[3H]pyrimidinone, ABPP) is an immunomodulating compound which has been shown to inhibit other viral infections. Oral (per os treatment) dosages of ABPP ranging from 50 to 400 mg/kg/day for 3 days resulted in increased numbers of infectious centers in the infected mice and increased splenomegaly and percentage of Ig+ (B cells) in spleens of infected and uninfected mice. Decreased percentages of total Thy-1.2+ (total T) cells and L3T4+ (T-helper) cells were seen in both uninfected and infected mice and a slightly decreased percentage of Ly-2+ (T-suppressor/cytotoxic) cells was observed in spleens of the infected mice. No effect on Ly2+ cells in spleens of uninfected mice was found. Intraperitoneal injection, single or multiple, of 20-200 mg/kg ABPP prior to FV injection resulted in increased spleen weights but had no effect on numbers of infectious centers in the spleens or on FV antibody titers in the plasma. Intraperitoneal treatment of uninfected mice with ABPP resulted in slight or no changes in percentages of Thy-1.2+, L3T4+ and Ly-2+ cells. Mice receiving multiple exposures of ABPP had an increase in percentage of splenic B cells and a depressed response to the T cell mitogen PHA. Treatment with ABPP induced the production of interferon (IFN); however, a state of hyporesponsive IFN production was seen following multiple administrations of ABPP. These data suggest that the immunomodulator ABPP may have an enhancing effect on this retroviral disease.
Antiviral Res 1992 Sep
PMID:Murine retroviral disease-enhancing effects of a pyrimidinone immunomodulator. 144 28

We present a rare case of bacterial endocarditis of tricuspid valve caused by temporary intracardiac pacing. The 48-year old male patient developed complete a-v block during the 1st day of acute inferior myocardial infarction. Intracardiac electrode was inserted for temporary pacing. After 4 days signs of bacterial endocarditis developed. Patient was markedly febrile, moderate tachycardia with gallop rhythm and systolic murmur of tricuspid valve insufficiency were present. Dullness to percussion was audible at the base of right lung. Hepato- and splenomegaly appeared during the second month of hospitalization. Laboratory tests revealed: elevated ESR, leukocytosis with a shift to the left, several blood cultures were positive to Staphylococcus aureus. On repeated chest X-ray patchy infiltrates with thin-walled translucent pools were visible. Transthoracic and transoesophageal++ echocardiography provided more precise informations. Bacterial vegetations were visualised on the tricuspid valve. Coronary angiography revealed proximal occlusion of the right coronary artery and 75-80% stenosis of the left circumflex artery. Antibacterial treatment guided by blood cultures was begun: vancomycin combined with netilmycin, then tienamycin and diflucan--after 10 weeks treatment was decided to be unsuccessful and the decision about surgical treatment was made. In extracorporeal circulation posterior left leaflet together with granular bacterial growths was excised. Septal and anterior leaflets were found normal. Cultures made of excised tissue were positive for Staphylococcus aureus and subsequent treatment with fluoroquinolones gave satisfactory result. Postoperative echocardiography revealed only small tricuspid valve insufficiency. Coronary by-pass surgery was performed later because of the high risk of simultaneous operation.(ABSTRACT TRUNCATED AT 250 WORDS)
Kardiol Pol 1992 Sep
PMID:[Surgical treatment of bacterial endocarditis of the tricuspid valve]. 147 72

The role of a previously described bacterial protein (F3'EP-Si), now designated P90, in the survival of Streptococcus intermedius in the host was investigated, and the immunosuppressive and B-cell-mitogenic effects of this protein were further characterized. C57BL6 mice treated with P90 were about 50 times more susceptible to infection with this bacterium than untreated mice. One of seven splenocytes of C57BL/6 mice were activated by P90. Marked splenomegaly was observed in mice treated with P90, with increased numbers of splenic mononuclear cells and polyclonal immunoglobulin-secreting plaque-forming cells. Peak responses were seen on day 3 for immunoglobulin M (IgM) and on day 5 for IgG, with an isotypic pattern consisting predominantly of IgG2a and IgG2b. When mice were treated with P90 before being primed with sheep erythrocytes, polyclonal immunoglobulin synthesis was accompanied by an ephemeral stimulation of the specific immune response against sheep erythrocytes that was quickly replaced by a dramatic immunosuppression. In contrast, when mice were treated with P90 after being primed, the polyclonal activation was comparatively much less evident and there was no suppression of the specific immune response. Immunosuppression was considerably reduced in mice thymectomized as adults or depleted of CD8+ cells. Adoptive transfer experiments showed that B cells obtained from P90-treated mice were less able to respond to an antigenic challenge, even in the presence of normal T cells, and that T cells obtained from P90-treated mice could actively suppress the specific immune response of normal B cells.
Infect Immun 1992 Sep
PMID:Protective effect of a T-cell-dependent immunosuppressive, B-cell-mitogenic protein (F3'EP-Si, or P90) produced by Streptococcus intermedius. 150 Jan 65

Twenty-seven Brazilian and 32 Sudanese patients with hepatosplenic schistosomiasis from areas where the disease is endemic were examined with ultrasound (US). Hyperechoic periportal areas indicating periportal fibrosis (PPF) were present in all patients irrespective of their origin. Nonspecific findings were splenomegaly (all patients), gallbladder wall thickening (81% and 92%, respectively, in Brazilian and Sudanese patients), portal vein (74% and 87%, respectively) and splenic vein (59% and 70%, respectively) enlargement, and portosystemic vascular shunts (62% and 61%, respectively). The hepatic alterations were congruent and the frequency of their occurrence was similar in both patient groups. With a standardized grading system, it was shown that grade of PPF was significantly correlated with a history of bleeding from endoscopically proved esophageal varices and with distention of the portal vein as measured with US. It was concluded that sonographic grading may be used in patients with hepatosplenic Schistosoma mansoni infection who originate from completely different endemic areas.
Radiology 1992 Sep
PMID:Hepatosplenic schistosomiasis: comparison of sonographic findings in Brazilian and Sudanese patients--correlation of sonographic findings with clinical symptoms. 150 54

We describe the unique clinical and histopathologic features of a child with biochemical and immunocytochemical features of Niemann-Pick disease type C (NPC). Clinically, she was found to have multiple xanthomas of the upper aerodigestive tract with dysphagia and expressive language delay, splenomegaly, bony infarcts, and type IIb hyperlipidemia. Neurologic examination was otherwise normal. Microscopy revealed foam cells in her bone marrow, liver, tongue, tonsils, glottis, and in normal-appearing peritonsillar mucosa. Lipid analysis of a liver biopsy specimen showed a small increase in phospholipids, a twofold increase in sphingomyelin, a fivefold increase in cholesterol, and a marked (25-fold) increase in bis(monoacylglycerol) phosphate. Lysosomal acid hydrolase activities in cultured skin fibroblasts were nondiagnostic. Biochemical and immunocytochemical studies of cultured fibroblasts demonstrated lysosomal accumulation of unesterified LDL-derived cholesterol as well as delayed induction of homeostatic responses to endogenous cholesterol consistent with a diagnosis of NPC. Based upon these observations, we speculate that this patient could have a new phenotypic expression of NPC or represents a new cholesterol lipidosis biochemically resembling NPC. The chance occurrence of two separate lipid disorders seems less likely.
Neurology 1992 Sep
PMID:Clinical, pathologic, and biochemical features of a cholesterol lipidosis accompanied by hyperlipidemia and xanthomas. 151 68

Malaria at an elevation of 1500 meters is uncommon and is usually unstable when it occurs. To confirm reports of a recent increase in the transmission of stable malaria in the Oksibil Valley, at an elevation of 1250-1500 meters in the Jayawijaya Mountains of Irian Jaya, Indonesia, 5 malariometric surveys were conducted in 4 villages between May 1990 and July 1991. A total of 3380 blood smears from 1949 people was examined. Prevalence rates over the survey period were consistent in each of the 4 villages,with averages of 10% for infants, 50% for children 1-4 years old, 35% for those 5-9 years old, 28% for those 10-14 years old, and 16% for adults (over 15 years of age). The spleen rate for those less than 5 years old was 96%, with an average enlarged spleen score of 2.32. Plasmodium falciparum accounted for 55% of the infections in the valley, but P. vivax was the predominant species in those less than 10 years old. In the village of Kutdol, at an elevation of 1500 meters, P. Malariae was identified in 43% of the positive smears. 4 cases were diagnosed as P. ovale. Infection with P. falciparum without obvious clinical symptoms was common in both adults and children. Entomologic and epidemiologic data suggested that the recent upsurge in transmission coincided with the replacement of traditional village huts with more modern social housing. This replacement required the extensive construction of drainage ditches, which inadvertently also served as additional vector breeding sites. The authors suspect that this manipulation of the environment, while attempting to improve the quality of life, created conditions which were conducive for the increased transmission of stable malaria.
Am J Trop Med Hyg 1992 Sep
PMID:Heightened transmission of stable malaria in an isolated population in the highlands of Irian Jaya, Indonesia. 152 48

Unusual clinicopathological features drew our attention to nine of 208 cases diagnosed as Hodgkin's disease. Lymph node biopsy specimens in these cases were immunostained with monoclonal antibodies against B-cell, T-cell and Reed-Sternberg cell associated antigens and epithelial membrane antigen (EMA). Reed-Sternberg-like and other atypical large cells were dispersed in a diffuse, small lymphocyte-rich background, consistent more often with the initial diagnosis of diffuse, lymphocyte predominance Hodgkin's disease. The clinical stage in these cases was unusually advanced (stages III and IV). Splenomegaly was a common feature (six of nine cases), the male to female ratio was 7:2 and the median age was 55 years (range 25-77). Response to recognized regimes for Hodgkin's disease treatment was poor in most cases, and three patients died early of their disease. Large cells were B-lymphocytes expressing EMA--an immunophenotype similar to nodular, lymphocyte predominance Hodgkin's disease. Reed-Sternberg cell and T-cell associated antigens were absent on large cells. Mature T-cells, with nuclear irregularities in some instances, predominated in the background. A more appropriate diagnostic category is, therefore, T-cell-rich B-cell lymphoma. The cases represent a 4-5% erroneous diagnosis of Hodgkin's disease and further suggest that there is a need for revision of criteria for the diagnosis of the diffuse, lymphocyte predominance variant.
Histopathology 1991 Sep
PMID:Large B-cell lymphoma rich in T-cells and simulating Hodgkin's disease. 165 14

The generalized lymphoproliferative disease (gld) and lymphoproliferation (lpr) mutations induce the development of strikingly similar autoimmune and lymphoproliferative syndromes in C57BL/6 mice (B6). These syndromes are characterized by hyperglobulinaemia, high levels of circulating autoantibodies and significant splenomegaly and lymphadenopathy resulting principally from the accumulation of a double negative CD4/CD8 T-cell population. These similarities led to the suggestion that the gld and lpr mutations affect two different steps of a common metabolic pathway controlling the differentiation of the T cells. By transferring haematopoietic cells into sublethally irradiated recipients we provide evidence for the different aetiology of the gld- and lpr-induced syndromes. The [gld----gld] chimaeras developed a gld-induced syndrome, like the [lpr----lpr] chimaeras developed a lpr-induced syndrome. However, in contrast to the severe lymphoid aplasia observed in the [lpr----wild] chimaeras, the [gld----wild] chimaeras showed an attenuated form of the gld-induced syndrome. The [lpr----gld] chimaeras developed a lymphoid aplasia (as in the [lpr----wild] chimaeras). This result shows that the gld environment cannot substitute for the lpr environment and allow for the emergence of an lpr-induced pathology.
Immunology 1991 Sep
PMID:Haematopoietic cell transfers between C57BL/6 mice differing at the lpr or gld locus. 168 43

Most of the circulating lymphocytes from three asymptomatic adults (one male, two female, age range 61-67 years) with isolated persistent lymphocytosis of between 7.1 and 10 x 10(9)/l possessed characteristic villous projections of the cell membrane. Morphological, histochemical, ultrastructural, immunological, and genotypic studies confirmed a clonal proliferation of tartrate-resistant acid phosphatase (TRAP)-negative CD5-CD10-CD25- and CD11c+ B-cells. In addition to CD11c, these cells expressed other adhesion receptors (LFA-1/CD11a, VLA-4/CD29/49d, ICAM-1/CD54, and LAM-1) and produced detectable amounts of interleukin-1 beta, interleukin-6, and in one case tumour necrosis factor-alpha mRNA. This monoclonal villous lymphocytosis (MVL) could be differentiated from B-cell chronic lymphocytic, prolymphocytic, and hairy cell leukaemias, and from previously recognized CD11c+ chronic B-cell leukaemia. A rare splenomegalic non-Hodgkin's lymphoma variant with circulating villous B-lymphocytes (SLVL), usually CD10+ and sometimes CD11c- and TRAP+, appears to be a closely related disorder. In all three patients the lymphocyte count increased very slowly, at a rate less than 5 x 10(9)/l per year, over 3-7.5 years of follow up, and a moderate splenomegaly eventually developed in one of the patients. Chemotherapy was never required. MVL may be a relatively benign clinical entity akin to SLVL within the group of CD11c+ B-cell lymphoproliferative disorders.
Leukemia 1991 Sep
PMID:Monoclonal lymphocytosis with villous lymphocytes: a chronic lymphoproliferative disease of CD11c+ B-cells. 168 36


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