Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019214 (hepatosplenomegaly)
4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty male patients (mean age 23 years) with Schistosoma mansoni infections (mean egg count 429 +/- 311/g feces) were each treated with oxamniquine orally in a single daily dose of 20 mg/kg for 3 consecutive days. Seventeen patients had hepatosplenomegaly, two of these had ascites. Three patients had diffuse colonic polyposis, one of these had ascites. Except for one who developed mild hematemesis 3 days after treatment, all patients tolerated the drug very well. However, 11 patients developed a fever 24 to 48 hours after completing treatment, which lasted for 2-3 days and coincided with increased excretion of schistosomal antigens in urine. Three months after completing therapy, all except one young patient ceased to have live egge in the stools or rectal tissue. Six months after treatment, three patients with colonic polyposis showed marked clinical improvement and sigmoidoscopic and barium enema examination demonstrated almost complete disappearance of all polyps.
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PMID:Treatment of complicated schistosomiasis mansoni with oxamniquine. 72 34

This is a case report of juvenile gastrointestinal polyposis involving the gastrointestinal system from the stomach to the rectum. Only few cases have been reported and extra-intestinal manifestations of this syndrome include macrocephaly, hepatosplenomegaly, hypotonia, clubbing of fingers, anemia and protein-losing enteropathy. The disease usually has a poor prognosis, and the children rarely live more than 2 years.
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PMID:Infantile Cronkhite-Canada syndrome?--Case report. 132 40

Patients with simple and/or complicated intestinal bilharziasis and normal controls were chosen for CBC, complete urine and stool analysis, liver function tests, liver biopsy, sigmoidoscopy and rectal biopsy. HLA typing was done by microcytotoxicity test. A statistically significant increased frequency of HLA-B8 antigen was present in with or without polyposis and with or without hepatosplenomegaly, as compared to controls (P less than 0.01). Also HLA-B8 showed a statistically significant increased frequency in those with or without polyposis and with hepatosplenomegaly and without polyposis (P less than 0.05). The HLA-A25 and A26 showed a significant increased frequency among all groups (P less than 0.05). HLA-A1, A9, B18 were variable in frequency among all groups. It was concluded that patients with bilharzial hepatosplenomegaly specially with intestinal polyposis have a high frequency of HLA-B8.
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PMID:HLA typing intestinohepatic schistosomiasis. 250 21

The present study was designed to examine the possible association of HLA antigens and schistosomal hepatosplenomegaly and colonic polyposis in a large number of patients and controls. The results show that hepatosplenomegaly was significantly associated with the presence of two HLA antigens: HLA-A1 and B5; and colonic polyposis to HLA-B5 and B8. Furthermore, asymptomatic infection was significantly seen more in subjects with CW2. These results point to possible gentic factors controlling the pathogenesis of disease and susceptibility to infection in human schistosomiasis.
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PMID:Association of HLA class I antigens (A1, B5, B8 and CW2) with disease manifestations and infection in human schistosomiasis mansoni in Egypt. 308 19

This is a report of a case of juvenile gastrointestinal polyposis consisting of widespread juvenile polyps encountered from the stomach into the rectum. Only few cases have been reported, and extra intestinal manifestations include clubbing of fingers, macrocephaly, hypotonia, hepatosplenomegaly, anemia, and protein-losing enteropathy. The outcome is usually dismal, the children barely becoming older than 2 years. Modern fibreoptic endoscopy with polypectomies performed via the upper and lower gastrointestinal intestinal tracts and via a midbowel ileostomy may offer a viable form of management.
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PMID:Juvenile gastrointestinal polyposis or the infantile Cronkhite-Canada syndrome. 379 50

Levels of vitamin A and components of its transport protein complex, retinol binding protein and prealbumin, as well as zinc and its major binding proteins albumin and alpha 2-macroglobulin were measured in plasma of ninety-one male Egyptian patients with active schistosomal infection and thirty-two healthy adult males. Patients were divided into four groups: Group I had active schistosomiasis without associated complications; Group II had schistosomiasis complicated by colonic polyposis; Group III had advanced schistosomiasis, hepatosplenomegaly and/or ascites; and Group IV had chronic salmonella septicaemia in addition to schistosomiasis. Results showed that all patient groups had subnormal levels of plasma vitamin A, retinol binding protein, prealbumin, zinc and albumin (P less than 0.005 for all parameters) but elevated alpha 2-macroglobulin (P less than 0.001) when compared to the control group. Alterations in the vitamin A transport proteins increased with schistosomal complications and were closely correlated to changes in vitamin A (P less than 0.005), but alterations of albumin and alpha 2-macroglobulin were less consistent and did not correlate with changes in zinc levels. There was a highly significant correlation, however, between plasma zinc concentrations and those of vitamin A, retinol binding protein and prealbumin (P less than 0.001 for all correlations) in all groups of patients as well as the control group. These findings indicate that the binding and transport proteins of both zinc and vitamin A were adversely affected by schistosomiasis and its various complications. Deficiencies of both zinc and vitamin A seem to be interrelated in this disease and the vitamin A transport system may be largely dependent on zinc status.
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PMID:The interaction of zinc and vitamin A in human schistosomiasis. 681 25

Following detection of Schistosoma mansoni in a 12 years old boy who had both schistosomal polyposis and periportal fibrosis with hepatosplenomegaly, epidemiological studies to confirm local transmission were performed in his and 30 other villages in Assiut Governorate, Egypt. The index case's brother and 6 of 380 inhabitants of his village who provided stool specimens were infected with S. mansoni and a farmer with dysentery and hepatosplenomegaly had a positive rectal snip biopsy. All had hepatic abnormalities on ultrasound examination. Two of 221 schoolchildren in another village had mixed infections with S. mansoni and S. haematobium; 17 others had only S. haematobium. None of 419 inhabitants living near the infected boys had S. mansoni infection. Snails from canals and drains near both villages were netted, identified, counted and checked for infection: in the second village one of 1039 Bulinus truncatus was infected with Schistosoma sp. but none of 859 Biomphalaria alexandrina was infected. Schistosomiasis mansoni is being focally transmitted in 2 villages in Assiut Governorate and appears to be spreading from Lower to Middle and Upper Egypt. We believe B. alexandrina, which has been present in some of the waterways for at least 15 years, were infected recently by local inhabitants returning from Iraq or by cattle traders or military recruits from the Delta.
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PMID:Foci of Schistosoma mansoni in Assiut province in middle Egypt. 824 64

We report a case of diffuse follicular center lymphoma (FCL), which is a morphological variant of follicular lymphoma, resembling multiple lymphomatous polyposis (mantle cell lymphoma of the intestine). The patient was a 48-year-old Japanese man who was found, by colonoscopy, to have numerous small polypoid lesions along the entire large intestine. Abdominal computed tomography revealed hepatosplenomegaly and enlargement of multiple mesenteric lymph nodes. Histologically, the lesion was characterized by diffuse proliferation of small- to medium-sized lymphocytes with cleaved nuclei in the mucosa and submucosa. Immunohistochemical studies showed that the tumor cells were CD20+, CD10+, BCL-2+, CD5-, surface IgM-, and cyclin D1-. Moreover, a cytogenetic study showed a translocation at (14;18)(q32;q21). Finally, this case was diagnosed as diffuse FCL, although the tumor was mimicking mantle cell lymphoma.
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PMID:Extranodal diffuse follicular center lymphoma mimicking mantle cell lymphoma of the intestine. 1463 13