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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Splenomegaly is an unusual finding in Crohn's disease and can be persistent or transient. In three febrile patients it was so prominent that the diagnosis of lymphoma was considered. In two additional patients the splenomegaly was an incidental finding. It is probably a nonspecific response to the basic disease in most cases, or occasionally a response to the chronic liver disease that can occur in inflammatory bowel disease.
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PMID:Variability of splenomegaly in Crohn's disease. 90 Jan 15

Portal vein thrombosis is a rare complication of ulcerative colitis and is invariably fatal. This report describes a patient with severe Crohn's disease who underwent elective surgery complicated by an anastomotic disruption with faecal peritonitis. Following emergency laparotomy he developed left hypochondrial pain which was a manifestation of splenomegaly consequent upon portal vein thrombosis. Anticoagulation was successful in preventing further spread of the thrombosis as monitored by colour Doppler ultrasound. Severe active disease, surgery and sepsis have been recognized as predisposing factors for thromboembolic complications in inflammatory bowel disease and this patient was exposed to all three. It is conceivable that portal vein thromboses occur more commonly than suspected and ultrasound scanning could ascertain the prevalence if performed prospectively.
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PMID:Portal vein thrombosis in a complicated case of Crohn's disease. 140 98

The clinical and laboratory findings of 37 patients with primary sclerosing cholangitis (PSC) were reviewed. Mean age was 43.8 years, sex ratio between males and females was 3:1; IBD was present in 91% of patients with 51% having ulcerative colitis, 23% unclassified colitis and 17% Crohn's disease. Twenty-seven patients (73%) were symptomatic presenting most commonly with fatigue, pruritus and hepato-splenomegaly. Cholangiography revealed abnormalities affecting both extrahepatic and intrahepatic biliary ductal systems in 51.8% of cases, and only the intrahepatic or extrahepatic biliary tree, respectively in 11.1% and in 37% of cases. The last prevalence was very high compared with that previously known. Clinical and biochemical data, when compared between asymptomatics and symptomatics, demonstrated a significant difference only for alkaline phosphatase which increased in the symptomatic group and for prothrombin activity which decreased among symptomatic patients. Nevertheless, predictive value of sALP for the presence of PSC was high when pts were pooled together with a randomly selected group of 36 non-affected persons that underwent ERCP for suspected primary sclerosing cholangitis: sensitivity was 94% and specificity 78%.
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PMID:Primary sclerosing cholangitis: an analysis of 37 retrospective cases. 148 78

An attempt was made to develop an animal model for the study of the etiology of noncirrhotic portal fibrosis or idiopathic portal hypertension based on the assumption that it is related to chronic abdominal infection. Rabbits were given killed nonpathogenic Escherichia coli intraportally or intravenously. The animals to which a mixture of killed E. coli and rabbit antiserum (aggregated E. coli) was given intraportally developed remarkable histologic changes in the liver. The early inflammatory reactions in the portal area and parenchyma were followed by rapid disappearance of inflammation and development of portal fibrosis with bile duct proliferation. Three intraportal challenges with aggregated E. coli were sufficient to produce pronounced portal fibrosis, although there was considerable variation in response among individual animals. This procedure also produced splenomegaly, and in some animals marked portal hypertension. Injection of nonaggregated killed E. coli into the portal vein or aggregated E. coli into the ear vein also caused similar hepatic changes, but they were milder in degree. These histologic changes resemble portal fibrosis seen in idiopathic portal hypertension and, less closely, pericholangitis associated with inflammatory bowel disease in humans.
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PMID:Experimental portal fibrosis produced by intraportal injection of killed nonpathogenic Escherichia coli in rabbits. 327 75

A woman 68 years of age had fever, malaise, diffuse lymphadenopathy, splenomegaly followed by abdominal pain, and diarrhea. A lymph node biopsy specimen showed nonspecific follicular hyperplasia. Symptoms were responsive initially to prednisone. Recurrent symptoms warranted colonic biopsy, which was consistent with Crohn's disease, and were responsive partially to prednisone and azulfidine. Because of progressive deterioration, a repeat lymph node biopsy was performed and showed the characteristic histologic feature of angioimmunoblastic lymphadenopathy (AILD). The evolution of the histopathologic features of the case is discussed, and gastrointestinal (GI) manifestations of AILD are reviewed. Although the GI tract is an unusual site for extra nodal AILD, colonic involvement can imitate the clinical and histologic features of inflammatory bowel disease.
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PMID:Colonic involvement in angioimmunoblastic lymphadenopathy resembling inflammatory bowel disease. 336 52

Campylobacter fetus subspecies jejuni was isolated fom the feces of 63 (3.2%) of the 1,953 patients who had stools cultured at the Mayo Clinic in 1979. In contrast, Salmonella and Shigella combined were isolated from 31 (1.6%) patients. Two patients had double infections with Salmonella species and C. fetus subsp jejuni. Three patients had no diarrhea at the time of stool culture. One patient, who had chronic lymphocytic leukemia, had both blood and stool cultures positive for C. fetus subsp jejuni. There was a seasonal incidence that peaked in July when 7.8% of all patients who had stools cultured had C. fetus subsp jejuni isolated. Thirteen cases occurred in children 5 years of age and younger and 29 cases occurred between the ages of 15 and 30 years. Clinical features often included a prodrome of malaise, which preceded the onset of abdominal cramps, diarrhea, anorexia, fever, nausea, and vomiting. Grossly bloody diarrhea occurred in 33 patients, and massive intestinal bleeding occurred in 1 patient as a late complication after diarrhea had resolved. Transient splenomegaly was attributed to C. fetus subsp jejuni on one occasion. Proctoscopic findings may be similar to those seen in inflammatory bowel disease or pseudomembranous colitis. Three patients were referred to this institution with newly diagnosed chronic ulcerative colitis, and one patient was referred with newly diagnosed Crohn's disease. C. fetus subsp jejuni was isolated from their stools, and the diagnosis of inflammatory bowel disease was subsequently dropped. A selected review of cases illustrates the variety of gastrointestinal manifestations seen with this organism.
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PMID:Diarrhea due to Campylobacter fetus subspecies jejuni. A clinical review of 63 cases. 725 3

The clinical presentation and outcome of 32 children with primary sclerosing cholangitis (PSC) are reviewed, the largest North American series. The majority of patients were diagnosed in their second decade (median age: 13 years). Four children presented before the age of 2 years, but none in the neonatal period. Seventeen patients had inflammatory bowel disease (IBD), all with colitis, 14 ulcerative colitis, and 3 Crohn's disease. Eight patients presented with chronic liver disease before clinical onset of IBD. Only 8 of 32 patients were jaundiced at presentation. Fifteen of 32 had a normal serum alkaline phosphatase (ALP) level at presentation. Nine children presented with features similar to those of autoimmune hepatitis. Cholangiography was performed in all cases and classified by a scoring system specifically developed for pediatric patients. Intrahepatic disease predominated; in only three cases a common bile duct stricture was identified requiring stenting. Findings on the initial liver biopsy were classified according to Ludwig's criteria for staging PSC: there were 15 biopsies in stages 1 to 2 and 17 biopsies stages 3 to 4. HLA class I and II antigens were determined in 27 patients. An increased incidence of HLA B8 and DR2(15) but not DRw52a (DRB3*0101) was found. Anti-neutrophil cytoplasmic antibody (ANCA) was positive in 10 of 24 patients tested. Survival analysis indicated that a later age at presentation, splenomegaly, and prolonged prothrombin time (PT) at presentation were significant contributors to the prediction of poor outcome (i.e., death or listing for transplantation). Liver transplantation was successfully performed in seven children. Physicians must maintain a high index of suspicion of PSC in any child or young adult presenting with chronic liver disease, especially in the presence of IBD, even with a normal serum alkaline phosphatase level.
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PMID:Primary sclerosing cholangitis in 32 children: clinical, laboratory, and radiographic features, with survival analysis. 759 Jun 57

We report on 56 children with sclerosing cholangitis (SC) seen between 1972 and 1992. The first symptoms occurred at a mean age of 3.7 years; 15 infants had neonatal cholestatic jaundice. At diagnosis, cholestatic jaundice was present in 25 children, hepatomegaly in 54, splenomegaly in 41, and ascites in 12. Serum alkaline phosphatase activity was increased in 49 patients and gamma-glutamyltransferase activity in all patients tested. Most often the histopathologic findings were extensive portal fibrosis and neoductular proliferation. Cholangiography showed abnormal intrahepatic bile ducts in all children and abnormal extrahepatic bile ducts in 35 (63%). The children were separated into three groups: (1) those with SC of neonatal onset (27%); (2) those with SC of postneonatal onset associated with another disease (55%)--histiocytosis X in 14 children, immunodeficiency syndromes in 8, chronic inflammatory bowel disease or autoimmune hepatitis in 8, and congenital psoriasis in 1; and (3) those with SC of postneonatal onset without an associated disease (18%). Biliary cirrhosis was present in all but three children after 6 months to 19.3 years of follow-up. Eleven children died of portal hypertension or liver failure, and six died of a complication related to the associated disease. Fifteen children had liver transplantation; 11 of these are alive 6 months to 6 1/2 years later without recurrence of SC. The overall estimated median survival time of children with SC was 10 years from clinical onset. These results indicate that SC should be suspected in all children with a chronic cholestatic disease and increased serum gamma-glutamyl transferase activity, especially when diseases known to be associated with SC are present. The prognosis is poor, but liver transplantation should be considered except in those with severe immunodeficiency syndromes.
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PMID:Sclerosing cholangitis in children. 828 75

The kallikrein-kinin (K-K) (contact) system is activated during acute and chronic relapsing phases of enterocolitis induced in genetically susceptible Lewis rats by intramural injection of peptidoglycan-polysaccharide (PG-APS). Using the selective plasma kallikrein inhibitor P8720, we investigate whether activation of the K-K system plays a primary role in chronic granulomatous intestinal and systemic inflammation in this model. Group I (negative control) received human serum albumin intramurally. Group II (treatment) received PG-APS intramurally and P8720 orally. Group III (positive control) received PG-APS intramurally and albumin orally. P8720 attenuated the consumption of the contact proteins, high molecular weight kininogen (P<0.03), and factor XI (P<0.04) in group II vs. group III. P8720 decreased chronic intestinal inflammation measured by blinded gross (P<0.01) and histologic (P<0.0005) scores as well as systemic complications (arthritis, splenomegaly, hepatomegaly, leukocytosis, and acute-phase reaction) (P<0.01) in group II as compared with group III. We conclude that relapsing chronic enterocolitis and systemic complications are in part due to plasma K-K system activation, and that inhibition of this pathway is a potential therapeutic approach to human inflammatory bowel disease and associated extraintestinal manifestations.
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PMID:Specific inhibition of plasma kallikrein modulates chronic granulomatous intestinal and systemic inflammation in genetically susceptible rats. 950 76

Primary sclerosing cholangitis (PSC) is increasingly diagnosed in children and adolescents, but its long-term prognosis remains uncertain. The aim of this longitudinal, cohort study was to determine the long-term outcome of children with PSC. Fifty-two children with cholangiography-proven PSC (34 boys and 18 girls; mean age 13.8 +/- 4.2 years; range, 1.5-19.6 years) who were seen at our institution over a 20-year period were followed-up for up to 16.7 years. Two thirds presented with symptoms and/or signs of PSC and 81% had concomitant inflammatory bowel disease (IBD). Twenty-five percent had total alkaline phosphatase activity within the normal range for the age group, but all of them had elevated gamma-glutamyl transpeptidase levels. Autoimmune hepatitis overlapping with PSC was present in 35% of children. A positive but transient clinical and/or biochemical response occurred under therapy with ursodeoxycholic acid, alone or in combination with immunosuppressive medications. During follow-up, 11 children underwent liver transplantation for end-stage PSC and 1 child died. The median (50%) survival free of liver transplantation was 12.7 years. Compared with an age- and gender-matched U.S. population, survival was significantly shorter in children with PSC (P <.001). In a Cox regression model, lower platelet count, splenomegaly, and older age were associated with shorter survival. Presence of autoimmune hepatitis overlapping with PSC (P =.2) or medical therapy (P =.2) did not affect survival. In conclusion, PSC significantly decreases survival in this child population. Although pharmacologic therapy may improve symptoms and liver test results initially, it does not seem to impact the long-term outcome.
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PMID:Primary sclerosing cholangitis in children: a long-term follow-up study. 1283 4


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