Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The role and value of endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric age group is not well established, because pancreatic and biliary diseases are less common in children. This however is not the case in areas like the Eastern Province of Saudi Arabia where sickle cell disease (SCD) and other hemoglobinopathies are common, with increased frequency of cholelithiasis and choledocholithiasis. The purpose of this study was to evaluate the indications, findings, safety and therapies of ERCP in children. One hundred and twenty five children had diagnostic and/or therapeutic ERCP as part of their management at our hospital. Their medical records were reviewed for: age at diagnosis, sex, Hb electrophoresis, indication for ERCP, findings, therapy and complications. There were 77 males and 48 females. Their age at presentation ranged from 5-18 year (mean 13.25 year). The majority of them had sickle cell disease (77.6%). The indications for ERCP were: obstructive jaundice (67.2%), recurrent biliary colic with or without jaundice (10.4%), acute and chronic pancreatitis (7.2%), postoperative bile leak (2.4%), cholangitis with obstructive jaundice (2.4%), hepatitis of unknown etiology (3.2%), cirrhosis of unknown etiology (4%), thalassemia with jaundice (0.8%), hemobilia (0.8%), acute cholecystitis with jaundice (0.8%), and sickle cell disease with ulcerative colitis and obstructive jaundice (0.8%). In six children, ERCP was done following laparoscopic cholecystectomy. ERCP was carried out under sedation in 91 (72.8%) children and under general anesthesia in 34. It was successful in 121 (96.8%) children while cannulation of the Ampulla failed in four. ERCP was normal in 43 children, but eight of them showed evidence of recent stone passage and in six, there were gallstones. In the remaining children, ERCP revealed: normal CBD with stones (18 patients), dilated CBD with stones (17 patients), dilated CBD without stones (19 patients), dilated biliary tree with stones (10 patients), dilated biliary tree without stones (six patients), bile leak (two patients), dilated biliary tree with stones and choledocho-duodenal fistula (one patient), choledochal cyst (two patients), septate gallbladder (one patient), normal ERCP with multiple pancreatic cysts (one patient) and biliary stricture (one patient). The following procedures were carried out: 35 had endoscopic sphincterotomy and stone extraction, 20 had endoscopic sphincterotomy, four had CBD stenting, one underwent removal of a stent, two had insertion of a nasobiliary tube and one had biliary endoprosethesis. There was no mortality. One had bleeding from the site of sphincterotomy which stopped after adrenaline injection. Four patients (3.2%) developed transient mild pancreatitis which settled conservatively. ERCP in the pediatric age group is safe both as a diagnostic and therapeutic procedure. ERCP can provide valuable information which aid in the diagnosis of biliary and pancreatic diseases in children as well as therapy with the technical feasibility of endoscopic sphincterotomy. This is specially so in the era of laparoscopic cholecystectomy, where ERCP should be the treatment of choice in children with CBD stones who are going or have previously undergone laparoscopic cholecystectomy.
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PMID:Diagnostic and therapeutic ERCP in the pediatric age group. 1714 28

We report the case of a patient with refractory ulcerative colitis who developed cholestatic acute liver damage after a single infusion of infliximab. Unusual aspects of this case were the early onset (after the first administration) of liver damage and the absence of antinuclear antibodies, alcohol intake, hepatotoxic drugs and all known viral and metabolic causes of hepatic injury. Moreover, no serological or morphological findings of primary sclerosing cholangitis were observed. The patient's liver damage resolved spontaneously within 6 weeks. Although a direct relationship between administration of infliximab and onset of acute liver damage could not be definitely established, our case suggests that infliximab may induce direct liver damage, the course of which is similar to acute cholestatic hepatitis and resolves following withdrawal of the drug.
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PMID:Onset of liver damage after a single administration of infliximab in a patient with refractory ulcerative colitis. 1716 3

High mobility group box 1 (HMGB1) protein plays multiple roles in transcription, replication, and cellular differentiation. HMGB1 is also secreted by activated monocytes and macrophages and passively released by necrotic or damaged cells, stimulating inflammation. HMGB1 is a novel antigen of anti-neutrophil cytoplasmic antibodies (ANCA) observed in the sera of patients with ulcerative colitis and autoimmune hepatitis, suggesting that HMGB1 is secreted from neutrophils to the extracellular milieu. However, the actual distribution of HMGB1 in the cytoplasm of neutrophils and the mechanisms responsible for it are obscure. Here we show that HMGB1 in neutrophils is post-translationally mono-methylated at Lys42. The methylation alters the conformation of HMGB1 and weakens its DNA binding activity, causing it to become largely distributed in the cytoplasm by passive diffusion out of the nucleus. Thus, post-translational methylation of HMGB1 causes its cytoplasmic localization in neutrophils. This novel pathway explains the distribution of nuclear HMGB1 to the cytoplasm and is important for understanding how neutrophils release HMGB1 to the extracellular milieu.
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PMID:Post-translational methylation of high mobility group box 1 (HMGB1) causes its cytoplasmic localization in neutrophils. 1740 84

The term ''overlap syndromes of liver diseases'' includes coexistence of autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), primary biliary cirrhosis (PBC). Due to their unknown etiology, as well as their variable presentation with mixed clinical and biochemical features, these overlap syndromes are often a diagnostic and therapeutic challenge for hepatologists. The most frequent association reported occurs between AIH and PBC. More rare is the overlap between AIH and PSC, typical in young age and often concomitant with an inflammatory bowel disease as ulcerative colitis. The treatment of choice is based on ursodeoxycholic acid and immunosoppressive drugs, used at the same time or consecutively, according to the course of disease. Histological examination seems an important tool, but often does not help for a correct diagnosis due to lack of specificity. Two particular forms of variant syndrome are the so called outlier syndromes, without clear characteristics of overlap: the autoimmune cholangitis, probably a form of PBC anti-mitochondrial antibodies negative, and the hepatitis C virus related with stigmata of autoimmunity, such as nonspecific autoantibodies at low titer. The diagnostic score system elaborated in 1999 by the International AIH Group can help for diagnosis, even if its definite validity is lacking.
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PMID:Diagnostic and therapeutic questions in overlap syndromes of autoimmune hepatitis. 1741 45

Oral 5-aminosalicylic acid (5-ASA) has been known as a first-choice drug for ulcerative colitis. However, hypersensitivity reactions, including pancreatitis, hepatitis, and skin rash, have been reported with 5-ASA. Topical formulations of 5-ASA like suppositories have been rarely reported to induce adverse reactions because of their limited absorption rate. We recently experienced a case of acute pancreatitis caused by 5-ASA suppositories in a patient with ulcerative colitis. A 26-year-old male was admitted with abdominal pain and diagnosed as ulcerative colitis. Acute pancreatitis occurred soon after 24 hours of treatment with oral mesalazine. Drug-induced pancreatitis was suspected and administration of mesalazine was discontinued. Then 5-ASA suppositories were started instead of oral mesalazine. Twenty-four hours after taking 5-ASA suppositories, he experienced severe abdominal pain, fever, and elevation of amylase levels. The suppositories were immediately stopped and symptoms resolved over next 48 hours. Herein, we suggest that, in patients treated with 5-ASA suppositories who complain of severe abdominal pain, drug-induced pancreatitis should be suspected.
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PMID:[A case of acute pancreatitis caused by 5-aminosalicylic acid suppositories in a patient with ulcerative colitis]. 1815 75

Ulcerative colitis and Crohn's disease represent the two major forms of inflammatory bowel disease. In this highlight topic series of articles we cover the latest developments in genetics and epidemiology, intestinal physiology, mucosal immunology, mechanisms of epithelial cell injury and restitution, current medical therapy, modern surgical management, important extra-intestinal complications such as primary sclerosing cholangitis, cholangiocellular carcinoma and autoimmune hepatitis as well as endoscopic and molecular screening, detection and prevention of small bowel and colorectal cancer.
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PMID:What's new in inflammatory bowel disease in 2008? 1820 Jun 55

Despite the accumulation of positive data, the role of azathioprine (AZA) in the maintenance of remission of ulcerative colitis is still controversial. We looked at the follow-up of the ulcerative colitis patients who, after responding to either steroids or cyclosporin (CsA), received AZA at our referral center for over a decade. The 39 patients (29 m/10f) were treated between 1991 and 2007. Twenty-five of them had responded to CsA, the remaining 14 to corticosteroids. AZA was usually overlapped with either of the two agents at the initial dose of 2mg/kg/day. The definitions of remission, relapse, and AZA toxicity followed commonly agreed criteria. The median duration of the AZA treatment was 14 months (<1-201). Fifty-two percent and 14%, respectively, of the CsA and the steroid responders needed surgery (overall rate=38%). The figures were 32 and 15 at the first year. The majority of the patients had 1-2 relapses often in connection with withdrawal of AZA; only 3 of these relapsers needed hospitalization. AZA caused toxicity in 16/39 (41%) patients, requiring withdrawal in 23% of the cases; leukopenia (17%) and hepatitis/cholestasis (10%) ranked first and second for frequency. All of the patients in whom AZA was stopped (or reduced) relapsed. In conclusion, the 1-year colectomy rates compare favorably with the figures reported by the literature. By contrast, the toxicity rates were higher than expected. Failure to genotype or to use escalating AZA doses can only be hypothesized as causes.
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PMID:The 17-year single-center experience with the use of azathioprine to maintain remission in ulcerative colitis. 1865 49

Pioderma gangrenosum (PG) is an uncommon ulcerative cutaneous dermatosis associated with a variety of systemic diseases, including inflammatory bowel disease (IBD), arthritis, leukaemia, hepatitis, and primary biliary cirrhosis. Other cutaneous ulceration resembling PG had been described in literature. There has been neither laboratory finding nor histological feature diagnostic of PG, and diagnosis of PG is mainly made based on the exclusion criteria. We present here a patient, with ulcerative colitis (UC) who was referred to the emergency section with a large and rapidly evolving cutaneous ulceration. Laboratory and microbiological investigation associated with histological findings of the ulcer specimen allowed us to exclude autoimmune and systemic diseases as well as immuno-proliferative disorders. An atypical presentation of PG with UC was diagnosed. Pulse boluses of i.v. methyl-prednisolone were started, and after tapering steroids, complete resolution of the skin lesion was achieved in 3 wk. The unusual rapid healing of the skin ulceration with steroid mono-therapy and the atypical cutaneous presentation in this patient as well as the risk of misdiagnosis of PG in the clinical practice were discussed.
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PMID:Atypical presentation of pioderma gangrenosum complicating ulcerative colitis: rapid disappearance with methylprednisolone. 1880 62

The role of cytomegalovirus (CMV) infection in patients with inflammatory bowel disease (IBD) is controversial. Although CMV has been specifically associated with refractory disease, the strength and nature of this association have been a subject of debate. The aim of this study was to evaluate the prevalence and outcome of acute cytomegalovirus infection in patients with severe refractory and complicated inflammatory bowel disease. Seventy-two patients with active IBD (both ulcerative colitis [UC] and Crohn's diseases [CD]) were included in this study. Thorough history taking and physical examination of all patients was made with special emphasis on symptoms and signs of CMV disease. Colonoscopic assessment was made for the extent and activity of IBD and collection of specimen. Prevalence of CMV infection was estimated by serology; anti-CMV IgM and IgG antibodies, and pathologic studies of colonic biopsies used conventional haematoxylin and eosin (H & E) and immunohistochemistry (IHC) with monoclonal antibodies. Complete blood count and liver function tests were done for all patients. Among 72 patients with active inflammatory bowel disease, 23 (31.9%) were resistant to intravenous steroids. CMV was detected in eight (six with UC and two with CD) of the 23 (34.8%) steroid-resistant patients and in only one (3.2%) patient in the remaining 31 patients under steroid treatment and was not detected in 18 IBD patients not using steroids. Among nine CMV-positive IBD patients, six (66.6%) were female and six had fever; cervical lymphadenopathy was found in five patients and splenomegaly in two, compared to no patients in the CMV-negative group (P = 0.01 and 0.03, respectively). Leucopenia and thrombocytopenia were predominantly seen in the CMV-positive versus CMV-negative patients (2.1+/-0.3 vs. 5.9+/-3.4 and 98+/-34 vs. 165+/-101, respectively). Pancolitis was found in five of nine CMV-positive IBD patients whereas in only two patients out of 63 in the CMV-negative group (P = 0.005). Acute CMV infection in patients with IBD is not rare and is often underestimated. CMV infection in patients with refractory or complicated IBD should be ruled out before aggressive immunosuppressive therapy. High clinical index of suspicion for the association of CMV infection with IBD should be directed towards female IBD patients presenting with fever, lymphadenopathy, splenomegaly, leucopenia, and mild hepatitis. CMV IHC is significantly more sensitive than routine H & E stain and should be considered as part of the routine evaluation of IBD patients with severe exacerbation or steroid-refractory disease before proceeding with other medical or surgical therapy that may not be necessary once the CMV is treated.
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PMID:Acute cytomegalovirus infection is a risk factor in refractory and complicated inflammatory bowel disease. 2009 80

Autoantibodies are disease markers of autoimmune hepatitis (AIH). Antinuclear antibodies, smooth muscle antibodies, antibodies to liver/kidney microsome type 1, and perinuclear antibodies to neutrophil cytoplasm constitute the ''conventional'' battery of autoantibodies, while an emerging interest to evaluate new autoantibodies as diagnostic or prognostic markers, such as the anti-Saccharomyces cerevisiae antibodies, is detectable (ASCA). This paper focuses mainly on the findings and the potential role of ASCA in AIH. These antibodies are present in 5-6.3% of blood donors and in the gastrointestinal setting, ASCA have been found most often in Crohn's disease and with lower frequency in the course of ulcerative colitis and celiac disease. Furthermore, they have been described, to a lesser extent, in patients with primary sclerosing cholangitis and primary biliary cirrhosis and in AIH. ASCA occur in 20-30% of patients suffering from AIH with a statistically significant increase observed only for IgG ASCA in type 1 AIH. This probably indicates collateral immune reactivities to the primary pathogenic process. The outcome of hepatitis is not influenced by the presence of ASCA. In conclusion, ASCA positivity does not imply that there exists a distinct subgroup of patients with AIH and these autoantibodies are not involved in the pathogenetic mechanism of AIH.
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PMID:Anti-Saccharomyces cerevisiae as unusual antibodies in autoimmune hepatitis. 1921 6


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