Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty human immunodeficiency virus (HIV)-positive patients were studied who presented with right upper quadrant abdominal pain, with or without abnormal biochemical liver function tests, in whom AIDS-related sclerosing cholangitis (ASC) was suspected. The results obtained from hepatobiliary scintigraphy using 99Tcm-IODIDA were compared with data from endoscopic retrograde cholangiopancreatography (ERCP), ultrasound and histological data from liver biopsy or post mortem. 99Tcm-IODIDA was abnormal in 14 patients. Liver biopsy, ERCP or post mortem confirmed ASC in 11 patients of whom 10 had an abnormal 99Tcm-IODIDA study. Ultrasound was performed in eight of the patients with confirmed ASC but was abnormal in only five of these. One patient with mild ASC on ERCP and Kaposi's sarcoma had a normal 99Tcm-IODIDA. In HIV-positive patients with right upper quadrant pain, imaging with 99Tcm-IODIDA provides a non-invasive screening test which may help to determine those patients who should be referred for ERCP.
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PMID:Hepatobiliary scintigraphy in the diagnosis of AIDS-related sclerosing cholangitis. 155 14

Sclerosing cholangitis, an inflammatory disease of the biliary tree that occurs infrequently in childhood, has been recognized in combination with papillary stenosis in adults with the acquired immunodeficiency syndrome. A 10-yr-old child with a familial immunodeficiency syndrome characterized by defective T-cell function and deficiencies of immunoglobulins A and G developed papillary stenosis and sclerosing cholangitis associated with cryptosporidium enteritis. The patient presented with fever, jaundice, right upper quadrant pain, and elevated serum concentrations of transaminases and alkaline phosphatase. The pain and jaundice resolved after endoscopic sphincterotomy, but the biochemical abnormalities persisted. This case demonstrates that the combination of papillary stenosis and sclerosing cholangitis can occur in children as well as adults and may be associated with immunodeficiency syndromes other than the acquired immunodeficiency syndrome. Endoscopic sphincterotomy can provide symptomatic treatment for papillary stenosis in children with this condition, although the effect of sphincterotomy on the natural history of the sclerosing cholangitis is uncertain.
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PMID:Papillary stenosis and sclerosing cholangitis in an immunodeficient child. 271 83

From September 1986 to September 1994, 34 emergency laparotomies were performed in human immunodeficiency virus (HIV) seropositive patients. Patients were divided into 2 groups. Group A included 11 HIV seropositive patients without acquired immunodeficiency syndrome (AIDS). In these patients, indications for exploration included right lower quadrant pain consistent with appendicitis in 6 patients, right upper quadrant pain consistent with cholecystitis in 3 patients, small bowel obstruction in 1 patient, and blunt abdominal trauma in 1 patient. No postoperative deaths were observed. Group B included 23 AIDS patients. Indications for exploration were diffuse peritonitis in 8 patients, right lower quadrant pain consistent with appendicitis in 6 patients, right upper quadrant pain consistent with cholecystitis in 5 patients, bowel obstruction in 2 patients, diffuse abdominal pain in 1 patient, and massive rectal hemorrhage in 1 patient. The mortality rate in this group was 35% (8 out of 23 patients). Five of the 8 patients with diffuse peritonitis died postoperatively (62%). The importance of early diagnosis and prompt surgery is emphasized to improve the prognosis in AIDS patients, because of their poor general condition and the severity of abdominal complications.
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PMID:[Abdominal surgical emergencies in human immunodeficiency virus (HIV) infected patients. Apropos of 34 cases]. 878 19

Both hepatic parenchymal and biliary tract diseases are common in patients with human immunodeficiency virus (HIV). In this paper, the authors focus mainly on clinical aspects of acquired immunodeficiency syndrome (AIDS)-related cholangiopathy. Although the etiology is unclear, several opportunistic infections (cytomegalovirus, Cryptosporidium and others) are suspected to cause it. Endoscopic retrograde cholangiopancreatography (ERCP) is the diagnostic gold standard and it offers a therapeutic means to provide symptomatic relief in case of papillary stenosis. The most common ERCP pattern is diffuse sclerosing cholangitis in combination with papillary stenosis. Clinically, the presentation may be variable, although right upper quadrant pain and fever accompanied by an elevated serum alkaline phosphatase (ALP) level are the most common manifestations. Jaundice is unusual suggesting that complete ductal obstruction is rare. While ERCP results and the need of sphincterotomy do not influence the prognosis, antiretroviral therapy is a protective factor and, on the contrary, high ALP level is related to a less favorable outcome. Regarding the possible pathogenic mechanisms through which HIV infection could be involved in AIDS-related cholangiopathy, in vitro experiments have shown that concurrent active HIV replication and Cryptosporidium parvum infection synergistically increase cholangiocyte apoptosis and thus jointly contribute to AIDS-related cholangiopathies.
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PMID:An update on AIDS-related cholangiopathy. 1921 10