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)

Trichomonas infections of the abdominal cavity are uncommon and case histories have not been published previously. A patient to whom reference is made this paper developed a combined immunodeficiency syndrome and portal hypertension with ascites on the basis of a cirrhosis of the liver. This seems to have been the precondition for a peritonitis caused by a Trichomonas species.
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PMID:Peritonitis caused by Trichomonas--a preliminary case report. 174 23

Sustained and significant reversal of antibody deficiencies is uncommon, although it has been noted in some cases during childhood and after viral infections. We report a case of reversal of IgG2 deficiency after splenectomy. A 46-year-old man who suffered recurrent sinusitis despite antibiotic therapy was noted to have IgG2 deficiency and cutaneous anergy. Replacement therapy with intravenous immunoglobulin ameliorated his symptoms. After 13 months of therapy, the patient had a diagnostic laparotomy and splenectomy because of portal hypertension, hypersplenism, and consideration of underlying malignancy. No evidence of malignancy or infection, including human immunodeficiency virus, active cytomegalovirus, or Epstein-Barr virus infection, was found. After splenectomy, the patient's serum IgG2 level normalized without replacement therapy. Subsequently, it fell, then normalized once more, and remains normal. The patient also demonstrated positive reactions to delayed hypersensitivity testing after splenectomy, but, subsequently, became anergic and remains anergic at the present time. Since the splenectomy he has remained clinically well without antibiotics or immunoglobulin replacement. In vitro analysis of his lymphocyte function demonstrated impaired T cell proliferation as well as an intrinsic B cell differentiation defect. This case demonstrates the potentially dynamic nature of IgG subclass deficiencies.
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PMID:Variable expression of IgG2 deficiency. 214 99

One of the main causes of cytopenia and cellular immunodeficiency in patients with cirrhosis of the liver and portal hypertension is increased portal pressure, which, in turn, induces increased storage of formed elements in the spleen. Shunting operations producing a stable decompressive effect correct cytopenia similar to splenectomy but at the same time are devoid of the menacing postoperative complications characteristic of splenectomy.
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PMID:[Pathogenesis of the cytopenia syndrome in patients with cirrhosis of the liver complicated by portal hypertension]. 270 11

Liver biopsy specimens previously taken from 16 haemophilic patients with chronic non-A, non-B hepatitis were reviewed. The degree of fibrosis correlated with serum procollagen III peptide (sPIIIP) concentrations, measured both at the time of biopsy and 4.25 years later. Two patients with extremely high sPIIIP concentrations had collateral veins on computed tomography, suggesting portal hypertension. Twenty eight of 47 patients (60%) had splenomegaly on computed tomography, and of 28 patients in whom intravenous contrast medium was used, seven (25%) had collateral oesophageal veins. Serum procollagen III peptide estimations and computed tomography, both non-invasive investigations, indicated that hepatic fibrosis and portal hypertension had developed in a proportion of haemophilic patients with non-A, non-B hepatitis. Infection with the human immunodeficiency virus (HIV) may modify the course of this presumably cytopathic virus infection of the liver.
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PMID:Non-invasive investigation of liver disease in haemophilic patients. 314 33

A total of 437 patients with chronic pancreatitis (CP) were examined. The authors detected interstitial or acute, parenchymal or recurrent, hyperplastic or pseudotumorous, and cystic variants in 91 (20.8%), 218 (49.9%), 78 (17.8%), 22 (5.1%), and 28 (6.4%) patients, respectively, Severe types due to the permanent pain syndrome, substantial weight loss and overall intoxication phenomena were seen in 21.5%. Complications of chronic pancreatitis were revealed in 32.3%. Pyoseptic complications (11.2%), anicteric cholestasis (8.5%), subhepatic portal hypertension (8.0%), cholestatic jaundice (7.8%) were most common. Immunodeficiency states developed with long-term treatment of CP. A small portion (0.9%) of patients with CP developed pancreatic carcinoma. 3% of patients had deaths directly due to the active course of the disease whose causes were pyoseptic processes, pancreatic carcinoma and profuse hemorrhages from exulcerations of the duodenal postbulbar part.
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PMID:[Severe and complicated forms of chronic pancreatitis]. 768 83

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

Five autopsy cases of Vibrio vulnificus infection with liver disease are reported. All five patients ate raw seafood 24 h before the onset of illness. The clinical presentation was of primary septicemia, with positive cultures in both the blood and cutaneous lesions. Stool cultures were positive for the organism in one patient with gastrointestinal symptoms. Autopsy examination revealed liver cirrhosis in three cases and alcoholic liver disease in two; all showed portal hypertension. Gastrointestinal mucosal changes were seen in four patients: edema, hemorrhagic necrosis, and lymphocyte infiltration. One case was of an human immunodeficiency virus infected patient in which histology showed a rare intestinal disease, phlegmonous colitis. We believe this is the first description of a case of concomitant phlegmonous enterocolitis and V. vulnificus infection. Patients with liver disease should be warned about the possibility of life-threatening infections and complications associated with the consumption of raw seafood.
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PMID:Vibrio vulnificus infection in patients with liver disease: report of five autopsy cases. 1211 Dec 6

Complete interferon-gamma receptor 1 (IFNgammaR1) deficiency is a primary immunodeficiency disease characterized by high susceptibility to recurrent, severe mycobacterial and other intracellular infections. We here report the first successful treatment of the disorder by bone marrow transplantation (BMT). The 8-year-old girl had suffered from recurrent mycobacterial infections in the past and had developed liver cirrhosis with portal hypertension. For conditioning, fractionated total body irradiation (TBI) was used in combination with cyclophosphamide and antithymocyte globulin (ATG). The patient received red cell-depleted bone marrow from her HLA-identical sister. The transplantation course was uneventful and 4 years later, the child remains in excellent clinical condition and free of mycobacterial infections. She has stable mixed lymphohematopoietic chimerism after repeat T-cell transfusions. Liver disease has not further deteriorated. This experience shows that correction of IFNgammaR1 deficiency is possible by BMT and complications of the disease can be controlled.
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PMID:Correction of complete interferon-gamma receptor 1 deficiency by bone marrow transplantation. 1239 76

This article focuses on pulmonary arterial hypertension, including both primary pulmonary hypertension (PPH) and those forms of pulmonary arterial hypertension that are related to other factors, including collagen vascular diseases, congenital shunts, portal hypertension, human immunodeficiency viral infection, and exposure to specific drugs and toxins. Risks for different types of pulmonary arterial hypertension are identified. The common pathogenesis for pulmonary arterial hypertension is discussed, and includes an overview of the role of key vasoactive substances such as nitric oxide, prostacyclin, endothelin, and thromboxane. Typical presenting clinical manifestations, recommendations for screening of patients at risk, and key diagnostic findings are discussed. The mainstay of treatment is identified as pharmacologic, and may include diuretics, digoxin, warfarin, calcium channel antagonists, and prostacyclin analogues such as epoprostenol. Surgical interventions are considered as a last resort, and may include unilateral or bilateral lung transplant or atrial septostomy. Treatment options for patients with pulmonary arterial hypertension hold more hope today than they did a decade ago and are identified so as to guide the advanced practice nurse in recognizing and then facilitating the appropriate management of patients with this rare but disabling disease.
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PMID:An overview of pulmonary arterial hypertension: risks, pathogenesis, clinical manifestations, and management. 1585 58

Immune thrombocytopenic purpura (ITP), alone or in combination with autoimmune hemolytic anemia (Evans syndrome) and/or autoimmune neutropenia, is frequent in patients with common variable immunodeficiency (CVID). A 34-year-old man with CVID had long-standing unresponsive ITP. The patient had a 9-year history of CVID on substitutive therapy with intravenous immunoglobulin (IVIG). The clinical course of CVID was complicated with refractory fistulizing inflammatory bowel disease, nodular regenerative hyperplasia of the liver, splenomegaly, severe portal hypertension, and hypercatabolism of IgG. ITP was refractory to medical therapy, including different combinations of corticosteroids, high-dose IVIG, azathioprine, and vincristine. Splenectomy was not performed because of severe portal hypertension. He received a total five doses of rituximab, a monoclonal antibody directed against CD20 antigen, at a dose of 375 mg/m(2). After an initially slow response, his platelet count increased to more than 50,000/microL by the fourth week of infusion. Therapy was well tolerated, and B lymphocytes were effectively depleted from the peripheral blood. The patient was completely tapered off glucocorticoids and maintained platelets at above 40,000/microL. The patient has not taken immunosuppressive agents for 11 months. Early treatment with rituximab might be an option for patients with CVID and ITP that do not respond to other treatments or for patients for whom a splenectomy is contraindicated.
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PMID:Partial response to anti-CD20 monoclonal antibody treatment of severe immune thrombocytopenic purpura in a patient with common variable immunodeficiency. 1612 7


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