Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-nine patients with severe aplastic anaemia (SAA) were submitted to bone marrow transplantation (BMT) and their immunological recovery analysed. Total lymphocyte counts, estimation of B lymphocytes, T lymphocytes and their subsets, natural-killer (NK) activity were performed. Cells with the CD8+ phenotype and NK activity were the first signs of immunological recovery, whereas the CD4+ subset recovered later in patients who suffered from acute graft versus host disease (GvHD) and infections. Acute and chronic GvHD, cirrhosis, rejection and HIV viral infection contributed to the persistence of the profound immunodeficiency status observed after BMT. Our results did not differ greatly from the others and confirmed that BMT may be performed in underdeveloped countries despite the difficulties it might pose.
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PMID:Immunological recovery after bone marrow transplantation for severe aplastic anaemia: a Brazilian experience. 792 58

This report describes the case of a 14-yr-old hemophiliac who died of complications of primary pulmonary hypertension. He was infected with the human immunodeficiency virus. The autopsy disclosed that he also had membranoproliferative glomerulonephritis type III and hepatic cirrhosis, both clinically unsuspected. This is the second report describing the association of membranoproliferative glomerulonephritis type III and primary pulmonary hypertension in an HIV-infected patient and the first to consider cirrhosis as a possible additional element of the syndrome.
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PMID:Pulmonary hypertension and HIV infection: new observations and review of the syndrome. 799 28

We report on eight children with severe diarrhea beginning in the first 6 months of life (< 1 month in six cases), who had a number of features in common. All were small for gestational age and had an abnormal phenotype, including facial dysmorphism, hypertelorism, and woolly, easily removable hair with trichorhexis nodosa. Two were products of consanguineous marriages. Severe secretory diarrhea persisted despite bowel rest (n = 7). Jejunal biopsy specimens showed total or subtotal villous atrophy with crypt necrosis, and inconstant T-cell activation in some cases (n = 3). Colon biopsy specimens showed moderate nonspecific colitis. All the patients had defective antibody responses despite normal serum immunoglobulin levels, and defective antigen-specific skin tests despite positive proliferative responses in vitro. Three had monoclonal hyper-immunoglobulinemia A. The course was marked by diffuse erythroderma in two cases and mental retardation in three. Treatment included bowel rest, intravenous administration of immune globulins, administration of corticosteroids (n = 6) and cyclosporine (n = 2), and bone marrow transplantation (n = 1). Five patients died between the ages of 2 and 5 years (of sepsis or cirrhosis), two are being fed enterally, and one continues to receive total parenteral nutrition. The cause of the combined low birth weight, dysmorphism, severe diarrhea, trichorrhexis, and immunodeficiency is unclear. These features may constitute a specific syndrome within the group of intractable diarrheas of infancy.
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PMID:Intractable infant diarrhea associated with phenotypic abnormalities and immunodeficiency. 802 82

This study examines the clinical and pathologic course of seven patients who developed giant cell hepatitis (GCH) after liver transplantation. Five of these patients also had GCH as their native liver disease and experienced a particularly aggressive course because of recurrent disease, beginning 1-21 months after transplantation. Two died and another two required hepatic retransplantation because of recurrent GCH; one of them had GCH recurrence in a second liver allograft. A remaining patient with recurrent GCH is alive 6 years after transplantation. Follow-up of the two patients who developed de novo GCH between 8 and 24 months after hepatic transplantation showed active micronodular cirrhosis in one and persistent giant cell transformation in the other at 4 years. All of the patients were serologically negative for hepatitis C virus, hepatitis B virus, and human immunodeficiency virus before transplantation. One patient became positive for hepatitis C virus after transplantation. Two patients had an associated autoimmune syndrome, which could have been accounted for by the GCH. None had a history of drug exposure. Interestingly, human papilloma virus (HPV) type 6 was detected by PCR analysis of liver tissues with GCH from one of three cases before and three of four cases after transplantation. This small series shows that GCH occurs in liver allografts, but it is uncommon. Documentation of recurrent disease in five of seven patients suggests that GCH in a subgroup of patients may be related to a transmissible agent, or that a particular recipient may injure livers in a way that elicits a giant cell reaction.
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PMID:Recurrent and de novo giant cell hepatitis after orthotopic liver transplantation. 803 95

We report the case of a patient suffering from acquired immunodeficiency syndrome and hepatitis B and D virus-related cirrhosis of the liver who was diagnosed as subclinical Crohn's disease. We attribute this clinical course to abnormality of intestinal immune system induced by the human immunodeficiency virus. Concomitant hepatitis B and D virus infection may have contributed. This observation supports the hypothesis of helper-inducer T cells (CD4 T cells) having a critical role in the immunopathogenesis of Crohn's disease and its clinical expression.
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PMID:[Subclinical Crohn disease in acquired immunodeficiency syndrome]. 812 96

Herpes simplex virus (HSV) infections causing severe disease are reported frequently in patients suffering from human immunodeficiency virus (HIV) infection. This disease pattern may also be seen in an immunocompromised disease state with other causes, however, as in the case presented in this paper. An 84-year-old woman had hepatic cirrhosis resulting from chronic hepatitis C virus infection. The woman developed ulcerative lesions in and around her mouth and in the genito-anal region, and these persisted for some months. Diagnosis of HSV infection was not obtained until after extensive laboratory investigations. Aciclovir infusion therapy started immediately afterwards led to dramatic improvement of the skin and mucous membrane changes. Complete clearing of lesions was not obtained, however, because the patient died as a result of the immunosuppression.
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PMID:[Chronic mucocutaneous herpes simplex infection. Occurrence within the scope of liver-induced immunodeficiency]. 822 77

Although a normal or increased anion gap (AG) is commonly used to help assess acid-base balance, decreased AG has aided in the diagnosis of halogen ingestion and myeloma. Substantially increased levels of IgG cause a decrease in the AG. Patients with polyclonal increases in immunoglobulins, especially hepatic cirrhosis, also exhibit decreased anion gaps. Patients with human immunodeficiency virus (HIV) infection commonly show polyclonal increases in immunoglobulins. A case is reported of a patient with HIV infection who exhibited a decreased AG associated with increased polyclonal IgG (63 g per L). Unlike the electrophoretic profile of patients with hepatic cirrhosis, which commonly shows a beta-gamma-globulin bridge, reflecting a decreased immunoglobulin degradation, the profile of the patient with HIV infection was consistent with an increased immunoglobulin synthesis. Examination of sera from 18 additional HIV positive patients indicated that, in general, the AG of HIV infected patients with normal renal function is significantly higher than in normal persons. The significance of this finding is as yet unclear. Nevertheless, decreased AG was associated with increased IgG. This may complicate the use of the AG in evaluating HIV infected patients because of frequent elevations in IgG. These relationships are now in the process of further investigation. Nevertheless, it is suggested that, with appropriate history and physical, identification of a decreased anion gap in conjunction with a polyclonal increase in gamma-globulin may be reason to consider a work up for infection by HIV.
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PMID:Human immunodeficiency virus infection and anion gap. 837 29

A pilot study of chronic hepatitis C treatment was conducted in 14 patients (13 had chronic active hepatitis and 1 had liver cirrhosis). All patients were asymptomatic for the human immunodeficiency virus (HIV) type 1 (mean CD4 count of 584 +/- 283 cells/mm3). Patients received 9 MU rIFN-alpha 2A per day for three months. After this, patients received 9 MU three times weekly for three months, 6 MU for another three months on the same protocol, and finally 3 MU again three times weekly for the last three months. After the first month of ALT treatment in 9 patients (64%) returned to normal; a significant decrease in ALT levels was observed with respect to the pretreatment values (mean of 42 IU/l, range 15-75 vs 152 IU/l, range 69-355; P < 0.01). Of the 9 patients who completed the treatment period, 5 had a complete response, and 4 of these 5 continued with normal ALT values during follow-up (sustained response) while the other patient relapsed within one month after cessation of therapy. The remaining 4 patients were non-responders (including one case with a break-through of the response). HCV-RNA was not detectable in 3 of the 5 responders at the end of therapy while during follow-up viral RNA became undetectable in the other 2 patients. 2/4 non-responder patients had detectable HCV-RNA during follow-up. Liver histology improved in all the patients. No changes were observed in the immunological status or HIV infection.
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PMID:Treatment with recombinant alpha-interferon of chronic hepatitis C in anti-HIV-positive patients. 839 52

In this work the results of the clinico-immunological evaluation of the therapeutic effectiveness of alpha-interferon preparations are presented and criteria suitable for use in screening patients with chronic virus hepatitis, sensitive to interferon therapy, are discussed. The study revealed that the use of alpha-interferon preparations in single doses of 1-3 x 10(6) in a prolonged course of treatment (6-12 months) facilitated essential improvement in the clinical course of the disease and ensures correction of the immune status in 55.6% of patients with chronic active hepatitis (CAH) resulting in cirrhosis of the liver and 57.9% of patients with CAH moderate activity. Indications for the use of alpha-interferon preparations in patients with CAH-induced cirrhosis were high activity of the cytolytic process, the presence of immunodeficiency, faintly pronounced autoimmune process and the presence of protein shifts.
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PMID:[The problem of the interferon therapy of patients with chronic viral hepatitis]. 852 15

The pharmacokinetics of zidovudine (ZDV) are established in patients with various stages of human immunodeficiency virus (HIV) disease. This study was conducted to determine the pharmacokinetic parameters of ZDV in patients with asymptomatic HIV infection and liver disease. HIV-infected volunteers with normal renal function were stratified according to the severity of liver disease (seven of eight were classified as mild). Each subject received a single intravenous dose of ZDV (120 mg) on the first day, followed by a single oral dose of ZDV (200 mg) on the second day. Blood samples were obtained over a 8-h collection interval, and concentrations of ZDV and its glucuronidated metabolite (GZDV) were determined by high-performance liquid chromatography. The following pharmacokinetic parameters were obtained after oral administration of ZDV to HIV-infected patients with mild hepatic disease; these values were compared with previously reported data in healthy volunteers. The area under the curve (AUC) (1,670 +/- 192 ng.h/ml), maximum concentration of drug in serum (1,751 +/- 180 ng/ml), and half-life (2.04 +/- 0.38 h) of ZDV were increased, while the apparent oral clearance (1.57 +/- 0.31 liter/h/kg of body weight) was decreased; AUC (7,685 +/- 1,222 ng.h/ml) and maximum concentration of drug in serum (5,220 +/- 1,350 ng/ml) of GZDV and the AUC ratio of GZDV to ZDV (2.79 +/- 0.43) after oral administration were decreased. ZDV absolute bioavailability was 0.75 +/- 0.15 in HIV-infected patients with hepatic disease. Although the ZDV apparent oral clearance was not impaired as significantly as in patients with biopsy-proven cirrhosis, our results suggest that ZDV, could accumulate in HIV-infected patients with mild hepatic disease because of impaired formation of GZDV. Patients with mild hepatic disease may require dosage adjustment to avoid accumulation of ZDV after extended therapy.
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PMID:Pharmacokinetics and bioavailability of zidovudine and its glucuronidated metabolite in patients with human immunodeficiency virus infection and hepatic disease (AIDS Clinical Trials Group protocol 062). 859 10


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