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)

Secretory component was assayed in serum and bile from 34 patients within 40 days after a first or a second (three cases) liver transplantation. Levels of serum secretory IgA and IgM and of a serum component referred to as immunoreactive free secretory component, identified by its reactivity with monoclonal and polyclonal antibodies specific to secretory component, were significantly elevated in all posttransplant patients compared with 45 healthy subjects and 10 kidney transplant patients (p less than 0.0001). The highest serum levels of bound secretory component and of immunoreactive free secretory component were observed in patients with acute rejection. The elevation of immunoreactive free secretory component was significantly higher in patients with rejection as compared with patients with a graft ischemia (p = 0.002) or an uncomplicated postoperative evolution (p = 0.01). The highest levels of immunoreactive free secretory component and secretory IgM were observed in a transplant patient with selective IgA deficiency. No significant difference was seen between the levels of serum immunoreactive free secretory component observed in patients with rejection and those of patients with cytomegalovirus hepatitis or sepsis. Immunoreactive free secretory component, secretory IgA and secretory IgM levels measured in the serum of three patients with primary nonfunction were lower than those observed in the other groups. Immunoreactive free secretory component bile/serum ratios calculated from 16 patients were significantly higher in patients with acute rejection than in infected patients. This study provides new insight into the mechanisms of increase of serum immunoreactive free secretory component, secretory IgA and secretory IgM in various types of liver dysfunction.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Serum and bile secretory immunoglobulins and secretory component during the early postoperative course after liver transplantation. 195 52

Intravenous immunoglobulin has been used for hypogammaglobulinaemic conditions treated at the Royal Children's Hospital, Melbourne since 1972. Fifty-four children have been treated. Nineteen have been males with congenital hypogammaglobulinaemia (including 12 with sex-linked agammaglobulinaemia and 5 with hypogammaglobulinaemia with IgM); 8 have had common variable immunodeficiency, and 11 have had severe combined immune deficiency. Intravenous immunoglobulin has also been used for some patients with transient hypogammaglobulinaemia, isolated IgG deficiency, isolated IgA deficiency and isolated IgM deficiency. Infusions are given four weekly at a dose of 5-7.5 ml/kg of a 6% preparation (300-450 mg/kg). At diagnosis, a loading dose is given of 10-15 ml/kg (600-900 mg/kg). Previous studies have demonstrated a half-life of 25 days. The median preinfusion IgG concentration for the 22 children receiving monthly infusions currently is 68 IU/ml. Hospitalisation rates for infective illness have been reduced with the use of intravenous gammaglobulin. No patients are known to have developed hepatitis. Reactions to infusions are experienced by 60% of patients. These have not been reduced significantly by the addition of 10% maltose, but have been lessened considerably by using intravenous methylprednisolone (1 mg/kg) before the commencement of infusion.
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PMID:The long term treatment of childhood hypogammaglobulinaemia in Melbourne with intravenous gammaglobulin, 1972-1985. 244 Jul 42

Hepatitis occurred in five out of 12 patients with selective IgA deficiency who had been detected by immunelectrophoretic screening of 2,600 individuals. This apparent increased susceptibility to hepatitis has not been reported before, and it may be due to the defect in their local and humoral immune mechanisms.
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PMID:Hepatitis in selective IgA deficiency. 475 72

The syndrome of nodular lymphoid hyperplasia of the small bowel with hypogammaglobulinemia is one of the hypogammaglobulinemic enteropathies. Chronic diarrhea and malabsorption are the most characteristic features of this disease, and they are frequently associated to hypogammaglobulinemia of various types (acquired, congenital non sex-linked) and to selective IgA deficiency. The immunological deficiency gives rise to the more characteristic features of the disease, namely: a) hypogammaglobulinemia; b) respiratory infections and dental caries; c) Giardia lamblia infestation of the small bowel; d) the characteristic radiological features; and, e) the histological aspect of the intestinal mucosa with absence of plasma cells. Periodical follow-up is needed because of the increased incidence of tumors in immunological deficiency states. A new case of nodular lymphoid hyperplasia associated to hemolytic anemia and granulomatous hepatitis is reported, and its possible pathogenesis is discussed.
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PMID:[Nodular lymphoid hyperplasia of the small bowel with IgA deficiency and hemolytic anemia (author's transl)]. 742 63

Shortly after the discovery of the hepatitis C virus, it was realized that this infectious agent caused more than just liver disease. A remarkable array of extrahepatic manifestations of hepatitis C has now been described. Many of these associated syndromes implicate the hepatitis C virus as a mediator of autoimmunity or of immune complex formation. These disorders include mixed essential cryoglobulinemia, autoimmune hepatitis, glomerulonephritis, thyroiditis, and possibly Sjogren's syndrome. The hepatitis C virus has also been strongly linked to two skin disorders: prophyria cutanea tarda and lichen planus. Other possible hepatitis-C-associated diseases described in the literature include idiopathic pulmonary fibrosis, IgA deficiency. Mooren's corneal ulcers, Behcet's syndrome, polyarthritis, Guillain-Barre' syndrome, idiopathic thrombocytopenic purpura, and others. A number of these reported diseases have either responded to or been cured by a therapeutic course of alpha interferon. This report discusses the reported extrahepatic manifestations of hepatitis C as of mid-1995.
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PMID:Extrahepatic manifestations of hepatitis C. 886 23

The X-linked hyper-IgM (XHIGM) syndrome is an uncommon primary immunodeficiency disease caused by mutations in the gene for CD40 ligand and characterized by normal or elevated serum IgM, reduced levels of IgG and IgA, and defective T-cell function. Because of its rarity, it has been difficult for any single investigator or institution to develop a comprehensive clinical picture of this disorder. Accordingly, a national registry was developed in the United States to provide demographic, genetic, immunologic, and clinical information on a relatively large number of patients with the XHIGM syndrome.A total of 79 patients from 60 unrelated families were registered between January 1997 and July 2002. The estimated minimal incidence was approximately 1/1,030,000 live births. All of the patients had significant IgG deficiency and most had IgA deficiency, but only one-half had elevated IgM levels. Most patients presented initially with a history of an increased susceptibility to infection including Pneumocystis carinii pneumonia. The average age of diagnosis was significantly earlier in patients born into a family with a previously affected individual. However, only one-third of the patients born into a family with a previously affected individual were diagnosed exclusively because of the presence of the positive family history before any clinical symptoms developed. Over half the patients developed symptoms of immunodeficiency and were diagnosed by 1 year of age, and over 90% by 4 years of age. The most prominent clinical infections were pneumonia (81% of patients), upper respiratory infections (49%) including sinusitis (43%) and recurrent otitis (43%), recurrent/protracted diarrhea (34%), central nervous system infections (14%), sepsis (13%), cellulitis (13%), hepatitis (9%), and osteomyelitis (1%). In addition to infections caused by encapsulated bacteria, opportunistic infections were relatively common and were caused by P. carinii, members of the herpes virus family (including cytomegalovirus), Cryptosporidium, Cryptococcus, Candida, Histoplasma, and Bartonella. Sclerosing cholangitis occurred in 5 patients and in 4 of these was associated with Cryptosporidium infection. Eight patients had died at the time of their entry into the Registry; 2 of pneumonia (1 P. carinii and 1 cytomegalovirus), 2 of encephalitis (1 ECHO virus and 1 cytomegalovirus), 2 of malignancy (both hepatocellular carcinoma), 1 of sclerosing cholangitis caused by Cryptosporidium, and 1 of hemolytic uremic syndrome.
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PMID:The X-linked hyper-IgM syndrome: clinical and immunologic features of 79 patients. 1466 87

Gluten enteropathy is a chronic all-life disease, characterized by typical inflammatory changes of the small bowel mucosa. It is the genetically determined autoimmune disease with permanent intolerance to gluten. Its clinical signs are very heterogeneous and also the severity of intestinal changes varies considerably. It frequently presents with atypical symptoms, as many as 80% of adult patients have no gastrointestinal troubles at all. The present estimated prevalence is 1:200 - 1:250. The diagnosis is based on criteria of the European Society of Paediatric Gastroenterology, Hepatology and Nutrition revised in 1990, above all on the detection of serum antibodies to endomysium and tissue transglutaminase. The five forms are distinguished: classic, subclinical, latent, potential and silent. Coeliac disease and dermatitis herpetiformis are considered to be two identical forms of the gluten enteropathy manifestations. Coeliac disease is frequently associated with other autoimmune diseases--diabetes mellitus, autoimmune thyreoiditis, hepatitis, IgA deficiency. Permanent gluten free diet is the cornerstone of the therapy; untreated gluten enteropathy is a serious illness, considered to be a significant precancerosis.
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PMID:[Gluten enteropathy--occurrence, diagnosis, therapy]. 1598 87

Liver disorders with a likely autoimmune pathogenesis in childhood include autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis (ASC), and de novo AIH after liver transplantation. AIH is divided into two subtypes according to seropositivity for smooth muscle and/or antinuclear antibody (SMA/ANA, type 1) or liver kidney microsomal antibody (LKM1, type 2). There is a female predominance in both. LKM1 positive patients tend to present more acutely, at a younger age, and commonly have partial IgA deficiency, while duration of symptoms before diagnosis, clinical signs, family history of autoimmunity, presence of associated autoimmune disorders, response to treatment, and long-term prognosis are similar in both groups. The most common type of paediatric sclerosing cholangitis is ASC. The clinical, biochemical, immunological, and histological presentation of ASC is often indistinguishable from that of AIH type 1. In both, there are high IgG, non-organ specific autoantibodies, and interface hepatitis. Diagnosis is made by cholangiography. Children with ASC respond to immunosuppression satisfactorily and similarly to AIH in respect to remission and relapse rates, times to normalization of biochemical parameters, and decreased inflammatory activity on follow up liver biopsies. However, the cholangiopathy can progress. There may be evolution from AIH to ASC over the years, despite treatment. De novo AIH after liver transplantation affects patients not transplanted for autoimmune disorders and is strikingly reminiscent of classical AIH, including elevated titres of serum antibodies, hypergammaglobulinaemia, and histological findings of interface hepatitis, bridging fibrosis, and collapse. Like classical AIH, it responds to treatment with prednisolone and azathioprine. De novo AIH post liver transplantation may derive from interference by calcineurin inhibitors with the intrathymic physiological mechanisms of T-cell maturation and selection. Whether this condition is a distinct entity or a form of atypical rejection in individuals susceptible to the development of autoimmune phenomena is unclear. Whatever its etiology, the recognition of this potentially life-threatening syndrome is important since its management differs from that of standard anti-rejection therapy.
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PMID:Autoimmune paediatric liver disease. 1852 33

Autoimmune hepatitis (AIH) is characterized by inflammatory liver histology, circulating non-organ-specific autoantibodies, and increased levels of immunoglobulin (Ig) G in the absence of a known etiology. Two types of childhood AIH are recognized according to seropositivity: smooth muscle antibody (SMA) and/or antinuclear antibody (ANA), which is AIH type 1; and antibodies to liver-kidney microsome type 1 (anti-LKM1), which is AIH type 2. There is a female predominance in both. Autoimmune hepatitis type 2 presents more acutely, at a younger age, and commonly with IgA deficiency; however, duration of symptoms before diagnosis, clinical signs, family history of autoimmunity, presence of associated autoimmune disorders, response to treatment, and long-term prognosis are similar in the two groups. Immunosuppressive treatment with steroids and azathioprine, which should be instituted promptly to avoid progression to cirrhosis, induces remission in 80% of cases. Relapses are common, often due to nonadherence. Drugs effective in refractory cases include cyclosporine and mycophenolate mofetil. Long-term treatment is usually required, with only some 20% of AIH type 1 patients able to discontinue therapy successfully. In childhood, sclerosing cholangitis with strong autoimmune features, including interface hepatitis and serological features identical to AIH type 1, is as prevalent as AIH, but it affects boys and girls equally. The differential diagnosis relies on cholangiographic studies. In autoimmune sclerosing cholangitis, liver parenchymal damage responds satisfactorily to immunosuppressive treatment, whereas bile duct disease tends to progress.
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PMID:Autoimmune hepatitis in children: what is different from adult AIH? 1967 2

Autoimmune hepatitis (AIH) is a severe hepatopathy characterised by female preponderance, hypertransaminasaemia, elevated levels of immunoglobulin (IgG), presence of serum autoantibodies and, histologically, by interface hepatitis. AIH occurs both in adults and children, being particularly aggressive in the latter. According to the type of serum autoantibodies, AIH can be differentiated in two forms: one positive for smooth muscle antibody (SMA) and/or antinuclear antibody (ANA) (type 1 AIH, AIH-1) and another positive for liver kidney microsomal antibody type 1 (LKM-1) (type 2 AIH, AIH-2). These two forms differ with regard to age at onset (earlier in the case of AIH-2), mode of presentation (fulminant hepatic failure more frequently observed in AIH-2) and association with IgA deficiency (more frequent in AIH-2). AIH responds satisfactorily to immunosuppressive treatment (corticosteroids with or without azathioprine) that should be started as soon as the diagnosis is made. Despite immune suppression, some 40% of patients experience relapse and 9% undergo liver transplantation. Though the exact mechanism leading to loss of immune-tolerance in AIH is still unclear, recent evidence has pointed to a numerical and functional defect of CD4(pos)CD25(pos) regulatory T-cells as a factor permitting autoaggressive CD4 and CD8 T-cells to react against liver autoantigens. The generation and expansion of regulatory T-cells with liver autoantigen specificity in vitro represents a potential immunotherapeutic tool for the reconstitution of immune-tolerance in AIH without the drawback of pan-immunosuppression.
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PMID:Autoimmune hepatitis. 2540 36


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