1/19. Primary sjogren's syndrome in children and adolescents: proposal for diagnostic criteria.OBJECTIVE: Primary sjogren's syndrome (pSS) in childhood is a rare disease. Diagnostic criteria are available for adult patients only. In order to establish diagnostic criteria for juvenile pSS an analysis of 7 girls and one boy suffering from pSS with early onset is reported. Due to the rarity of the disease, data on patients with pSS reported in the literature are included in the proposal for modified diagnostic criteria. methods: The diagnosis of pSS was established according to the criteria for adulthood pSS, duly modified, which include clinical symptoms and laboratory immunological evaluation. RESULTS: The average age of our patients at clinical onset was 13.5 years (range: 10-17 yrs.). Clinical signs included systemic (fever, fatigue) as well as local (parotitis, vulvovaginitis, conjunctivitis) symptoms. paralysis due to hypokalemia linked to renal tubular acidosis and central nervous system (CNS) involvement was seen in one patient. Asymptomatic renal tubular acidosis was diagnosed in another 2 patients. Autoimmune hepatitis was present in 2 patients. All patients had laboratory abnormalities: hyperimmunoglobulinemia IgG, high titers of antinuclear antibodies (anti-SS-A and/or anti-SS-B) and elevated serum amylases. Sicca syndrome was never seen during childhood, although it developed later in 3 patients, after 7 to 10 years of follow-up. CONCLUSIONS: It has been stressed that the classical diagnostic criteria for adult sjogren's syndrome, especially sicca syndrome, are not applicable to a pediatric onset of the disease. On the other hand, the presence of typical laboratory abnormalities can allow the diagnosis of these patients in the early stages. Both laboratory and clinical symptoms typical for childhood are included in our proposal for diagnostic criteria applicable to juvenile pSS. life-threatening conditions such as hypokalemic paralysis, CNS involvement and hepatitis may also occur in children. Sicca syndrome tends to develop much later in pediatric patients.- - - - - - - - - - ranking = 1keywords = hepatitis (Clic here for more details about this article) |
2/19. Biclonal gammopathy and platelet antibodies in a patient with chronic hepatitis c virus infection and mixed cryoglobulinemia.The presence of biclonal gammopathy [immunoglobulin (Ig)Mk IgAlambda] and platelet antibodies in a patient with chronic hepatitis c virus (HCV) infection is reported. A type-II cryoglobulinemia (IgMk and polyclonal IgG) has also been detected. The IgAlambda monoclonal component still persists after removal of the cryoprecipitate. Two populations of atypical lymphoid cells with plasmocytoid features in bone marrow have been detected. They express IgM with k restriction and IgA with lambda restriction, respectively. These cells are CD19 , CD5 , bc12 , and Ki67-. Liver biopsy shows lymphoid infiltrates with features of B follicles. thrombocytopenia with platelet antibodies but without splenomegaly is also present. This case is interesting because two hematologic extrahepatic manifestations simultaneously occur in a patient with HCV infection. Moreover the association between HCV and biclonal gammopathies has never been reported.- - - - - - - - - - ranking = 2.5keywords = hepatitis (Clic here for more details about this article) |
3/19. Autoimmune hepatitis concomitant with hypergammaglobulinemic purpura, immune thrombocytopenia, and sjogren's syndrome.sjogren's syndrome occurs as an occasional complication of autoimmune hepatitis, and purpura or thrombocytopenia develops in some patients with this syndrome. This report describes a 62-year-old woman with a 6-year history of autoimmune hepatitis who concurrently had hypergammaglobulinemic purpura, immune thrombocytopenia and sjogren's syndrome. Treatment with prednisolone resulted in marked improvement of biochemical, hematological and dermatological abnormalities. This case emphasizes the manifestation of purpura or thrombocytopenia as an associated disorder during the course of autoimmune hepatitis concomitant with sjogren's syndrome.- - - - - - - - - - ranking = 3.5keywords = hepatitis (Clic here for more details about this article) |
4/19. A case of posthepatitic HBs-positive chronic hepatitis with transient monoclonal gammopathy.Report of a case of HBsAg-positive chronic aggressive hepatitis associated with a transient monoclonal gammopathy.- - - - - - - - - - ranking = 2.5keywords = hepatitis (Clic here for more details about this article) |
5/19. Transverse myelitis associated with chronic hepatitis c.An infectious etiology is one of the postulated mechanisms for autoimmune diseases. An emergence of autoimmune phenomena associated with hepatitis c virus (HCV) infection has been reported. Transverse myelitis is an inflammatory disease of the spinal vasculature attributed to viral infections and to autoimmune diseases including systemic lupus erythematosus and the antiphospholipid syndrome. A 34-year-old male was admitted for the rapid onset of numbness of the lower extremities and urinary retention. His past history included hepatitis c infection. The neurological examination and MRI of the thoracic spine confirmed the diagnosis of transverse myelitis. Abnormal laboratory results were hyperglobulinemia, abnormal liver function tests, and positive ANF, anti-dsDNA antibodies, and p and c-ANCA. The patient was treated with high dose prednisone, then tapered to a low dose, but regained only partial neurological function after 15 months of prednisone therapy. Persistent neurological deficits and elevated ANF and ANCA were present at a follow-up of 30 months. We describe the first reported case of a patient with chronic HCV who developed transverse myelitis.- - - - - - - - - - ranking = 3keywords = hepatitis (Clic here for more details about this article) |
6/19. Elevated interleukin-6 and gamma-globulin during interferon therapy of hepatitis B.A 49-yr-old man with chronic hepatitis b manifested hypergammaglobulinemia, lymphadenopathy, and a high serum interleukin-6 level following treatment with recombinant human alpha-interferon. One month later, when the patient was treated with natural beta-interferon, serum levels of interleukin-6 and gamma-globulin increased again. The serum gamma-globulin decreased to the pretreatment level after discontinuation of interferon therapy. The serum alanine aminotransferase level remained normal for 6 months. In this case, hypergammaglobulinemia and lymphadenopathy, as well as the elevated serum interleukin-6 level, were considered to be signs of highly enhanced humoral immunity related to alpha- and beta-interferon therapy.- - - - - - - - - - ranking = 2.5keywords = hepatitis (Clic here for more details about this article) |
7/19. Nonmyeloablative hematopoietic stem cell transplant for X-linked hyper-immunoglobulin m syndrome with cholangiopathy.OBJECTIVE: X-linked hyper-immunoglobulin m (X-HIM) syndrome is a rare genetic immunodeficiency syndrome caused by mutations in the gene encoding cd40 ligand (CD40L, CD154). Allogeneic hematopoietic stem cell transplantation (HSCT) offers the prospect of immune reconstitution in X-HIM syndrome. Standard HSCT using high-dose chemoradiotherapy can be followed by serious hepatic problems, including veno-occlusive disease, graft-versus-host disease, and/or drug-induced hepatotoxicity. In patients whose liver function is compromised before HSCT, such as in X-HIM syndrome caused by cholangiopathy and hepatitis related to opportunistic infections, there is a higher likelihood of hepatotoxicity. We explored nonmyeloablative HSCT in 2 patients with X-HIM syndrome. Nonmyeloablative HSCT without liver transplant for X-HIM syndrome, to our knowledge, has not been described previously. methods: Two children with X-HIM syndrome and persistent infections had documented cholangiopathy on liver biopsy. Both children underwent nonmyeloablative HSCT from HLA-matched siblings with fludarabine, busulfan, and anti-thymocyte globulin as their preparative regimen. Graft-versus-host disease prophylaxis consisted of cyclosporine. RESULTS: Both children are >2 years after their HSCT. One remains a mixed chimera, and the other shows 100% donor chimerism. Both children are now free of infections and are no longer dependent on intravenous gammaglobulin. Both show response to immunizations. Both have had resolution of their cholangiopathy. CONCLUSIONS: Nonmyeloablative HSCT from HLA-matched siblings can offer immune reconstitution without hepatotoxicity in patients with X-HIM syndrome and preexisting cholangiopathy. Even with stable mixed chimerism after allogeneic HSCT, patients may be able to enjoy a normal phenotype. Nonmyeloablative HSCT warrants additional study in X-HIM syndrome.- - - - - - - - - - ranking = 0.5keywords = hepatitis (Clic here for more details about this article) |
8/19. Relapsing hepatitis due to cytomegalovirus?A case of agammaglobulinaemia with hyper IgM (the 'hyper-IgM syndrome') is described, in which a serological diagnosis of cytomegalovirus infection was made during repeated episodes of hepatitis. Three unrelated serological tests agreed with each other, but eventually it appeared that the reactions were non-specific. This shows the limitation of confirmation by tests which are used routinely.- - - - - - - - - - ranking = 2.5keywords = hepatitis (Clic here for more details about this article) |
9/19. A case report of primary hepatic carcinoma with prolonged HB virus infection and mono-clonal gammopathy.A case of alpha-feto protein (AFP) positive hepatic cell carcinoma has been presented in which the appearance of a mono-clonal gammopathy can be explained by persistent infection with HB virus (HBV). The patient, a fifty year old male, developed acute jaundice with hepatitis in July, 1952, and died of an hepatic cell carcinoma in December, 1973. The interest of this case lies in the fact that during the seven months prior to be patient's demise, a mono-clonal gammopathy of IgG (L type), together with positive titeres for HBs Ag (adr) and AFP, was demonstrated.- - - - - - - - - - ranking = 0.5keywords = hepatitis (Clic here for more details about this article) |
10/19. Chronic active hepatitis in childhood with detection of liver-pancreas-specific autoantibodies.This paper describes a special form of chronic active hepatitis (CAH) with hypergammaglobulinemia in a ten-year-old girl. Because of unclear symptoms she was admitted to our hospital for exclusion of leukemia. However, further clinical and laboratory investigations suggested an autoimmune hepatitis although the typical antibodies could not be found in the immunofluorescence test. Only the detection of liver-pancreas-specific, complement-fixing autoantibodies (LP-antibodies) confirmed the diagnosis of an autoimmune hepatitis. histology showed typical infiltrations of plasma cells and lymphocytes in the periportal area. LP-antibodies had been recently described in 20 adults with HBs-Ag-negative CAH[6]. To our knowledge this is the first report on LP-positive hepatitis in childhood. As a therapeutic response is likely, early immunosuppressive treatment is indicated.- - - - - - - - - - ranking = 4keywords = hepatitis (Clic here for more details about this article) |
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