Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Indian childhood cirrhosis is a significant cause of morbidity and mortality in young children in India. One hundred patients with ICC, 66 boys and 34 girls, were studied. Pedigree analysis yielded a segregation ratio of 0-2196, suggestive of an autosomal recessive inheritance. Serum alpha1-antitrypsin level was normal. Serum alpha-foetoprotein (AFP) concentration was increased in all the patients, parents and in some siblings. Serum immunoglobulins G, A, M, and D were elevated. Haemolytic complement and C3 were low. Electrophoretically altered complement components were detected in 36% of patients. There was an inverse relationship between C3 concentration and immunoconglutinin titre. Circulatingimmune complexes were detected in the sera of six out of ten patients who had significant proteinuria. Hepatitis B surface antigen (HBsAg) was present in the serum, ascitic fluid, saliva, urine and faeces of ICC patients more frequently than in controls. HBsAb was detected less often. Lymphocyte response to HBsAg was impaired. The first-degree relatives had a higher incidence of HBsAg and HBsAb than healthy controls. It is suggested that ICC occurs in infants with an inherited hepatocyte vulnerability and that one of the precipitating causes of liver cell necrosis is infection with hepatitis virus(es). The consequent immunologic epiphenomena contribute to progressive hepatic damage ending in death.
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PMID:Indian childhood cirrhosis: genealogic data, alpha-foetoprotein, hepatitis antigen and circulating immune complexes. 6 6

A total of 163 Japanese children (90 boys and 73 girls, ranging in age from 3 to 15) with proteinuria and/or hematuria were studied for renal histopathology using biopsy materials by light microscopy, electron microscopy, and immunofluolescence method. Eleven patients were diagnosed as membranous nephropathy (MN) while the other 152 patients had various renal diseases other than MN. All patients with MN did not have any known predisposing of associated caused. Hepatitis B virus surface antigen (HBsAg) in the serum, as determined by a reversed passive hemagglutination method (RPHA), was positive in 100% of the patients with MN, while it was positive in 4.6% of the patients with other renal diseases. The difference was statistically significant. Of the 11 mothers of the children with MN, six were positive for HBsAg, and one of the remaining 2 was positive for antibody to HGsAg (anti-HBs) and another was not examined. These findings suggest that MN among Japanese children are mainly, if not exclusively, caused by hepatitis B virus (HBV) and that the virus is transmitted from the mother to child in most instances. In each case of HBsAg-associated glomerulonephritis reported, HBsAg was detected, by immunofluorescence, in the glomeruli, with a distribution similar to that of immunoglobulins. However deposits of HBsAg could not be demonstrated in the glomeruli of the 9 patients with MN studied. Pathogenic immune complexes in the glomerular lesions with subepithelial deposits have been shown to weigh less than 1 million daltons. Since the intact 20-nm HBsAg has a molecular weight of more than 2.4 million daltons, probably most immune complexes containing it would be very large and rapidly cleared by the reticuloendothelial system. Therefore, this failure to detect glomerular staining with anti-HBs antiserum may mean that MN is caused by some other antigen, of a lower molecular weight, associated with HBV, but not necessarily by HB surface antigen.
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PMID:[Etiology of membranous nephropathy in children: Association between membranous nephropathy and hepatitis B virus infection (author's transl)]. 54 9

The long-term clinical course of patients with primary Type II essential mixed cryoglobulinaemia is unclear as many reports fail to separate this group from patients with Type III disease. We have reviewed 13 patients with Type II essential mixed cryoglobulinaemia who presented to the Hammersmith Hospital between 1976 and 1990. All patients had a cryoglobulin level greater than 0.1 mg/ml (range 0.27-6.50 mg/ml), and characterization of the cryoglobulin in all cases revealed the presence of a monoclonal IgM kappa component with rheumatoid factor activity together with polyclonal IgG. All patients had evidence of activation of the classical pathway of complement with greatly reduced levels of C4, while C3 levels were moderately reduced in three patients. All patients had skin disease and joint symptoms were reported by nine patients, with erosive arthritis in one. Eight patients had peripheral sensorimotor neuropathy. Renal disease was observed in 10 patients, manifesting as raised creatinine level, proteinuria or haematuria. Renal tissue was examined in eight patients: in six the appearances were those of a mesangiocapillary glomerulonephritis Type I while in the other two patients there was a mesangioproliferative glomerulonephritis, in one diffuse and in the other focal and segmental. Glomerular capillary 'hyaline thrombi' were found in six biopsies, extracapillary proliferation was found in three and evidence of vasculitis was found in all eight. Liver biopsy showed macronodular cirrhosis in one patient, while a second with recurrent episodes of jaundice showed only chronic inflammatory changes. No patient was positive for hepatitis B surface antigen; however one patient had low titre anti-hepatitis B surface antibody. Normochromic normocytic anaemia was present in nine patients. Bone marrow examination was carried out in 13 patients at presentation to our unit: 10 showed no evidence of a lymphoproliferative disorder, while three suggested the presence of a non-Hodgkin's lymphoma (some years after original presentation in all three). Unusual clinical features included one patient with retinal vasculitis and one patient with severe pulmonary haemorrhage.
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PMID:Type II essential mixed cryoglobulinaemia: presentation, treatment and outcome in 13 patients. 162 Aug 12

To evaluate the clinical features, immunopathology and the prognosis of hepatitis B virus-associated membranous nephropathy (HBVMN), 34 patients (25 boys and 9 girls) from April 1981 to November 1986 were studied. With Fab fragments of monoclonal antibodies, hepatitis B e antigen (HBeAg) was detected in the glomerular deposits from 30 cases (88.2%) and in the sera from 32 cases (94.1%). These results suggest that HBe Ag plays an important role in the development of HBVMN. In patients without corticosteroid treatment, HBV DNA was found as only episomal molecules with 3.2 kb in macrophage, T and B cells. The HBV cellular DNA disappeared within 12 months. In a HBVMN patient with corticosteroid treatment, even three years later, cellular HBV DNA was still detectable in T cells. They also had occasional proteinuria. From the in vitro study, we also demonstrated that corticosteroid stimulated endogenous HBsAg and HBeAg production from patient's mononuclear cells. Therefore, the use of corticosteroid could lead to a potential risk of enhancing viral replication. In addition, clinical trials of 32 cases demonstrate a relatively poor response to the steroid therapy with persistent heavy proteinuria (32.4%) or a high frequent relapse rate (38.2%); only one case (3.1%) had early response. Four cases received follow-up renal biopsy, progressive sclerosis with interstitial fibrosis being noted in each instance. The stage of membranous nephropathy in light microscope had progressed from stage I or II into III. One had impaired renal function. Therefore, HBVMN does not always take a benign course. Usage of corticosteroid in HBVMN patients should be avoided.
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PMID:Clinical features and natural course of HBV-related glomerulopathy in children. 177 Jul 10

The clinical and pathologic features of progressive renal disease in 4 patients with Down's syndrome are described. All patients were male, between 20 and 30 years of age at the time of clinical presentation. Three out of 4 had proteinuria, and 2 had hematuria. Serologic tests for hepatitis B virus infection and antinuclear antibodies performed in 2 patients were negative. Examination of renal tissue from biopsy and/or from autopsy revealed mesangiocapillary glomerulonephritis (MCGN), type 1. While an increased incidence of congenital heart disease and acute leukemias has been documented in Down's syndrome, an association with MCGN has not been reported previously to our knowledge. This probably represents a form of idiopathic MCGN and may be related to the long survival of these individuals.
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PMID:Mesangiocapillary glomerulonephritis in Down's syndrome. 183 96

The complement fragment Ba is a 33 kD activation product of factor B which suppresses human B-lymphocyte functions in vitro. We report that plasma levels of Ba are highly elevated in patients with chronic renal failure (4.84 +/- 3.58 micrograms/ml) and in patients with end-stage renal disease undergoing regular hemodialysis (16.1 +/- 6.1 micrograms/ml) as compared to normals (1.01 +/- 0.30 micrograms/ml). Ba levels were strictly correlated with the creatinine clearance. The urinary excretion of Ba was 165-fold higher in patients with tubular proteinuria than in normals. These results indicate that the kidney is the major catabolic site for Ba. In addition, direct evidence was obtained for an enhanced turnover of the alternative pathway of complement in renal failure that, although it appears to be less important than the renal retention of Ba, contributes to elevated Ba plasma levels in these patients. Ba concentrations in dialysis patients who responded to hepatitis B vaccination were significantly lower than in non-responders. Furthermore, the in vitro IgM synthesis by purified mononuclear cells was negatively correlated with Ba concentrations determined in the plasma of these patients. These results suggest that the accumulation of Ba contributes to the defective immune response in patients with renal failure.
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PMID:Elevated plasma levels of the immunosuppressive complement fragment Ba in renal failure. 183 62

Previously we found that corticosteroid treatment in the hepatitis B virus (HBV)-associated membranous nephropathy (HBVMN) was not associated with a favorable outcome. To distinguish the differences of the HBV DNA in macrophage, T and B cells among HBVMN patients with or without corticosteroid treatment, serial studies at different time points were investigated. HBV DNA appeared as an "episomal" molecule as with 3.2 kb in macrophage, T and B cells. This molecule disappeared after 12 months among HBVMN patients without corticosteroid treatment. HBV DNA, by contrast, appeared as episomal form even three years later in T cells, with frequent proteinuria among HBVMN patients with corticosteroid treatment. This finding indicates that the use of corticosteroids leads to a potential risk of enhancing HBV viral replication in T cells. We studied 24 HBVMN patients who had previously received corticosteroid treatment and had persistent proteinuria, who were administered combination therapy with adenine arabinoside for two weeks and thymic extract (Thymostimulin) for six months to decrease urine protein loss and obtain seroconversion. These 24 patients had heavy (22 of 24, 91.6%) or mild (2 of 24, 8.4%) proteinuria prior to adenine arabinoside and thymostimulin treatment. All 24 patients demonstrated HBV DNA in mononuclear cells and simultaneously exhibited sera positive with HBsAg and HBeAg. In contrast, after treatment only one case (4.2%) had heavy and two cases (8.4%) mild proteinuria; HBV DNA was demonstrated in macrophage (4 of 24, 16.7%), T cells (9 of 24, 37.5%), and B cells (6 of 24, 25%) as well as serum (24 of 24, 100%) prior to treatment.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Treatment of hepatitis B virus-associated membranous nephropathy with adenine arabinoside and thymic extract. 200 43

Hepatitis B virus carriers, a 30-year-old man (case 1) and a 31-year-old man (case 2), associated with nephrotic syndrome were treated with interferon-beta. The nephrotic syndrome did not respond to corticosteroid therapy. Their HBs-Ag, HBe-Ag and HBc-Ab were positive. Renal biopsies revealed membranous glomerulonephritis in case 1 and mixed membranous and proliferative glomerulonephritis in case 2. Direct immunofluorescence studies showed strong granular staining of the GBM with IgG and using sandwich technique with anti-HBe antiserum, granular deposits were seen throughout the GBM. Patients were administrated mainly 3-6 x 10(6) IU/day interferon-beta intravenously for four weeks. After transitory elevation of serum transaminase, HBe-Ag and DNA-polymerase have disappeared with development of HBe-Ab (seroconversion) about six months after the end of interferon-beta administration. Then nephrotic syndrome has recovered in incomplete remission after a year and a half follow-up. The secondary renal biopsy in case 1 showed less intense deposits of HBe-Ag along GBM. These facts suggest that the improvement of proteinuria is associated with the decrease in HBV replication due to interferon therapy.
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PMID:[Clinical and histological observation of HBV glomerulonephritis treated with interferon-beta]. 208 50

To evaluate the clinical features, immunological profiles and the prognosis of hepatitis B virus-associated membraneous nephropathy (HBVMN), 34 patients (25 boys and 9 girls) were studied from April 1981 to November 1987. With Fab fragments of monoclonal antibodies, hepatitis B e antigen (HBeAg) was detected in the glomerular deposits from 30 cases (88.2%) and in the sera from 32 cases (94.1%). These results suggest that HBeAg plays an important role in the development of HBVMN. In addition, clinical trials of 32 cases demonstrate a relatively poor response to the steroid therapy with persistent heavy proteinuria (32.4%) or a high frequent relapse rate (38.2%); only 1 case (3.1%) had early response. In 4 cases follow-up renal biopsy was performed, progressive sclerosis with interstitial fibrosis being noted in each instance. The stage of membraneous nephropathy examined under the light microscope, had progressed from stage I or II to stage III. One had impaired renal function. Therefore, HBVMN does not always take a benign course. In the immunological profiles, significant hypocomplementemia with low C3, C4 and properdin factor B levels were found during the initial 6 months after the onset of disease. Hepatitis B surface antigen (HBsAg) circulating immune complexes (CIC) were also significantly higher. However, the levels of HBsAg CIC did not correlate with the degree of proteinuria or hematuria. In patients with persistent HBaAg carriage, serum HBeAg status alone did not correlate with remission rate, and remission occurred usually before the HBeAg seroconversion to anti-HBe. These results suggest that factors other than HBeAg play important roles in HBVMN.
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PMID:Hepatitis B virus-associated membraneous nephropathy: clinical features, immunological profiles and outcome. 219 Dec 32

The therapeutic benefits and risks of short-term corticosteroid were investigated in 8 patients with membranous nephropathy and hepatitis B surface antigenaemia. Seven patients presented with nephrotic syndrome, and the remaining patient had significant proteinuria. Their liver function tests were normal on repeated examination. Their sera demonstrated the persistent presence of hepatitis B virus surface antigen and high titres of antibody to hepatitis B virus core antigen. Hepatitis B virus e antigens were present in the sera of 4 patients at initial presentation. Their clinical responses were compared with 7 similar patients previously treated with diuretic therapy alone and acting as historic controls. Short-term corticosteroid (6 months) with stepwise reduction resulted in an early regression of the nephrotic syndrome in 3 patients. Five patients had persistent but reduced proteinuria. Transient liver impairment was observed in 3 patients. Corticosteroid therapy induced transient viral replication with increased serum concentration of hepatitis B virus e antigen and hepatitis B virus DNA. Two of the 7 patients receiving diuretics developed spontaneous remission though apparently later than those receiving corticosteroid. Yet complications such as liver dysfunction and hypertension were not observed in the patients treated with diuretics. Our findings suggest that corticosteroid therapy could be harmful in membranous nephropathy related to hepatitis B surface antigenaemia, as activation of viral replication could occur with corticosteroid therapy.
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PMID:The therapeutic dilemma of the usage of corticosteroid in patients with membranous nephropathy and persistent hepatitis B virus surface antigenaemia. 224 84


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