Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Diagnosis, Differential , Empyema, Pleural , Fatal Outcome , Humans , Immunoglobulin M/immunology , Immunoglobulin M/metabolism , Male , Middle Aged , Multiple Myeloma/diagnosis , Multiple Myeloma/drug therapy , Multiple Myeloma/immunology , Multiple Myeloma/pathology , Multiple Myeloma/physiopathology , Pleural Effusion, Malignant/diagnosis , Pleural Effusion, Malignant/drug therapy , Pleural Effusion, Malignant/immunology , Pleural Effusion, Malignant/pathology , Pleural Effusion, Malignant/physiopathology , Pneumonia , Radiography, Thoracic , Radiotherapy , Renal Insufficiency , Steroids/therapeutic useABSTRACT
BACKGROUND/AIMS: According to recent prevalence of hepatitis A virus (HAV) infection, acute liver failure (ALF) due to HAV infection is observed frequently in parallel. The aim of this study was to elucidate the clinical, laboratory, and pathologic features of patients who have undergone emergency liver transplantation (LT) due to fulminant HAV infection. METHODS: Clinical, laboratory, and pathologic data of 11 transplant recipients with anti-HAV IgM-positive ALF between December 2007 and May 2009 were analyzed, and compared with data of 10 recipients who underwent LT for the management of ALF due to other causes. RESULTS: The median age of the patients with HAV-related ALF was 34 years (range: 15-43 years). The levels of hemoglobin, aspartate aminotransferase (AST), alanine aminotransferase (ALT), and creatinine were higher and the level of bilirubin was lower in the HAV-related ALF group than in the other group (P=0.005, 0.001, 0.001, 0.010, and 0.003, respectively). The time from the onset of initial symptoms to the development of encephalopathy was shorter in the HAV-related ALF group than in the other group (median 5 days, range: 4-13 days; P<0.001). In patients with HAV-related ALF, laboratory findings and clinical prognostic parameters including the Acute Liver Failure Study Group prognostic index, King's College criteria, and model for endstage liver disease (MELD) and Child-Pugh scores were not associated with the grade of hepatic encephalopathy or time of progression to encephalopathy. CONCLUSIONS: The results of this study indicate that the clinical condition of patients with HAV-related ALF requiring emergency LT aggravates rapidly. Prognostic parameters are not sufficient for discriminating transplant candidates in patients with fulminant hepatitis A.
Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Alanine Transaminase/blood , Aspartate Aminotransferases/blood , Bilirubin/blood , Creatine/blood , Emergencies , Hemoglobins/analysis , Hepatitis A/complications , Hepatitis A Antibodies/immunology , Immunoglobulin M/metabolism , Liver Failure, Acute/complications , Liver Transplantation , Prognosis , Severity of Illness Index , Time FactorsABSTRACT
PURPOSE: Kawasaki disease is a systemic vasculitis, and its etiology and pathogenesis are still not clear. Our study was undertaken to investigate the characteristics of the activation of B cells in the peripheral blood of Kawasaki disease (KD) patients and evidence of stimulation by superantigens. MATERIALS AND METHODS: Blood samples were obtained from three patients (2 males, 1 female) with KD, who were admitted to our Hospital, Seoul, Korea. The mean age was 1.2 years. Distribution of B cells was studied in the acute and subacute phases of KD patients. From the RNA of B cells, we obtained complementary DNA (cDNA) and performed polymerase chain reaction (PCR). To determine the oligoclonal expansion of immunoglobulin M (IgM) VH family, we cloned and sequenced the PCR products from each group and analyzed DNA. RESULTS: In the peripheral blood of acute phase patients, T cells were significantly decreased (p < 0.05), whereas B cells were significantly increased (p < 0.05). When the first PCR was done on the B cell chains, VH1 to VH6 were all found to be expressed. The number of micro gene clones obtained from 3 patients was 312, and they belonged to VH3, VH4 and VH5 family. M99686 germ line was most frequently used and the next most frequently used, were X92224/J, L21967 and L21964. A similar order was seen in patients. Among the clones, 20 sets of clones showed the same base sequence and this was frequent between VH2 and VH5. There was one set, which showed almost the same base sequence between different patients, and the homology was 99.5%. Twenty sets of clones that had the same base sequence showed high similarity to the germ line (94 - 100%). Among these, the clones that utilized the M99686 germ line were 4 sets which were most frequent. The 3-dimensional structure of one of these clones showed typical beta, sheet structure of immunoglobulin chains. CONCLUSION: The IgM transcripts expressed by the B cells in the peripheral blood of KD patients in the acute phase of the disease clearly showed an oligoclonal expansion, suggesting that KD is caused not by stimulation of a superantigen, but rather by a conventional antigen.
Subject(s)
Female , Humans , Infant , Male , B-Lymphocytes/metabolism , Immunoglobulin M/metabolism , Immunoglobulin Variable Region/genetics , Mucocutaneous Lymph Node Syndrome/genetics , Reverse Transcriptase Polymerase Chain Reaction , Sequence Analysis, DNAABSTRACT
PURPOSE: Behcet's disease (BD) is a disease of unknown etiology, which has multisystemic involvement. This multisystemic involvement might be the clue for an autoimmune pathogenesis. In order to evaluate an autoimmune pathogenesis, we examined immunoreactans depositions in the skin of BD patients. MATERIALS AND METHODS: The skin samples of 108 BD patients (28 perilesional skin, 44 positive pathergy test site, 22 negative pathergy test site, 14 normal skin) were examined for the depositions of immunoglobulin (Ig)M, IgG, IgA, complement 3 (C3), and fibrinogen (F) using direct immunofluorescence (DIF). The data were statistically compared to the DIF of 36 systemic lupus erythematosus (SLE) patients and 20 healthy controls using chi-square Fisher exact test. RESULTS: Highly significant immunoreactans depositions were obtained in BD (deposition rates: IgM 70.3%, IgG 0%, IgA 20.3%, C3 62.9%, F 83.3%). The comparison with SLE revealed no differences in IgM, IgA, and C3. However, IgG deposition was higher in SLE while F deposition was higher in BD. In both BD and SLE, the Ig depositions were highly significant when the data were compared with the healthy controls. CONCLUSION: The significant deposition of immunoreactans in BD, especially in the negative pathergy and the normal skin sites, were observed. This study is the first controlled study revealing positive Ig depositions in BD, and it is expected to help us to reconsider the autoimmune pathogenesis in BD.
Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Behcet Syndrome/metabolism , Case-Control Studies , Fluorescent Antibody Technique, Direct/methods , Immunoglobulin A/metabolism , Immunoglobulin G/metabolism , Immunoglobulin M/metabolism , Skin/metabolismABSTRACT
PURPOSE: Kawasaki disease is a systemic vasculitis, and its etiology and pathogenesis are still not clear. Our study was undertaken to investigate the characteristics of the activation of B cells in the peripheral blood of Kawasaki disease (KD) patients and evidence of stimulation by superantigens. MATERIALS AND METHODS: Blood samples were obtained from three patients (2 males, 1 female) with KD, who were admitted to our Hospital, Seoul, Korea. The mean age was 1.2 years. Distribution of B cells was studied in the acute and subacute phases of KD patients. From the RNA of B cells, we obtained complementary DNA (cDNA) and performed polymerase chain reaction (PCR). To determine the oligoclonal expansion of immunoglobulin M (IgM) VH family, we cloned and sequenced the PCR products from each group and analyzed DNA. RESULTS: In the peripheral blood of acute phase patients, T cells were significantly decreased (p < 0.05), whereas B cells were significantly increased (p < 0.05). When the first PCR was done on the B cell chains, VH1 to VH6 were all found to be expressed. The number of micro gene clones obtained from 3 patients was 312, and they belonged to VH3, VH4 and VH5 family. M99686 germ line was most frequently used and the next most frequently used, were X92224/J, L21967 and L21964. A similar order was seen in patients. Among the clones, 20 sets of clones showed the same base sequence and this was frequent between VH2 and VH5. There was one set, which showed almost the same base sequence between different patients, and the homology was 99.5%. Twenty sets of clones that had the same base sequence showed high similarity to the germ line (94 - 100%). Among these, the clones that utilized the M99686 germ line were 4 sets which were most frequent. The 3-dimensional structure of one of these clones showed typical beta, sheet structure of immunoglobulin chains. CONCLUSION: The IgM transcripts expressed by the B cells in the peripheral blood of KD patients in the acute phase of the disease clearly showed an oligoclonal expansion, suggesting that KD is caused not by stimulation of a superantigen, but rather by a conventional antigen.
Subject(s)
Female , Humans , Infant , Male , B-Lymphocytes/metabolism , Immunoglobulin M/metabolism , Immunoglobulin Variable Region/genetics , Mucocutaneous Lymph Node Syndrome/genetics , Reverse Transcriptase Polymerase Chain Reaction , Sequence Analysis, DNAABSTRACT
PURPOSE: Behcet's disease (BD) is a disease of unknown etiology, which has multisystemic involvement. This multisystemic involvement might be the clue for an autoimmune pathogenesis. In order to evaluate an autoimmune pathogenesis, we examined immunoreactans depositions in the skin of BD patients. MATERIALS AND METHODS: The skin samples of 108 BD patients (28 perilesional skin, 44 positive pathergy test site, 22 negative pathergy test site, 14 normal skin) were examined for the depositions of immunoglobulin (Ig)M, IgG, IgA, complement 3 (C3), and fibrinogen (F) using direct immunofluorescence (DIF). The data were statistically compared to the DIF of 36 systemic lupus erythematosus (SLE) patients and 20 healthy controls using chi-square Fisher exact test. RESULTS: Highly significant immunoreactans depositions were obtained in BD (deposition rates: IgM 70.3%, IgG 0%, IgA 20.3%, C3 62.9%, F 83.3%). The comparison with SLE revealed no differences in IgM, IgA, and C3. However, IgG deposition was higher in SLE while F deposition was higher in BD. In both BD and SLE, the Ig depositions were highly significant when the data were compared with the healthy controls. CONCLUSION: The significant deposition of immunoreactans in BD, especially in the negative pathergy and the normal skin sites, were observed. This study is the first controlled study revealing positive Ig depositions in BD, and it is expected to help us to reconsider the autoimmune pathogenesis in BD.
Subject(s)
Adolescent , Adult , Aged , Female , Humans , Male , Middle Aged , Young Adult , Behcet Syndrome/metabolism , Case-Control Studies , Fluorescent Antibody Technique, Direct/methods , Immunoglobulin A/metabolism , Immunoglobulin G/metabolism , Immunoglobulin M/metabolism , Skin/metabolismABSTRACT
OBJETIVO: Verificar a influência do tipo de parto sobre a concentração das imunoglobulinas (Ig) A, G e M no colostro materno. MÉTODOS: Foram selecionadas 82 puérperas com idade cronológica entre 21 e 41 anos, idade gestacional de 37 ou mais semanas, paridade até IV gesta, bom estado nutricional e sem patologias associadas durante a gestação e o puerpério. Foram também critérios de inclusão para os recém-nascidos: peso > 2.500 g, escore de Apgar > 7 no primeiro minuto e aleitamento materno exclusivo durante o período da internação. As puérperas foram divididas em três grupos: A - parto vaginal; B - cesárea precedida de trabalho de parto; e C - cesárea eletiva. O colostro foi colhido manualmente entre 48 e 72 horas pós-parto. IgA, IgG e IgM foram dosadas pela técnica de ELISA RESULTADOS: Não se observou diferença significativa entre os tempos de coleta do colostro nos três grupos maternos estudados. Quanto menor o tempo de coleta, maior foi a concentração de IgA no colostro materno; quanto menor a paridade, maior foi a concentração de IgA e IgM no colostro materno. O grupo de puérperas submetidas a cesárea precedida de trabalho de parto apresentou concentração mais elevada de IgA no colostro do que o grupo de puérperas que havia dado à luz por parto normal. A concentração de IgM e IgG no colostro materno não foi influenciada pelo tipo de parto. CONCLUSAO: A ocorrência do trabalho de parto, somada ao estresse cirúrgico, induz a uma concentração mais elevada de IgA no colostro materno na puérpera submetida a cesárea precedida de trabalho de parto.
Subject(s)
Humans , Female , Pregnancy , Infant, Newborn , Adult , Middle Aged , Colostrum/chemistry , Delivery, Obstetric , Immunoglobulins/analysis , Immunoglobulins/metabolism , Infant, Newborn/immunology , Birth Weight , Cesarean Section , Colostrum/immunology , Enzyme-Linked Immunosorbent Assay , Gestational Age , Immunoglobulin A/analysis , Immunoglobulin A/metabolism , Immunoglobulin G/analysis , Immunoglobulin G/metabolism , Immunoglobulin M/analysis , Immunoglobulin M/metabolism , Maternal Age , Postpartum Period , Time FactorsABSTRACT
A hipogamaglobulinemia com imunoglobulina M (IgM) normal ou elevada é uma imunodeficiência raramente observada, sendo também conhecida com o nome de síndrome de Hiper IgM. Esta síndrome é diagnosticada em pacientes portadores de níveis séricos de imunoglobulinas (IgG e IgA) diminuídos, com níveis de imunoglobulina M (IgM) normais ou elevados. O objetivo do presente relato é descrever as características clínico-laboratoriais e a evoluçäo de dois pacientes portadores desta síndrome, com idades de 2 anos e 10 meses e 10 anos, acompanhados na Unidade de Alergia e Imunologia do ICr HCFMUSP. As manifestaçöes clínicas foram precoces, com infecçöes recorrentes de vias aéreas e trato gastrointestinal. A avaliaçäo imunológica encontramos diminuiçäo dos níveis de IgG e IgA, com níveis de IgM normais no primeiro paciente e elevados (acima do percentil 97,5) no segundo paciente. Após o diagnóstico, os pacientes receberam gamaglobulina, antibióticos e orientaçäo quanto à nutriçäo e ambiente físico, com melhora evidente dos sintomas apresentados e sem o desenvolvimento de complicaçöes. Os autores ressaltam a importância do diagnóstico precoce da síndrome de Hiper IgM para tratamento adequado e controle dos processos infecciosos.
Subject(s)
Humans , Male , Female , Child , Child, Preschool , Agammaglobulinemia/blood , Hypergammaglobulinemia/metabolism , Immunoglobulin M/metabolism , Immunologic Deficiency Syndromes/blood , Immunoglobulin A/blood , Immunoglobulin G/bloodABSTRACT
The mechanism whereby the immune system avoids self-aggression is one of the central issues of Immunology. The discovery of natural autoantibodies, mainly of IgM isotype, and of idiotypic interactions between antibodies indicates that elements of the immune system interact with self constituents and with themselves. Results of studies with soluble antibodies have indicated that the pool of circulating IgM represents the end result of a highly selective process of B cell activation and differentiation by self proteins resulting in the formation of a network. The objective of the present work was to determine the frequency of self-reacting B cells in normal mice. We were able to detect B cells that recognize self proteins present in extracts of different organs in normal adult, 2-3-month old, BALB/c and C57BL/6 mice with an ELISA spot assay. About 1 per cent of total IgM-secreting cells among small, LPS-stimulated spleen cells reacted with organ extracts, whereas among large spleen cells the frequency was 5- to 10- fold lower. Immunization induced an increase in the frequency of IgM-secreting cells. The present results provide cellular evidence for the results of studies done at the serological level. The physiological role of these self-recognizing cells, as well as their participation in autoimmune processes, remain to be established.
Subject(s)
Mice , Animals , Autoimmunity/physiology , B-Lymphocytes/immunology , Immunoglobulin M/metabolism , Spleen/immunology , Cell Culture Techniques , Electrophoresis, Polyacrylamide Gel , Enzyme-Linked Immunosorbent Assay , Mice, Inbred BALB C , Mice, Inbred C57BLABSTRACT
Schistosomal nephropathy has long been related to the hepatosplenic form of schistosomiasis. In the last few years, 24 patients with hepatointestinal schistosomiasis and the nephrotic syndrome were studied. Aiming at evaluating a possible etiologic participation of schistosomiasis in the development of the nephropathy, this group was comparatively studied with a group of 37 patients with idiopathic nephrotic syndrome. Both groups had a different distribution of the histologic lesions. In the group with schistosomiasis there was a statistically significant prevalence of proliferative mesangial glomerulonephritis (33.3%), whereas in the control group there was prevalence of membranous glomerulonephritis (32.4%). On immunofluorescence, IgM was positive in 94.4% of the patients with schistosomiasis versus 55.0% in the control group (P < 0.01). In the group with schistosomiasis, 8 patients evidenced mesangial proliferative glomerulonephritis and 5, membranoproliferative glomerulonephritis. In both histological types immunofluorescence showed IgM and C3 granular deposits in the glomeruli. The data in this study suggests that mesangial proliferative and membranoproliferative glomerulonephritis, with glomerular granular IgM and C3 deposits, represent the renal lesions of the schistosomiasis associated nephropathy