The Experts below are selected from a list of 10293 Experts worldwide ranked by ideXlab platform
Megan A. Cooper - One of the best experts on this subject based on the ideXlab platform.
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Hypogammaglobulinemia in pediatric systemic lupus erythematosus
Lupus, 2013Co-Authors: Emilina Lim, Yu Tao, Andrew J. White, Anthony R. French, Megan A. CooperAbstract:ObjectiveSystemic lupus erythematosus (SLE) is a systemic autoimmune disease typically associated with elevated serum immunoglobulin G (IgG). Hypogammaglobulinemia in SLE patients has been attributed to immunosuppressive treatment or a transient effect associated with nephrotic syndrome. We retrospectively reviewed pediatric SLE patients from a single institution to identify patients with Hypogammaglobulinemia and risk factors for Hypogammaglobulinemia.MethodsA total of 116 pediatric SLE cases from 1997 to 2011 were reviewed and patients with Hypogammaglobulinemia (IgG
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Hypogammaglobulinemia in pediatric systemic lupus erythematosus
Lupus, 2013Co-Authors: Emilina Lim, Yu Tao, Andrew J. White, Anthony R. French, Megan A. CooperAbstract:ObjectiveSystemic lupus erythematosus (SLE) is a systemic autoimmune disease typically associated with elevated serum immunoglobulin G (IgG). Hypogammaglobulinemia in SLE patients has been attributed to immunosuppressive treatment or a transient effect associated with nephrotic syndrome. We retrospectively reviewed pediatric SLE patients from a single institution to identify patients with Hypogammaglobulinemia and risk factors for Hypogammaglobulinemia.MethodsA total of 116 pediatric SLE cases from 1997 to 2011 were reviewed and patients with Hypogammaglobulinemia (IgG < 500 mg/dl) were identified. The two cohorts were evaluated for association with age, sex, presence of lupus nephritis at SLE diagnosis, disease activity at diagnosis, initial IgG level, and drug treatment.ResultsEighty-six patients were included in our study, with a median age of 15 years and a median follow-up of 39.5 months. Seven percent (six of 86) of patients had Hypogammaglobulinemia with a median onset of 27 months (0–72 months) af...
Erwin W. Gelfand - One of the best experts on this subject based on the ideXlab platform.
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Humoral immunity in steroid-dependent children with asthma and Hypogammaglobulinemia
The Journal of pediatrics, 1996Co-Authors: Gideon Lack, Hans D. Ochs, Erwin W. GelfandAbstract:Abstract OBJECTIVE: To determine primary and secondary antibody responses in children with Hypogammaglobulinemia attributed to corticosteroid use. RESULTS: In seven patients with steroid-dependent asthma and significant Hypogammaglobulinemia (IgG concentration, 275 to 443 mg/dl), antibody responses to protein and polysaccharide antigens were shown to be normal, as were primary and secondary responses to a neoantigen, bacteriophage ΦXC174. CONCLUSIONS: Patients with asthma, and with Hypogammaglobulinemia resulting from steroid therapy, have normal humoral immunity, and immunoglobulin replacement therapy is not indicated. (J Pediatr 1996;129:898-903)
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Ureaplasma urealyticum chronic osteomyelitis in a patient with Hypogammaglobulinemia.
Journal of Allergy and Clinical Immunology, 1991Co-Authors: Ahmed A. Mohiuddin, Ronald J. Harbeck, Jonathan Corren, Jeri L. Teague, Michael Volz, Erwin W. GelfandAbstract:Mycoplasma species are recognized as important pathogens in patients with Hypogammaglobulinemia. In this article we describe, for the first time, a patient with Hypogammaglobulinemia who developed osteomyelitis of the hip caused by Ureaplasma urealyticum. This article emphasizes the need for considering infection with Mycoplasma species in patients with antibody deficiency.
László Maródi - One of the best experts on this subject based on the ideXlab platform.
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WHIM-szindróma = WHIM syndrome
2007Co-Authors: Melinda Erdős, László MaródiAbstract:A WHIM-szindroma ritka, autoszomalis dominans oroklődesmenetű primer immunhiany-betegseg, amelyre virusos szemolcsok, hypogammaglobulinaemia, visszaterő fertőzesek es myelokathexis jellemző. A kozlemenyben a szerzők egy esetismertetes kapcsan mutatjak be a betegseg klinikumat, laboratoriumi eltereseit, osszefoglaljak a korkep molekularis patomechanizmusaval kapcsolatos ismereteket es kezelesenek lehetősegeit. A szerzők szerint a betegseg inkomplett megjelenese a kesői felismeres es kezeles gyakori oka gyermekkorban. | The WHIM syndrome is a rare, autosomal dominant primary immunodeficiency disorder characterized by warts, Hypogammaglobulinemia, recurrent infections, and myelokathexis. The authors summarize current knowledge on molecular basis, diagnostic criteria, therapy, and clinical manifestations of WHIM syndrome. The authors propose that delayed diagnosis and treatment of children with WHIM may be due to incomplete presentation of the disease.
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whim szindroma whim syndrome
2007Co-Authors: Melinda Erdős, László MaródiAbstract:A WHIM-szindroma ritka, autoszomalis dominans oroklődesmenetű primer immunhiany-betegseg, amelyre virusos szemolcsok, hypogammaglobulinaemia, visszaterő fertőzesek es myelokathexis jellemző. A kozlemenyben a szerzők egy esetismertetes kapcsan mutatjak be a betegseg klinikumat, laboratoriumi eltereseit, osszefoglaljak a korkep molekularis patomechanizmusaval kapcsolatos ismereteket es kezelesenek lehetősegeit. A szerzők szerint a betegseg inkomplett megjelenese a kesői felismeres es kezeles gyakori oka gyermekkorban. | The WHIM syndrome is a rare, autosomal dominant primary immunodeficiency disorder characterized by warts, Hypogammaglobulinemia, recurrent infections, and myelokathexis. The authors summarize current knowledge on molecular basis, diagnostic criteria, therapy, and clinical manifestations of WHIM syndrome. The authors propose that delayed diagnosis and treatment of children with WHIM may be due to incomplete presentation of the disease.
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Recurrent CXCR4 sequence variation in a girl with WHIM syndrome.
European journal of haematology, 2006Co-Authors: Krisztina Alapi, Melinda Erdos, Gabriella Kovács, László MaródiAbstract:WHIM (warts-Hypogammaglobulinemia-infections-myelokathexis) syndrome is a recently described primary immunodeficiency disorder caused by mutation of the CXCR4 chemokine receptor gene. We report here of a 6.5-yr-old girl with bacterial infections, severe chronic neutropenia, and Hypogammaglobulinemia. Sequencing the CXCR4 gene revealed a c.1013C>G sequence variant suggesting WHIM syndrome. Recurrent c.1013C>G sequence variant of the CXCR4 gene resulting in p.S338X truncation mutation of this chemokine receptor protein is first reported here.
Goncalo Boleto - One of the best experts on this subject based on the ideXlab platform.
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op0230 predictors of Hypogammaglobulinemia during rituximab maintenance therapy in rheumatoid arthritis a 12 year longitudinal multi centre study
Annals of the Rheumatic Diseases, 2018Co-Authors: Goncalo Boleto, Jerome Avouac, Julien Wipff, Marine Forien, M Dougados, Christian Roux, Andre Kahan, Philippe Dieude, Yannick AllanoreAbstract:Background Rituximab (RTX) is an anti-CD20 monoclonal antibody that selectively depletes B-cell population. One of the drawbacks of a prolonged peripheral B-cell depletion is the suppression of protective antibodies and an increased risk for infectious events. However, few long-term data are available on predictors for the development of low levels of serum immunoglobulins in patients receiving repeated courses of RTX. Objectives We aimed at the identification of predictors for Hypogammaglobulinemia occurrence in RA patients long-term treated with RTX in a ‘real-life’ setting. Methods Multicenter longitudinal observational usual care study including RA patients according to ACR 1987 and/or ACR/ EULAR 2010 classification criteria followed and treated with RTX. A previous study assessing the safety profile of RTX in patients with RA reported a median follow-up of 30 months. 1 Therefore, we decided to include RA patients on RTX maintenance therapy, after a minimal exposition of 30 months. Serum protein electrophoresis was performed before each RTX infusion. Hypogammaglobulinemia and severe Hypogammaglobulinemia were defined as total gammaglobulin Results 134 patients met inclusion criteria: 113 female subjects (84.3%); mean age 52.1±11.4 years. Mean follow-up was 79.5±24.6 months and analysis was based on 854.9 patient-years (pt-yrs). Mean RTX cumulative dose was 12.0±4.9 g. Hypogammaglobulinemia ( Conclusions Our results show that gammaglobulin levels of less than 8 g/L at baseline is a strong independent risk factor for developing subsequent Hypogammaglobulinemia, whereas concomitant MTX therapy seems to be a protective factor in RA patients treated long-term with RTX. Identifying such predictors will raise clinicians’ awareness and allow more tailored monitoring of RA patients long-term treated with RTX. Reference [1] Isvy, et al. Joint Bone Spine2012. Disclosure of Interest None declared
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predictors of Hypogammaglobulinemia during rituximab maintenance therapy in rheumatoid arthritis a 12 year longitudinal multi center study
Seminars in Arthritis and Rheumatism, 2018Co-Authors: Goncalo Boleto, Jerome Avouac, Julien Wipff, Marine Forien, M Dougados, Christian Roux, Andre Kahan, Philippe Dieude, Yannick AllanoreAbstract:Abstract Objective Rituximab (RTX) is an anti-CD20 monoclonal antibody that selectively depletes B-cell population. Thus, it presents a potential risk for the development of Hypogammaglobulinemia and related infectious events. Our aim was to identify predictors of Hypogammaglobulinemia in RA patients long-term treated with RTX. Methods Multicenter observational usual care study of patients with RA on RTX maintenance therapy (minimal exposition of 30 months). Serum protein electrophoresis was performed before each RTX infusion. Hypogammaglobulinemia and severe Hypogammaglobulinemia were defined as total gammaglobulin Results 134 patients met inclusion criteria and were followed-up for 79.5 ± 24.6 months. Hypogammaglobulinemia occurred during the follow-up period in 23 patients (2.7 events per 100 pt-yrs). The mean time to development of Hypogammaglobulinemia was 64 ± 23 months. Patients who developed Hypogammaglobulinemia were more likely to experience severe infections (26.1% vs. 6.3%, P = 0.033). Multivariate Cox analysis identified gammaglobulin levels Conclusion Our results show that gammaglobulin levels of less than 8 g/L at baseline is a strong independent risk factor for developing subsequent Hypogammaglobulinemia, whereas concomitant MTX therapy seems to be a protective factor in RA patients treated long-term with RTX.
Yannick Allanore - One of the best experts on this subject based on the ideXlab platform.
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op0230 predictors of Hypogammaglobulinemia during rituximab maintenance therapy in rheumatoid arthritis a 12 year longitudinal multi centre study
Annals of the Rheumatic Diseases, 2018Co-Authors: Goncalo Boleto, Jerome Avouac, Julien Wipff, Marine Forien, M Dougados, Christian Roux, Andre Kahan, Philippe Dieude, Yannick AllanoreAbstract:Background Rituximab (RTX) is an anti-CD20 monoclonal antibody that selectively depletes B-cell population. One of the drawbacks of a prolonged peripheral B-cell depletion is the suppression of protective antibodies and an increased risk for infectious events. However, few long-term data are available on predictors for the development of low levels of serum immunoglobulins in patients receiving repeated courses of RTX. Objectives We aimed at the identification of predictors for Hypogammaglobulinemia occurrence in RA patients long-term treated with RTX in a ‘real-life’ setting. Methods Multicenter longitudinal observational usual care study including RA patients according to ACR 1987 and/or ACR/ EULAR 2010 classification criteria followed and treated with RTX. A previous study assessing the safety profile of RTX in patients with RA reported a median follow-up of 30 months. 1 Therefore, we decided to include RA patients on RTX maintenance therapy, after a minimal exposition of 30 months. Serum protein electrophoresis was performed before each RTX infusion. Hypogammaglobulinemia and severe Hypogammaglobulinemia were defined as total gammaglobulin Results 134 patients met inclusion criteria: 113 female subjects (84.3%); mean age 52.1±11.4 years. Mean follow-up was 79.5±24.6 months and analysis was based on 854.9 patient-years (pt-yrs). Mean RTX cumulative dose was 12.0±4.9 g. Hypogammaglobulinemia ( Conclusions Our results show that gammaglobulin levels of less than 8 g/L at baseline is a strong independent risk factor for developing subsequent Hypogammaglobulinemia, whereas concomitant MTX therapy seems to be a protective factor in RA patients treated long-term with RTX. Identifying such predictors will raise clinicians’ awareness and allow more tailored monitoring of RA patients long-term treated with RTX. Reference [1] Isvy, et al. Joint Bone Spine2012. Disclosure of Interest None declared
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predictors of Hypogammaglobulinemia during rituximab maintenance therapy in rheumatoid arthritis a 12 year longitudinal multi center study
Seminars in Arthritis and Rheumatism, 2018Co-Authors: Goncalo Boleto, Jerome Avouac, Julien Wipff, Marine Forien, M Dougados, Christian Roux, Andre Kahan, Philippe Dieude, Yannick AllanoreAbstract:Abstract Objective Rituximab (RTX) is an anti-CD20 monoclonal antibody that selectively depletes B-cell population. Thus, it presents a potential risk for the development of Hypogammaglobulinemia and related infectious events. Our aim was to identify predictors of Hypogammaglobulinemia in RA patients long-term treated with RTX. Methods Multicenter observational usual care study of patients with RA on RTX maintenance therapy (minimal exposition of 30 months). Serum protein electrophoresis was performed before each RTX infusion. Hypogammaglobulinemia and severe Hypogammaglobulinemia were defined as total gammaglobulin Results 134 patients met inclusion criteria and were followed-up for 79.5 ± 24.6 months. Hypogammaglobulinemia occurred during the follow-up period in 23 patients (2.7 events per 100 pt-yrs). The mean time to development of Hypogammaglobulinemia was 64 ± 23 months. Patients who developed Hypogammaglobulinemia were more likely to experience severe infections (26.1% vs. 6.3%, P = 0.033). Multivariate Cox analysis identified gammaglobulin levels Conclusion Our results show that gammaglobulin levels of less than 8 g/L at baseline is a strong independent risk factor for developing subsequent Hypogammaglobulinemia, whereas concomitant MTX therapy seems to be a protective factor in RA patients treated long-term with RTX.