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Primary antibody deficiency in a tertiary referral hospital: A 30-year experiment
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نویسنده
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mohammadinejad p. ,pourhamdi s. ,abolhassani h. ,mirminachi b. ,havaei a. ,masoom s.n. ,sadeghi b. ,ghajar a. ,afarideh m. ,parvaneh n. ,mirsaeed-ghazi b. ,movahedi m. ,gharagozlou m. ,chavoushzadeh z. ,mahdaviani a. ,zandieh f. ,sherkat r. ,sadeghi-shabestari m. ,faridhosseini r. ,jabbari-azad f. ,ahanchian h. ,zandkarimi m. ,cherghi t. ,fayezi a. ,mohammadzadeh i. ,amin r. ,aleyasin s. ,moghtaderi m. ,ghaffari j. ,bemanian m. ,shafiei a. ,kalantari n. ,ahmadiafshar a. ,khazaei h.a. ,mohammadi j. ,nabavi m. ,rezaei n. ,aghamohammadi a.
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منبع
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journal of investigational allergology and clinical immunology - 2015 - دوره : 25 - شماره : 6 - صفحه:416 -425
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چکیده
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Background: primary antibody deficiency (pad) is the most common group of primary immunodeficiency disorders (pid),with a broad spectrum of clinical features ranging from severe and recurrent infections to asymptomatic disease. objectives: the current study was performed to evaluate and compare demographic and clinical data in the most common types of pad. materials and methods: we performed a retrospective review of the medical records of all pad patients with a confirmed diagnosis of common variable immunodeficiency (cvid),hyper igm syndrome (higm),selective iga deficiency (sigad),and x-linked agammaglobulinemia (xla) who were diagnosed during the last 30 years at the children’s medical center,tehran,iran. results: a total number of 280 cases of pad (125 cvid,32 higm,63 sigad,and 60 xla) were enrolled in the study. the median (range) age at the onset of disease in cvid,higm,sigad,and xla was 2 (0-46),0.91 (0-9),1 (0-26),and 1 (0-10) years,respectively. gastrointestinal infections were more prevalent in cvid patients,as were central nervous system infections in xla patients. autoimmune complications were more prevalent in higm patients,malignancies in cvid patients,and allergies in sigad patients. the mortality rate for cvid,higm,and xla was 27.2%,28.1%,and 25%,respectively. no deaths were reported in sigad patients. conclusions: sigad patients had the best prognosis. while all pad patients should be monitored for infectious complications,special attention should be paid to the finding of malignancy and autoimmune disorders in cvid and higm patients,respectively. © 2015 esmon publicidad.
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کلیدواژه
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Common variable immunodeficiency; Hyper-IgM syndrome; Immunoglobulin A deficiency; Infection; X-linked agammaglobulinemia
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آدرس
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research center for immunodeficiencies,pediatrics center of excellence,children’s medical center,tehran university of medical sciences,tehran, ایران, research center for immunodeficiencies,pediatrics center of excellence,children’s medical center,tehran university of medical sciences,tehran, ایران, research center for immunodeficiencies,pediatrics center of excellence,children’s medical center,tehran university of medical sciences,tehran,iran,division of clinical immunology,department of laboratory medicine,karolinska institutet at karolinska university hospital huddinge,stockholm, Sweden, research center for immunodeficiencies,pediatrics center of excellence,children’s medical center,tehran university of medical sciences,tehran, ایران, research center for immunodeficiencies,pediatrics center of excellence,children’s medical center,tehran university of medical sciences,tehran, ایران, research center for immunodeficiencies,pediatrics center of excellence,children’s medical center,tehran university of medical sciences,tehran, ایران, research center for immunodeficiencies,pediatrics center of excellence,children’s medical center,tehran university of medical sciences,tehran, ایران, research center for immunodeficiencies,pediatrics center of excellence,children’s medical center,tehran university of medical sciences,tehran, ایران, research center for immunodeficiencies,pediatrics center of excellence,children’s medical center,tehran university of medical sciences,tehran, ایران, research center for immunodeficiencies,pediatrics center of excellence,children’s medical center,tehran university of medical sciences,tehran, ایران, department of immunology,bahrami hospital,tehran university of medical sciences,tehran, ایران, department of allergy and clinical immunology,pediatrics center of excellence,children’s medical center,tehran university of medical sciences,tehran, ایران, department of allergy and clinical immunology,pediatrics center of excellence,children’s medical center,tehran university of medical sciences,tehran, ایران, pediatric infectious research center,mofid children's hospital,shahid beheshti university of medical sciences,tehran, ایران, pediatric respiratory disease research center,national research institute of tuberculosis and lung disease,masih daneshvari hospital,shahid beheshti university of medical sciences,tehran, ایران, department of immunology,bahrami hospital,tehran university of medical sciences,tehran, ایران, acquired immunodeficiency research center,al-zahra hospital,isfahan university of medical sciences,isfahan, ایران, department of immunology and allergy,tabriz university of medical sciences,tabriz, ایران, allergy research center,school of medicine,mashhad university of medical sciences,mashhad, ایران, allergy research center,school of medicine,mashhad university of medical sciences,mashhad, ایران, allergy research center,school of medicine,mashhad university of medical sciences,mashhad, ایران, allergy research center,school of medicine,mashhad university of medical sciences,mashhad, ایران, department of pediatrics,17th shahrivar children's hospital,guilan university of medical sciences,rasht, ایران, department of immunology and allergy,ahvaz university of medical sciences,ahvaz, ایران, noncommunicable pediatric diseases research center,amirkola hospital,babol university of medical sciences,babol, ایران, department of pediatric immunology and allergy,namazi hospital,shiraz university of medical sciences,shiraz, ایران, department of pediatric immunology and allergy,namazi hospital,shiraz university of medical sciences,shiraz, ایران, department of pediatric immunology and allergy,namazi hospital,shiraz university of medical sciences,shiraz, ایران, department of pediatrics,mazandaran university of medical sciences,sari, ایران, department of immunology and allergy,yazd university of medical sciences,yazd, ایران, department of immunology,bahrami hospital,tehran university of medical sciences,tehran,iran,department of immunology and allergy,yazd university of medical sciences,yazd, ایران, department of immunology and allergy,golestan university of medical sciences,gorgan, ایران, department of immunology and allergy,ghazvin university of medical sciences,ghazvin, ایران, department of immunology and hematology,zahedan medical sciences university,zahedan, ایران, department of life science,university of tehran,tehran, ایران, department of allergy and clinical immunology,rasool e akram hospital,iran university of medical sciences,tehran, ایران, research center for immunodeficiencies,pediatrics center of excellence,children’s medical center,tehran university of medical sciences,tehran,iran,molecular immunology research center and department of immunology,school of medicine,tehran university of medical sciences,tehran, ایران, research center for immunodeficiencies,pediatrics center of excellence,children’s medical center,tehran university of medical sciences,tehran, ایران
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Authors
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