کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
3201055 | 1201948 | 2009 | 21 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Improving cellular therapy for primary immune deficiency diseases: Recognition, diagnosis, and management
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کلمات کلیدی
TMP/SMXGvHDCIDCenter for International Blood and Marrow Transplant ResearchPIDPIDTCHLHPCPHCTCGDSCIDnatural killer - (سلول های) کشنده طبیعیadenosine deaminase - آدنوزین دآمینازAlc - آلکWAS - بودPrimary immunodeficiency disease - بیماری ابتلا به کمبود ایمنیGraft-versus-host disease - بیماری مرض در برابر میزبانchronic granulomatous disease - بیماری گرانولوماتوز مزمنTrimethoprim-sulfamethoxazole - ترمثوپریم سولفامتوکسازولcomputed tomography - توموگرافی کامپیوتری یا سی تی اسکن یا مقطعنگاری رایانهایWiskott-Aldrich syndrome - سندرم Wiskott-Aldrichcytomegalovirus - سیتومگالوویروسCMV - سیتومگالوویروسCyclophosphamide - سیکلوفسفامید Absolute lymphocyte count - شمارش لنفوسیت مطلقHemophagocytic lymphohistiocytosis - لنفوهیستسیتوز هموفوگوسیتیکPhysical examination - معاینه بالینیADA - وجود داردAllogeneic hematopoietic stem cell transplantation - پیوند سلول های بنیادی خون آلوژنیکHematopoietic stem cell transplantation - پیوند مغز استخوانGene therapy - ژن درمانیClinical trial - کارآزمایی بالینیprimary immunodeficiency - کمبود ایمنی اولیهSevere combined immune deficiency - کمبود شدید کمبود ایمنی
موضوعات مرتبط
علوم زیستی و بیوفناوری
ایمنی شناسی و میکروب شناسی
ایمونولوژی
پیش نمایش صفحه اول مقاله
چکیده انگلیسی
More than 20 North American academic centers account for the majority of hematopoietic stem cell transplantation (HCT) procedures for primary immunodeficiency diseases (PIDs), with smaller numbers performed at additional sites. Given the importance of a timely diagnosis of these rare diseases and the diversity of practice sites, there is a need for guidance as to best practices in management of patients with PIDs before, during, and in follow-up for definitive treatment. In this conference report of immune deficiency experts and HCT physicians who care for patients with PIDs, we present expert guidance for (1) PID diagnoses that are indications for HCT, including severe combined immunodeficiency disease (SCID), combined immunodeficiency disease, and other non-SCID diseases; (2) the critical importance of a high degree of suspicion of the primary care physician and timeliness of diagnosis for PIDs; (3) the need for rapid referral to an immune deficiency expert, center with experience in HCT, or both for patients with PIDs; (4) medical management of a child with suspicion of SCID/combined immunodeficiency disease while confirming the diagnosis, including infectious disease management and workup; (5) the posttransplantation follow-up visit schedule; (6) antimicrobial prophylaxis after transplantation, including gamma globulin administration; and (7) important indications for return to the transplantation center after discharge. Finally, we discuss the role of high-quality databases in treatment of PIDs and HCT as an element of the infrastructure that will be needed for productive multicenter clinical trials in these rare diseases.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Journal of Allergy and Clinical Immunology - Volume 124, Issue 6, December 2009, Pages 1152-1160.e12
Journal: Journal of Allergy and Clinical Immunology - Volume 124, Issue 6, December 2009, Pages 1152-1160.e12
نویسندگان
Linda M. MD, PhD, Morton J. MD, Luigi D. MD, Jennifer M. MD, Rebecca H. MD, Fabio MD, Mary Ellen MD, Thomas A. MD, H. Bobby MD, PhD, Donald B. MD, Hans D. MD, Richard J. MD, J. Douglas MD, MS, Chaim M. MD, FRCP, FCACB, Trudy N. MD, William T. MD, PhD,