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为非典型和继发性抗体缺陷患者开具免疫球蛋白替代治疗处方:对英国和爱尔兰共和国临床免疫学家实践的分析。

Prescribing Immunoglobulin Replacement Therapy for Patients with Non-classical and Secondary Antibody Deficiency: an Analysis of the Practice of Clinical Immunologists in the UK and Republic of Ireland.

机构信息

Regional Immunology Service, The Royal Hospitals, Belfast Health & Social Care Trust and Queen's University Belfast, Belfast, Northern Ireland, UK.

Clinical Immunology Service, Birmingham Medical School & University Hospitals, Birmingham NHS Foundation Trust, Queen Elizabeth Hospital, Birmingham, B15 2GW, UK.

出版信息

J Clin Immunol. 2018 Feb;38(2):204-213. doi: 10.1007/s10875-017-0469-4. Epub 2018 Feb 8.

Abstract

BACKGROUND

Immunologists are increasingly being asked to assess patients with non-classical and secondary antibody deficiency to determine their potential need for immunoglobulin replacement therapy (IGRT). Immunoglobulin is a limited, expensive resource and no clear guidance exists for this broad patient group. The purpose of this survey is to establish what factors influence the decision to commence IGRT in adult patients, when diagnostic criteria for primary antibody deficiency are not fulfilled.

METHODS

Under the auspices of the United Kingdom Primary Immunodeficiency Network (UKPIN), a study group was established which circulated an online questionnaire to the consultant body across the UK and Ireland. Results provided a snapshot of the current clinical practice of 71% of consultant immunologists, from 30 centers.

RESULTS

In order of importance, factors which influence the decision to commence IGRT include number of hospital admissions with infection, serum IgG level, bronchiectasis, radiologically proven pneumonia, number of positive sputum cultures, number of antibiotic courses, and results of immunization studies. The commonest test vaccine used was Pneumovax 23 with measurement of serotype-specific responses at 4 weeks, with a threshold of 0.35 μg/ml in 2/3 of serotypes measured. Eighty-six percent of patients are treated with a trial of prophylactic antibiotics prior to consideration of IGRT. Efficacy of IGRT trial is assessed at between 6 and 12 months.

CONCLUSIONS

There was consistency in clinical practice using a combination of clinical history, evidence of infections, and vaccination testing for diagnosis. However, there was some variation in the implementation of this practice, particularly in vaccine choice and assessment of response to vaccination.

摘要

背景

免疫学家越来越多地被要求评估非典型和继发性抗体缺陷患者,以确定他们是否需要免疫球蛋白替代治疗(IGRT)。免疫球蛋白是一种有限且昂贵的资源,对于这一大类患者,目前尚无明确的指导意见。这项调查的目的是确定在未满足原发性抗体缺陷诊断标准的情况下,哪些因素会影响开始 IGRT 的决定。

方法

在英国原发性免疫缺陷网络(UKPIN)的支持下,成立了一个研究小组,该小组向英国和爱尔兰的顾问机构分发了在线问卷。结果反映了 30 个中心的 71%顾问免疫学家的当前临床实践情况。

结果

按重要性顺序排列,影响开始 IGRT 决定的因素包括感染住院次数、血清 IgG 水平、支气管扩张、放射学证实的肺炎、阳性痰培养次数、抗生素疗程数和免疫接种研究结果。最常用的测试疫苗是 23 价肺炎球菌疫苗,在 4 周时测量血清型特异性反应,2/3 测量的血清型中阈值为 0.35μg/ml。86%的患者在考虑 IGRT 之前,会先进行预防性抗生素试验治疗。IGRT 试验的疗效在 6 至 12 个月时进行评估。

结论

临床实践中使用临床病史、感染证据和疫苗接种测试相结合的方法进行诊断具有一致性。然而,在实践的实施方面存在一些差异,特别是在疫苗选择和接种反应评估方面。

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