Protocols of Standard of Care for Adult Patients with Primary Antibody Deficiencies Will Improve Timing of Diagnosis, Survival, and Quality of Life.

IF 1.2 4区 医学 Q4 ALLERGY Iranian journal of allergy, asthma, and immunology Pub Date : 2022-08-12 DOI:10.18502/ijaai.v21i4.10285
Katarzyna Napiórkowska-Baran, Ewa Więsik-Szewczyk, Marcin Ziętkiewicz, Aleksandra Matyja-Bednarczyk, Sylwia Kołtan, Natalia Bąkowska-Kocik, Tomasz Rosada, Karolina Baranowska, Ewa Alska, Marta Tykwińska, Ewa Szynkiewicz, Robert Zacniewski, Zbigniew Bartuzi
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引用次数: 2

Abstract

The majority of primary immunodeficiencies (PIDs) are antibody deficiencies (PADs), and not all of them are rare diseases; As an example, Caucasian individuals suffer from selective IgA deficiency at a frequency of 1:500. In addition to infections, symptomatic patients with PAD are more likely to develop neoplastic, autoimmune, and allergic diseases. In the event that PAD is neglected or delayed for more than ten years, complications develop, eventually resulting in death. No studies have been conducted to devise and report detailed ready-to-use protocols for managing PAD to date. This study aimed to propose protocols and guidelines for the adult PAD patients' standard care. Preparing the protocol, we considered the frequency and type of laboratory tests, imaging, endoscopic examinations, specialist consultations, and standardized recommendations for further care in the place of residence.  As a result of the proposed monitoring scheme, patients can be provided with complete care in terms of their underlying conditions and comorbidities, as well as early detection of complications. This protocol will serve as a guide for physicians dealing with these patients and enable comparisons of patient groups across a variety of treatment centers, even far away from each other. A national consultant in the field of clinical immunology verified the protocol mainly developed by Polish experts from reference immunology centres for adults.

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成人一抗缺乏患者的标准治疗方案将改善诊断时间、生存率和生活质量。
原发性免疫缺陷(pid)的大多数是抗体缺陷(PADs),并不是所有的是罕见的疾病;例如,高加索人患选择性IgA缺乏症的频率为1:500。除了感染外,有症状的PAD患者更容易发生肿瘤、自身免疫性疾病和过敏性疾病。如果PAD被忽视或延迟超过十年,就会出现并发症,最终导致死亡。迄今为止,还没有进行任何研究来设计和报告管理PAD的详细即用方案。本研究旨在为成人PAD患者的标准护理提供方案和指南。在制定方案时,我们考虑了实验室检查的频率和类型、成像、内窥镜检查、专家咨询以及在居住地进一步护理的标准化建议。由于拟议的监测方案,可以为患者提供有关其潜在疾病和合并症的完整护理,以及早期发现并发症。该协议将作为医生处理这些患者的指南,并使不同治疗中心的患者群体能够进行比较,即使彼此相距遥远。临床免疫学领域的一名国家顾问验证了主要由成人参考免疫学中心的波兰专家制定的方案。
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来源期刊
CiteScore
2.60
自引率
6.70%
发文量
64
审稿时长
>12 weeks
期刊介绍: The Iranian Journal of Allergy, Asthma and Immunology (IJAAI), an international peer-reviewed scientific and research journal, seeks to publish original papers, selected review articles, case-based reviews, and other articles of special interest related to the fields of asthma, allergy and immunology. The journal is an official publication of the Iranian Society of Asthma and Allergy (ISAA), which is supported by the Immunology, Asthma and Allergy Research Institute (IAARI) and published by Tehran University of Medical Sciences (TUMS). The journal seeks to provide its readers with the highest quality materials published through a process of careful peer reviews and editorial comments. All papers are published in English.
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