Pediatric Infectious Diseases, Rheumatology and Immunology, University Hospital Virgen del Rocío, Institute of Biomedicine of Seville (IBiS), Sevilla, Spain.
French National Reference Center for Primary Immunodeficiencies (CEREDIH), Necker-Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France.
Clin Exp Immunol. 2024 Oct 16;218(2):136-150. doi: 10.1093/cei/uxae059.
Prevention and treatment of infections are primary goals of treatment of children and adults with primary immune deficiencies due to decreased antibody production. Approaches to these goals include immunoglobulin replacement therapy, vaccination, and prophylactic treatment with antimicrobials. In this review, the infectious and non-infectious complications of antibody deficiencies will be discussed along with the limited number of studies that support the effective use of the available therapies and to drive the development of new therapies. Some illustrative case studies will be presented and the outlook for additional controlled clinical trials and potential for therapies driven by the underlying disease genetics will be considered.
预防和治疗感染是因抗体产生减少而导致原发性免疫缺陷的儿童和成人治疗的主要目标。实现这些目标的方法包括免疫球蛋白替代疗法、疫苗接种和预防性使用抗生素。在这篇综述中，将讨论抗体缺陷的感染和非感染性并发症，以及支持有效利用现有疗法和推动新疗法发展的为数不多的研究。将提出一些说明性病例研究，并考虑进行更多对照临床试验的前景以及潜在的针对潜在疾病遗传学的治疗方法。
