出版社：Grupo de Pesquisa Metodologias em Ensino e Aprendizagem em Ciências
摘要：Introduction: The Primary Immunodeficiency Diseases comprise more than 400 disorders of the immune system, are rare and associated with abnormal development of immune system cells. Objective: To analyze how nursing care for patients with primary immunodeficiency in health systems is described in the scientific literature. Method: An integrative literature review that included journals in Portuguese, English or Spanish available in full and free text in the CINAHL, MEDLINE, EMBASE, Web of Science and Virtual Health Library databases, from 2000 to 2021. Abstracts, editorials and other systematic or integrative literature reviews were excluded. Results: Nine publications were selected. Descriptive studies predominated, with the qualitative approach being the most prevalent. Regarding the evaluation performed with the CASP standardized instrument for analyzing articles, approximately 33% of the selected studies were classified as level A. Discussion: Nursing has numerous nursing attributions in this scenario, such as: performing actions before, during and after immunoglobulin infusion; informing and supporting family members/caregivers; be aware of the patient's social context; promote self-care; prevent and control infections related to health care and encourage adherence to treatment, among other attributions. Conclusion: It is possible to acknowledge the need for trained professionals with scientific knowledge to correctly guide and offer quality care to patients with PID. In addition, further studies are needed on the role of nursing in this scenario, aiming at the propagation of knowledge.
