Pulmonary Complications in a Patient with Common Variable Hypogammaglobulinemia

IF 0.3 Q3 MEDICINE, GENERAL & INTERNAL Clinical Management Issues Pub Date : 2023-01-31 DOI:10.7175/cmi.v17i1.1521
P. Ghiringhelli, F. Foieni, Girolamo Sala, A. Diana, B. Valvo, Mariella Ciola, Michele Zaza, R. Bertolini, C. Simoni, Lorenzo Bellintani, A. Agostinelli, F. Macchi, S. Limbiati, M. Provisione
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Abstract

Common variable immunodeficiency (CVID) is a rare disorder characterized by primary antibody deficiency leading to hypogammaglobulinemia and increased risk of infections. Pulmonary hypertension (PH) is an unusual complication of CVID and may be associated with chronic hypoxemic respiratory failure.We described the case of a 47-year-old female patient hospitalized with worsening dyspnea, which had emerged about 10 days before and was associated with productive cough. 26 years before, she was diagnosed with common variable hypogammaglobulinemia, that was treated with intravenous immunoglobulin infusions for at least 20 years. She had suffered from recurrent secondary infections of the respiratory tract with inveterate bronchiectasis, pulmonary hypertension, and chronic respiratory failure.This case suggests that Internal Medicine wards, due to their global vision of the patient, are well suited to manage subjects with serious, complex, and genetically determined pathologies.
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常变型低丙种球蛋白血症患者的肺部并发症
常见可变免疫缺陷(CVID)是一种罕见的疾病,其特征是一级抗体缺乏导致低丙种球蛋白血症和感染风险增加。肺动脉高压(PH)是CVID的一种不常见并发症,可能与慢性低氧性呼吸衰竭有关。我们描述了一名47岁的女性患者因呼吸困难恶化住院的病例,该患者在大约10天前出现,并与生产性咳嗽有关。26年前,她被诊断为常见的可变低丙种球蛋白血症,接受了至少20年的静脉注射免疫球蛋白治疗。她患有反复发作的呼吸道继发感染,伴有根深蒂固的支气管扩张、肺动脉高压和慢性呼吸衰竭。这一案例表明,内科病房由于其对患者的全球视野,非常适合管理具有严重、复杂和遗传决定病理的受试者。
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来源期刊
Clinical Management Issues
Clinical Management Issues MEDICINE, GENERAL & INTERNAL-
自引率
0.00%
发文量
3
审稿时长
13 weeks
期刊介绍: Clinical Management Issues is an open access, peer-reviewed journal published by SEEd Medical Publishers (online ISSN = 2283-3137). The aim of the published case reports is to expand medical knowledge, allowing a better explanation of the practical application of a clinical guideline, or including an up-to-date review of medical knowledge in that field, or helping doctors to make better decisions in a “grey area”, or explaining how to manage a disease with an integrated approach between different specialists involved. Clinical Management Issues also publishes unusual case reports (i.e. unusual side effects or adverse interactions involving medications, unexpected or unusual presentations of a disease, etc.), articles on clinical management of a disease, case series, editorials, and brief reports. Acceptance rate of submitted articles is about 90%. Content is subject to peer-review and is editorially independent. This journal provides immediate open access to all of its articles (both HTML and PDF versions). Authors are asked to state any professional and financial situations that might be perceived as causing a conflict of interest with respect to integrity of content. The Declaration of Financial Competing Interests, that should be filled, signed and sent to the Publisher, is downloadable here.
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