From the Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania; and.
Division of General Internal Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
Allergy Asthma Proc. 2020 Sep 1;41(5):386-388. doi: 10.2500/aap.2020.41.200039.
Terminal complement component deficiencies are risk factors for neisserial infections. To review the clinical characteristics, the diagnosis and the management of patients with a terminal complement component deficiency. Pertinent articles were selected and reviewed in relation to a case presentation of C6 deficiency. A case of a 56-year old patient with a history of meningitis, chronic rash, and C6 deficiency was presented, followed by discussion of clinical characteristics, diagnosis, and management of terminal complement component deficiencies. Clinical pearls and pitfalls were reviewed for the practicing allergist/immunologist and fellow-in-training. C6 deficiency is the most common terminal complement component deficiency and can present later in age with N. meningitidis infections. Patients can be screened for terminal complement component deficiency by checking CH50.
末端补体成分缺陷是奈瑟菌感染的危险因素。本文旨在回顾末端补体成分缺陷患者的临床特征、诊断和治疗方法。我们选取了与 C6 缺陷病例相关的文章进行复习，并报道了 1 例 56 岁患者，该患者有脑膜炎、慢性皮疹和 C6 缺陷病史。随后，我们讨论了末端补体成分缺陷的临床特征、诊断和治疗方法。本文为过敏症/免疫学家和培训医师提供了临床要点和注意事项。C6 缺陷是最常见的末端补体成分缺陷，可在较晚年龄因脑膜炎奈瑟菌感染而发病。可通过检查 CH50 对末端补体成分缺陷进行筛查。
