诊断

需根据反复感染的病史和体格检查进行诊断。血液检查和基因检测或可确认诊断结果。

治疗

XLA 尚无治愈方法。治疗的目标是增强免疫系统以预防感染。在发生感染时,也可针对感染进行快速治疗。

药物

治疗 XLA 的药物包括:

  • 丙种球蛋白。这是一种存在于血液中的蛋白质,含有抗感染的抗体。每 2 到 4 周经静脉给药(输注)一次,或每周注射给药一次。

    对丙种球蛋白的反应可能包括头痛、寒战、背痛和恶心。在病毒感染(如感冒)期间更容易发生反应。

  • 抗生素。一些 XLA 患者一直服用抗生素来预防感染。另一些患者要比非 XLA 患者服用更长时间的抗生素来对抗细菌感染。

医疗护理专业人员可能会建议您每 6 到 12 个月复诊一次,以筛查 XLA 的并发症。还可能建议您不要接种活疫苗,例如脊髓灰质炎、麻疹-流行性腮腺炎-风疹或水痘活疫苗。

临床试验

探索 Mayo Clinic 的研究 测试新的治疗、干预与检查方法,旨在预防、检测、治疗或控制这种疾病。

April 17, 2024

Living with x 连锁无丙种球蛋白血症?

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  1. X-linked agammaglobulinemia. Genetic and Rare Diseases Information Center. https://rarediseases.info.nih.gov/diseases/1033/x-linked-agammaglobulinemia. Accessed Sept. 28, 2023.
  2. Hernandez-Trujillo VP. Agammaglobulinemia. https://www.uptodate.com/contents/search. Accessed Sept. 28,2023.
  3. X-linked agammaglobulinemia. Merck Manual Professional Version. https://www.merckmanuals.com/professional/immunology-allergic-disorders/immunodeficiency-disorders/x-linked-agammaglobulinemia. Accessed Nov. Sept. 28, 2023.
  4. AskMayoExpert. Common variable immunodeficiency. Mayo Clinic; 2023.
  5. Agammaglobulinemia: X-linked and autosomal recessive. Immune Deficiency Foundation. https://primaryimmune.org/about-primary-immunodeficiencies/specific-disease-types/agammaglobulinemia-x-linked-autosomal-recessive/?gclid=EAIaIQobChMIuPyMwJ2D5gIVyEXVCh2P3AYZEAAYASAAEgJY_fD_BwE. Accessed Nov. 24, 2019.
  6. Brown AY. Allscripts EPSi. Mayo Clinic. Nov.14, 2019.

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