【摘要】 背景目前在我国关于免疫缺陷病相关疫苗衍生性脊髓灰质炎(iVAPP)的病例报道少见。目的 总结iVAPP的临床表现、免疫学指标及转归。设计病例系列报告。方法 纳入2018年5月至2023年6月在复旦大学附属儿科医院临床免疫科住院的iVAPP患儿，截取患儿的人口学资料、接种脊髓灰质炎病毒(PV)疫苗的相关情况、临床表现、发病时免疫相关指标、病原学检测结果、治疗、转归和肌力恢复情况。主要结局指标临床表现和免疫相关指标。结果 4例iVAPP患儿均为男孩，发病年龄为7～12月龄，处于口服脊髓灰质炎病毒疫苗(OPV)4～7个月后；首剂均在3月龄接种OPV,例1、2、4分别接种OPV 1、2、3剂，例3接种OPV 2剂、灭活脊髓灰质炎疫苗(IPV)1剂。2例为联合免疫缺陷病相关疫苗衍生性脊髓灰质炎(CID-VAPP),因“肺部感染、腋下淋巴结破溃”入院，T、B细胞数量均严重降低，呈现多部位肢体瘫痪，存在多侧肢体的肌力减退，积极治疗后未发生缓解；感染累及部位多，感染病原种类多，均发生卡介苗相关结核分枝杆菌感染、重症肺炎，在1周岁内因严重感染死亡。2例为原发性抗体缺陷病(PAD)-VAPP,因“肢体活动障碍”入院，仅B细胞数量降低和抗体分泌障碍，肢体瘫痪症状可累及一侧或双侧，经治疗后肌力可恢复正常，且感染症状轻，预后良好。结论CID和PAD患儿口服OPV均有可能导致iVAPP,患儿的预后主要取决于原发病。更多还原

【Abstract】 Background Rare cases of immunodeficiency disease-related vaccine-derived poliomyelitis(iVAPP) have been reported in China. Objective To analyze the clinical features, immunodeficiency phenotype, treatment as well as prognosis among different iVAPP patients. Design Case series report. Methods Retrospective analysis was performed for the clinical data of 4 children with iVAPP admitted to Department of Clinical Immunology at Children’s Hospital of Fudan University from May 2018 to June 2023. The intercepted information from medical records included poliovirus(PV) vaccination, clinical manifestations, immune-related indicators, etiological test results, treatment, outcome and muscle strength recovery. Main outcome measures Clinical manifestations and immunology-related indicators. Results All 4 patients with iVAPP were boys. Their paralysis symptoms were presented at 4-7 months after taking OPV, and the first dose was received at 3 months of age. Case 1, 2, 4 received 1, 2, and 3 doses of OPV respectively, and case 3 received 2 doses of OPV and 1 dose of IPV. Two cases of combined immunodeficiency-related vaccine-derived polio(CID-VAPP) were admitted to the hospital due to lung infection and axillary lymph node inflammation with severely reduced counts of T and B cells. Their paralysis of multiple limbs and muscle weakness of multiple limbs could not be relieved by active treatment. The infections involved multiple sites and were caused by various pathogens. Mycobacterium tuberculosis complex and severe pneumonia were found. Deaths occurred within the first year of life due to severe infections. The other 2 cases were primary antibody deficiency disease(PAD)-VAPP, who were admitted to the hospital at the age of 12 months and 9 months respectively due to limb disability, showing a decrease in the number of B cells as well as impaired antibody secretion. The symptoms of limb paralysis involved one or more sides of the body, and the muscle strength could return to normal after treatment, with mild infection symptoms and a good prognosis. Conclusion Intaking OPV may lead to iVAPP in both CID and PAD children, and the prognosis mainly depends on the primary disease.更多还原