学龄期肾病综合征男童咳嗽1月伴气促半月

A school-aged boy with nephrotic syndrome with cough for one month and shortness of breath for half a month

摘要:

患儿，男，6岁11个月，因诊断肾病综合征2年，咳嗽1个月，气促15 d入院。患儿有长期使用激素和免疫抑制剂病史，出现咳嗽和气促后完善胸部CT提示双肺弥漫性磨玻璃影，G试验阳性，呼吸道分泌物检测结果示巨细胞病毒核酸阳性，给予抗真菌及抗病毒治疗后一过性好转，短期内再次加重，肺泡灌洗液六胺银染色发现肺孢子菌。该患儿诊断为（1）重症肺炎（耶氏肺孢子菌、巨细胞病毒感染），（2）急性呼吸窘迫综合征，（3）肾病综合征。予复方新诺明、更昔洛韦抗感染治疗和呼吸支持治疗，呼吸困难仍进行性加重，并出现张力性气胸，有创呼吸机治疗第13天予体外膜肺氧合（ECMO）支持。ECMO期间经复方新诺明、更昔洛韦、利奈唑胺等抗感染、抗凝、镇静、营养和呼吸道综合管理，于72 d后成功撤离ECMO，住院时间共134 d。出院随访6个月肺部CT明显好转，无脏器功能不全。耶氏肺孢子菌肺炎对免疫低下的患儿是一种潜在的威胁生命的感染，ECMO的应用能有效改善重度呼吸窘迫综合征患儿的预后。

Abstract:

A boy, aged 6 years and 11 months, was admitted due to nephrotic syndrome for 2 years, cough for 1 month, and shortness of breath for 15 days. The boy had a history of treatment with hormone and immunosuppressant. Chest CT after the onset of cough and shortness of breath showed diffuse ground-glass opacities in both lungs. Serum (1,3)-beta-D glucan was tested positive, and the nucleic acid of cytomegalovirus was detected in respiratory secretions. After the anti-fungal and anti-viral treatment, the child improved temporarily but worsened again within a short period of time. Pneumocystis jirovecii was identified by Gomori's methenamine silver staining in bronchoalveolar lavage fluid. The child was diagnosed with severe pneumonia (Pneumocystis jirovecii and cytomegalovirus infection), acute respiratory distress syndrome, and nephrotic syndrome. After anti-infective therapy with sulfamethoxazole/trimethoprim and ganciclovir and respiratory support, the child still experienced progressive aggravation of dyspnea and tension pneumothorax, and extracorporeal membrane oxygenation (ECMO) was given on day 13 of invasive ventilation. Anti-infective therapy with sulfamethoxazole/trimethoprim, ganciclovir, and linezolid, anticoagulation therapy, sedation therapy, nutrition, and comprehensive management of the respiratory tract were given during ECMO. The child was successfully weaned from ECMO after 72 days, resulting in a length of hospital stay of 134 days. The child was followed up for 6 months after discharge, and there was a significant improvement on lung CT, without organ dysfunction. It is concluded that Pneumocystis jirovecii pneumonia is a potential lifethreatening infection for children with low immunity, and that ECMO can effectively improve the prognosis of children with severe respiratory distress syndrome.