| 摘要: |
| ［摘要］ 目的 探讨非HIV相关儿童马尔尼菲篮状菌（TM）感染的临床特点、治疗和预后。方法 回顾性分析2015年10月至2023年10月广州医科大学附属第一医院儿科收治的9例HIV阴性TM感染患儿的临床资料，包括临床表现、肺部影像学、病原检测、组织病理特点、治疗和预后等。结果 9例非HIV相关TM感染患儿以男性为主，中位年龄为40(3～164)个月。主要临床表现为发热、咳嗽、浅表淋巴结大，皮疹、肝脾肿大、腹痛或腹泻少见。大部分患儿C反应蛋白、降钙素原以及血沉升高，伴有血红蛋白降低。8例TM患儿免疫球蛋白G、免疫球蛋白A有不同程度降低。胸部CT显示肺部弥漫性或实质性病变。痰/血培养TM阳性8例。6例经支气管镜肺组织病理活检（1例同时行淋巴结活检），其中2例肺组织病理为肉芽组织形成，1例淋巴结及肺组织细胞质内可见圆形或腊肠状伴有横隔的孢子样物质，其余3例肺组织病理为非特异性炎性病变组织。8例患儿行基因全外显子测序，5例检出明确致病基因。9例患儿初始均采用伏立康唑治疗，4例预后良好，2例改用两性霉素B治疗后好转，3例因病情进展死亡。结论 非HIV相关TM患儿起病隐匿，病程长，需注意原发病的追踪。血、肺泡灌洗液培养有助于尽早诊断TM感染，尽早进行抗真菌治疗有助于改善预后。 |
| 关键词: 儿童 马尔尼菲篮状菌感染 原发性免疫缺陷病 |
| DOI：10.3969/j.issn.1674-3806.2024.11.05 |
| 分类号: |
| 基金项目:广东省医学科学技术研究基金项目（编号：A2023345）；广东省基础与应用基础研究基金项目（编号：2021A1515220043） |
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| Clinical analysis of 9 cases of Talaromyces marneffei infection in non-HIV-associated children |
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LI Hongwei, WANG Yanhong, WU Shangzhi, CHEN Xiaowen, HU Yingjie, HUANG Zhanhang, LU Chengyu, CHEN Dehui
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Department of Pediatrics, the First Affiliated Hospital of Guangzhou Medical University, Guangdong 510120, China
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| Abstract: |
| ［Abstract］ Objective To explore the clinical features, treatment and prognosis of Talaromyces marneffei(TM) infection in non-HIV-associated children. Methods The clinical data of 9 HIV-negative pediatric patients with TM infection who were admitted to Department of Pediatrics, the First Affiliated Hospital of Guangzhou Medical University from October 2015 to October 2023 were retrospectively analyzed. The clinical data of the pediatric patients included clinical manifestations, lung imaging, pathogen detection, histopathological features, treatment and prognosis. Results Among the 9 non-HIV-associated pediatric patients with TM infection, the most patients were male, with a median age of 40(3-164)months. The main clinical manifestations of the pediatric patients included fever, cough, large superficial lymph nodes, skin rash, hepatosplenomegaly, abdominal pain or rare diarrhea. Elevated C-reactive protein, procalcitonin and erythrocyte sedimentation rate, with a decrease in hemoglobin were found in most of the pediatric patients. Immunoglobulin G and immunoglobulin A levels were reduced to varying degrees in 8 pediatric patients with TM infection. The results of chest computed tomography(CT) scan showed diffuse or solid lesions in the lungs of the pediatric patients. The TM culture of sputum/blood showed positive results in 8 pediatric patients. Six pediatric patients underwent bronchoscopic lung tissue biopsy(one of them undergoing lymph node biopsy simultaneously), and among the 6 pediatric patients, 2 pediatric patients had granulation tissue formation in lung tissue pathology, and 1 pediatric patient had circular or sausage-like and spore-like substance with transverse septate in lymph node and lung tissue cytoplasm, and the other 3 pediatric patients had non-specific inflammatory lesion tissues in lung tissue pathology. Eight pediatric patients underwent whole-exome sequencing, and 5 of them were found to have clear pathogenic genes. All the 9 pediatric patients were treated with intravenous voriconazole in the initial treatment, among whom 4 pediatric patients had a good prognosis, and 2 pediatric patients were improved after switching to amphotericin B treatment, and 3 pediatric patients died due to disease progression. Conclusion The onset of TM in non-HIV-associated pediatric patients is obscure and the course of the disease is long, so attention should be paid to the tracking of the primary disease in the pediatric patients. Blood and bronchoalveolar lavage fluid culture can help to diagnose TM infection as early as possible, and antifungal treatment as early as possible can improve the prognosis. |
| Key words: Children Talaromyces marneffei infection Primary immunodeficiency diseases |
