Abstract:

Objective　To analyze the characteristics of disseminated penicillium marneffei（PSM） in children and to deepen the understanding of PSM in the context of non-HIV. Methods　The clinical data，treatment program and prognosis of 15 children were retrospectively analyzed，who were diagnosed with non-HIV disseminated PSM in the First Affiliated Hospital of Guangzhou Medical University from Jan. 2005 to June 2016. Results　The 15 children（male∶female＝9∶6） had a median age of 23 months with a range of 3 months to 4 years and 10 months of age. All of them had clinical manifestations of fever and hepatomegaly on admission，which were often associated with cough，tachypnea，splenomegaly and lymphadenectasis. ESR was elevated by 93.3%（14/15） in laboratory tests，80%（8/10） was positive in fungal G tests，and 87.5%（7/8） in fungal GM tests. Chest imaging studies revealed that the lungs were all involved and showed various forms. Bone marrow culture and lymph node biopsy showed the highest positive rate of PM，more than 90%. The prognosis was related to the duration of the disease and  anti-fungal treatment. The duration of the death group（n＝7） was significantly longer than that of the cured group（n＝8）（P＜0.05）. The duration of anti-fungal treatment  for death groups was less than 2 weeks with the main death reason of septic shock and multiple organ failure. The cured group was given amphotericin B or voriconazole intravenously 2-4 weeks and later it was changed to itraconazole for oral maintenance；there was no recurrence after six months of follow-up. Conclusion　Non-HIV disseminated PSM in children occurs more often in infants under 3 years of age，and clinical and laboratory diagnosis lack specificity. Multi-site culture or biopsy（especially bone marrow culture and lymph node biopsy） can help confirm the diagnosis. Patients with long course of disease without timely anti-fungal treatment are associated with infectious shock and multiple organ failure，which are the main cause of death.

Key words: child, penicillium marneffei, HIV, disseminated

摘要：

目的　总结非人类免疫缺陷病毒（HIV）感染相关儿童播散性马尔尼菲青霉菌病（penicillium marneffei，PSM）特点，提高对其的认识。 方法　回顾性分析广州医科大学附属第一医院儿科2005年1月至2016年6月诊断的15例非HIV感染相关播散性PSM患儿的临床资料、治疗方案及转归。 结果　15例患儿中，男∶女＝9∶6，中位年龄23个月（3个月至4岁10个月）。入院时均有发热、肝大，多伴有咳嗽气喘、脾大、淋巴结肿大。实验室检查红细胞沉降率升高93.3%（14/15），真菌G试验阳性80.0%（8/10），真菌GM试验阳性87.5%（7/8）。胸部影像学检查提示15例肺部均有累及，表现形态多样。骨髓培养和淋巴结组织活检马尔尼菲菌阳性率最高（＞90%）。预后与病程长短及抗真菌治疗疗程相关，死亡的7例患儿病程明显长于治愈的8例（P＜0.05），抗真菌治疗时间均＜2周，主要死亡原因是感染性休克及多脏器衰竭。治愈患儿采取两性霉素B或伏立康唑静滴2～4周后改为伊曲康唑口服维持，随访半年以上无复发。结论　儿童非HIV感染相关播散性PSM好发于婴幼儿，临床及实验室诊断缺乏特异性。同时进行多部位体液培养或组织活检（尤其是骨髓培养和淋巴结活检）有助于明确诊断。病程偏长、未进行及时抗真菌治疗的患儿容易合并感染性休克及多脏器衰竭，是导致死亡的主要原因。

关键词: 儿童, 马尔尼菲青霉菌病, 人类免疫缺陷病毒, 播散性