对14例免疫功能基本正常儿童肺隐球菌病的再认识

目的深入认识儿童肺隐球菌病的表现、诊断进展、治疗和预后,以提高对儿童肺隐球菌病的诊断和治疗水平,防止发生全身播散。方法回顾分析2005-01—2006-06北京儿童医院收治的免疫功能基本正常的14例肺隐球菌病患儿的临床资料。结果14例患儿最小年龄为1岁8个月。症状主要为长期发热,咳嗽不重或持续时间较短暂。肺部查体12例正常,2例闻及干啰音和中水泡音?级廾飨灾卸局⒆础?例伴有间断腹痛,查体时有肝和(或)脾肿大。从发病到确诊至少23d,最长为5个月。实验室检查:13例外周血白细胞和中性粒细胞升高、C反应蛋白(CRP)明显升高。肺部影像学表现:11例患儿表现为双肺弥漫或散在小结节影,并有胸膜下分布。3例未发生肺外播散,11例伴有肺外隐球菌病(播散性隐球菌病)。病原学检查:8例找到隐球菌孢子。12例进行血清隐球菌荚膜多糖抗原检查,仅1例阴性,其余2例单独肺隐球菌病患儿以及9例播散性隐球菌病患儿全部阳性。动物接触史:6例有接触鸽子史,5例无动物接触史,3例接触家鸡。在11例伴有肺外隐球菌病的患儿中,9例静脉联合应用二性霉素B和氟康唑等治疗,病情控制,改为口服氟康唑继续治疗。1例在确诊前死亡,未行抗真菌治疗。在3例单独的肺隐球菌病患儿中,2例静脉应用二性霉素B;1例静脉应用氟康唑治疗病情控制,改为口服氟康唑继续治疗。结论儿童肺隐球菌病可发生于免疫功能正常的儿童,可无动物接触史,尽管长期发热、外周血白细胞和中性粒细胞升高、CRP升高,无特异性,但临床进展缓慢,咳嗽不重、无明显中毒症状、嗜酸性粒细胞升高,IgE升高是其相对特异的临床表现。双肺弥漫或散在小结节影,和(或)纵隔、肺门淋巴结肿大,为影像学表现。除隐球菌培养外,血肺隐球菌抗原检测有助于诊断。二性霉素B和(或)氟康唑治疗可控制隐球菌病。

Pulmonary cryptococcosis in immunocompetent children

Objective To elucidate pulmonary cryptococcosis in immunocompetent children and enhance the knowledge about it.Methods Pulmonary cryptococcosis in 14 children were reviewed for host immune status,symptoms and signs,radiographic patterns,diagnostic procedures such as culture and serum cryptococcal antigen,treatment,and follow-up.Results The 14 children with pulmonary cryptococcosis had normal Ig and CD series.The presenting symptom in all patients was persistent high fever(>3w),which was associated with mild and temporal cough in 11 cases.WBC,neutrophil counts and CRP were elevated in 13 patients.Seven patients had esophilia.Moist rales in lung exsited in 2 cases.There were no abnormalities in lung on physical exam.The common chest CT findings in 11 patients were poor-defined small nodules,in diffuse distribution(n=7) or scatter distribution(n=4),localized in parenchyma and subpleura;associated findings included hilar or mediastinal lymphadenopathy(n=4),segmental consolidation(n=2).The predominant CT finding in another 3 patients was mediastinal or hilar lymphadenopathy,confluent to masses(n=2),associated alveolar infiltration and scatter small nodules(n=2).No pleural effusion,cavitation and calcification were present in all patients.Three patients had no evidence of dissemination,but another 11 patients disseminated to other organs.Among them,10 cases had hepatic or spleen and abdominal lymph node cryptococcosis and 4 of 9 also had meningitis.One case was with complicated meningitis.Spore was found in mediastinal node(n=1),hepatic lesion and abdominal lymph node(n=2),cervical node(n=3),CSF(n=4).Culture was performed in sputum or BALF in 10 patients and 1 case had positive culture in sputum(2/9) and 1 case in BALF.A serum cryptococcal antigen(sCRAG) was investigated in 12 patients(12/14).sCRAG was positive in all cases with disseminated cryptococcosis and in 2 of 3 cases without dissemination.A negative sCRAG was observed in 1 patient without dissemination.Amphotericin B and/or fluconazole treatment controlled pulmonary cryptococcosis and complicated disease.Conclusion Pulmonary cryptococcosis can occur in immunocompetent children.High fever associated with mild and temporal cough,elevated WBC,Neutrophil,CRP and esophilia are manifestations.The most common CT finding in pulmonary small nodules,or hilar or mediastinal lymphadenopathy.Pulmonary cryptococcosis should be considered in the differential diagnosis of pulmonary nodules,alveolar infiltration and lymphadenopathy even if in immunocompetent children.The sensitivity of sCRAG was higher than culture.A positive sCRAG is very helpful to diagnosis and might imply dissemination.Amphotericin B and /or fluconazole treatment should be given to all patients and try to prevent pulmonary cryptococcosis from disseminating.