Chaudhary Mohammed Wajid, Sardana Kabir, Kumar Praveen, Dewan Vivek, Anand V K
Department of Pediatric Medicine, Lady Hardinge Medical College, New Delhi, India.
Indian J Pediatr. 2005 Jan;72(1):85.
Disseminated cryptococcosis is a rare and often fatal disease in children. The majority of cases usually occur in individuals with defective cell-mediated immunity, most commonly due to HIV infection. The authors here in report an 8-year-old girl from Nepal who presented with fever, cough, headache, lymphadenopathy, hepatosplenomegaly and cutaneous lesions. Lymph node biopsy revealed multiple granulomas composed of histiocytes and epitheliold cells along with numerous yeast forms of cryptococcus. Cultures of CSF, sputum and urine yielded cryptococcus neoformans. Surprisingly,the immune function in terms of T-cell number, CD4 : CD8 ratio, serum immunoglobulins and HIV serology was normal. After the diagnosis of disseminated cryptococcosis was established, the patient was treated with 5-fluorocytosine (100 mg/kg/day) for initial two weeks and amphotericin B (1 mg/kg/day) for 13 weeks. Patient responded well to the treatment with disappearance of presenting symptoms, cutaneous lesions, and lymphadenopathy, though she still had hepatosplenomegaly, which also decreased. Unfortunately, she developed loss of vision in 10th week of therapy. The patient was discharged on oral fluconazole (6 mg/kg/day) and no recurrence was found during the follow-up period of more than 9 months. This is the first case of disseminated cryptococcosis with no detectable immune deficit, from India.
播散性隐球菌病在儿童中是一种罕见且往往致命的疾病。大多数病例通常发生在细胞介导免疫功能缺陷的个体中,最常见的原因是HIV感染。本文作者报告了一名来自尼泊尔的8岁女孩,她出现发热、咳嗽、头痛、淋巴结病、肝脾肿大和皮肤病变。淋巴结活检显示多个肉芽肿,由组织细胞和上皮样细胞组成,伴有大量隐球菌酵母形式。脑脊液、痰液和尿液培养均培养出新型隐球菌。令人惊讶的是,其T细胞数量、CD4:CD8比值、血清免疫球蛋白和HIV血清学方面的免疫功能正常。在确诊播散性隐球菌病后,患者最初两周接受5-氟胞嘧啶(100mg/kg/天)治疗,随后13周接受两性霉素B(1mg/kg/天)治疗。患者对治疗反应良好,出现的症状、皮肤病变和淋巴结病消失,尽管仍有肝脾肿大,但也有所减轻。不幸的是,她在治疗第10周出现视力丧失。患者出院时口服氟康唑(6mg/kg/天),在超过9个月的随访期内未发现复发。这是印度首例无明显免疫缺陷的播散性隐球菌病病例。