Bartram U, Thul J, Bauer J, Wössmann W, Schranz D
Department of Pediatric Cardiology, University Children's Hospital Giessen, Giessen, Germany.
J Heart Lung Transplant. 2006 Oct;25(10):1263-7. doi: 10.1016/j.healun.2006.06.011. Epub 2006 Sep 12.
Sarcoidosis is a granulomatous disease of unknown etiology and is only rarely seen in infants and children. We present the case of a 9-year-old boy who developed sarcoidosis with multi-organ involvement 9 years after cardiac transplantation for Shone complex. The patient was on immunosuppressive therapy with tacrolimus and mycophenolate mofetil. He presented with severe respiratory distress due to marked mediastinal lymphadenopathy and bilateral pulmonary infiltrates in association with fatigue, low-grade fever, hepatosplenomegaly and generalized lymphadenopathy. Lymph node histology showed non-caseating epitheloid cell granulomas and giant cells. Initialization of therapy with prednisolone resulted in prompt clinical recovery and resolution of all symptoms except for the development of mild pulmonary fibrosis. Tapering of the steroids led to recurrence of mediastinal lymphadenopathy 5 months after the initial disease, which responded to an increase in steroid dose. The clinical course, the medical management, and the possible role of immunosuppression in the etiology of the disease are discussed.
结节病是一种病因不明的肉芽肿性疾病，在婴幼儿中极为罕见。我们报告一例9岁男孩，他在因先天性心脏病Shone综合征接受心脏移植9年后发生了多器官受累的结节病。该患者正在接受他克莫司和霉酚酸酯的免疫抑制治疗。他因明显的纵隔淋巴结肿大和双侧肺部浸润出现严重呼吸窘迫，同时伴有疲劳、低热、肝脾肿大和全身淋巴结肿大。淋巴结组织学检查显示非干酪样上皮样细胞肉芽肿和巨细胞。使用泼尼松龙治疗后临床迅速恢复，除出现轻度肺纤维化外，所有症状均得到缓解。激素减量导致初始疾病5个月后纵隔淋巴结病复发，增加激素剂量后病情得到缓解。本文讨论了该疾病的临床过程、治疗管理以及免疫抑制在疾病病因学中可能的作用。
