PDF(Pubmed)
Abstract：
Rosai-Dorfman disease (RDD) is a rare, multisystemic, histiocytic disorder that usually presents with painless cervical lymphadenopathy. We describe a case of constrictive effusive pericarditis with congestive cardiac failure in a six-year-old child as an initial presentation of RDD. The child underwent pericardiectomy and was treated with steroids, following which the symptoms resolved entirely. While tuberculosis accounts for the majority of cases presenting with constrictive pericarditis in developing countries, the presentation of RDD can be easily missed if not kept in mind as a possibility. This case report documents the unique presentation of a rare disease.
摘要：
Rosai-Dorfman病（RDD）是一种罕见的疾病，多系统，组织细胞疾病，通常表现为无痛性颈淋巴结肿大。我们描述了一个6岁儿童的缩窄性渗出性心包炎伴充血性心力衰竭的病例，作为RDD的初步表现。这个孩子接受了心包切除术，并接受了类固醇治疗，之后症状完全缓解。虽然结核病占发展中国家缩窄性心包炎病例的大多数，RDD的呈现很容易错过，如果不记住作为一种可能性。此病例报告记录了罕见疾病的独特表现。
