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本文报告16例小儿组织细胞增生症X,其中勒雪氏病(LS)10例,韩薛柯氏病(HSC)5例及介于两者之间的中间型1例。发病年龄LS 1个月～2岁3个月,HSC 1岁11个月～12岁,中间型为5岁。主要临床表现LS 有发热、皮疹、肝、脾和淋巴结肿大,HSC 有头部肿块或缺损、突眼和尿崩症,中间型有发热和肝脾肿大。LS 和HSC 各有4例以临床及X 线为诊断依据,病理检查确诊LS6例,HSC 及中间型各1例。复习有关文献对本病的病因、发病年龄、特殊类型、临床表现、X 线检查、诊断、治疗和预后等作了简要的讨论。
In this paper, 16 cases of pediatric histiocytosis X were reported. Among them, 10 cases of Leech’s disease (LS), 5 cases of Han Xue Xue Kesi disease (HSC) and 1 case of intermediate type between them. The age of onset LS 1 month to 2 years 3 months, HSC 1 year old 11 months to 12 years old, middle type is 5 years old. The main clinical manifestations of LS have fever, rash, liver, spleen and lymph nodes, HSC head lumps or defects, exophthalmos and diabetes insipidus, fever and hepatosplenomegaly. LS and HSC each have 4 cases based on clinical and X-ray diagnosis, pathological examination confirmed LS6 cases, HSC and intermediate type in 1 case. Review the literature on the etiology, age of onset, special types, clinical manifestations, X-ray examination, diagnosis, treatment and prognosis were briefly discussed.