Sims D G
Arch Dis Child. 1977 Jun;52(6):433-40. doi: 10.1136/adc.52.6.433.
Over a 29-year period, 43 cases of histiocytosis X presented in children under the age of 12 years. 29 patients (67%) have survived, and of these, 15 (52%) have a detectable disability. It was confirmed that young age at presentation and evidence of soft tissue involvement were associated with a worse prognosis. The majority of deaths were associated with pulmonary involvement. 14 patients developed diabetes insipidus. 5 of the surviving adults have heights below the 3rd centile. Puberty usually occurred at a normal age. Follow-up studies on 12 survivors showed no evidence of residual abnormality of haematology, deficient lymphocyte function, or yeast opsonization. HLA typing showed no unusual pattern. Mild carbon monoxide diffusion deffects were present in 4 patients and other abnormalities were detected on lung function tests.
在29年期间,43例朗格汉斯细胞组织细胞增多症出现在12岁以下儿童中。29名患者(67%)存活,其中15名(52%)有可检测到的残疾。已证实发病时年龄小和软组织受累证据与预后较差有关。大多数死亡与肺部受累有关。14名患者发生尿崩症。5名存活的成年人身高低于第3百分位数。青春期通常在正常年龄出现。对12名幸存者的随访研究未发现血液学残留异常、淋巴细胞功能缺陷或酵母调理作用的证据。HLA分型未显示异常模式。4名患者存在轻度一氧化碳弥散缺陷,肺功能测试检测到其他异常。