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[儿童朗格汉斯细胞组织细胞增生症的治疗]

[Treatment of langerhans cell histiocytosis in children].

作者信息

Bilić Ernest, Bojanić Katarina, Pavlović Maja, Konja Josip, Femenić Ranka, Dapić Tomislav, Antabak Anko, Anticević Darko, Murat-Susić Slobodna, Husar Karmela, Potocki Kristina, Rajić Ljubica

机构信息

Klinika za pedijatriju Medicinskog fakulteta Sveuclista u Zagrebu, KBC Zagreb.

出版信息

Lijec Vjesn. 2011 Nov-Dec;133(11-12):376-84.

PMID:22329293
Abstract

Langerhans' cell histiocytosis (LCH) is a disease characterised by pathologic accumulation and proliferation of histiocytes, cells from the monocyte-macrophage system, in various tissues and organs. In this retrospective study we analyzed patients charts treated in the Department of pediatric hematology and oncology at the University Hospital Zagreb with the diagnosis of LCH. Twenty-two children were diagnosed between January 1st 1996 and December 31st 2010, and all were treated with chemotherapy. 19 patients survived (86%) and the remaining 3 (14%), all under the age of 2 with multisystem disease, died. At the time of diagnosis 12 children (55%) presented with single-system disease, the most common were bone lesions in 8 children (36%). All children were treated according to protocols LCH-I and LCH -III. Eight children had mild complications of treatment and the disease itself. Diabetes insipidus remains in 4 children.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种以组织细胞(来自单核巨噬细胞系统的细胞)在各种组织和器官中病理性积聚和增殖为特征的疾病。在这项回顾性研究中,我们分析了萨格勒布大学医院儿科血液学和肿瘤学部门诊断为LCH的患者病历。1996年1月1日至2010年12月31日期间诊断出22名儿童,所有患者均接受了化疗。19名患者存活(86%),其余3名(14%)均为2岁以下患有多系统疾病的儿童,死亡。诊断时,12名儿童(55%)表现为单系统疾病,最常见的是8名儿童(36%)出现骨病变。所有儿童均按照LCH-I和LCH-III方案进行治疗。8名儿童出现了治疗及疾病本身的轻度并发症。4名儿童仍患有尿崩症。

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