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儿童重症免疫性血小板减少症缓解的预测因素

Predictors of Remission in Severe Childhood Immune Thrombocytopenia.

作者信息

Cheng Chao-Neng, Yang Yuan-Ning, Yeh Yun-Hsuan, Chen Li-Wen, Chen Jiann-Shiuh, Lin Yung-Chieh

机构信息

Department of Pediatrics, National Cheng Kung University Hospital, College of Medicine, National Cheng-Kung University, Tainan 704302, Taiwan.

Department of Pediatrics, College of Medicine, National Cheng-Kung University, Tainan 701401, Taiwan.

出版信息

Diagnostics (Basel). 2023 Jan 17;13(3):341. doi: 10.3390/diagnostics13030341.

Abstract

Childhood immune thrombocytopenia (ITP; platelet count < 100 × 10/L) is the most common bleeding disorder in children. A total of 3-5% of children with ITP face a greater risk of bleeding, resulting in significant morbidity and mortality. Childhood ITP is often benign and self-limited; however, children with severe ITP (platelet count < 30 × 10/L) require investigation and monitoring. In addition, 20% of ITP patients may not go into remission (platelet counts < 100 × 10/L by 12 months after diagnosis) and may develop chronic ITP. The early identifying predictors associated with the resolution of severe ITP at the time of diagnosis may be helpful for family guidance. However, there is still controversy about the associations between the clinical factors at the time of initial diagnosis and the definitions of disease remission assessed at different timepoints after diagnosis. This retrospective study aimed to analyze the shared clinical factors among the disease remission definitions at three arbitrarily set timepoints-3, 6, and 12 months after diagnosis. This study retrieved records for hospitalized children aged under 18 years and diagnosed with ITP from the hospital registry in a tertiary university hospital. Clinical variables were recorded by reviewing the medical records with structured data entry for ITP admission. The serial follow-up platelet counts within 12 months after diagnosis were recorded. The times of ITP remission were identified by experienced pediatric hematologists. Patients with mild-form ITP (platelet counts ≥ 30 × 10/L) at diagnosis or who were lost to follow-up within 3 months were excluded. From 1988 to 2019, 546 children were enrolled, and a total of 497 children with severe ITP were included in the further analysis. In total, one (0.2%) died of an intracranial hemorrhage, 363 (73.2%) children went into remission at 3 months, 40 (8.1%) went into remission between 6 and 12 months, and 104 (20.9%) developed chronic ITP. The shared significant predictors for remission by the third, sixth, and twelfth months included pre-adolescent age (<10 years) at diagnosis, abrupt onset (duration of symptoms prior to admission ≤ 2 weeks), and speedy recovery (platelet count > 100 × 10/L at 1 month post diagnosis). ITP patients with positive viral serology tests or vaccination within 4 weeks had trends of delayed remission. In conclusion, diagnosis before preadolescent age, abrupt onset, and speedy recovery may share favorable factors for the remission of childhood ITP assessed at different timepoints.

摘要

儿童免疫性血小板减少症(ITP;血小板计数<100×10⁹/L)是儿童中最常见的出血性疾病。共有3%-5%的ITP患儿面临更高的出血风险,导致显著的发病率和死亡率。儿童ITP通常为良性且具有自限性;然而,重度ITP患儿(血小板计数<30×10⁹/L)需要进行检查和监测。此外,20%的ITP患者可能无法缓解(诊断后12个月血小板计数<100×10⁹/L)并可能发展为慢性ITP。诊断时与重度ITP缓解相关的早期识别预测因素可能有助于为家庭提供指导。然而,关于初始诊断时的临床因素与诊断后不同时间点评估的疾病缓解定义之间的关联仍存在争议。这项回顾性研究旨在分析在诊断后3、6和12个月这三个任意设定时间点的疾病缓解定义中共同的临床因素。本研究从一所三级大学医院的医院登记处检索了18岁以下确诊为ITP的住院儿童的记录。通过审查ITP入院结构化数据录入的病历记录临床变量。记录诊断后12个月内的系列随访血小板计数。由经验丰富的儿科血液学家确定ITP缓解时间。诊断时为轻度ITP(血小板计数≥30×10⁹/L)或在3个月内失访的患者被排除。从1988年到2019年,共纳入546名儿童,共有497名重度ITP患儿纳入进一步分析。总共1名(0.2%)死于颅内出血,363名(73.2%)儿童在3个月时缓解,40名(8.1%)在6至12个月时缓解,104名(20.9%)发展为慢性ITP。在第三个月、第六个月和第十二个月缓解的共同显著预测因素包括诊断时青春期前年龄(<10岁)、起病急(入院前症状持续时间≤2周)和恢复快(诊断后1个月血小板计数>100×10⁹/L)。病毒血清学检测阳性或在4周内接种疫苗的ITP患者有缓解延迟的趋势。总之,青春期前年龄前诊断、起病急和恢复快可能是在不同时间点评估的儿童ITP缓解的共同有利因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0442/9914323/710ea101b230/diagnostics-13-00341-g001.jpg

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