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Etiology, clinical outcome, and laboratory features in children with neutropenia: analysis of 104 cases

机译:中性粒细胞减少症患儿的病因,临床结局和实验室特征:104例病例分析

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摘要

Neutropenia is not uncommon in childhood. The aim of our study was toudanalyze the underlying causes of neutropenia and to evaluate its clinical significance inuda series of children referred to our center.udMethods: One hundred and four consecutive children with neutropenia were enrolledudin this study. Clinical and laboratory features were analyzed.udResults: The majority of patients (63.5%) showed chronic neutropenia. Among alludchronic forms, the most frequent was chronic idiopathic neutropenia (CIN), followedudby autoimmune neutropenia (AIN). Congenital neutropenia was identified in 6udpatients. Acute neutropenia was mainly due to infections.Overall, at the time of firstuddetection, neutropenia was more frequently severe or moderate. One-third of ourudpatients who presented with severe neutropenia were ultimately diagnosed with a postinfectiousudacute form. Conversely, nearly half patients with CIN, AIN, or congenitaludneutropenia showed moderate/mild neutropenia at onset.Among patients with AINudand CIN, nearly half recovered between 7 months and 46 months and approximatelyudone-fourth experienced infectious episodes during follow-up. No significant differenceudwas noticed in terms of mean ANC between patients with and without remission,udneither between patients with and without infections.udConclusions: Our study confirms the great etiological heterogeneity of neutropenia inudchildren. We could not demonstrate a correlation between ANC level at onset and theudunderlying disorder, nor a correlation between mean ANC and duration ofudneutropenia or infectious episodes during follow-up. Neutropenia remains a diseaseof concern to pediatricians, requiring several laboratory investigations, prolongedfollow-up, and, in few cases, advanced molecular methods.
机译:中性粒细胞减少症在儿童时期并不罕见。我们的研究目的是分析中性粒细胞减少症的根本原因,并评估其在我们中心转诊的一系列儿童中的临床意义。 ud方法:本研究共纳入104例连续的中性粒细胞减少症儿童。结果:大多数患者(63.5%)表现出慢性中性粒细胞减少。在所有非历时性形式中,最常见的是慢性特发性中性粒细胞减少症(CIN),其次是自身免疫性中性粒细胞减少症(AIN)。在6名患者中发现了先天性中性粒细胞减少症。急性中性粒细胞减少症主要是由于感染引起的。总体而言,在首次发现时,中性粒细胞减少症的发生频率通常为严重或中度。患有严重中性粒细胞减少症的 udud患者中,最终有1/3被诊断为感染后 udacute形式。相反,将近一半的CIN,AIN或先天性嗜中性白血球减少症患者在发作时表现出中度/轻度中性粒细胞减少症。在AIN udand CIN的患者中,近一半在7个月至46个月之间恢复,约有1/4的患者在随访期间经历了感染-向上。在有缓解和无缓解的患者之间,以及在有感染和无感染的患者之间,ANC的平均ANC均无显着差异。 ud结论:我们的研究证实了中性粒细胞减少症在儿童中的巨大病因异质性。我们无法证明发作时的ANC水平与 udundering障碍之间的相关性,也无法证明平均ANC与随访期间 udneutropenia持续时间或传染性发作之间的相关性。中性粒细胞减少症仍然是儿科医生关注的疾病,需要进行一些实验室研究,长期随访以及在少数情况下需要先进的分子方法。

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