Children Hospital, Pediatric Pulmonology and Allergology Unit CHU Toulouse, Toulouse, France.
Children Hospital, Pediatric Pulmonology and Allergology Unit CHU Toulouse, Toulouse, France.
J Pediatr. 2020 Feb;217:158-164.e1. doi: 10.1016/j.jpeds.2019.10.014. Epub 2019 Nov 21.
To collect all published cases up to January 2019 of pulmonary alveolar microlithiasis (PAM) in patients age 5 years and under and to compare their characteristics with those of the 1022 cases in the most recent all-age cohort published in 2015.
We identified 28 cases of PAM worldwide in children age 5 years and under, accounting for only 2%-3% of all cases.
Children seem more frequently symptomatic, notably with more cough and severe acute respiratory failure, but had no reported extrapulmonary manifestation. Children with PAM evidenced less typical radiologic findings, with frequent ground glass opacities not reported in adult cases and milder calcifications as less frequent, smaller, and mainly restricted to the lower lobes.
PAM remains an uncommon diagnosis in young children, as symptoms and radiologic findings are less specific. Physicians should be aware to look for calcifications in chest computed tomography at mediastinal window and avoid elution of the bronchoalveolar lavage to find microliths. Collecting longitudinal data through an international registry would help in characterizing PAM to predict disease progression and plan lung transplantation.
收集截至 2019 年 1 月的所有 5 岁及以下儿童肺泡微石症(PAM)病例,并将其特征与 2015 年发表的最新全年龄段 1022 例病例进行比较。
我们在全球范围内确定了 28 例 5 岁及以下儿童的 PAM 病例,仅占所有病例的 2%-3%。
儿童似乎更常出现症状,尤其是咳嗽和严重急性呼吸衰竭,但无报道的肺外表现。PAM 患儿的影像学表现不那么典型,常见磨玻璃影在成人病例中未见报道,钙化程度较轻,频率较低,体积较小,主要局限于下叶。
PAM 在幼儿中仍然是一种罕见的诊断,因为症状和影像学表现不具特异性。医生应注意在纵隔窗胸部 CT 上寻找钙化,避免洗脱支气管肺泡灌洗液以寻找微石。通过国际登记处收集纵向数据有助于对 PAM 进行特征描述,以预测疾病进展并计划肺移植。