Kuo Christin S, Young Lisa R
aDivision of Pediatric Pulmonary Medicine, Lucile Packard Children's Hospital, Stanford University School of Medicine, Stanford, California bDivision of Pediatric Pulmonary Medicine, Monroe Carell Jr Children's Hospital at Vanderbilt cDivision of Allergy, Pulmonary, and Critical Care, Department of Medicine, Vanderbilt University School of Medicine, Nashville, Tennessee, USA.
Curr Opin Pediatr. 2014 Jun;26(3):320-7. doi: 10.1097/MOP.0000000000000094.
There has been tremendous progress in the approach to childhood interstitial lung diseases (chILD), with particular recognition that interstitial lung disease (ILD) in infants is often distinct from the forms that occur in older children and adults. Diagnosis is challenging because of the rarity of ILD and the fact that the presenting symptoms of ILD often overlap those of common respiratory disorders. This review summarizes the newly published recommendations for diagnosis and management, and highlights the recent scientific advances in several specific forms of chILD.
Clinical practice guidelines emphasize the role for chest computed tomography, genetic testing, and lung biopsy in the diagnostic evaluation of children with suspected ILD. Recent studies have better defined the characteristics and molecular understanding of several different forms of ILD, including neuroendocrine cell hyperplasia of infancy and ILD, due to mutations in genes affecting surfactant production and metabolism. Despite significant progress, definitive therapies are often lacking.
chILD encompasses a collection of rare, diffuse lung diseases. Timely recognition of children with suspected ILD and initiation of appropriate diagnostic evaluations will facilitate medical management. Systematic approaches to clinical care and further studies are needed to improve the outcomes of children with these rare disorders.
儿童间质性肺疾病（chILD）的诊疗方法取得了巨大进展，尤其是认识到婴儿期的间质性肺疾病（ILD）通常与大龄儿童及成人的间质性肺疾病形式不同。由于ILD罕见，且ILD的首发症状常与常见呼吸系统疾病的症状重叠，因此诊断具有挑战性。本综述总结了新发布的诊断和管理建议，并重点介绍了几种特定形式的chILD最近的科学进展。
临床实践指南强调胸部计算机断层扫描、基因检测和肺活检在疑似ILD儿童诊断评估中的作用。最近的研究更好地明确了几种不同形式ILD的特征和分子认识，包括婴儿期神经内分泌细胞增生症和因影响表面活性剂产生和代谢的基因突变导致的ILD。尽管取得了重大进展，但往往缺乏确切的治疗方法。
chILD包括一系列罕见的弥漫性肺部疾病。及时识别疑似ILD的儿童并启动适当的诊断评估将有助于医疗管理。需要系统的临床护理方法和进一步的研究来改善这些罕见疾病患儿的治疗效果。
