Lelii Mara, Patria Maria Francesca, Pinzani Raffaella, Tenconi Rossana, Mori Alessandro, Bonelli Nicola, Principi Nicola, Esposito Susanna
Pediatric Highly Intensive Care Unit, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy.
Pediatric Radiology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy.
Int J Environ Res Public Health. 2017 Sep 25;14(10):1113. doi: 10.3390/ijerph14101113.
: Chronic interstitial lung diseases in children (chILD) are a heterogeneous group of disorders that can represent a clinical challenge for pediatric pneumologists. Among them, neuroendocrine cell hyperplasia of infancy (NEHI) is a diffuse lung disease prevalent in the first years of life that spontaneously improves over time. The clinical presentation of NEHI is indistinguishable from other interstitial lung diseases, so a correct and non-invasive diagnosis by chest computed tomography (CT) without lung biopsy might not be simple. : An 8-month-old male infant presented with a history of chronic tachypnoea and dyspnoea since 6 months of age. The patient was born at term, with APGAR scores of 9 and 10 at 1 and 5 min, respectively. Since his second month of life, the patient suffered from abnormal breathing, which was characterized by mild tachypnoea and costal retractions that worsened during breastfeeding, crying, and respiratory infections. Bilateral inspiratory crackles, preferential to the lung bases, without oxygen desaturation were detected. A chest X-ray showed a diffuse over-inflation of the lungs, but laboratory tests did not reveal any abnormalities. High-resolution chest CT documented patchy areas of ground-glass opacity involving the right upper lobe, middle lobe, and lingula, and showed mosaic areas of air-trapping, suggesting a diagnosis of NEHI. The infant was discharged without therapy and gradually improved over time. At 1 year of age, the patient was eupnoeic and chest auscultation had normalized. : NEHI is an interstitial disease of infancy characterized by tachypnoea from the first months of life, with a good prognosis and for which a rational diagnostic approach is crucial for making a specific, early diagnosis. Initially, clinical suspicions can be confirmed with reasonable accuracy by a CT scan of the chest. Other more invasive and more expensive investigations should be reserved for selected cases that do not show a spontaneous, favourable clinical evolution.
儿童慢性间质性肺疾病(chILD)是一组异质性疾病,对儿科肺病专家而言可能构成临床挑战。其中,婴儿期神经内分泌细胞增生症(NEHI)是一种在生命最初几年中普遍存在的弥漫性肺部疾病,会随时间自发改善。NEHI的临床表现与其他间质性肺疾病难以区分,因此在不进行肺活检的情况下通过胸部计算机断层扫描(CT)进行正确且非侵入性的诊断可能并非易事。
一名8个月大的男婴自6个月大起就有慢性呼吸急促和呼吸困难的病史。该患儿足月出生,1分钟和5分钟时的阿氏评分分别为9分和10分。自出生第二个月起,患儿就出现呼吸异常,表现为轻度呼吸急促和肋间凹陷,在母乳喂养、哭闹及呼吸道感染时加重。检测到双侧吸气性啰音,以肺底部为主,无血氧饱和度下降。胸部X线显示肺部弥漫性过度充气,但实验室检查未发现任何异常。高分辨率胸部CT记录到右肺上叶、中叶及舌叶有斑片状磨玻璃影,并显示有空气潴留的马赛克样区域,提示诊断为NEHI。该婴儿未经治疗出院,随时间推移逐渐好转。1岁时,患儿呼吸正常,胸部听诊已恢复正常。
NEHI是一种婴儿期的间质性疾病,自出生头几个月起即表现为呼吸急促,预后良好,合理的诊断方法对于做出特异性的早期诊断至关重要。最初,通过胸部CT扫描可在合理程度上准确证实临床怀疑。其他更具侵入性且更昂贵的检查应仅用于那些未表现出自发性良好临床进展的特定病例。