Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Center for Pulmonary Imaging Research, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
Pediatr Pulmonol. 2019 Jun;54(6):822-827. doi: 10.1002/ppul.24301. Epub 2019 Mar 6.
Neuroendocrine cell hyperplasia of infancy (NEHI) is a rare pediatric interstitial lung disease (ILD). Distinct chest computed tomography (CT) define its radiographic appearance-specifically, ground-glass (GG) opacities most prominent in the right middle lobe (RML) and lingula. We sought to quantitatively validate this description and correlate radiologic findings with clinical presentation.
Twenty-one children with NEHI were identified retrospectively, alongside 10 age-matched controls without lung disease. Clinical histories were reviewed for NEHI subjects. Semiautomated image analysis was used to measure lung volume and density. A patient-specific Hounsfield unit threshold defining GG was developed to quantify GG and assess its distribution in each subject.
NEHI subjects had more GG than controls (37.9 ± 11.3% vs 14.0 ± 2.7%, P < 0.0001). The proportion of GG in the RML and lingula was greater in NEHI patients compared to controls (1.43 ± 0.37 vs 0.45 ± 0.21, P < 0.0001). GG preferentially involved the RML and lingula in 20/21 NEHI subjects. There was more GG distribution in NEHI subjects who were prescribed continuous oxygen compared with those using only nocturnal oxygen (45.7 ± 8.9% vs 29.3 ± 6.1%, P = 0.003).
We confirm the previously reported finding that most patients with childhood ILD and a distinctive pattern of GG distribution on CT scan are likely to have NEHI. The amount of GG may be a biomarker for severity of respiratory disease.
婴儿期神经内分泌细胞增生症（NEHI）是一种罕见的儿科间质性肺病（ILD）。独特的胸部计算机断层扫描（CT）定义了其放射影像学表现——特别是在右中叶（RML）和舌段最明显的磨玻璃（GG）混浊。我们试图定量验证这一描述，并将影像学发现与临床表现相关联。
回顾性地确定了 21 名患有 NEHI 的儿童，以及 10 名年龄匹配的无肺部疾病的对照组。对 NEHI 患者的临床病史进行了回顾。使用半自动图像分析来测量肺容量和密度。开发了一个特定于患者的亨氏单位阈值来定量 GG，并评估其在每个患者中的分布。
NEHI 患者的 GG 比对照组多（37.9±11.3%比 14.0±2.7%，P<0.0001）。与对照组相比，NEHI 患者的 RML 和舌段的 GG 比例更大（1.43±0.37比 0.45±0.21，P<0.0001）。在 20/21 的 NEHI 患者中，GG 优先累及 RML 和舌段。与仅使用夜间吸氧的患者相比，需要持续吸氧的 NEHI 患者的 GG 分布更多（45.7±8.9%比 29.3±6.1%，P=0.003）。
我们证实了之前报道的发现，即大多数患有儿童ILD 和 CT 扫描上具有独特 GG 分布模式的患者很可能患有 NEHI。GG 的量可能是呼吸疾病严重程度的生物标志物。
