Department of Pediatric Pneumonology and Allergy, Medical University of Warsaw, Warsaw, Poland.
Department of Epidemiology and Biostatistics, Medical University of Warsaw, Warsaw, Poland.
Pediatr Pulmonol. 2024 Nov;59(11):2885-2890. doi: 10.1002/ppul.27148. Epub 2024 Jun 27.
Neuroendocrine cell hyperplasia of infancy (NEHI) is a form of childhood interstitial lung disease of unknown origin associated with hyperplasia of pulmonary neuroendocrine cells (PNECs). Diagnosis is based on the characteristic clinical picture and typical radiological imaging, and, in some cases, on lung biopsies. To date, no biochemical indicators of the disease have been identified.
We aimed to determine biomarkers that could be useful in the management of children diagnosed with NEHI.
Patients with NEHI and healthy children were enrolled. Concentrations of serum biomarkers secreted by PNECs (calcitonin gene-related peptide and gastrin-releasing peptide) and biomarkers of the destruction of alveolar capillary membrane (surfactant proteins A and D [SP-A and SP-D]; glycoprotein Krebs von den Lungen-6 [KL-6]; metalloproteinases 7 and 9 [MMP-7 and MMP-9]; tissue inhibitor of metalloprotease 1) were measured.
Fifty-two children with NEHI and 23 healthy children were included in the study. The median age of children with NEHI was 3.9 years. There were no differences in serum levels of biomarkers secreted by PNECs between groups. KL-6 levels were significantly higher in children with NEHI than in healthy ones (median 119.6 vs. 92.1 U/mL, p = 0.003); however, concentrations of KL-6 were low in both groups. No significant differences existed between groups for the remaining biomarkers associated with the destruction of the alveolar-capillary membrane.
Measurement of serum biomarkers released by PNECs and those associated with the destruction of the alveolar-capillary membrane does not appear to be useful in the management of children with NEHI.
婴儿神经内分泌细胞增生症（NEHI）是一种病因不明的儿童间质性肺疾病，其特征为肺神经内分泌细胞（PNEC）增生。诊断基于特征性临床表现和典型影像学表现，在某些情况下还需要进行肺活检。迄今为止，尚未发现该疾病的生化标志物。
我们旨在确定可用于管理诊断为 NEHI 的儿童的生物标志物。
招募了 NEHI 患儿和健康儿童。测量了由 PNEC 分泌的血清生物标志物（降钙素基因相关肽和胃泌素释放肽）和肺泡毛细血管膜破坏的生物标志物（表面活性蛋白 A 和 D [SP-A 和 SP-D]；肺表面活性剂相关蛋白-6 [KL-6]；基质金属蛋白酶 7 和 9 [MMP-7 和 MMP-9]；基质金属蛋白酶组织抑制剂 1）的浓度。
本研究纳入了 52 例 NEHI 患儿和 23 例健康儿童。NEHI 患儿的中位年龄为 3.9 岁。两组间由 PNEC 分泌的生物标志物的血清水平无差异。NEHI 患儿的 KL-6 水平显著高于健康儿童（中位数 119.6 vs. 92.1 U/mL，p=0.003）；然而，两组的 KL-6 浓度均较低。与肺泡毛细血管膜破坏相关的其余生物标志物在两组间无显著差异。
PNEC 释放的血清生物标志物和与肺泡毛细血管膜破坏相关的生物标志物的测量似乎对 NEHI 患儿的管理无用。
