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疾病发病年龄是特发性肺含铁血黄素沉着症患儿身体生长发育的独立危险因素。

Age at disease onset is an independent risk factor for physical growth of children with idiopathic pulmonary hemosiderosis.

作者信息

Li Siyun, Li Tingting, Zhou Lanzhi, Mou Dandan, Sun Meng

机构信息

Department of Infants, Qingdao Women and Children's Hospital, Qingdao, China.

Department of Children Health Care, Yantaishan Hospital, Qingdao, China.

出版信息

Transl Pediatr. 2025 Aug 31;14(8):1961-1973. doi: 10.21037/tp-2025-263. Epub 2025 Aug 27.

Abstract

BACKGROUND

Idiopathic pulmonary hemosiderosis (IPH), a pediatric pulmonary disorder requiring prolonged corticosteroid therapy raises concerns about growth impairment. While corticosteroid-induced stunting is well-documented, its specific impact on the height of IPH patients remains unclear. This study aimed to evaluate the impact of corticosteroid exposure on height outcomes and identify key predictors of growth restriction in children with IPH.

METHODS

In this retrospective cohort study, 45 IPH patients [2018-2023] were stratified into normal-stature (n=37) and short-stature (n=8) groups based on anthropometric criteria. Multivariable Cox regression and comparative analyses assessed age at onset, diagnostic delay, corticosteroid duration, nutritional parameters, pulmonary function, and growth hormone (GH) therapy efficacy.

RESULTS

Short-stature patients demonstrated an earlier disease onset (P=0.004) and prolonged diagnostic delay (P=0.002). A younger age at onset independently predicted impaired height growth [hazard ratio (HR) =0.435, 95% confidence interval (CI): 0.206-0.920, P=0.02], while corticosteroid duration showed no association (P=0.45). Secondary analyses identified synergistic effects of anemia (P=0.02), malnutrition (P=0.045), and pulmonary fibrosis (P=0.008). GH therapy significantly improved height velocity (P<0.001) and bone metabolism markers (P<0.05).

CONCLUSIONS

Early IPH onset, rather than corticosteroid exposure, drives growth impairment in children. Timely diagnosis, nutritional optimization, and adjunctive GH therapy may mitigate height deficits, underscoring the significance of multidisciplinary management strategies.

摘要

背景

特发性肺含铁血黄素沉着症(IPH)是一种需要长期使用皮质类固醇治疗的儿科肺部疾病,这引发了人们对生长发育受损的担忧。虽然皮质类固醇导致发育迟缓已有充分记录,但其对IPH患者身高的具体影响仍不清楚。本研究旨在评估皮质类固醇暴露对身高结果的影响,并确定IPH儿童生长受限的关键预测因素。

方法

在这项回顾性队列研究中,45例IPH患者[2018 - 2023年]根据人体测量标准分为正常身高组(n = 37)和矮身材组(n = 8)。多变量Cox回归和比较分析评估了发病年龄、诊断延迟、皮质类固醇使用时间、营养参数、肺功能和生长激素(GH)治疗效果。

结果

矮身材患者发病较早(P = 0.004),诊断延迟时间较长(P = 0.002)。发病年龄较小独立预测身高增长受损[风险比(HR)= 0.435,95%置信区间(CI):0.206 - 0.920,P = 0.02],而皮质类固醇使用时间未显示出相关性(P = 0.45)。二次分析确定了贫血(P = 0.02)、营养不良(P = 0.045)和肺纤维化(P = 0.008)的协同作用。GH治疗显著改善了身高增长速度(P < 0.001)和骨代谢标志物(P < 0.05)。

结论

儿童IPH生长发育受损的原因是发病早,而非皮质类固醇暴露。及时诊断、营养优化和辅助GH治疗可能减轻身高缺陷,强调了多学科管理策略的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0b7c/12433028/2ba78275c696/tp-14-08-1961-f1.jpg

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