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

发病年龄是特发性肺含铁血黄素沉着症患儿体格发育的独立危险因素。

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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.

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