Hepatocyte Growth Factor in Children of Primary Ciliary Dyskinesia and Its Relation to Disease Severity. A case-control study.

Abstract

Background: Primary Ciliary Dyskinesia (PCD) is a rare genetic disorder causing impaired mucociliary clearance, recurrent respiratory infections, and progressive bronchiectasis. Hepatocyte Growth Factor (HGF) plays a role in lung repair and inflammation, but its relationship with clinical and radiological severity in pediatric PCD remains unclear. Objective: To evaluate serum and bronchoalveolar lavage (BAL) hepatocyte growth factor (HGF) levels in children with genetically confirmed primary ciliary dyskinesia (PCD) compared with healthy controls, and to investigate their associations with clinical, functional, and radiological measures of disease severity. Methods: This case-control study was conducted from July 2023 to June 2024 at Ain Shams University Pediatric Tertiary Hospital. Thirty children aged 1-18 years with genetically confirmed (PCD) were included and compared with 30 age- and sex-matched healthy controls. All patient group underwent clinical, functional, and radiological evaluation. Spirometry Pulmonary function was assessed while radiological severity was evaluated by the modified Bhalla score. Nutritional status was assessed by body mass index (BMI) Z-scores, and airway colonization was documented. Serum and bronchoalveolar lavage (BAL) HGF levels were measured in all participants. The relationship between HGF levels and disease severity was evaluated using both univariate and multivariate linear regression analyses. A p-value < 0.05 was considered statistically significant. Results: In multivariable analysis, the modified Bhalla score was identified as an independent predictor of both BAL and serum HGF levels (P = 0.004 and P = 0.010, respectively), while BAL cell count independently predicted BAL HGF (P = 0.048). Serum HGF showed a significant negative correlation with FVC (r = -0.446, P = 0.029) and FEV₁ (r = -0.418, P = 0.042). BAL HGF was negatively correlated with FVC (r = -0.479, P = 0.018). Conclusions: HGF levels in both serum and BAL fluid are significantly associated with disease severity in children with primary ciliary dyskinesia.