Impact of Prognostic Nutritional Index on Clinical Outcomes in Hereditary Angioedema: A single center experience
Öz
Abstract
Background/Aims: Hereditary angioedema (HAE) is a rare genetic disorder (prevalence approximately 1:50,000) marked by recurrent episodes of severe swelling due to a deficiency in the C1 inhibitor (C1-INH) protein, affecting the complement and contact systems. This study aims to evaluate the impact of the Prognostic Nutritional Index (PNI) on clinical outcomes in HAE patients.
Methods: A single-center retrospective cohort of 60 patients was analyzed. Clinical characteristics and PNI were assessed, calculated using the formula: [Serum Albumin (g/L)] + [5 × Peripheral Blood Lymphocyte Count (×10⁹/L)]. Patients were stratified into low and high PNI groups based on a cut-off value of 51.725, derived from receiver operating characteristic (ROC) curve analysis (AUC = 0.902, p < 0.001). Statistical significance was set at p < 0.05.
Results: Low PNI was significantly associated with increased attack frequency (p < 0.001), extended diagnostic delays (p = 0.022), and greater laryngeal (p = 0.003) and gastrointestinal involvement (p = 0.005). Furthermore, low PNI was negatively correlated with monthly attack frequency (Spearman’s rho = -0.653, p < 0.001) and positively correlated with C1-INH function (Spearman’s rho = 0.408, p = 0.001).
Conclusions: These findings underscore the importance of incorporating nutritional evaluations into HAE management, as poor nutritional status may exacerbate disease severity. Further prospective studies are needed to validate these associations and explore nutritional interventions as a complementary strategy in HAE treatment.
Anahtar Kelimeler
Hereditary angioedema (HAE), Prognostic Nutritional Index (PNI), Clinical outcomes, Nutritional status, Attack frequency
Kaynakça
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