Herediter Anjiyoödemde Prognostik Nutrisyonel İndeksin Klinik Sonuçlar Üzerine Etkisi: Tek Merkez Deneyimi

Year 2025, , 285 - 292, 30.04.2025

Mehmet Emin Gerek , Fatih Çölkesen , Tuğba Önalan , Fatma Arzu Akkuş , Recep Evcen , Mehmet Kılınç , Selim Kahraman , Şevket Arslan

https://doi.org/10.54005/geneltip.1600277

Abstract

Özet
Giriş/Amaç: Herediter anjiyoödem (HAE), C1 inhibitör (C1-INH) protein eksikliğine bağlı olarak kompleman ve kontakt sistemlerini etkileyen, yaklaşık 1:50.000 prevalans ile seyreden nadir bir genetik hastalıktır. Hastalık, tekrarlayan ciddi şişlik atakları ile karakterizedir. Bu çalışmanın amacı, Prognostik Nutrisyonel İndeksin (PNI) HAE hastalarındaki klinik sonuçlar üzerindeki etkisini değerlendirmektir.
Yöntem: Tek merkezli retrospektif bir kohort çalışması kapsamında 60 hasta analiz edilmiştir. Hastaların klinik özellikleri ve PNI değerleri incelenmiş, PNI şu formülle hesaplanmıştır: [Serum Albümini (g/L)] + [5 × Periferik Kan Lenfosit Sayısı (×10⁹/L)]. Alıcı çalıştırma özellik (ROC) eğrisi analiziyle belirlenen 51.725 kesim değeri esas alınarak hastalar düşük ve yüksek PNI gruplarına ayrılmıştır (AUC = 0.902, p <0.001). İstatistiksel anlamlılık seviyesi p <0.05 olarak kabul edilmiştir.
Bulgular: Düşük PNI, artmış atak sıklığı (p <0.001), uzamış tanı gecikmesi (p = 0.022), ve daha sık larinks (p = 0.003) ve gastrointestinal tutulum (p = 0.005) ile anlamlı olarak ilişkilendirilmiştir. Ayrıca, düşük PNI ile aylık atak sıklığı arasında negatif bir korelasyon (Spearman rho = -0.653, p < 0.001) ve C1-INH fonksiyonu ile pozitif bir korelasyon (Spearman rho = 0.408, p = 0.001) bulunmuştur.
Sonuç: Bu bulgular, HAE yönetiminde beslenme değerlendirmelerinin önemini vurgulamaktadır. Zayıf beslenme durumu, hastalık şiddetini artırabilir. Bu ilişkilerin doğrulanması ve HAE tedavisinde tamamlayıcı bir strateji olarak beslenme müdahalelerinin etkilerinin araştırılması için ileriye dönük çalışmalara ihtiyaç vardır.

Keywords

Herediter anjiyoödem (HAE), Prognostik Nutrisyonel İndeks (PNI), Beslenme durumu, Atak sıklığı, Klinik sonuçlar

Impact of Prognostic Nutritional Index on Clinical Outcomes in Hereditary Angioedema: A single center experience

Abstract

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.

Keywords

Hereditary angioedema (HAE), Prognostic Nutritional Index (PNI), Clinical outcomes, Nutritional status, Attack frequency

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