The Effects of Elexacaftor, Tezacaftor, and Ivacaftor (ETI) on Blood Glucose in Patients With Cystic Fibrosis: A Systematic Review

Cystic fibrosis (CF) is an autosomal recessive genetic disorder resulting from defects in the cystic transmembrane conductance regulator (CFTR) protein, which turn results a multi-systemic disorder. There are numerous known CF alleles associated with different mutations of CFTR gene, most common allele being three-base-pair deletion as ΔF508. One manifestation glycemic dysregulation decreased insulin secretion, often progressing into distinct form diabetes fibrosis-related (CFRD). In past decade, class drugs modulators has entered clinical practice. These interact protein to restore its function, (or combinations modulators) suitable for patients mutations. Previous research established that modulator ivacaftor effective decreasing blood glucose and sometimes resolving CFRD certain (class III mutations). However, early therapies individuals ΔF508 mutation (e.g., combination lumacaftor ivacaftor) have largely proven ineffective improving regulation. More recently, therapy three modulators, namely elexacaftor, tezacaftor, (ETI), practice mutation. it not clear whether this treating dysglycemia. We searched studies any design examined effects ETI on measures glucose. All available were observational comparing before after initiating therapy. Measures daily-life (those obtained continuous monitoring systems or by measuring glycated hemoglobin (HbA1c)) post-prandial spikes oral tolerance tests showed significant improvements at least some studies. The majority pre- post-ETI one more measures. While interpretation evidence complicated lack randomized controlled trials, appears improved regulation