Fomepizole as an adjunct in acetylcysteine treated acetaminophen overdose patients: a case series.

INTRODUCTION: Acetaminophen (N-acetyl-para-aminophenol or APAP) is the leading cause of acute liver failure worldwide. Standard therapy for APAP overdose is with IV N-acetylcysteine (NAC). However, overdose patients treated with NAC can still incur hepatotoxicity in some circumstances. Fomepizole has proven safety in methanol and ethylene glycol poisoning and is a potent CYP2E1 and c-Jun-N-terminal Kinase (JNK) inhibitor that is effective even in the metabolic phase. METHODS: We present a prospective case series of 14 consecutive, high-risk patients who had elevated APAP levels after overdose who were treated with fomepizole as an adjunct to standard IV-NAC. The attending toxicologist utilized clinical judgement to determine the use of fomepizole, especially if APAP levels persisted due to altered half-life or risk factors for toxicity. RESULTS: There were no unfavorable outcomes in any patient, which were better than expected. CONCLUSIONS: This case series has demonstrated the safety of fomepizole in high-risk APAP overdose. The efficacy of fomepizole needs to be further elucidated through controlled clinical trials on a larger scale. In massive APAP overdoses, fomepizole should be considered as an adjunct due to the known failure rate of NAC and the safety profile of fomepizole.

Review of Rapid Advances in Cystic Fibrosis.

Cystic fibrosis (CF) is an autosomal recessive disease that was previously a fatal pediatric disease with no treatment; however, due to scientific advancements, the median age of survival for the CF population born in 2018 has increased from 29 in 1989 to 47.4 in 2018. This is an innovative era for the treatment of CF as advanced research continues to evolve and novel treatments for the disease and related illnesses are discovered.