Alpha-1 antitrypsin for cystic fibrosis complicated by severe cytokinemic COVID-19.

ABSTRACT:

Background

The clinical course of severe COVID-19 in cystic fibrosis (CF) is incompletely understood. We describe the use of alpha-1 antitrypsin (AAT) as a salvage therapy in a critically unwell patient with CF (PWCF) who developed COVID-19 while awaiting lung transplantation.

Methods

IV AAT was administered at 120 mg/kg/week for 4 consecutive weeks. Levels of interleukin (IL)-1β, IL-6, IL-8, and soluble TNF receptor 1 (sTNFR1) were assessed at regular intervals in plasma, with IL-1β, IL-6, IL-8 and neutrophil elastase (NE) activity measured in airway secretions. Levels were compared to baseline and historic severe exacerbation measurements.

Results

Systemic and airway inflammatory markers were increased compared to both prior exacerbation and baseline levels, in part

SUBMITTER: McElvaney OJ 

PROVIDER: S-EPMC7678455 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature