wqe94v9

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?: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)-1beta, IL-6, IL-8, and soluble TNF receptor 1 (sTNFR1) were assessed at regular intervals in plasma, with IL-1beta, 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 particular IL-6, IL-1beta and NE activity Following each AAT dose, rapid decreases in each inflammatory parameter were observed These were matched by marked clinical and radiographic improvement CONCLUSIONS: The results support further investigation of AAT as a COVID-19 therapeutic, and re-exploration of its use in CF 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)-1ß, IL-6, IL-8, and soluble TNF receptor 1 (sTNFR1) were assessed at regular intervals in plasma, with IL-1ß, 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 particular IL-6, IL-1ß and NE activity. Following each AAT dose, rapid decreases in each inflammatory parameter were observed. These were matched by marked clinical and radiographic improvement. CONCLUSIONS: The results support further investigation of AAT as a COVID-19 therapeutic, and re-exploration of its use in CF.
is ?:annotates of	<https://research.tib.eu/covid-19/entity/6wqe94v9_hasAnnotation_C1363984> <https://research.tib.eu/covid-19/entity/6wqe94v9_hasAnnotation_C3537219>
?:creator	<https://research.tib.eu/covid-19/entity/McElvaney%2C_O._J.%3B_O039%2C%3B_Connor%2C_E.%3B_McEvoy%2C_N._L.%3B_Fraughan%2C_D._D.%3B_Clarke%2C_J.%3B_McElvaney%2C_O._F.%3B_Gunaratnam%2C_C.%3B_Rourke%2C_J.%3B_Curley%2C_G._F.%3B_McElvaney%2C_N._G.> <https://research.tib.eu/covid-19/entity/McElvaney%2C_Oliver_J%3B_O039%2C%3B_Connor%2C_Eoin%3B_McEvoy%2C_Natalie_L%3B_Fraughan%2C_Daniel_D%3B_Clarke%2C_Jennifer%3B_McElvaney%2C_Ois%C3%ADn_F%3B_Gunaratnam%2C_Cedric%3B_Rourke%2C_James%3B_Curley%2C_Gerard_F%3B_McElvaney%2C_Noel_G>
?:journal	J._cyst._fibros Journal_of_Cystic_Fibrosis
?:license	unk
?:publication_isRelatedTo_Disease	<https://research.tib.eu/covid-19/entity/COVID-19>
is ?:relation_isRelatedTo_publication of	<https://research.tib.eu/covid-19/entity/6wqe94v9_HAS_C0002191_TREATS_C0010674> <https://research.tib.eu/covid-19/entity/6wqe94v9_HAS_C5203670_COEXISTS_WITH_C0010674> <https://research.tib.eu/covid-19/entity/6wqe94v9_HAS_C5203670_COMPLICATES_C0010674>
?:source	WHO
?:title	Alpha-1 antitrypsin for cystic fibrosis complicated by severe cytokinemic COVID-19
?:type	<https://research.tib.eu/covid-19/vocab/Publication>
?:who_covidence_id	#1065291 #939037
?:year	2020 2021

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?: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)-1beta, IL-6, IL-8, and soluble TNF receptor 1 (sTNFR1) were assessed at regular intervals in plasma, with IL-1beta, 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 particular IL-6, IL-1beta and NE activity Following each AAT dose, rapid decreases in each inflammatory parameter were observed These were matched by marked clinical and radiographic improvement CONCLUSIONS: The results support further investigation of AAT as a COVID-19 therapeutic, and re-exploration of its use in CF
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)-1ß, IL-6, IL-8, and soluble TNF receptor 1 (sTNFR1) were assessed at regular intervals in plasma, with IL-1ß, 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 particular IL-6, IL-1ß and NE activity. Following each AAT dose, rapid decreases in each inflammatory parameter were observed. These were matched by marked clinical and radiographic improvement. CONCLUSIONS: The results support further investigation of AAT as a COVID-19 therapeutic, and re-exploration of its use in CF.

is ?:annotates of