Table 1 Demographic and clinical information for the five included pediatric patients, including two pediatric patients with CF and three pediatric controls (CO).

From: The single-cell transcriptional landscape of the pediatric cystic fibrosis lung from minimally invasive respiratory specimens

 

CF1

CF2

CO1

CO2

CO3

Age, years

0.9

16.3

0.6

17.6

4.2

Sex

Male

Female

Male

Female

Female

Primary pulmonary

diagnosis

Cystic

fibrosis

Cystic fibrosis

Bronchomalacia

None

Asthma

CFTR variants

F508del/

R1162X

F508del/

F508del

Unknown

Unknown

K166N

Lowest sweat

chloride value

(mmol/L)

QNS

84

NP

NP

18

Pancreatic exocrine

insufficiency

Yes

Yes

No

No

No

FEV1, % pred

NP

61

NP

79

NP

Staph aureus

colonization

Yes

Yes

No

No

Yes

Pseudomonas

colonization

No

No

No

No

No

CFTR modulator

use

No

Yes†

No

No

No

Dornase alfa

use

Yes

Yes

No

No

No

Hypertonic saline

use

Yes

No

No

No

Yes

Bronchodilator use

Yes

Yes

No

No

Yes

Inhaled antibiotic

use

No

No

No

No

No

Oral/IV antibiotics

within 6 months

Yes

Yes

No

No

Yes

Inhaled corticosteroid

use

Yes

No

No

No

Yes

Oral/IV corticosteroids

within 6 months

Yes

No

No

No

Yes

  1. †CF2 was on elexacaftor-tezacaftor-ivacaftor at the time of flexible bronchoscopy and for over eighteen months prior to the procedure. CFTR: Cystic fibrosis transmembrane conductance regulator; FEV1, % pred: Forced expiratory volume in one second as percent predicted; IV: Intravenous; NP: Not performed; QNS: Quantity not sufficient.