Lumacaftor/ivacaftor therapy is associated with reduced hepatic steatosis in cystic fibrosis patients

Table 3 Magnetic resonance imaging proton density fat fraction and biochemistry for all subjects and stratified by modulator (lumacaftor/ivacaftor) status

	Reference range	All subjects, n = 20	CFTR modulator, n = 9	No CFTR modulator, n = 11	P value
PDFF (%)	< 5%	3.0 (0.0-21.0)	2.0 (0.0-6.4)	4.1 (2.7-21.0)	0.002
HbA1C (%)	< 5.8%	5.8 (5.2-8.2)	6.3 (5.3-8.2)	5.7 (5.2-7.4)	0.21
Alk Phos (U/L)	1	110 (44-310)	66 (44-178)	155 (103-310)	0.01
Alk Phos (SD)	-2.0-2.0	1.2 (-1.5-4.8)	-0.3 (-1.5-4.7)	1.8 (-0.6-4.8)	0.07
Total bilirubin (µmol/L)2	0-20.5	6.8 (3.4-20.5)	5.1 (3.4-6.8)	8.6 (3.4-20.5)	0.003
AST (U/L)	9-39	23 (11-45)	20 (11-42)	27 (11-45)	0.39
ALT (U/L)	10-52	22 (10-58)	18 (10-45)	29 (12-58)	0.19
GGT (U/L)	5-64	14 (9-29)	12 (9-24)	19 (10-29)	0.07
Triglyceride (mmol/L)3	< 1.7	1.02 (0.36-2.55)	0.9 (0.64-2.55)	1.25 (0.36-2.35)	0.79

¹Reference range is age dependent;

²To convert from μmol/L to mg/dL multiply by 0.0585;

³To convert to mg/dL multiply by 88.5. Results of magnetic resonance imaging proton density fat fraction and key laboratory parameters for all subjects and stratified by cystic fibrosis transmembrane conductance regulator modulator (lumacaftor/ivacaftor) use. Data presented as median (range) or number (percent) as appropriate. CFTR: Cystic fibrosis transmembrane conductance regulator; PDFF: Proton density fat fraction; AST: Aspartate transaminase; ALT: Alanine transaminase; GGT: Gamma-glutamyltransferase.