Table 3:

Studies that have evaluated pulmonary functions in Ol

Study	Number enrolled	Age	Major Findings
Falvo et al 1973	“severe disease” 4 “moderate disease” 6 “mild disease” 1	4–34 yrs	Reduction of VC and increase in RV and RV/TLC ratio were found only in patients with kyphoscoliosis
Widmann et al 1999	“OI tarda” 8 “OI congenita” 7	21–45 yrs	“Restrictive disease” in 53% Diminished VC with scoliosis of greater than 600
Takken et al 2004	OI I 17	8–21 yrs	Decrease in percent predicted FEV1 and FVC with normalized height but not measured height Decreased exercise tolerance
Thiele et al 2012	OI III 23 OI IV 23	4–20 yrs	FVC, VC, and TLC decrease with age Decline in volumes is greater in type III OI Scoliosis contributes to decline in volumes; however decline observed even in absence of scoliosis 57% diagnosed with “restrictive disease” 22% diagnosed with “obstructive disease”
LoMauro et al 2012	OI III 7 OI IV 15	Mean (SD) OI III 26 (16) yrs Mean (SD) OI IV 16 (11) yrs	Lower FVC and FEV1 compared to predicted values Paradoxical movement of chest wall and thoracoabdominal asynchrony in type III OI
Wekre et al 2014	OI I 60 OI III 3 OI IV 10	Mean (SD) 44 (12)	Use of arm span-imputed height decreased percent predicted FVC and FEV1 compared to measure height FVC and FEV1 correlated negatively with scoliosis