Review Article

Vitamin D Deficiency in Cystic Fibrosis

Table 2

Vitamin D Therapy Trials in CF.

ArticleDesignPatientsInterventionOutcomeLimitations

Hillman et al. [32]Double-blinded RCT15 children aged 7–132000 D3 1 g Ca versus 2000 D3 versus 400 D3 1 g Ca versus 400 D3 for 6 monthsNo change in 25OHD, 1,25OHD levels in any armShort duration of treatment, small study, young patients only

Boyle et al. [9]Open label CT66 adult vitamin D deficient (25OHD 75 nmol/L [30 ng/mL]) patientsSequential efforts of 50,000 IU D2 weekly for 8 weeks or twice weekly for 8 weeksOnly 5 (8%) patients achieved repletion (25OHD 75 nmol/L [30 ng/mL])Many follow up 25OHD levels may have been drawn after supplementation ended.

Haworth et al. [37]Double-blinded RCT30 adult patients with BMD z score 1 in lumbar spine, femur or distal forearm1 g cal 1700 IU D3 (treatment) versus 900 IU D3 (control) over 12 monthsTreatment group showed reduced rate of bone loss, no significant change in 25OHD levelSmall sample size, changes in BMD not statistically significant

Brown et al. [38]Open Label10 adult CF patients10 healthy patients5 microg calcitriol bid for 14 daysIncreased short term study showing improved calcium absorption in both healthy and CF patients. In CF, calcitriol also decreased urine NTX in CF patients.Short duration of treatment, Long term effects unknown. Calcitriol requires close monitoring to prevent hypecalciuria & hypercalcemia

Stephenson et al. [35]Open Label, dose escalating study215 CF adults patients with 25OHD 50 nmol/L (20 ng/mL)400 IU D3, 800 IU D3, 1000 IU D3, 1000 IU D3, compliance counseling92% achieved 25OHD 50 nmol/L (20 ng/mL) with average dose of 1800 IU/day
Only 18% of these patients achieved 25OHD 75 nmol/L
No control group, no differentiation in outcome between intervention arms

Khazai et al. [29] Open label RCT 30 CF adult patients50,000 IU D2 weekly100% D3 achieved 25OHD 75 nmol/L (30 ng/mL)Small sample size, limited compliance in sun lamp arm
50,000 IU D3 weekly,60% D2 achieved 25OHD 75 nmol/L (30 ng/mL)
2%–5% UVB lamps55% sunlamps achieved 25OHD 75 nmol/L (30 ng/mL)

Green 2008 [71]Retrospective chart review262 pediatric CF patients50,000 IU D2 1X weekly, 2X weekly or 3X weekly for variable lengths of time33% weekly achieved 25OHD 75 nmol/L (30 ng/mL)
26% twice weekly achieved 25OHD 75 nmol/L (30 ng/mL)
43% thrice weekly 25OHD 75 nmol/L (30 ng/mL)
While there was some improvement in levels, no treatment was significantly better than controls

Gronowitz et al. [72]Open Label Controlled Trial30 pts aged 9–40 mean initial 25OHD 53 nmol/L (21.2 ng/mL) in control and 55 nmol/L (22 ng/mL) in treatment at baselineRoutine Supplementation (400-2250 IU D3 daily) versus Routine Supplementation + UVB lamps for 12 weeks25OHD level post 8 weeks: Control 48 nmol/L (19.2 ng/mL) Intervention 110 nmol/L (44 ng/mL)
Post 12 weeks: Control 63 nmol/L (25.2 ng/ml) Intervention 125 nmol/L/mL (50 ng/mL)
Limited compliance to treatment regimen due to skin type side effects with prolonged UVB exposure