25-hydroxyvitamin-d-2 and Cystic-Fibrosis

The objective was to develop evidence-based clinical care guidelines for the screening, diagnosis, management, and treatment of vitamin D deficiency in individuals with cystic fibrosis (CF).. The guidelines committee was comprised of physicians, registered dietitians, a pharmacist, a nurse, a parent of an individual with CF, and a health scientist, all with experience in CF.. Committee members developed questions specific to vitamin D health in individuals with CF. Systematic reviews were completed for each question. The committee reviewed and graded the available evidence and developed evidence-based recommendations and consensus recommendations when insufficient evidence was available. Each consensus recommendation was voted upon by an anonymous process.. Vitamin D deficiency is common in CF. Given the limited evidence specific to CF, the committee provided consensus recommendations for most of the recommendations. The committee recommends yearly screening for vitamin D status, preferably at the end of winter, using the serum 25-hydroxyvitamin D measurement, with a minimal 25-hydroxyvitamin D concentration of 30 ng/ml (75 nmol/liter) considered vitamin D sufficient in individuals with CF. Recommendations for age-specific vitamin D intake for all individuals with CF, form of vitamin D, and a stepwise approach to increase vitamin D intake when optimal vitamin D status is not achieved are delineated.

Topics: 25-Hydroxyvitamin D 2; Adolescent; Adult; Age Factors; Calcifediol; Child; Cholecalciferol; Cystic Fibrosis; Dietary Supplements; Ergocalciferols; Evidence-Based Practice; Humans; Infant; Malabsorption Syndromes; Mass Screening; Seasons; Vitamin D; Vitamin D Deficiency

Topics: 25-Hydroxyvitamin D 2; Cystic Fibrosis; Humans; Pain; Vitamin D; Vitamin D Deficiency

The effectiveness of current treatment recommendations for vitamin D insufficiency in children with CF is unknown. Therefore, we assessed the effectiveness of vitamin D(2) 50,000 IU once daily for 28 days for vitamin D insufficiency.. Retrospective chart review of pediatric CF patients from 2006-2008. Vitamin D(2) 50,000 IU daily for 28 days was given to patients with 25-OHD <30 ng/mL and repeat 25-OHD levels were obtained after completion of therapy.. One hundred forty-seven levels from 97 individuals were assessed. Success of treatment was 54% (n=80/147). Seventeen of 39 patients (43%) followed for an additional 6-18 months were able to maintain levels of >or=30 ng/mL.. Vitamin D(2) 50,000 IU daily for 28 days was effective in correcting vitamin D insufficiency in approximately 50% of subjects. However, almost half of successfully treated patients were unable to maintain normal 25-OHD levels >6 months after completion of therapy, implying that this effect is transient.

Topics: 25-Hydroxyvitamin D 2; Adolescent; Calcifediol; Child; Child, Preschool; Cystic Fibrosis; Dose-Response Relationship, Drug; Drug Administration Schedule; Ergocalciferols; Female; Follow-Up Studies; Humans; Male; Prevalence; Retrospective Studies; Seasons; Time Factors; Treatment Outcome; Vitamin D Deficiency; Vitamins

Young adults with cystic fibrosis (CF) frequently develop bone disease. One suggested aetiological factor is suboptimal vitamin K status with impaired carboxylation of osteocalcin and abnormal bone formation.. We measured bone mineralization and turnover in thirty-two 8-12 year old CF patients (14 boys) using Dual Energy X-ray absorptiometry (whole body (WB) and lumbar spine (LS)), 25-OH Vitamin D, PTH and markers of bone formation (plasma osteocalcin, N-terminal pro-peptide of type 1 collagen (P1NP)), plus an indirect measure of vitamin K status, undercarboxylated osteocalcin (uc-OC).. LS bone mineral density (BMD) standard deviation (SD) scores were < -1.0 in 20% of subjects. Size-adjusted LS and WB bone mass was normal. Compared to reference data, % uc-OC was high and P1NP low. LS bone mass was predicted by % uc-OC but not other markers (0.4% decrease in size-adjusted LSBMC (p=0.05); 0.04 SD decrease in LSBMAD (p=0.04) per 1% increase in uc-OC).. Markers suggestive of sub-optimal vitamin K status and low bone formation were present despite normal size-adjusted bone mass. The association between LSBMC and % uc-OC is consistent with the hypothesis that sub-optimal vitamin K status is a risk factor for CF bone disease. This should ideally be investigated in an intervention trial.

Topics: 25-Hydroxyvitamin D 2; Absorptiometry, Photon; Bone Density; Bone Remodeling; Child; Cohort Studies; Cystic Fibrosis; Female; Humans; Male; Osteocalcin; Osteoporosis; Parathyroid Hormone; Vitamin K Deficiency

To assess mineral metabolism in patients with cystic fibrosis and to study the effects of season and sunlight exposure on generation of vitamin D metabolites, we quantified serum levels of calcidiol and calcitriol, other measures of bone metabolism, and radiographic bone mass in 20 adolescents and young adults with CF and 20 age-matched normal volunteers. Levels of calcidiol were lower in patients with CF than in controls and lower in Massachusetts than in Arizona in both study groups. Controls in Arizona had higher (P less than 0.05) levels of calcitriol than in Massachusetts throughout the year. All control subjects in both states had higher levels of calcitriol than did patients with CF. Patients in Massachusetts had significantly lower levels of calcitriol in winter than in summer. Summer levels of calcitriol in CF were significantly higher in Massachusetts than in Arizona; during winter, lower levels were found in Massachusetts than in Arizona. Mean bone density in patients with CF was 88% and 89% of normal American standards in Massachusetts and Arizona, respectively. These data indicate a seasonal, sunlight-related influence on levels of vitamin D metabolites in patients with CF receiving approximately 1000 IU vitamin D per day. Older patients with CF with progressively diminishing sunlight exposure may be at increased risk for development of osteopenia. The detected radiographic abnormalities of bone mineralization may also be related to malabsorptive deficiencies of calcium and phosphorus.

Topics: 25-Hydroxyvitamin D 2; Adolescent; Adult; Arizona; Bone and Bones; Calcitriol; Calcium; Child; Chromatography, High Pressure Liquid; Cystic Fibrosis; Ergocalciferols; Female; Humans; Male; Massachusetts; Minerals; Radiography; Seasons; Sunlight