cholecalciferol and Cystic-Fibrosis

Vitamin D deficiency occurs frequently in patients with cystic fibrosis (CF). Vitamin D is important for optimal mineralization of bone and may be important for other comorbidities commonly occurring in patients with CF. Vitamin D deficiency in patients with CF can arise from various causes including pancreatic exocrine insufficiency, lack of outdoor activity, and alterations of vitamin D metabolism. Due to fat malabsorption stemming from pancreatic insufficiency, higher oral doses of vitamin D are necessary to correct and maintain optimal vitamin D status in patients with CF. Recent studies have demonstrated that higher vitamin D status is associated with better lung function and that vitamin D therapy may help recovery from pulmonary exacerbations of CF. The mechanisms by which vitamin D may exert its beneficial actions in CF are unclear but likely related to the role vitamin D has in modulating the adaptive and innate immune response. Large randomized clinical studies to evaluate the potential role of vitamin D as adjunctive therapy in CF that goes beyond bone are necessary.

Topics: Bone Diseases; Cholecalciferol; Clinical Trials as Topic; Comorbidity; Cystic Fibrosis; Ergocalciferols; Humans; Inflammation; Lung; Randomized Controlled Trials as Topic; Respiratory Function Tests; Vitamin D; Vitamin D Deficiency

The existing guidelines on screening and treatment are confusing because different guidelines target different populations. The IOM and AAP guidelines target generally healthy populations, whereas the Endocrine Society and other subspecialty guidelines target individuals with specific medical conditions associated with increased bone fragility. These distinctions have not always been well articulated. For healthy adolescents, the AAP does not recommend universal screening or screening of obese or dark-skinned individuals. Increased dietary intake of vitamin D is recommended, and vitamin D supplementation can be considered if the RDA cannot be met. For adolescents with chronic medical illnesses associated with increased fracture risk, screening for vitamin D deficiency should be performed by obtaining a serum 25-OHD level. Those found to be deficient (25-OHD level < 20 ng/mL) should be treated with doses of vitamin D2 or vitamin D3 higher than the daily requirement (as discussed in the section on vitamin D and chronic disease), followed by a maintenance dose. A repeat 25-OHD level should be obtained after the therapeutic course is completed. Some experts advocate for achievement of 25-OHD levels greater than 30 ng/mL in conditions associated with increased bone fragility, and several pediatric subspecialty organizations have made recommendations specific to the diseases they treat. In such instances, the recommendations of the pediatric subspecialty organizations should take precedence over the AAP recommendations for adolescents with chronic illnesses associated with increased bone fragility because the AAP recommendations were primarily targeted at a healthy population.

Topics: Adolescent; Celiac Disease; Cholecalciferol; Comorbidity; Cystic Fibrosis; Ergocalciferols; Feeding and Eating Disorders; Humans; Inflammatory Bowel Diseases; Mass Screening; Practice Guidelines as Topic; Rheumatic Diseases; Vitamin D; Vitamin D Deficiency; Vitamins

This review summarizes recently published data on the epidemiology, pathophysiology and treatment of cystic fibrosis (CF)-related low bone mineral density (BMD).. A systematic literature review reports that the pooled prevalence of osteoporosis in adults with CF is 23.5% [95% confidence interval (CI) 16.6-31.0] and the pooled prevalences of radiologically confirmed vertebral and nonvertebral fractures are 14% (95% CI 7.8-21.7) and 19.7% (95% CI 6.0-38.8), respectively. Recent data suggest that the cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in bone cells and that CFTR dysfunction affects bone cell activity. The secondary effects of CFTR dysfunction also influence bone metabolism. For example, bone resorption increases during CF pulmonary exacerbations due to the stimulatory effects of proinflammatory cytokines on osteoclast activity. Bisphosphonates inhibit osteoclastic bone resorption and recent data show that both oral and intravenous bisphosphonates improve BMD in patients with CF.. CF-related low BMD is a common but treatable complication of CF.

Topics: Bone Density; Calcium; Cholecalciferol; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Diphosphonates; Humans; Osteoporosis; Vitamin K

Vitamin D plays an important role in inflammatory responses after antigen exposure. Interleukin-23 (Il-23) promotes Il-17-dependent inflammation during Pseudomonas aeruginosa (P. aeruginosa) pulmonary infection. We aimed to compare the ability of calcitriol and cholecalciferol to modulate the inflammatory response of the CF airways infected with P. aeruginosa.. The level of Il-17A in EBC significantly decreased in calcitriol group from 0,475 pg/mL (± SD 0,515 pg/mL) to 0,384 pg/mL (± SD 0,429 pg/mL) (p = 0,008); there was no change in cholecalciferol group (p = 0,074). The level of Il-23 in EBC did not significantly change in calcitriol group (p = 0,086); there was significant decrease in cholecalciferol group from 8,90 pg/mL (± SD 4,07 pg/mL) to 7,33 pg/mL (± SD 3,88 pg/mL) (p = 0,001). In calcitriol group serum phosphorus and PTH significantly decreased (p = 0,021 and p = 0,019 respectively), the concentration of calcium significantly increased (p = 0,001); there were no changes in cholecalciferol group.. Both analogs of vitamin D revealed their anti-inflammatory effect and reduced the level of Il-17A and Il-23 in the airway of CF patients with chronic P. aeruginosa infection. We observed improvement in calcium-phosphorus metabolism after supplementation with calcitriol, without adverse effects. It is recommended to use vitamin D in CF patients.

Topics: Adolescent; Breath Tests; Calcitriol; Calcium; Child; Cholecalciferol; Creatinine; Cross-Over Studies; Cystic Fibrosis; Double-Blind Method; Humans; Interleukin-17; Interleukin-23; Lung; Male; Parathyroid Hormone; Phosphorus; Pilot Projects; Pneumonia, Bacterial; Pseudomonas aeruginosa; Pseudomonas Infections; Vitamin D; Vitamins; Young Adult

Despite the availability of consensus guidelines for the treatment of vitamin D deficiency, prospective trials are lacking to examine alternative dosing strategies for adult patients with cystic fibrosis (CF) who do not meet therapeutic goals with standard regimens.. The primary objective of this study was to determine the efficacy of high-dose cholecalciferol supplementation in increasing serum vitamin D (25-OHD) levels in adult patients with CF.. Patients were eligible for inclusion if they were 18 years or older, had baseline 25-OHD levels lower than 30 ng/ml, and were diagnosed with CF and pancreatic insufficiency. Patients were given a single dose of cholecalciferol 300,000 or 500,000 IU based on baseline 25-OHD levels. Response was defined by 25-OHD and ionized calcium levels at 3 months. At 6 months, responders received a second dose of the same strength, and nonresponders were given a weekly supplement of cholecalciferol 50,000 IU in addition to cholecalciferol 500,000 IU. A second 25-OHD level was obtained at 9 months.. Of the 46 patients enrolled, 32 patients (70%) completed the study. Baseline levels of 25-OHD significantly increased over time in the per protocol population at 3 and 9 months. A total of 16 patients (50%) were considered nonresponders and required weekly supplementation.. A protocol using high-dose cholecalciferol or high-dose plus weekly cholecalciferol is safe and effective in treating adult patients with CF and pancreatic insufficiency.

Topics: Adult; Cholecalciferol; Cystic Fibrosis; Dietary Supplements; Dose-Response Relationship, Drug; Exocrine Pancreatic Insufficiency; Female; Humans; Male; Prospective Studies; Vitamin D Deficiency; Vitamins; Young Adult

Disruption of gut microbiota may exacerbate severity of cystic fibrosis (CF). Vitamin D deficiency is a common comorbidity in patients with CF that may influence composition of the gut microbiota.. Compare microbiota of vitamin D-sufficient and -insufficient CF patients and assess impact of a weekly high-dose vitamin D3 bolus regimen on gut and airway microbiome in adults with CF and vitamin D insufficiency (25-hydroxyvitamin D < 30 ng/mL).. Forty-one subjects with CF were classified into two groups: vitamin D insufficient (n = 23) and vitamin D sufficient (n = 18). Subjects with vitamin D insufficiency were randomized to receive 50,000 IU of oral vitamin D3 or placebo weekly for 12 weeks. Sputum and stool samples were obtained pre- and postintervention and 16S ribosomal RNA genes sequenced using Illumina MiSeq technology.. Gut microbiota differed significantly based on vitamin D status with Gammaproteobacteria, which contain numerous, potentially pathogenic species enriched in the vitamin D-insufficient group. Principal coordinates analysis showed differential gut microbiota composition within the vitamin D-insufficient patients following 12 weeks treatment with placebo or vitamin D3 (permutation multivariate analysis of variance = 0.024), with Lactococcus significantly enriched in subjects treated with vitamin D3, whereas Veillonella and Erysipelotrichaceae were significantly enriched in patients treated with placebo.. This exploratory study suggests that vitamin D insufficiency is associated with alterations in microbiota composition that may promote inflammation and that supplementation with vitamin D has the potential to impact microbiota composition. Additional studies to determine the impact of vitamin D on microbiota benefit clinical outcomes in CF are warranted.

Topics: Adult; Cholecalciferol; Cystic Fibrosis; Dietary Supplements; Double-Blind Method; Drug Administration Schedule; Female; Gastrointestinal Microbiome; Humans; Male; Microbiota; Middle Aged; Pilot Projects; Respiratory System; Vitamin D Deficiency; Young Adult

Persistent inflammatory response in cystic fibrosis (CF) airways is believed to play a central role in the progression of lung damage. Anti-inflammatory treatment may slow lung disease progression, but adverse side effects have limited its use. Vitamin D has immunoregulatory properties. We randomized 16 CF patients to receive vitamin D2, vitamin D3 or to serve as controls, and investigated the effect of vitamin D supplementation on soluble immunological parameters, myeloid dendritic cells (mDCs) and T cell activation. Three months of vitamin D treatment were followed by two washout months. Vitamin D status at baseline was correlated negatively with haptoglobin, erythrocyte sedimentation rate and immunoglobulin A concentration. Total vitamin D dose per kg bodyweight correlated with the down-modulation of the co-stimulatory receptor CD86 on mDCs. Vitamin D treatment was associated with reduced CD279 (PD-1) expression on CD4

Topics: Adolescent; ADP-ribosyl Cyclase 1; B7-2 Antigen; CD4-Positive T-Lymphocytes; CD8-Positive T-Lymphocytes; Child; Cholecalciferol; Cystic Fibrosis; Dendritic Cells; Dietary Supplements; Ergocalciferols; Female; Haptoglobins; HLA-DR Antigens; Humans; Immunomodulation; Lymphocyte Activation; Male; Membrane Glycoproteins; Pilot Projects; Programmed Cell Death 1 Receptor; Pseudomonas aeruginosa; Vitamin D

There is little consensus on the most efficacious vehicle substance for vitamin D supplements. Fat malabsorption may impede the ability of patients with cystic fibrosis (CF) to absorb vitamin D in an oil vehicle. We hypothesized that vitamin D contained in a powder vehicle would be absorbed more efficiently than vitamin D contained in an oil vehicle in patients with CF.. In this double-blind, randomized controlled trial, hospitalized adults with CF were given a one-time bolus dose of 100,000 IU of cholecalciferol (D. This trial was completed by 15 patients with CF. The median (interquartile range) age, body mass index, and forced expiratory volume in 1 second were 23.7 (19.9-33.2) years, 19.9 (18.6-22.6) kg/m. In adults with CF, cholecalciferol is more efficiently absorbed in a powder compared with an oil vehicle. Physicians should consider prescribing vitamin D in a powder vehicle in patients with CF to improve the absorption of vitamin D from supplements.

Topics: Adult; Cholecalciferol; Cystic Fibrosis; Dietary Supplements; Dose-Response Relationship, Drug; Double-Blind Method; Female; Humans; Lipid Metabolism; Male; Parathyroid Hormone; Pilot Projects; Powders; Prospective Studies; Treatment Outcome; Vitamin D; Vitamin D Deficiency; Young Adult

Vitamin D insufficiency in cystic fibrosis is common. Vitamin D3 is currently preferred over D2. We aimed to study the efficacy of vitamin D2 and D3 at increasing serum 25-hydroxyvitamin D (s25OHD) concentrations and their effect on respiratory health in cystic fibrosis.. Sixteen CF patients were randomized to receive vitamin D2 or D3 or to serve as controls. The starting dose of 5000 IU (<16 years old) or 7143 IU/day (⩾16 years old) was further individually adjusted. Three months of intervention were followed by two of washout (ClinicalTrials.gov NCT01321905).. To increase s25OHD, the mean daily dose of vitamin D2 and D3 had to be increased up to 15650 and 8184 IU, respectively. The combined group of vitamin D2 and D3 treated patients decreased plasma IL-8 (P<0.05). Patients provided vitamin D3 improved FVC at the end of the trial (P<0.05). Change in s25OHD was positively correlated with changes in the adult Quality-of-Life respiratory score at the end of supplementation (P=0.006, r=0.90), and with changes in FEV1 (P=0.042, r=0.62) and FVC (P=0.036, r=0.63) at one month of washout.. Vitamin D supplementation may contribute to reduced inflammation and improved lung function in CF.

Topics: Adolescent; Adult; Child; Cholecalciferol; Cystic Fibrosis; Dietary Supplements; Ergocalciferols; Female; Humans; Lung; Male; Pilot Projects; Treatment Outcome; Vital Capacity; Vitamin D; Vitamin D Deficiency; Vitamins; Young Adult

Vitamin D insufficiency is common in children with cystic fibrosis (CF), yet data are sparse regarding the most effective form of vitamin D supplementation. The aim of this study was to compare two different vitamin D replacement regimens.. We conducted a randomized controlled trial comparing 50,000 IU of ergocalciferol (vitamin D2) twice weekly for 8 weeks versus 50,000 IU of cholecalciferol (vitamin D3) weekly in patients with CF, pancreatic insufficiency, age 6-21 years and a 25(OH)D<30 ng/mL. The primary outcome was change in serum 25(OH)D concentration. For secondary analyses, we examined changes in IgG, IgE and CRP in patients who normalized their vitamin D levels.. A total of 47 patients completed the trial. The mean pre-treatment 25(OH)D concentration was 23.1 (SD 4.7) ng/mL. The overall mean increase in 25(OH)D was 11.1 (11.9) ng/mL and 31/47 (66%) achieved a 25(OH)D concentration ≥ 30 ng/mL; of the 26 participants who received D2, 18 (69%) achieved sufficiency while 13/21 (62%) participants treated with D3 achieved sufficiency. There was no difference between groups in change of 25(OH)D (p=0.65). Similarly, there was no difference in the number of patients to achieve vitamin D sufficiency between treatments (p=0.6).. Ergocalciferol administered as 50,000 IU twice weekly is as effective as cholecalciferol 50,000 IU weekly for 8 weeks in pediatric patients with CF and vitamin D insufficiency. Only 66% of the patients studied achieved the desired 25(OH)D concentration.

Topics: Administration, Oral; Adolescent; Biomarkers; Child; Cholecalciferol; Cystic Fibrosis; Dose-Response Relationship, Drug; Drug Administration Schedule; Drug Therapy, Combination; Ergocalciferols; Exocrine Pancreatic Insufficiency; Female; Follow-Up Studies; Humans; Male; Treatment Outcome; Vitamin D; Vitamin D Deficiency; Vitamins; Young Adult

The optimal treatment for correcting or preventing vitamin D insufficiency in cystic fibrosis (CF) patients has not been established.. The aim of the study was to assess the relative efficacy of three modes of vitamin D therapy: cholecalciferol (D3), ergocalciferol (D2), and UV light in raising or maintaining 25(OH)D levels above 30 ng/ml.. Thirty adult CF subjects with vitamin D insufficiency were randomized into one of three treatment arms: D3, D2, or UV light. Subjects randomized to D3 or D2 ingested 50,000 IU of vitamin D weekly, and those randomized to UV exposed their skin to UV light from a lamp five times a week. Serum was collected for 25(OH)D and PTH at baseline and at 12 wk.. Treatment with D3 and D2 raised 25(OH)D levels significantly, from a mean of 21.2 +/- 10.18 to 47.1 +/- 20.5 ng/ml (P < 0.001) and 24.4 +/- 10.3 to 32.7+/- 9.7 ng/ml (P = 0.01), with 100% and 60% reaching 25(OH)D levels above 30 ng/ml, respectively. Treatment with UV did not raise 25(OH)D levels significantly; however, only 55% of subjects were adherent with UV therapy.. This study demonstrates that CF subjects are able to achieve or maintain optimal vitamin D status (>30 ng/ml) with two oral regimens of either D3 or D2 treatment, the former being more efficacious. A confounding variable for this observation is the fact that the D3 and D2 capsules contained different carriers, powder-based vs. oil-based, respectively. UV therapy did not alter vitamin D status, possibly due to poor adherence to UV therapy.

Topics: Adolescent; Adult; Aged; Cholecalciferol; Cystic Fibrosis; Ergocalciferols; Female; Humans; Hydroxycholecalciferols; Male; Middle Aged; Parathyroid Hormone; Patient Compliance; Ultraviolet Therapy; Vitamin D Deficiency; Young Adult

The effect of a high dose oral cholecalciferol repletion strategy in Vitamin D insufficient adults with CF is still unknown. Therefore, we assessed the effectiveness of our current approach, giving oral vitamin D3 supplementation at a dose of 10,000 IU from Monday to Friday for a total of 50,000 IU D3 weekly in vitamin D insufficient adult with CF.. We performed a retrospective chart review of all 59 adult CF patients between the ages of 17 and 64 years routinely followed at the CF Adult Program of Winnipeg Health Sciences Centre. Through consultation with the endocrinologist, our clinic vitamin D repletion protocol for treating CF adult patients who have serum 25-hydroxyvitamin D (25-OHD) < 30 ng/ml (<75 nmol/L) was to prescribe vitamin D3 10,000 IU orally from Monday to Friday (or the weekly equivalent of 50,000 IU) for 12 weeks in addition to their regular CF vitamin that supplied from 800 to 2000 IU vitamin D3 daily. Cholecalciferol was conveniently administered orally as either one capsule (oil-based) 10,000 IU or one tablet (powder-based) 10,000 IU. All patients were instructed to obtain follow-up serum 25-OHD levels post completion of treatment.. Of the 59 adult patients at our CF Clinic, 35 patients (59%) had below optimal serum 25-OHD levels. Of the 35 patients identified, 10 patients with insufficient serum 25-OHD levels between 10 and 30 ng/ml (25-75 nmol/L) fulfilled the inclusion criteria. A significant increase in serum 25-OHD levels was observed (P < 0.01) from mean value of 21.6 ± 5.9 ng/ml (54.1 ± 14.8 nmol/L) at baseline to 31.7 ± 9.1 ng/ml (79.3 ± 22.8 nmol/L) ≥ 2 months post intervention. The current treatment approach was successful in treating Vitamin D insufficiency in 70% of the patients with low 25-OHD levels.. The results of this study demonstrate that a large number of adults attending Winnipeg Health Sciences Centre CF Clinic have serum 25-OHD levels below 30 ng/ml (75 nmol/L). This supports the need for dedicated and individualized approach to manage this condition. High dose therapy of vitamin D3, although a more aggressive treatment approach, may result in achieving optimal levels of serum 25-OHD in adults with CF.

Topics: Adolescent; Adult; Body Mass Index; Cholecalciferol; Cystic Fibrosis; Diet; Dietary Supplements; Dose-Response Relationship, Drug; Female; Humans; Male; Middle Aged; Retrospective Studies; Seasons; Vitamin D Deficiency; Young Adult

Currently vitamin D3 (VD3) or cholecalciferol is considered an immunomodulator that may be implicated in nasal polyposis (NP) pathophysiology.. This study aimed to investigate if deficiency of VD3 is associated with the presence of NP in patients with cystic fibrosis (CF) and patients with chronic rhinosinusitis (CRS).. In total, 152 adult participants were included in five phenotypic groups: CF with NP (CFwNP) (n = 27), CF without NP (CFsNP) (n = 31), CRS with NP (CRSwNP) (n = 32), CRS without NP (CRSsNP) (n = 30), and controls (n = 32). The serum levels of 25(OH)-VD3 < 20 ng/mL are considered as a deficiency, 21-29 ng/mL as insufficiency, and >30 ng/mL as sufficiency. Endoscopic and imaging staging of the mucosal disease performed with the Lund-Kennedy (LK) and Lund-Mackay (LM) scoring systems, respectively. The genotype of the patients with CF and the nasal microbial colonization of the patients with CF and patients with CRS were also recorded.. The patients with CFwNP had the lowest percentage of sufficiency in VD3 and the highest percentage in insufficiency among all the groups. The LM imaging scores were inversely correlated with the VD3 levels in both arms of the study (CF and CRS). Moreover, the LK endoscopic scores had a similar correlation in the CF groups; however, this was not the case with the CRS groups. The genotype of the patients with CF was not correlated with the VD3 serum levels. The patients with positive microbial colonization (mainly Pseudomonas and Staphylococcus aureus) had significantly lower VD3 serum levels in both the CF and CRS process.. VD3 deficiency seemed to be associated with the presence of nasal polyps in the patients with CRS and in the patients with CF in a similar manner. The lower the level of serum VD3, the more severe the mucosal disease was found in the imaging studies and the more frequent microbial colonization of the patients with CF and the patients with CRS.

Topics: Adolescent; Adult; Cholecalciferol; Chronic Disease; Cystic Fibrosis; Female; Humans; Male; Middle Aged; Nasal Polyps; Rhinitis; Sinusitis; Young Adult

Significant increase in serum 25(OH)D levels from baseline (25.9 ± 10.3 ng/mL) to follow-up (37.0 ± 11.4 ng/mL) (P ≤ 0.001). At follow-up, increased doses during the winter improved serum 25(OH)D levels to a degree comparable to the summer.. This supplementation protocol is efficient and needs to be tested in other CF adult cohorts and correlated to potential health benefit measurements.

Topics: Adult; Body Mass Index; Cholecalciferol; Cystic Fibrosis; Dietary Supplements; Dose-Response Relationship, Drug; Female; Follow-Up Studies; Humans; Male; Retrospective Studies; Seasons; Vitamin D Deficiency; Young Adult

Vitamin D has immunomodulatory properties in the defence against pathogens. Its insufficiency is a widespread feature of cystic fibrosis (CF) patients, which are repeatedly suffering from rhinovirus (RV)-induced pulmonary exacerbations.To investigate whether vitamin D has antiviral activity, primary bronchial epithelial cells from CF children were pre-treated with vitamin D and infected with RV16. Antiviral and anti-inflammatory activity of vitamin D was assessed. RV and LL-37 levels were measured in bronchoalveolar lavage (BAL) of CF children infected with RV.Vitamin D reduced RV16 load in a dose-dependent manner in CF cells (10(-7 )M, p<0.01). The antiviral response mediated by interferons remained unchanged by vitamin D in CF cells. Vitamin D did not exert anti-inflammatory properties in RV-infected CF cells. Vitamin D increased the expression of the antimicrobial peptide LL-37 up to 17.4-fold (p<0.05). Addition of exogenous LL-37 decreased viral replication by 4.4-fold in CF cells (p<0.05). An inverse correlation between viral load and LL-37 levels in CF BAL (r=-0.48, p<0.05) was observed.RV replication in primary CF bronchial cells was reduced by vitamin D through the induction of LL-37. Clinical studies are needed to determine the importance of an adequate control of vitamin D for prevention of virus-induced pulmonary CF exacerbations.

Topics: Adolescent; Antimicrobial Cationic Peptides; Bronchi; Bronchoalveolar Lavage Fluid; Case-Control Studies; Cathelicidins; Child; Child, Preschool; Cholecalciferol; Cystic Fibrosis; Epithelial Cells; Female; Humans; Infant; Male; Real-Time Polymerase Chain Reaction; Rhinovirus; Viral Load; Virus Replication; Vitamin D; Vitamins

To determine the safety and efficacy of stoss therapy on vitamin D levels over a 12 month period in children with cystic fibrosis and vitamin D deficiency (<75 nmol/L).. Retrospective chart review of 142 paediatric CF patients from 2007 till 2011.. Thirty eight children received stoss therapy and 37 children with vitamin D deficiency were not treated and served as a control group. The stoss treated group had a significant and sustained increase in 25-hydroxyvitamin D levels measured at 1, 3, 6 and 12 months post treatment compared to controls (94.82 ± 41.0 nmol/L, p=0.001; 81.54 ± 24.6 nmol/L, p=0.001; 92.18 ± 36.5 nmol/L, p=0.008 and 64.6 ± 20.0 nmol/L, p=0.006 respectively). At 12 months post intervention, the mean difference in vitamin D levels from baseline between the stoss treated group and controls was significant at 15 nmol/L compared to 5 nmol/L (p=0.038).. Stoss therapy effectively achieves and maintains levels of 25-hydroxyvitamin D greater than 75 nmol/L over 12 months.

Topics: Administration, Oral; Case-Control Studies; Child; Cholecalciferol; Cystic Fibrosis; Female; Humans; Male; Retrospective Studies; Treatment Outcome; Vitamin D Deficiency

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

As decreased bone mineral density (BMD) is a common problem in cystic fibrosis (CF) and milk products may have pivotal dietary role affecting BMD, we aimed to assess the potential influence of adult-type hypolactasia (ATH) and lactose malabsorption (LM) on BMD in adolescent and young adult patients. In 95 CF pancreatic-insufficient patients aged 10-25 years (without liver cirrhosis, steatosis and cholestasis, diabetes mellitus, systemic glucocorticoid therapy), lumbar BMD, the nutritional status, pulmonary function, vitamin D3 concentration, calcium intake and single-nucleotide polymorphism upstream of the lactase gene were assessed. In subjects with the -13910 C/C genotype predisposing to ATH, the presence of LM was determined with the use of a hydrogen-methane breath test (BT). BMD and calcium intake were significantly lower in patients with the C/C genotype (P<0.028 and P<0.043, respectively). The abnormal BMD was stated more frequently in patients with the C/C genotype (P<0.042) and with LM (P<0.007). BMD, daily calcium intake and serum vitamin D concentration were significantly lower in LM subjects than in the other patients (P<0.037, P<0.000004 and P<0.0038, respectively). In logistic regression analysis, the relationship between examined parameters and BMD, was found to be statistically significant (P<0.001). However, only standardized body weight and LM were documented to influence BMD (P<0.025 and P<0.044, respectively). In conclusion, LM seems to be an independent risk factor for decreased BMD in CF patients.

Topics: Adult; Body Weight; Bone Demineralization, Pathologic; Bone Density; Breath Tests; Calcium; Child; Cholecalciferol; Cystic Fibrosis; Female; Humans; Lactase; Lactose Intolerance; Male; Polymorphism, Single Nucleotide; Promoter Regions, Genetic; Risk Factors

Allergic bronchopulmonary aspergillosis (ABPA) is caused by a dominant Th2 immune response to antigens derived from the opportunistic mold Aspergillus, most commonly Aspergillus fumigatus. It occurs in 4%-15% of patients with cystic fibrosis (CF); however, not all patients with CF infected with A. fumigatus develop ABPA. Therefore, we compared cohorts of A. fumigatus-colonized CF patients with and without ABPA to identify factors mediating tolerance versus sensitization. We found that the costimulatory molecule OX40 ligand (OX40L) was critical in driving Th2 responses to A. fumigatus in peripheral CD4+ T cells isolated from patients with ABPA. In contrast, CD4+ T cells from the non-ABPA cohort did not mount enhanced Th2 responses in vitro and contained a higher frequency of TGF-beta-expressing regulatory T cells. Heightened Th2 reactivity in the ABPA cohort correlated with lower mean serum vitamin D levels. Further, in vitro addition of 1,25 OH-vitamin D3 substantially reduced DC expression of OX40L and increased DC expression of TGF-beta. This in vitro treatment also resulted in increased Treg TGF-beta expression and reduced Th2 responses by CD4+ T cells from patients with ABPA. These data provide rationale for a therapeutic trial of vitamin D to prevent or treat ABPA in patients with CF.

Topics: Adult; Aspergillosis, Allergic Bronchopulmonary; Aspergillus; Aspergillus fumigatus; CD4-Positive T-Lymphocytes; Cholecalciferol; Cystic Fibrosis; Female; Humans; Male; Receptors, OX40; T-Lymphocytes, Regulatory; Th2 Cells; Transforming Growth Factor beta

Hypovitaminosis D is a risk factor for transplant-related osteoporosis. Its contribution to severe hypocalcemia in transplant recipients is less well recognized. We present 2 cases to illustrate how risk factors specific to transplant recipients significantly increase the risk of development of severe hypocalcemia, on a background of unrecognized vitamin D deficiency. Regular surveillance of calcium homeostasis should be incorporated into routine clinical care of transplant recipients.

Topics: Calcitriol; Calcium; Cholecalciferol; Cystic Fibrosis; Humans; Hypocalcemia; Immunosuppressive Agents; Lung Transplantation; Magnesium; Male; Middle Aged; Pulmonary Fibrosis; Reference Values; Treatment Outcome; Vitamin D; Vitamin D Deficiency

Topics: Adolescent; Cholecalciferol; Cystic Fibrosis; Dietary Supplements; Female; Humans; Male; Retrospective Studies; Sunlight; Vitamin D; Vitamin D Deficiency

Vitamin D deficiency is increasingly being recognized and treated in patients with cystic fibrosis, although the treatment guidelines are not proven and the effectiveness of vitamin D preparations is untested.. The aims of this study were to determine the prevalence of 25-hydroxyvitamin D [25(OH)D] deficiency in a large cohort of adults with cystic fibrosis and to evaluate the effectiveness of supplementation with cholecalciferol.. In this retrospective cohort design, baseline 25(OH)D concentrations were measured, and the effects of clinical interventions that involved either counseling on compliance or increasing supplemental cholecalciferol on serum 25(OH)D concentrations in those subjects with baseline concentrations

Topics: Adolescent; Adult; Body Mass Index; Cholecalciferol; Cohort Studies; Cystic Fibrosis; Dietary Supplements; Female; Humans; Male; Middle Aged; Nutritional Requirements; Nutritional Status; Prevalence; Retrospective Studies; Seasons; Treatment Outcome; Vitamin D; Vitamin D Deficiency; Vitamins

Cystic fibrosis (CF) patients suffer from malabsorption of fat-soluble vitamins (A, D, E and K). These vitamins are available as water-dispersible (A, D(3) and E) or water-soluble grades (K(3)), which is favoured in CF patients as they fail to absorb oil-based products. The objective of this study was to determine stability of these raw materials after opening the original package and to develop a compounded formulation of acceptable quality, stability and taste, allowing flexible dose adaptation and being appropriate for administration to children and elderly people.. The raw materials were stored after opening their original package for 8 months at 8 degrees C and room temperature (RT). Stability was assessed using a validated HPLC method after extraction of the vitamin from the cold water-soluble matrix (vitamin A acetate, D(3) and E) or using a spectrophotometrical method (vitamin K(3)). These materials were mixed with an appropriate lactose grade (lactose 80 m for vitamins A and D(3); lactose 90 m for vitamin E, lactose very fine powder for vitamin K(3)) and filled in hard gelatin capsules. Mass and content uniformity were determined and stability of the vitamins in the capsules was assessed after 2 months storage at 8 degrees C and RT.. All raw materials showed good stability during storage in the opened original package for 8 months storage at 8 degrees C as well as RT (>95% of the initial content). The compounded formulations complied with the requirements of the European Pharmacopoeia for mass and content uniformity and can be stored for 2 months at 8 degrees C or RT while maintaining the vitamin content between 90% and 110%.. As these fat-soluble vitamins are not commercially available on the Belgian market, compounded formulations are a valuable alternative for prophylactic administration of these vitamins to CF patients, i.e. a stable formulation, having an acceptable taste, allowing flexible dose adaptation and being appropriate for administration to children and elderly people.

Topics: Capsules; Cholecalciferol; Chromatography, High Pressure Liquid; Cystic Fibrosis; Drug Combinations; Drug Compounding; Drug Stability; Drug Storage; Humans; Spectrophotometry; Taste; Vitamin A; Vitamin E; Vitamin K 3; Vitamins