Supplementation For Cystic Fibrosis Research Articles

634 High-dose (stoss) vitamin D supplementation in cystic fibrosis

634 High-dose (stoss) vitamin D supplementation in cystic fibrosis

61 High-dose vitamin D supplementation in cystic fibrosis: A quality improvement project

61 High-dose vitamin D supplementation in cystic fibrosis: A quality improvement project

Vitamin K supplementation for cystic fibrosis.

Malabsorption and deficiency of fat-soluble vitamins K may occur in cystic fibrosis, a genetic disorder affecting multiple organs. Vitamin K is known to play an important role in both blood coagulation and bone formation, hence the role of supplementation of vitamin K in this category needs to be reviewed. This is an updated version of the review. To assess the effects of vitamin K supplementation in people with cystic fibrosis and to investigate the hypotheses that vitamin K will decrease deficiency-related coagulopathy, increase bone mineral density, decrease risk of fractures and improve quality of life in people with CF. Also to determine the optimal dose and route of administration of vitamin K for people with CF (for both routine and therapeutic use). We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Most recent search: 12 August 2019. Randomised controlled trials of all preparations of vitamin K used as a supplement compared to either no supplementation (or placebo) at any dose or route and for any duration, in patients with cystic fibrosis. Two authors independently screened papers, extracted trial details and assessed their risk of bias. The quality of the evidence was assessed using the GRADE criteria. Three trials (total 70 participants, aged 8 to 46 years) assessed as having a moderate risk of bias were included. One trial compared vitamin K to placebo, a second to no supplementation and the third compared two doses of vitamin K. No trial in either comparison reported our primary outcomes of coagulation and quality of life or the secondary outcomes of nutritional parameters and adverse events. Vitamin K versus control Two trials compared vitamin K to control, but data were not available for analysis. One 12-month trial (n = 38) compared 10 mg vitamin K daily or placebo in a parallel design and one trial (n = 18) was of cross-over design with no washout period and compared 5 mg vitamin K/week for four-weeks to no supplementation for four-weeks. Only the 12-month trial reported on the primary outcome of bone formation; we are very uncertain whether vitamin K supplementation has any effect on bone mineral density at the femoral hip or lumbar spine (very low-quality evidence). Both trials reported an increase in serum vitamin K levels and a decrease in undercarboxylated osteocalcin levels. The cross-over trial also reported that levels of proteins induced by vitamin K absence (PIVKA) showed a decrease and a return to normal following supplementation, but due to the very low-quality evidence we are not certain that this is due to the intervention. High-dose versus low-dose vitamin K One parallel trial (n = 14) compared 1 mg vitamin K/day to 5 mg vitamin K/day for four weeks. The trial did report that there did not appear to be any difference in serum undercarboxylated osteocalcin or vitamin K levels (very low-quality evidence). While the trial reported that serum vitamin K levels improved with supplementation, there was no difference between the high-dose and low-dose groups. There is very low-quality evidence of any effect of vitamin K in people with cystic fibrosis. While there is no evidence of harm, until better evidence is available the ongoing recommendations by national CF guidelines should be followed.

EPS4.08 Sodium supplementation in cystic fibrosis: is it worth it?

ePS4.08 Sodium supplementation in cystic fibrosis: is it worth it?

Antioxidant Micronutrients and Essential Fatty Acids Supplementation on Cystic Fibrosis Outcomes: A Systematic Review

BackgroundAntioxidant micronutrients and essential fatty acids supplementation intake appears to have a protective effect in some diseases such as cardiovascular disease, cancer, and asthma. ObjectiveThe aim of this study was to perform a systematic review to evaluate the effects of these nutrients on nutritional and clinical outcomes of patients with cystic fibrosis (CF). MethodsThis is a systematic review of randomized clinical trials (RCTs) in CF. MEDLINE (via PubMed), Embase, and Scopus databases were searched for RCTs published from 1948 through February 2019. Two investigators independently reviewed the titles and abstracts and then extracted the data from the included studies using a standardized predesigned form. Two reviewers independently performed the quality assessment of the RCTs according to the Cochrane risk of bias tools. ResultsA total of 4,792 studies were identified, and 23 were eligible (8 antioxidant micronutrient and 15 essential fatty acids). The interventions found were beta-carotene, zinc, magnesium, multivitamin, docosahexaenoic acid (DHA), eicosapentaenoic acid (EPA), linoleic acid and lipid matrix with choline supplementation. A significant improvement was observed in: (a) pulmonary function with magnesium (n=1) and essential fatty acids (n=2) supplementation; (b) less pulmonary exacerbations with beta-carotene (n=1), zinc (n=1), antioxidant-enriched multivitamin (n=1) and essential fatty acids (n=2) supplementation. One study with antioxidant-enriched multivitamin and four studies with EPA/DHA supplementation reported significant reductions in inflammatory markers. Nutritional status was not modified by antioxidants supplementation in any of the studies, while in five studies there was an improvement with fatty acids supplementation. The risk of bias of the majority of the parallel studies was high. ConclusionsThe benefits of antioxidants or DHA/EPA supplementation for CF, although observed in some studies, are not consistent enough to recommend routine use of these supplements. The mechanisms of action of these nutrients, dose levels and timing should be further explored in future studies.

Ready-to-Use Supplemental Food for Nutritional Supplementation in Cystic Fibrosis

ABSTRACTUndernutrition is common in cystic fibrosis (CF) and is correlated with long-term outcomes, yet current nutritional interventions have not demonstrated consistent improvements in energy intake, and subsequently, growth. Development of novel nutritional interventions to increase energy intake is essential to improve clinical outcomes of individuals with CF. Ready-to-use supplemental food (RUSF) is a modifiable, inexpensive, palatable, safe, and nutrient-dense food for treatment or prevention of acute malnutrition in developing countries. Utilizing a linear-programming tool we identified 6 RUSF formulations with sufficient nutrient density (495 kcal/100 g), protein, and fat for children with CF. Palatability was established by a taste-trial and affirmed by a 2-wk tolerability assessment that demonstrated consistent consumption and tolerance of the RUSF. Although preliminary, this study demonstrates the potential for developing RUSF as a nutritional supplement for increasing energy intake in children with CF.

Adherence to Nutritional Supplementation in Cystic Fibrosis

PurposeThe purpose of this study was to examine patterns of adherence to a novel dietary supplement in pediatric cystic fibrosis. Adherence to dietary supplementation in cystic fibrosis is challenging, and examination of patterns of adherence behavior over time is needed to better characterize subgroups of patients who need self-management support. Design and methodsWe prospectively examined adherence to Lym-X-Sorb™ (LXS), an organized lipid matrix dietary supplementation for patients with cystic fibrosis (CF) and pancreatic insufficiency (PI), over a 12-month period. Adherence for participants aged 5–17 years with CF and PI (N = 109) was monitored monthly via supplement packet counts. Group-based trajectory modeling was employed to examine patterns in adherence behavior over time. ResultsFour distinct trajectories best characterized adherence in this sample, with 18% of participants demonstrating near perfect adherence, 42% demonstrating good adherence (at or above 80%), 16% demonstrating poor adherence that declined over time, and 24% demonstrating significant non-adherence (< 30%). ConclusionsSome patients with CF and PI who are prescribed nutritional supplements will require intensive, individualized behavioral intervention to enhance adherence. Identifying patients who will have difficulty adhering to dietary interventions may result in better treatment-to-patient matching and improved adherence promotion efforts.Practice Implications.Assessment of adherence to dietary supplementation over time can identify patients at risk for continued difficulty with self-management and provide opportunities for early intervention.

Choline Supplementation in Cystic Fibrosis-The Metabolic and Clinical Impact.

Background: Choline is essential for the synthesis of liver phosphatidylcholine (PC), parenchymal maintenance, bile formation, and lipoprotein assembly to secrete triglycerides. In choline deficiency, the liver accretes choline/PC at the expense of lung tissue, thereby impairing pulmonary PC homoeostasis. In cystic fibrosis (CF), exocrine pancreas insufficiency results in impaired cleavage of bile PC and subsequent fecal choline loss. In these patients, the plasma choline concentration is low and correlates with lung function. We therefore investigated the effect of choline supplementation on plasma choline/PC concentration and metabolism, lung function, and liver fat. Methods: 10 adult male CF patients were recruited (11/2014–1/2016), and orally supplemented with 3 × 1 g choline chloride for 84 (84–91) days. Pre-/post-supplementation, patients were spiked with 3.6 mg/kg [methyl-D9]choline chloride to assess choline/PC metabolism. Mass spectrometry, spirometry, and hepatic nuclear resonance spectrometry served for analysis. Results: Supplementation increased plasma choline from 4.8 (4.1–6.2) µmol/L to 10.5 (8.5–15.5) µmol/L at d84 (p < 0.01). Whereas plasma PC concentration remained unchanged, D9-labeled PC was decreased (12.2 [10.5–18.3] µmol/L vs. 17.7 [15.5–22.4] µmol/L, p < 0.01), indicating D9-tracer dilution due to higher choline pools. Supplementation increased Forced Expiratory Volume in 1 second percent of predicted (ppFEV1) from 70.0 (50.9–74.8)% to 78.3 (60.1–83.9)% (p < 0.05), and decreased liver fat from 1.58 (0.37–8.82)% to 0.84 (0.56–1.17)% (p < 0.01). Plasma choline returned to baseline concentration within 60 h. Conclusions: Choline supplementation normalized plasma choline concentration and increased choline-containing PC precursor pools in adult CF patients. Improved lung function and decreased liver fat suggest that in CF correcting choline deficiency is clinically important. Choline supplementation of CF patients should be further investigated in randomized, placebo-controlled trials.

Vitamin D and pediatrics respiratory diseases

The better understanding of the global activity of vitamin D has led to an intense search for its involvement in non-skeletal diseases. This article presents an updated review of the relationship between vitamin D and pediatric respiratory pathology. A literature search was performed in PUBMED using free terms and MESH terms: vitamin D, asthma, respiratory system diseases, and bronchiolitis. Stu dies in human patients younger than 18 years and animals, published in English and Spanish until 2017 were included. 507 articles were found, of which 43 were included. Indirect evidence suggests a role of vitamin D and fetal lung maturation. In relation to pediatric pulmonary pathology, studies are scarce and inconclusive. Recent meta-analyses performed with individualized evaluation of the participants shows an important protective role of vitamin D supplementation in the prevention of severe asthma exacerbations and acute viral infections. In bronchiolitis, the results are contradictory, with no clear relationship between plasma levels and severity. There is not enough evidence to assess the benefits of vitamin D supplementation in cystic fibrosis and tuberculosis. A direct relationship between the severity of sleep-related breathing disorders and vitamin D plasma levels has recently been proposed, although the exact mechanisms involved in this association are unknown. Current information suggests that vitamin D supplementation may represent a cost-effective strategy in redu cing important causes of infant morbidity and mortality.

Vitamin K supplementation for cystic fibrosis.

Cystic fibrosis is a genetic disorder which can lead to multiorgan dysfunction. Malabsorption of fat and fat-soluble vitamins (A, D, E, K) may occur and can cause subclinical deficiencies of some of these vitamins. Vitamin K is known to play an important role in both blood coagulation and bone formation. Supplementation with vitamin K appears to be one way of addressing the deficiency, but there is very limited agreement on the appropriate dose and frequency of use of these supplements. This is an updated version of the review. To assess the effects of vitamin K supplementation in people with cystic fibrosis and to determine the optimal dose and route of administration of vitamin K for both routine and therapeutic use. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Most recent search: 30 January 2017. Randomised and quasi-randomised controlled trials of all preparations of vitamin K used as a supplement compared to either no supplementation (or placebo) at any dose or route and for any duration, in children or adults diagnosed with cystic fibrosis (by sweat test or genetic testing). Two authors independently screened papers, extracted trial details and assessed their risk of bias. Two trials (total of 32 participants) each lasting one month were included in the review and were assessed as having a moderate risk of bias. One was a dose-ranging parallel group trial in children (aged 8 to 18 years); and the other (with an older cohort) had a cross-over design comparing supplements to no treatment, but no separate data were reported for the first intervention period. Neither of the trials addressed any of the primary outcomes (coagulation, bone formation and quality of life). Both trials reported the restoration of serum vitamin K and undercarboxylated osteocalcin levels to the normal range after one month of daily supplementation with 1 mg of vitamin K. Evidence from randomised controlled trials on the benefits of routine vitamin K supplementation for people with CF is currently weak and limited to two small trials of short duration. However, no harm was found and until further evidence is available, the present recommendations should be adhered to.

The clinical benefits of long-term supplementation with omega-3 fatty acids in cystic fibrosis patients – A pilot study

Effectiveness of omega-3 supplementation in cystic fibrosis (CF) remains controversial. This study sought to evaluate clinical status, exercise tolerance, inflammatory parameters, and erythrocyte fatty acid profile after 1 year of oral omega-3 supplementation in CF patients. Fifteen ΔF508-homozygous patients undergoing chronic azithromycin were randomized to receive omega-3 fish oil supplementation at a dose of 60mg/Kg/day or placebo. In comparison with the previous year, in the supplemented group, the number of pulmonary exacerbations decreased at 12 months (1.7 vs. 3.0, p<0.01), as did the duration of antibiotic therapy (26.5 days vs. 60.0 days, p<0.025). Supplementation significantly increased the levels of eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) as early as <3 months of administration, with concomitant decreases in arachidonic acid (AA) levels. This pilot study suggests that long-term omega-3 supplementation offers several clinical benefits as to the number of exacerbations and duration of antibiotic therapy in CF patients.

Glutamine supplementation in cystic fibrosis: A randomized placebo-controlled trial.

Pulmonary infection and malnutrition in cystic fibrosis are associated with decreased survival. Glutamine has a possible anti-microbial effect, with a specific impact against Pseudomonas aeruginosa. We aimed to test the hypothesis that oral glutamine supplementation (21 g/day) for 8 weeks in adults with cystic fibrosis would decrease pulmonary inflammation and improve clinical status. The study design was a randomized double-blind placebo-controlled study design with an iso-nitrogenous placebo. The primary analysis was intention to treat, and the primary outcome was change in induced sputum neutrophils. Thirty-nine individuals were recruited and thirty-six completed the study. Glutamine supplementation had no impact on any of the outcome measures in the intention-to-treat analysis. In the per protocol analysis, glutamine supplementation was associated with an increase in induced sputum neutrophils (P = 0.046), total cells (P = 0.03), and in Pseudomonas isolation agar colony forming units (P = 0.04) compared to placebo. There was no effect of glutamine supplementation on markers of pulmonary inflammation in the intention-to-treat analysis.

Serum 25‐hydroxyvitamin‐D 3 Response to a Large Bolus Dose of Cholecalciferol in Adult Patients with CF

The best vehicle substance for vitamin D supplementation in cystic fibrosis (CF) is unknown. Oil based vehicles may not be well absorbed in patients with CF given the high prevalence of pancreatic ...

Antioxidant supplementation for lung disease in cystic fibrosis

Airway infection leads to progressive damage of the lungs in cystic fibrosis and oxidative stress has been implicated in the etiology. Supplementation of antioxidant micronutrients (vitamin E, vitamin C, ß-carotene and selenium) or glutathione may therefore potentially help maintain an oxidant-antioxidant balance. Current literature suggests a relationship between oxidative status and lung function. To synthesize existing knowledge of the effect of antioxidants such as vitamin C, vitamin E, ß-carotene, selenium and glutathione in cystic fibrosis lung disease. The Cochrane Cystic Fibrosis and Genetic Disorders Group's Cystic Fibrosis Trials Register and PubMed were searched using detailed search strategies. We contacted authors of included studies and checked reference lists of these studies for additional, potentially relevant studies.Last search of Cystic Fibrosis Trials Register: 29 August 2013. Randomized controlled studies and quasi-randomized controlled studies of people with cystic fibrosis comparing antioxidants as listed above (individually or in combination) in more than a single administration to placebo or standard care. Two authors independently selected studies, extracted data and assessed the risk of bias in the included studies. We contacted trial investigators to obtain missing information. Primary outcomes are lung function and quality of life; secondary outcomes are oxidative stress, inflammation, nutritional status, days on antibiotics and adverse events during supplementation. If meta-analysed, studies were subgrouped according to method of administration and the duration of supplementation. One quasi-randomized and nine randomized controlled studies were included, with a total of 436 participants. Eight studies analyzed oral supplementation with antioxidants and two inhaled supplements.One study (n = 46) of an oral combined supplement demonstrated a significant difference in forced expiratory volume at one second expressed as per cent predicted after two weeks in favour of the control group, mean difference -4.30 (95% confidence interval -5.64 to -2.96); however a further study (n = 41) of oral supplementation with glutathione showed a significant improvement in this outcome and in forced vital capacity after six months from the treatment start, mean difference 17.40 (95% confidence interval 13.69 to 21.11) and 14.80 (95% confidence interval 9.66 to 19.94) respectively. The combined supplement study also indicated a significant improvement in quality of life favouring control, mean difference -0.06 points on the quality of well-being scale (95% confidence interval -0.12 to -0.01). Based on one study (n = 41) of oral glutathione supplementation in children, the supplements had a positive effect on the nutritional status (body mass index %) of the patients, mean difference 17.20 (95% confidence interval 12.17 to 22.23). In two studies (n = 83) that supplemented vitamin E, there was an improvement after two months in the blood levels of vitamin E, mean difference 11.78 μM/L (95% confidence interval 10.14 to 13.42).Based on one of the two studies of inhaled glutathione supplementation, there was an improvement in the forced expiratory volume at one second expressed as per cent predicted after three and six months (n = 153), mean difference 2.57 (95% confidence interval 2.24 to 2.90) and 0.97 (95% confidence interval 0.65 to 1.29) respectively. Only one of the studies reported quality of life data that could be analysed, but data showed no significant differences between treatment and control.None of the 10 included studies was judged to be free of bias. There appears to be conflicting evidence regarding the clinical effectiveness of antioxidant supplementation in cystic fibrosis. Based on the available evidence, glutathione (administered either orally or by inhalation) appears to improve lung function in some cases and decrease oxidative stress; however, due to the very intensive antibiotic treatment and other treatments that cystic fibrosis patients receive, the beneficial effect of antioxidants is very difficult to assess in patients with chronic infection without a very large population sample and a long-term (at least six months) study period. Further studies, especially in very young patients, examining clinically relevant outcomes, dose levels, timing and the elucidation of clear biological pathways by which oxidative stress is involved in cystic fibrosis, are necessary before a firm conclusion regarding effects of antioxidants supplementation can be drawn.

Vitamin A supplementation for cystic fibrosis.

People with cystic fibrosis and pancreatic insufficiency are at risk of fat soluble vitamin deficiency as these vitamins (A, D, E and K) are co-absorbed with fat. Thus, some cystic fibrosis centres routinely administer these vitamins as supplements but the centres vary in their approach of addressing the possible development of deficiencies in these vitamins. Vitamin A deficiency causes predominantly eye and skin problems while supplementation of vitamin A to excessive levels may cause harm to the respiratory and skeletal systems in children. Thus a systematic review on vitamin A supplementation in people with cystic fibrosis would help guide clinical practice. To determine if vitamin A supplementation in children and adults with cystic fibrosis:1. reduces the frequency of vitamin A deficiency disorders;2. improves general and respiratory health;3. increases the frequency of vitamin A toxicity. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search of the Group's Cystic Fibrosis Trials Register: 07 April 2014. All randomised or quasi-randomised controlled trials comparing all preparations of oral vitamin A used as a supplement compared to either no supplementation (or placebo) at any dose and for any duration, in children or adults with cystic fibrosis (defined by sweat tests or genetic testing) with and without pancreatic insufficiency. No relevant studies for inclusion were identified in the search. No studies were included in this review. As there were no randomised or quasi-randomised controlled trials identified, we cannot draw any conclusions on the benefits (or otherwise) of regular administration of vitamin A in people with cystic fibrosis. Until further data are available, country or region specific guidelines on the use of vitamin A in people with cystic fibrosis should be followed.

Vitamin D supplementation for cystic fibrosis.

Cystic fibrosis (CF) is a genetic disorder with multiorgan effects. In a subgroup with pancreatic insufficiency malabsorption of the fat soluble vitamins (A, D, E, K) may occur. Vitamin D is involved in calcium homeostasis and bone mineralisation and may have extraskeletal effects. This review examines the evidence for vitamin D supplementation in cystic fibrosis. To assess the effects of vitamin D supplementation on the frequency of vitamin D deficiency, respiratory outcomes and vitamin D toxicity in the cystic fibrosis population. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 08 July 2013. Randomised and quasi-randomised controlled studies of vitamin D supplementation compared to placebo in the cystic fibrosis population regardless of exocrine pancreatic function. Both authors independently assessed the risk of bias of each included study and extracted outcome data (from published study information) for assessment of bone mineralization, growth and nutritional status, frequency of vitamin D deficiency, respiratory status, quality of life and adverse events. Six studies (239 participants) are included, although only three studies provided data from 69 adults and children with cystic fibrosis for analysis. One study compared a single high dose of vitamin D (250,000 IU) to placebo at the time of hospital admission with a respiratory exacerbation in 30 pancreatic insufficient adults with cystic fibrosis. The second study compared supplemental 800 international units (IU) vitamin D and placebo for 12 months in 30 osteopenic pancreatic insufficient adults; both groups continued 900 IU vitamin D daily. The third study compared supplemental 1 g calcium alone, 1600 IU vitamin D alone, 1600 IU vitamin D and 1 g calcium and placebo in a double-blind randomised cross-over study; only nine children who completed both vitamin D and placebo groups after six-months supplementation and a three-month washout period are included; pancreatic sufficiency or disease status of participants are not defined. The studies are not directly comparable due to differences in supplementation, outcome reporting and possibly participant characteristics (e.g. severity of lung disease, growth and nutrition, pancreatic sufficiency).The only outcome for which we could combine data from more than two studies was 25-hydroxyvitamin D levels; patients receiving vitamin D supplementation had significantly higher levels, mean difference 7.24 ng/ml (95% confidence interval 5.01 to 9.46). However, ironically one study reported 1,25(OH)2D with levels significantly favouring the placebo group, mean difference -30.30 pmol/ml (95% confidence interval -59.89 to -0.71). Bone mineral density was measured in two studies; both described no significant change between groups. There were no adverse events in any study.The remaining three studies are published as abstracts only and did not provide data for analysis. These abstracts include: a report of pre-intervention data in a study comparing daily calcitriol (0.25 or 0.5 micrograms) with placebo in pancreatic insufficient children and young adults; an interim report of a double-blind randomised control study comparing 5000 IU vitamin D daily for 12 weeks during winter in 67 adult cystic fibrosis patients; and a comparison of the effect of three months of vitamin D supplementation (dose not specified) with placebo on bone mineral density in 42 children with cystic fibrosis and low bone mineral density.Risk of bias was highly variable between all studies. Only one study had a low risk of bias for the five main criteria (random sequence generation, allocation, blinding, attrition and reporting). The rest of the studies had unclear or high risks of bias. Two studies had a low risk of bias for blinding and another two studies for attrition bias. In the studies published as abstracts, assessment of the risks of bias was uncertain in many aspects. In patients receiving vitamin D supplementation, 25-hydroxyvitamin D levels are significantly higher. However, there is no evidence of clinical benefit or harm in the limited number of small-sized published studies. Adherence to relevant cystic fibrosis guidelines on vitamin D supplementation should be considered until further evidence is available.

Review article: enzyme supplementation in cystic fibrosis, chronic pancreatitis, pancreatic and periampullary cancer

Over 11000 UK patients each year develop pancreatic exocrine insufficiency--the major causes are not rare: cystic fibrosis (>300 new cases/year), pancreatic cancer (>7000 new cases/year) and chronic pancreatitis (>4000 new cases/year). Affected patients present in diverse ways, and for chronic pancreatitis, diagnosis is frequently made rather late in the course of the disease. To raise awareness of key clinical issues specific to patients with pancreatic exocrine insufficiency through experience from UK clinicians, and to offer advice regarding appropriate treatment with pancreatic enzymes. Three case studies describe clinical issues relating to pancreatic enzyme supplementation that may lead to underuse in patients with cystic fibrosis, pancreatic and periampullary cancer or chronic pancreatitis. The efficacy of the treatment of exocrine pancreatic insufficiency is dependent on adequate meal-time enzyme replacement therapy. Improvements in patients' weight and nutritional status are what is aimed for - an important reason for all doctors, nurses and dieticians to give this therapy close attention. Pancreatic exocrine insufficiency may result in malnutrition, but enzyme supplementation can greatly improve quality of life in these patients.

Taurine supplementation in cystic fibrosis.

Taurine supplementation in cystic fibrosis.

Decreased Total Serum Coenzyme-Q 10 Concentrations: A Longitudinal Study in Children with Cystic Fibrosis

To assess total serum levels of coenzyme Q(10) (Co-Q(10)), an important antioxidant, in children with cystic fibrosis (CF) and to investigate an association between Co-Q(10) level and clinical outcome. Co-Q(10) levels were measured annually in a prospective cohort study of 381 children with CF. A total of 1092 serum levels of total Co-Q(10) were obtained by high-performance liquid chromatography and ultraviolet light detection. Associations of Co-Q(10) with demographic variables and clinical outcomes were investigated. Of the 381 initial total serum Co-Q(10) measurements, 188 were in the deficient range. Low Co-Q(10) was significantly more prevalent in patients with pancreatic insufficiency (PI) (55%) compared with patients with pancreatic sufficiency (PS) (3%); 22% of the patients with PI exhibited persistently low Co-Q(10) levels. Low Co-Q(10) levels were significantly associated with Pseudomonas aeruginosa colonization in patients with PI and CF under age 24 months, but not with subsequent lung function or hospitalization rates. Low Co-Q(10) levels were related to other markers of nutritional status, including total lipids, beta-carotene, and alpha-tocopherol. Persistently low total serum Co-Q(10) levels are common in children with CF and PI. A prospective study is indicated to determine whether Co-Q(10) supplementation in CF is beneficial.

The Effect of Zinc Supplements in Cystic Fibrosis Patients

Aim: To study the effect of Zn supplements in cystic fibrosis (CF) on disease evolution. Methods: A retrospective study of all CF patients treated with Zn supplements (January 2002 to December 2004). Data from patient files for the year before and the first year of supplementation were compared. The controls were CF patients with normal serum Zn and without Zn supplementation. Results: 21 patients (7 females), median age 8.9 (interquartile range 13.1) years, received 5 mg/kg Zn sulfate/day (maximum 150 mg). The number of infections decreased from 3 (1.25) to 2 (2.0) (tied p < 0.02) and the forced expiratory volume in 1 s (FEV₁) increased from 72.0 (38.4) to 76.5 (52)% (p < 0.02). Energy intake improved (92.3 (14.5) to 117.0 (28.5)%; tied p < 0.02), as did weight for height (W/H; 90 (9.4) to 94 (8.5)%; tied p = 0.043). In the CF control patients the number of infections (2.0 (2.0)), energy intake (116 (43.3)%) and nutritional status remained stable (W/H 99 (17.2)%), but pulmonary function decreased significantly (ΔFEV₁ loss of 2.0 (8.0)%). There was a significantly different evolution for the change in forced vital capacity (p < 0.004) and ΔFEV₁ (p < 0.001) between supplemented and control patients. Conclusions: Analysis of the clinical data on Zn supplementation in CF showed beneficial effects in Zn-deficient CF patients. These results must be confirmed in a prospective double-blind randomized control trial.