Nutritional status of an adult cystic fibrosis population

Nutritional management and dietary recommendations in patients with cystic fibrosis (CF) have changed considerably over the past 10-15 y. The nutritional status of adult CF patients was assessed in a clinical survey before these changes in nutritional management. The aim of the study was to assess the current nutritional status of the CF population and compare the results with those of the previous study. Forty-three (24 male, 19 female) subjects participated in this study. Subjects' height, weight, mid-upper-arm circumference, and skinfolds at four sites were measured. Nutritional intake was measured by using a 7-d food intake diary including documentation of supplements taken. Compared with the 1983 study, the incidence of malnutrition, as indicated by a body mass index of less than 20, has decreased from 62% to 9%. Furthermore, there have been significant improvements in the weight, height, and body mass index of both males (P < 0.001) and females (P < 0.04). Individuals with CF are no longer subject to growth arrest, as their mean height is now comparable to the Australian average. Mid-upper-arm circumference (P < 0.0001), triceps skinfold (P < 0.0001), and percentage of body fat (P < 0.05) of males and females have also significantly increased. The fat intake (P < 0.02) of females and males and energy intake (P < 0.03) of females have increased significantly, and the mean energy intake of subjects has exceeded the recommended 120% of the recommended daily intake. A significant number of patients in the present study receive dietary oral and/or enteral supplements. Multiple linear regression analysis indicated that nutritional management was principally responsible for improvements in nutritional status. The findings suggest that there has been a significant improvement in the nutritional status of the adult CF population, which may be due primarily to changes in nutritional management.     

Nutritional status, perceived body image and eating behaviours in adults with cystic fibrosis

BACKGROUND & AIMS: Achieving and maintaining an ideal nutritional status is the primary aim of the nutritional management of cystic fibrosis (CF). It is unclear how nutritional interventions impact on patients' perceptions and behaviours concerning body image and eating. This work aimed to provide a psychosocial profile and compare CF patients receiving (a) enteral tube feeding, (b) nutritional supplements, (c) no nutritional interventions, and (d) healthy controls. METHODS: A cross-sectional questionnaire design was employed. Age, gender, lung function, and body mass index were recorded. Subjects completed measures of eating attitudes, perceived and desired body shape, body image, self-esteem and quality of life (QoL). RESULTS: A minority of CF patients reported disordered eating. Those receiving nutritional interventions engaged in less dieting behaviour. All CF groups, especially intervention groups, received more pressure from others to eat. For females, control groups desired to be slimmer whereas intervention groups desired to be heavier. Healthy males were content with their body whereas CF males wished to be heavier. Patients receiving enteral tube feeding were less satisfied with their body image, reported lower self-esteem and poorer QoL. CONCLUSION: Body image and eating behaviours are important considerations of nutritional interventions for maintaining QoL.     

Nutritional status of infants with cystic fibrosis associated with early diagnosis and intervention

The purpose of this study was to characterize the nutritional status of infants diagnosed with cystic fibrosis (CF) through neonatal screening and to determine if they would achieve normal nutrition when managed with early intervention. In addition, nutrient intake was assessed to determine energy and macronutrient-consumption patterns. Evaluation of growth revealed that normal patterns could be achieved with mean energy intake values at ages 6 and 12 mo of 481 and 426 kJ/kg body wt (115 and 102 kcal/kg body wt), respectively. Biochemical assessment demonstrated low alpha-tocopherol and linoleic acid values at diagnosis in the majority of infants whereas one-third had abnormal indices of protein nutriture. Essential fatty acid deficiency was also demonstrated at diagnosis by abnormal triene-tetraene ratio values in 27% of screened infants. With predigested formula and dietary supplementation, there was improvement in all indices of nutritional status and only a low percentage of patients showed mild biochemical abnormalities at age 12 mo.     

Nutritional management of cystic fibrosis patients

PURPOSE OF REVIEW: We critique recent advances in nutritional care of patients with cystic fibrosis focusing on our understanding of the role of fecal elastase as a pancreatic function test, enzyme replacement therapy and bone health. RECENT FINDINGS: Fecal elastase measured by the monoclonal antibody method is a useful screening test for delineating cystic fibrosis patients with pancreatic insufficiency (needing enzyme replacement therapy) from those with pancreatic sufficiency. Cutoff levels, however, have not been clearly defined, particularly in infancy. Newer enzyme products that attempt to improve the intralumenal intestinal pH with bicarbonate or using a combination of unprotected and enteric coated products fail to completely correct nutrient maldigestion. Compromised bone health in cystic fibrosis patients may be due to multiple factors including calcium, vitamin D, and vitamin K deficiencies and lung inflammation. Current recommendations for treatment of bone health in cystic fibrosis are not evidence-based. SUMMARY: Fecal elastase is a useful marker of pancreatic function but limitations for this test exist. The presence of an acidic intestinal milieu and hitherto poorly defined intestinal factors may contribute to the failure of current enzyme preparations to correct nutrient assimilation in cystic fibrosis. The many factors that contribute to bone health must be evaluated when developing treatment strategies.     

Nutritional status is an important predictor of diaphragm strength in young patients with cystic fibrosis

BACKGROUND: The effect of nutritional status and lung disease progression on diaphragm strength in young patients with cystic fibrosis remains unclear. OBJECTIVE: The aim of this study was to investigate the effect of nutritional status and airway obstruction on diaphragm strength. DESIGN: Twitch transdiaphragmatic pressure (Tw Pdi) obtained by bilateral anterior magnetic phrenic nerve stimulation, body mass index (BMI) z score, fat mass, fat-free mass (FFM), arm muscle circumference (AMC), forced expiratory volume in 1 s (FEV(1)), and functional residual capacity (FRC) were measured in 20 patients aged 15.1 +/- 2.8 y (x +/- SD). Values were expressed as a percentage of predicted values. RESULTS: Mean (+/-SD) Tw Pdi was 24.3 +/- 5.5 cm H(2)O. Univariate regression analysis showed positive correlations between Tw Pdi and nutrition scores (BMI z score: r = 0.63, P = 0.003; FFM: r = 0.47, P = 0.04; AMC: r = 0.45, P = 0.04), airway obstruction (FEV(1): r = 0.68, P = 0.001), and arterial oxygen partial pressure (r = 0.68, P = 0.001). Negative correlations were observed between Tw Pdi and dynamic hyperinflation (FRC: r = -0.65, P = 0.005) and arterial carbon dioxide pressure (r = -0.50, P = 0.03). Furthermore, stepwise regression analysis showed that Tw Pdi correlated with BMI z score (r = 0.75, P = 0.0002) and FEV(1) (r = 0.69, P = 0.001). CONCLUSIONS: Diaphragm strength is relatively well preserved in young patients with cystic fibrosis. However, the strength of the diaphragm decreases with the progression of malnutrition and airway obstruction.     

Nutritional management of cystic fibrosis in Australia and New Zealand

Aim:  To document current practice in the nutritional management of cystic fibrosis in Australia and New Zealand and to examine changes in practice since 1996.
Methods:  Thirty-four cystic fibrosis services in Australia and New Zealand responded to a survey that examined current nutritional management practices and dietetic staffing levels. The questionnaire was based on a previous survey conducted in 1996.
Results:  Cystic fibrosis dietetic staffing levels were low. No service met the staffing level recommended by the UK Cystic Fibrosis Trust. Minor practice variations existed in nutrition assessment and monitoring: nutrition support and vitamin supplementation. Changes in the management of pancreatic enzyme replacement therapy and cystic fibrosis-related diabetes were reported since 1996.
Conclusion:  This survey highlights that improvements in consistency of nutrition management have been achieved since 1996 and reflect adherence to available clinical guidelines for pancreatic enzyme replacement therapy. While there are many areas of agreement in nutrition care for people with cystic fibrosis around Australia and New Zealand, there are still practice differences, implying that Australian- and New Zealand-specific clinical guidelines are warranted. The implementation of the Australasian Clinical Practice Guidelines for Nutrition in Cystic Fibrosis will be important in standardising and improving cystic fibrosis care.     

囊性纤维化患者的营养干预与随诊

目的　探讨规范化营养评价、干预、监测对囊性纤维化患者营养状况的影响。方法　纳入2005年6月至2015年6月间北京协和医院诊断囊性纤维化的病例6例，评价临床资料及其营养状况，进行营养干预，随访4例患者，评价营养干预对营养状况的影响及患者依从性。结果　6例囊性纤维化患者疾病诊断时平均年龄（19±6）岁，患者均存在反复的肺部感染和不同程度的低氧血症。所有患者营养状况均不同程度受损。20岁以下的3例患者体质量指数（BMI）与同年龄同性别人群BMI生长曲线相比均小于第3百分位。2例存在轻度贫血（均为小细胞低色素性     

Nutritional rehabilitation of malnourished patients with cystic fibrosis

We examined the effect of nutritional rehabilitation in cystic fibrosis patients with severe disease. Thirteen malnourished patients (seven males, six females, age 7-27 yr) were studied over 7-16 mo. Oral supplementation was attempted initially in 12 patients (mean duration 6.7 mo); only 2 patients gained weight, 2 withdrew, and 1 died. The remaining 7 patients failed to achieve adequate weight gain and were commenced on nasogastric supplementation with a semisynthetic formula. An additional patient was entered without a prior period of oral supplementation because of the severity of malnutrition. Weight gain was achieved in 7 of 8 patients with nasogastric supplementation (mean duration 6.4 mo). Weight gain was associated with an increase in lean body mass, total body fat, and height velocity. While pulmonary function and biochemical parameters were unchanged, patient well-being improved and episodes of pneumonia decreased.     

Zinc status and vitamin A transport in cystic fibrosis

Zinc status and the retinol transport system were examined in 18 retinol supplemented cystic fibrosis (CF) patients and 40 age-matched controls. Plasma vitamin A was significantly lower in the CF group as compared to the controls and correlated positively with plasma retinol-binding protein (RBP) in both the CF and control groups. Plasma zinc of the CF group was not significantly lower than controls whereas hair zinc was. Plasma zinc was positively correlated with plasma RBP, vitamin A, and albumin in the CF group but not in the controls. Plasma concentrations of vitamin A, RBP, albumin, and zinc decreased with age in the CF group but not in the controls. The data support previous suggestions that low plasma vitamin A levels in CF are due to defects in the retinol transport system. The zinc status of the CF groups as a whole was judged to be low-normal however a subgroup of CF patients were in the marginal to deficient category. This subgroup also had lower levels of plasma vitamin A and RBP. The data suggest that zinc may be a contributing factor in the low plasma vitamin A/RBP levels of CF patients with marginal or deficient zinc status.     

Nutrient status of adults with cystic fibrosis

Nutrition is thought to influence disease status in patients with cystic fibrosis (CF). This cross-sectional study sought to evaluate nutrient intake and anthropometric data from 64 adult outpatients with cystic fibrosis. Nutrient intake from food and supplements was compared with the Dietary Reference Intakes for 16 nutrients and outcomes influenced by nutritional status. Attention was given to vitamin D and calcium given potential skeletal implications due to cystic fibrosis. Measurements included weight, height, body composition, pulmonary function, and serum metabolic parameters. Participants were interviewed about dietary intake, supplement use, pulmonary function, sunlight exposure, and pain. The participants’ mean body mass index (±standard deviation) was 21.8±4.9 and pulmonary function tests were normal. Seventy-eight percent used pancreatic enzyme replacement for malabsorption. Vitamin D deficiency [25-hydroxyvitamin D (25OHD)<37.5 nmol/L] was common: 25 (39%) were deficient despite adequate vitamin D intake. Lipid profiles were normal in the majority, even though total and saturated fat consumption represented 33.0% and 16.8% of energy intake, respectively. Reported protein intake represented 16.9% of total energy intake (range 10%-25%). For several nutrients, including vitamin D and calcium, intake from food and supplements in many participants exceeded recommended Tolerable Upper Intake Levels. Among adults with cystic fibrosis, vitamin D deficiency was common despite reported adequate intake, and lipid profiles were normal despite a relatively high fat intake. Mean protein consumption was adequate, but the range of intake was concerning, as both inadequate or excessive intake may have deleterious skeletal effects. These findings call into question the applicability of established nutrient thresholds for patients with cystic fibrosis.     

Nutritional, glycometabolic and genetic factors affecting menarcheal age in cystic fibrosis

Aims of this study were to investigate menarcheal age (MA) and menarcheal determinants in 25 girls with cystic fibrosis (CF) and to compare their MA with their respective mothers'. Patients' MA (13.3 +/- 1.1 yr) was on average significantly higher (p<0.0005) than that of the respective mothers (12.2 +/- 1.0 yr) and positively related to it (r=0.055, p<0.005). Six girls experienced menarche after 14.2 yr, ie after the uppest limit of their mothers' MA range. The only parameter which significantly differentiated these 6 patients from the remaining 19 cases was body mass percentile (BMP). Moreover, in the entire patient series a negative correlation was found between MA and BMP. None of the other clinical parameters correlated significantly with MA. No differences in terms of MA were detected in the subgroups of patients with a different glucose tolerance (GT) status and the 12 girls with a pathological GT were not older at menarche than those with normal GT. No correlations were found between either glucose or insulin areas during oral GT test and MA. In the subgroups of patients with a different genotype menarche occurred at a similar age, irrespectively of their genotype. On the basis of our findings we conclude that: a) a menarcheal delay of approximately 1 yr exists between CF girls and their mothers; b) menarcheal delay in CF is not related to either genotype or disease severity or glycometabolic status; c) the only two factors which are able to affect MA in CF are maternal MA and nutritional status.     

Altered copper status in adult men with cystic fibrosis

OBJECTIVES: To measure indices of copper status in adult men with cystic fibrosis (CF). A previous study in children showed changes in copper homeostasis compared to controls. This study was designed to investigate whether this observation persisted into adulthood. METHODS: This was a case-control age-matched study using seven men with CF and six healthy men. Blood samples were drawn into metal free tubes and fractionated into plasma, polymorphonuclear cells, mononuclear cells and erythrocytes. Cell fractions were assayed for copper and CuZn-superoxide dismutase; plasma was assayed for ceruloplasmin. RESULTS: The men with cystic fibrosis had significantly greater plasma copper and ceruloplasmin activity, yet had significantly lower copper-zinc superoxide dismutase activity in mononuclear and polymorphonuclear cells. Furthermore, the mononuclear cells of the cystic fibrosis subjects had about 45% percent less copper-zinc superoxide dismutase protein. Cellular copper levels were not statistically different between the two groups. A significant correlation was found between lung function and copper-zinc superoxide dismutase activity in the polymorphonuclear cells. Iron status was normal. CONCLUSIONS: The results indicate that individuals with cystic fibrosis have altered copper distribution compared to control individuals. Some aspects are characteristic of an inflammatory response; however, other measures suggest that copper homeostasis may be abnormal. It is not known whether the deviation in copper homeostasis in these individuals is a result of poor copper absorption, inadequate dietary intake, a result of their chronic inflammation or a direct effect due to the defect in ion transport caused by the disease. However, this research suggests that the severity of the disease and the activity of a copper dependent enzyme may be related. Further work will be necessary to determine the cause of the abnormal copper homeostasis and whether correcting it has any bearing on the course of the disease.     

The nutritional status of children with cystic fibrosis

The importance of nutritional intervention for children with cystic fibrosis (CF) is well recognised. It would be expected that the increase in knowledge over the past decade would be reflected in improvements in nutritional status for the CF paediatric population. The aim of the present paper was to evaluate the nutritional status of children with CF, cross-sectionally and longitudinally. Body cell mass adjusted for gender and size (BCM/Htp) was measured in sixty-four children with CF to represent nutritional status and expressed as a Z-score. The cross-sectional results showed a mean BCM/Htp Z-score of 0.54 (sd 1.21), with males having a slightly higher Z-score than females but with a larger variation. At the initial measurement, only one female and one male were considered sub-optimally nourished. The longitudinal analysis after 2 years showed that the mean population had a significantly decreased BCM/Htp Z-score; however, when each gender was analysed separately, this decrease was significant only in the males. At the final measurement, only two females and three males were considered sub-optimally nourished. It is evident from our results that children with CF are well nourished, with only a small percentage considered malnourished. It appears that nutritional status decreases with age, with this decline being more evident in males. These results signify that although children with CF are better nourished with current treatment support, intervention needs to continue throughout a CF patient's life to counteract the changes that occur with age.     

Nutritional status and pulmonary function in patients with cystic fibrosis with and without Burkholderia cepacia colonization: role of specialist dietetic support

We have studied the impact of the introduction of a specialist dietetic service and Burkholderia cepacia colonization on nutrition and lung function in cystic fibrosis (CF) patients in Nottingham, over a 3-year period from 1990 to 1993. We performed cross-sectional analyses of all patients attending the adult clinic (42 in 1990 and 48 in 1993) and longitudinal analysis of the 34 patients who were present in both years. Nutritional status improved in the whole clinic population following the introduction of a specialist dietetic service in 1991. The presence of colonization with Burkholderia cepacia was associated with an accelerated decline in lung function compared with patients colonized with other organisms. Despite this decline, nutritional status was well maintained in both groups of patients. This study shows that the introduction of a specialized dietetic service can result in better nutrition even in patients with declining lung function.     

Nutritional supplement attenuates selected oxidative stress markers in pediatric patients with cystic fibrosis

Our aim was to test the hypothesis that nutritional supplement (AquADEKs; Axcan Scandipharm Inc., Birmingham, Ala, USA) with various pharmaceutical forms of such as chewable tablets, capsules, and liquid administered daily for 12 weeks would reduce oxidative stress and enhance antioxidant status in pediatric patients with cystic fibrosis (CF). A total of 50 patients with CF and 21 healthy children were included in the study. Patients were divided into 4 groups: group A received supplementation with vitamins A (3 mg daily), E (200 mg daily), and D₃ (20 μg daily); group B was supplemented with AquADEKs chewable tablets; group C received the recommended amount of AquADEKs capsules; and group D was supplemented with AquADEKs liquid. The level of oxidative stress was determined by the analysis of activities of enzymes neutralizing reactive oxygen species and by the estimated markers of intensity of free radical processes. There was no difference in the activity of erythrocyte catalase, hydroperoxides level, and sulfhydryl group content in blood plasma between patients with CF and healthy children. The plasma total antioxidant status was decreased in all CF groups compared with the control. The supplementation with either AquADEKs chewable tablets or capsules normalized the malondialdehyde level in plasma. AquADEKs in various pharmaceutical forms normalized the sulfhydryl group content of erythrocytes. The superoxide dismutase activity was increased to near control level in the patients supplemented with either AquADEKs chewable tablets or liquid as compared with the group supplemented with vitamins or with AquADEKs capsules. In conclusion, AquADEKs attenuates selected oxidative stress markers in pediatric patients with CF.