Malnutrition in adults with cystic fibrosis

OBJECTIVE: To determine the prevalence and clinical features of malnutrition and its relationship with the CFTR genotype in a cystic fibrosis (CF) adult population. DESIGN: Cross-sectional study. SETTING: Department of Pulmonology, Cochin Hospital, Paris, France. SUBJECTS: 163 CF adults seen between 1997 and 1999. RESULTS: Mean age was 28.8 y. Mean body mass index (BMI) was 19.1 kg/m2. Malnutrition (BMI<18.5 kg/m2) was seen in 81 patients (49.7%). Its severity was associated with diagnosis of CF before the age of 18 y (P<0.01), FEV1 values below 30% (P<0.01), the yearly decline of FEV1 (P<0.01), pancreatic insufficiency (P<0.01) and gastro-oesophageal reflux (P<0.01). Malnutrition was observed in 58.7% of patients with a severe CFTR genotype but in 28.6% of patients with a mild genotype (P<0.001). CONCLUSION: Malnutrition remains frequent in adults with CF except in patients presenting with a mild CFTR genotype (leading to a mild phenotype and to later diagnosis).     

Nutrition in adults with cystic fibrosis

Improved survival has been associated with better nutritional status in patients with cystic fibrosis (CF). In this study we examined the relationship between nutritional state and other measures of clinical severity in adult patients with CF, attending a regional centre. Eighty-one patients (median age 21 years) were studied. Patients with CF were significantly under weight, compared to healthy individuals but were of similar height. Measurements of lung function, FEV1 and FVC were significantly related to body mass index. Lung function was poorer in patients with chronic pseudomonal infection but body weight and body mass index were not significantly different compared to those without such infection. In 53 patients who were alive 4 years later, FEV1 had declined by -10.5 (2.1)% (P < 0.001) but there was no significant change in body weight 1.5 (6.5) kgs. In 23 patients who died or had lung transplantation the change from 1994 to the date of death or transplantation the FEV1 was reduced by -7.9 (11.2)% (P = 0.004) and body weight -2.8 (4.4) kgs (P < 0.01). In 12 patients who had supplemental enteral feeding, the median increase in body weight was 7|kgs over a period of 12 months. This study confirms that young adult patients with CF are significantly under weight and declining health is associated with significant weight loss. In patients with severe malnutrition significant improvement can be achieved by enteral feeding.     

Reproductive health in women with cystic fibrosis

The life expectancy of women with cystic fibrosis has doubled in the last 20 years. A major implication of this is the advent of previously unseen reproductive health problems. We review the management problems presented by these women throughout their reproductive lives, including pregnancy.     

The first heart-lung transplantation in a Hungarian patient with cystic fibrosis

INTRODUCTION: Progressive lung disease and respiratory insufficiency is the leading cause of early death for patients with cystic fibrosis. METHOD: Successful heart-lung transplantation was performed in the 24 years old man with cystic fibrosis for end stage lung diseases and for concomitant dilatative cardiomyopathy. RESULTS: Six months following the operation the patient returned to full-time his job, and leads an active life like other fellow-men of his age. CONCLUSIONS: The attention is called with this case report to a rarely necessary intervention available for Hungarian patients as well. The criteria of the lung transplantation in cystic fibrosis, the preoperative screening and the tasks of the postoperative care are summarised.     

Parenteral nutrition in a pregnant cystic fibrosis patient

This case report describes a 30-year-old pregnant woman with cystsic fibrosis who had marasmus as evidenced by clinical examination. The patient was maintained on central venous hyperalimentation for 18 days in her last trimester. The patient delivered a full-term infant via cesarian section with Apgar of 8 and 9, at 1 and 5 minutes, respectively, and adverse affects.     

Gout and hyperuricaemia in adults with cystic fibrosis

Gout has not been described previously as a complication in cystic fibrosis (CF). Here we present data on nine CF patients who have presented with symptoms of acute gout. This gives an estimated prevalence of gout of around 2.5% in our adult CF population, compared to a previously described prevalence in the non-CF population of just over 1%. Serum urate is measured routinely at the annual review in our unit. Mean (SD) serum urate was 0.40 (0.09) mmol/L in male CF patients (n = 108) and 0.31 (0.08) mmol/L in female patients (n = 74). This was significantly greater than in historical controls. Thirty-seven percent of male CF patients and 36% of female patients had serum urate levels above the upper limit of normal.     

Nitrogen deposition in malnourished children with cystic fibrosis

To determine the protein nutritional status of 21 malnourished children with cystic fibrosis (CF), total body nitrogen (TBN) was measured and the results were compared with 21 control subjects. CF patients demonstrated a lower TBN (P less than 0.001). When matched for height (n = 10) or bone age (n = 13), the CF patients still had a depressed TBN/height or TBN/lean body mass (P less than 0.05). To assess nitrogen deposition during nutritional rehabilitation, repeat TBN measurements were performed on the 21 CF patients. Nitrogen deposition ranged from -230 to 550 g/y and correlated with weight velocity (r = 0.78, P less than 0.001). Increased nitrogen deposition (greater than 150 g/y) was generally associated with normal height gain (height velocity SD score greater than -2.00) and weight gain (greater than 2.0 kg/y). Decreased nitrogen deposition was associated with poor weight gain but did not preclude normal linear growth. These data suggest an important role for TBN estimations in defining protein nutritional status in children and indicate that skeletal growth can continue in the presence of minimal nitrogen deposition.     

Telehealth in cystic fibrosis: a systematic review

We conducted a systematic review of the use of telehealth in people with Cystic Fibrosis (CF). The studies reviewed were of adults and children with CF, and incorporated telehealth for monitoring symptoms, assessing adherence to prescribed therapies or providing a therapeutic intervention. Searches of four electronic databases returned 293 references. Eight studies met the inclusion criteria. Variability in study design and outcome measures precluded meta-analysis. Seven studies assessed telemonitoring feasibility for patient usability and acceptance, or for physiological monitoring. Two studies were randomised controlled trials, although only one showed differences in outcome between the intervention and usual care with improved spirometry stability and significantly increased antibiotic use in the intervention group. In four studies participants were asked to transmit data on spirometry (FEV(1)) or symptoms. Participant non-compliance with data reporting ranged from 43-63%. Generally, participants reported being able to use the required technology. There is insufficient evidence to reach a firm conclusion about the benefits of telehealth in people with CF, but it remains a promising area for future investigation.     

Mycobacterium abscessus and children with cystic fibrosis

We prospectively studied 298 patients with cystic fibrosis (mean age 11.3 years; range 2 months to 32 years; sex ratio, 0.47) for nontuberculous mycobacteria in respiratory samples from January 1, 1996, to December 31, 1999. Mycobacterium abscessus was by far the most prevalent nontuberculous mycobacterium: 15 patients (6 male, 9 female; mean age 11.9 years; range 2.5-22 years) had at least one positive sample for this microorganism (versus 6 patients positive for M. avium complex), including 10 with >3 positive samples (versus 3 patients for M. avium complex). The M. abscessus isolates from 14 patients were typed by pulsed-field gel electrophoresis: each of the 14 patients harbored a unique strain, ruling out a common environmental reservoir or person-to-person transmission. Water samples collected in the cystic fibrosis center were negative for M. abscessus. This major mycobacterial pathogen in children and teenagers with cystic fibrosis does not appear to be acquired nosocomially.     

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.     

Nutrition for patients with cystic fibrosis.

Cystic fibrosis (CF) is the most frequent, lethal genetic disorder among northern Europeans. The etiology of this autosomal recessive disease is known to be a defect in the cAMP activation of chloride (Cl-) channels in secretory cells in many organs of the body. Although this defect usually leads to severe lung disease, many of these patients also have nutritional deficiencies. Nutrition is one of the key components in the management of CF. Patients are at high risk for malnutrition, which may result in accelerated progression of the disease and increased morbidity. This review will discuss nutrition recommendations for calories, protein, vitamins and minerals, and enteral and parenteral nutrition support practices.