Abstracts
Carnitine & CF
Carnitine (1)
Carnitine metabolites were evaluated in cystic fibrosis infants (#23). They were treated with a carnitine and a predigested formula for 6 to 12 months. At the time of diagnosis, total, free and long chain plasma concentrations of carnitines were significantly less than the 48 controls. The total and free carnitine concentrations were normalized with nutritional supplementation.
There may be a disturbed regulatory control resulting in increased utilization in cystic fibrosis patients. It is noted acylcarnitine is low in cord blood in patients with cystic fibrosis. This suggests there may be an alteration in fatty acid metabolism in utero.
"Carnitine Metabolites in Infants With Cystic Fibrosis: A Prospective Study", Lloyd-Still, John D., et al, Acta Pediatrica, 1993;82:145-9.
Carnitine (2)
Carnitine is derived from the amino acid lysine, which is absorbed normally in cystic fibrosis patients. Abnormal carnitine metabolism found in the cystic fibrosis cord blood may be secondary to disturbed fatty acid metabolism.
Carnitine is involved in the oxidation of long chain fatty acids, medium chain fatty acids and substrates in the peroxisomes, and transports an acyl group into the cell to be used as a substrate reaction in that compartment.
"Acetylcarnitine is Low in Cord Blood and Cystic Fibrosis", Lloyd-Still, J.D., et al, ACTA Pediatr. Scand., 1990;79:427-439.
Antioxidants & CF
Carotenoid Levels
Lowered plasma carotenoid levels in the cystic fibrosis patients compared to the normal children may be due to rapid turnover of carotenoids, needed for quenching of toxic oxygen species generated in theinflammatory states of cystic fibrosis. Supplementation of antioxidants such as beta-carotene, warrant further study in cystic fibrosis patients.
"Carotenoid Levels in Normal Children and in Children With Cystic Fibrosis", Homnick, Douglas N et al, Journal of Pediatrics, May 1993;122(5/Part I):703-707.
Free Radicals and Antioxidant Status
Cystic fibrosis patients (#24) were compared to controls without the disease for antioxidant status.
Cystic fibrosis children were found to have elevated levels of ascorbic acid (94.6 vs 65.6 umol/L), plasma uric acid (330.8 vs 198.0) and sulfhydryl group (518 vs 363 umol/L). Vitamin E levels were lower.
The total radical-trapping antioxidant potential in cystic fibrosis patients was negatively correlated with high ascorbic acid concentration suggesting a pro-oxidant effect of vitamin C. With oral supplementation of vitamin C, there was a reduced total radical-trapping antioxidant potential.
"Reduced Free-Radical-Trapping Capacity and Altered Plasma Antioxidant Status in Cystic Fibrosis", Langley, S.C., Pediatric Research, 1993;33(3):247-250.
EFAs & CF
Essential Fatty Acids (1)
A study of 16 cystic fibrosis patients between 6.6 and 20 years of age compared to 16 healthy controls showed lean body mass, fat-free mass and fat mass were similar in the two groups. There was a higher ingestion of macronutrients by the cystic fibrosis patients compared to controls. Plasma phospholipid palmitoleic acid and eicosatrienoic acid were higher, while linoleic acid and docosahexaenoic acid were lower in the cystic fibrosis patients. The linoleic acid/arachidonic acid ratio was lower and the eicosatrienoic acid/arachidonic acid ratio was higher in cystic fibrosis subjects compared to controls. Essential fatty acid deficiency is present in young cystic fibrosis subjects in the absence of protein- energy malnutrition.
Roulet, M., et al: Essential Fatty Acid Deficiency in Well Nourished Young Cystic Fibrosis Patients, European Journal of Pediatrics, 1997;156:952-956.
Essential Fatty Acids (2)
Cystic fibrosis infants showed deficient patterns in a variety of omega-6 and omega-3 polyunsaturated fatty acids. Total saturated fatty acids and monounsaturated fatty acids were significantly elevated compared with normal infants. There appeared to be less fluidity in the plasma phospholipids of cystic fibrosis patients. Nutritional supplementation of omega-6 and omega-3 polyunsaturated fatty acids is warranted.
"Essential Fatty Acid Status and Fluidity of Plasma Phospholipids in Cystic Fibrosis Infants", Lloyd-Still, John D., et al, American Journal of Clinical Nutrition, 1991;54:1029-35.
Fat Soluble Vitamins
Reviews the importance of supplementation of fat soluble vitamins in cystic fibrosis patients. Studies have documented low levels of fat soluble vitamins in patients with cystic fibrosis
Pancreatic enzyme replacement therapy and routine vitamin supplementation eliminated many clinical deficiencies of fat soluble vitamins in children who were diagnosed and treated early.
Cystic fibrosis patients are still at risk for vitamin deficiency if the diagnosis is made late, if part of the bowel has been resected, or if fat soluble vitamin supplements are not taken.
As the prognosis for cystic fibrosis patients continues to improve, clinical problems due to fat soluble vitamin deficiencies will become more prevalent. Those at greatest risk are those with severe malabsorption, poor compliance or those with liver disease.
"Fat-Soluble Vitamins in Cystic Fibrosis," Rayner, RJ. Proceedings of the Nutrition Society, 1992;51:245-250.
Vitamins & CF
Vitamin A, Vitamin E
Cystic fibrosis patients with low levels of vitamins A and E exhibited poor lung function, and needed more intravenous antibiotic therapy than those with normal vitamin levels. In 68 patients with cystic fibrosis, 46% had normal vitamin A and E levels, 26% had low levels of either vitamin A or vitamin E, and 28% had low levels of both. According to this study, deficient individuals require regular monitoring of fat-soluble vitamins in the serum. Supplementation may also be necessary.
Watkin, S., et al: Vitamin A and E and Pulmonary Function in Cystic Fibrosis, Medical Science Research, 1998;26:97-99.
Vitamin A Deficiency and Electro-Oculogram
Vitamin A deficiency can cause an abnormal electro-oculogram. These findings are important for patient populations who are susceptible to vitamin A deficiency and night blindness (includes patients with cystic fibrosis, jejunal bypass surgery, and cirrhosis of the liver). Supplementation with 25,000 I.U. of vitamin A returned serum levels to low normal ranges and normalized the electro-oculogram. The electro-oculogram may be a beneficial diagnostic tool in vitamin A deficiency.
"Electro-Oculogram in Vitamin A Deficiency Associated With Cystic Fibrosis", Leguire, L et al, Ophthalmic Pediatrics, 1992;13(3):187-189.
Vitamin E
Evaluated the short term changes in erythrocyte à-tocopherol after a single dose of 100 mgs of all-rac-à-tocopheryl acetate/kg in 10 vitamin E-deficient cystic fibrosis patients.
Oral all-rac-à-tocopheryl acetate can be rapidly incorporated into erythrocyte membranes in vitamin E-deficient cystic fibrosis patients.
"Short-Term Changes in Erythrocyte à-Tocopherol Content of Vitamin E-Deficient Patients With Cystic Fibrosis", Winklhofer-Roob, Brigitte M., et al, The American Journal of Clinical Nutrition, 1992;55:100-3.
Vitamin Therapy
Reviews the current status of vitamin supplementation in cystic fibrosis.
Clinical fat-soluble vitamin deficiency has been recognized in cystic fibrosis for years. Cystic fibrosis seems to affect vitamin A metabolism because high liver vitamin A levels have been found in the face of low serum levels. This suggests a defect in mobilization or transport.
Vitamin D can be synthesized in the body regardless of gut function. Most cystic fibrosis patients taking 400 to 800 units of vitamin D per day have serum levels in the low normal range.
Vitamin K deficiency has not been reported in cystic fibrosis outside the new-born period.
Blood levels of vitamin E, which plays an antioxidant role in human metabolism, are generally low in cystic fibrosis patients. Water-miscible forms of 1 mg/kg per day of vitamin E was shown to absorb better than fat-soluble forms.
Vitamin A in a water-miscible form has been shown to be better absorbed.
Using enzyme testing, clinical deficiencies of vitamins B1, B2 and B6 have not been shown in cystic fibrosis patients.
Vitamin C levels are generally normal in cystic fibrosis patients, except for children in the low normal range.
Recommended dosages for vitamin A for cystic fibrosis patients range between 5,000 to
10,000 u; vitamin D at 400 to 800 u; and vitamin E at 50 to 200 u. Vitamin K may be needed if there is a prolonged prothrombin time. Cystic fibrosis patients should take twice the Recommended Daily Allowance of complex vitamins B and C.
"Vitamin Therapy and Cystic Fibrosis - A Review in Rationale", Peters, S. A. & Rolles, C. J., Journal of Clinical Pharmacy and Therapeutics, 1993;18:33-38.
Intestinal permeability & CF
Intestinal Permeability
There was an increase in permeability to 51Cr-EDTA in patients with cystic fibrosis, compared with the control group. Orocecal transit time was prolonged and mean breath hydrogen concentrations were significantly greater.
There is a severe problem with intestinal permeability and a prolonged small intestinal transit time in children with cystic fibrosis.
"Intestinal Permeability to 51Cr-EDTA and Orocecal Transit Time and Cystic Fibrosis", Escobar, H., et al, J of Pediatric Gastroenterology and Nutrition, 1992;14(2):204-207.
Leukotriene & CF
Leukotriene B4
Leukotriene B4 was the predominant eicosanoid in the cystic fibrosis airway. Prostaglandins and thromboxanes were elevated in cystic fibrosis compared to the healthy subjects. This data suggests that in cystic fibrosis, airways contain sufficient amounts of leukotriene B4 both to recruit neutrophils into the airways and to stimulate neutrophils to release their injurious products. Therapies that can interfere with the production or action of leukotriene B4 may be beneficial in cystic fibrosis and other lung diseases with a significant neutrophil response.
"Leukotriene B4 Markedly Elevated in Epithelial Lining Fluid of Patients With Cystic Fibrosis", Konstan, MW et al., American Review of Respiratory Diseases, 1993;148:896-901.
Nutritional Assessment & CF
Contents:
Beta-Carotene
Nutritional Assessment and Management
Nutritional State and Lung Disease
Beta-Carotene
Cystic fibrosis (CF) patients exhibit significantly lower levels of plasma beta-carotene concentrations than those of a control group. This study, conducted on 24 cystic fibrosis patients and 14 healthy controls, revealed that oral beta-carotene supplementation was effective in normalizing status of beta-carotene and malondialdehyde levels in CF patients. Beta-carotene concentrations in the cystic fibrosis supplemented group increased rapidly and reached a value of 0.6 umol/l after 12 weeks of supplementation. Total antioxidant capacity of the cystic fibrosis supplemented group increased after 12 weeks of supplementation at an extent of 12%.
Rust, P., et al: Effects of Long Term Oral -Carotene Supplementation on Lipid Peroxidation in Patients With Cystic Fibrosis, International Journal of Vitamin and Nutrition Research, 1998;68:83-87.
Nutritional Assessment and Management
A consensus meeting convened by the Cystic Fibrosis Foundation regarding nutrition and cystic fibrosis.
Assessment of nutritional status includes weight, height, head circumference, midarm circumference and triceps skin fold test thickness. Dietary intake and a 3 day fat balance is recommended. Laboratory studies include a complete blood count, serum and plasma retinol, serum and plasma à-tocopherol, albumin, electrolytes and acid-base status.
There are 5 categories of nutritional management for a cystic fibrosis patient: routine management, anticipatory guidance, support of intervention, rehabilitative care and resuscitative and palliative care.
Nutrients are as follows: A basic multivitamin and mineral supplementation with specific emphasis on vitamins A and E and sometimes vitamin D. Vitamin K deficiency in cystic fibrosis needs further evaluation.
Children between 2 and 8 years of age need a standard multiple vitamin containing 400 I.U. of vitamin D, 5,000 I.U. of vitamin A and a dose of 1 tablet. Older children, adolescents and adults need a standard multivitamin and mineral preparation, 1 or 2 tablets daily.
In infants less than 2 years of age, 1 ml of Polyvisol from Mead at Johnson Nutritionals, Evansville, Indiana, or a similar liquid multivitamin, is recommended daily. Vitamin E is given as Aquasol E from Rover Pharmaceuticals in Fort Washington, Pennsylvania or Liqui-E from Twin Labs in Ronkonkama, New York for the first year or two.
After the first year or two capsules of à-tocopherol or D-à-tocopheryl acetate may be used. High dose vitamin E greater than 1,000 I.U. per day may exacerbate the coagulopathy associated with vitamin K deficiency. Vitamin K supplementation may be given at the following dosages: Ages 0-12 months - 2.5 mg/wk or 2.5 mg twice weekly on antibiotics; ages greater than 1 year - 5.0 mg twice a week when on antibiotics or if cholestatic disease is present.
"Nutritional Assessment and Management in Cystic Fibrosis: A Consensus Report", Ramsey, Bonnie W., et al, American Journal of Clinical Nutrition, 1992;55:108-16.
Nutritional State and Lung Disease
Reviews the potential benefit of nutrition and lung disease in cystic fibrosis. Prevention of malnutrition results in improved lung function and improved survival. With nutritional intervention there may be an improvement in body weight, lung function and exercise tolerance, especially if the intervention is combined with exercise training. This improvement can be maintained with the consumption of a high energy, high fat diet, and daily exercise training at home.
"Nutritional State and Lung Disease in Cystic Fibrosis", Bakker, W., Netherlands Journal of Medicine, 1992;41:130-136.
Taurine & CF
Taurine is a conditionally essential amino acid that can improve the micellar phase of fat digestion.
Taurine may be a useful adjunct in patients with cystic fibrosis and severe steatorrhea.
"Taurine Decreases Fecal Fatty Acid and Sterol Excretion in Cystic Fibrosis: A Randomized, Double-Blind Trial", Smith, LJ et al, AJDC, 1991;145:1401-1404.
Bioelectric impedance & CF
Bioelectric impedance analysis (BIA)
Bioelectric impedance analysis (BIA), has been validated as a tool for nutritional assessment in healthy adults but it must be validated in patient populations with specific disease-related nutritional problems, such as CF.
BIA is a rapid, portable, and painless method for measuring body composition that correlates well with the deuterium-dilution method.
Are bioelectric impedance measurements valid in patients with cystic fibrosis? Borowitz-D; Conboy-K. J-Pediatr-Gastroenterol-Nutr. 1994 May; 18(4): 453-6.
Bioelectrical impedance (2)
Compared the use of bioelectrical impedance analysis (BIA) with anthropometry for the prediction of changes in total body potassium (TBK) in a group (n = 31) of children with cystic fibrosis.
The significant difference in the change in TBK between the groups was detectable by concomitant changes in impedance and weight but not by changes in height, FFM, or weight and height Z scores.
Results suggest that serial BIA measures may be useful as a predictor of progressive undernutrition and poor growth in children with cystic fibrosis.
Quirk PC et al., Evaluation of bioelectrical impedance for prospective nutritional assessment in cystic fibrosis. Nutrition, 1997 May, 13:5, 412-6.
GI Tract & CF
Contents:
Gastrointestinal manifestations
Gastrointestinal tract
Gastrointestinal manifestations
Cystic fibrosis is clearly a multisystem disease, which has the potential for prominent gastrointestinal and hepatobiliary involvement.
While the routine patient with pancreatic insufficiency may be easily managed with pancreatic enzyme and nutritional therapy, a large number of individuals with cystic fibrosis will present the clinician with gastrointestinal problems requiring additional thoughtful evaluation and treatment.
Familiarity with the common gastrointestinal manifestations of cystic fibrosis and emerging therapies is necessary to provide the comprehensive care these patients deserve.
Riedel BD: Gastrointestinal manifestations of cystic fibrosis. Pediatr Ann, 1997 Apr, 26:4, 235-41.
Gastrointestinal tract
Cystic fibrosis is known to be a serious genetic disease among those of northern European ancestry. Well known to nurses are the pulmonary insults to survival for children with cystic fibrosis. Not often found in the nursing literature is a focus on cystic fibrosis gastrointestinal presentation.
Duffield-RA Cystic fibrosis and the gastrointestinal tract. J. of Pediatric Health Care. 1996 Mar-Apr; 10(2): 51-7. (31 ref)
Malnutrition & CF
Malnourished children
The sweat test was performed by the method of Gibson and Cooke on children (# 36) with second and third degree malnutrition, aged from 2 months to 4 years.
Determinations were made of sodium and chloride concentrations, chloride/sodium ratio, and the sum of the concentrations of the two electrolytes in each sample.
The malnourished children were found to have higher sodium and chloride concentrations than the well nourished ones. None of the normal or malnourished children had a sweat chloride value greater than 60 mmol/l; chloride values within a suspicious range (between 40 and 60 mmol/l) were found in two malnourished children.
Malnourished children have raised sweat sodium and chloride concentrations when compared with well nourished children. The electrolyte values for most of them are not in a suspicious range or consistent with a diagnosis of cystic fibrosis. Unlike that found in cystic fibrosis, the sodium/chloride ratio in our subjects was less than 1 and the sum of both electrolytes never exceeded 140 mmol/l.
Rodrigues-ME et al: Concentration of electrolytes in the sweat of malnourished children. Arch-Dis-Child. 1994 Aug; 71(2): 141-3.
Supplemental feeding in CF
Investigated weight gain and clinical course of patients with cystic fibrosis (CF), aged 7 to 23 years, who received long-term nocturnal supplemental feedings by means of percutaneous endoscopic gastrostomies (PEGs).
After 1 year of supplemental feedings, body weight had increased by 6.0 kg (SD 3.6 kg) and weight-for-height value by 9.0% (SD 6.1%) of predicted compared with baseline values. Lung function also improved significantly: vital capacity increased by 8.2% (SD6.3%) and FEV1 by 3.9% (SD 4.2%) of predicted values (p < 0.01).
The total duration of nocturnal feedings was 26 months per patient.
Nocturnal PEG feedings of malnourished patients with CF improve nutritional status and lung function without major side effects.
Improvement of nutritional status and lung function after long-term nocturnal gastrostomy feedings in cystic fibrosis. Steinkamp, G & von-der-Hardt, H. J-Pediatr. 1994 Feb; 124(2): 244-9.
Fish oil & CF
Fish Oil and Leukotriene B4
Much of the lung damage that limits life expectancy of young adults with cystic fibrosis is due to proteases and oxygen metabolites, generated by neutrophils. Leukotriene B4 has been suggested as a local mediator of this process. Leukotriene B4 metabolism can be modulated with dietary fish oil (Eicosapentaenoic Acid).
Leukotriene B4 has a pathogenic role in lung damage of cystic fibrosis.
"Eicosapentaenoic Acid in Cystic Fibrosis: Evidence of a Pathogenetic Role for Leukotriene B4", Lawrence, Richard and Sorrell, Tania, The Lancet, August 21, 1993;342:465-469.
Neonates & CF
Contents:
Infant nutrition
Neonatal screening
Infant nutrition
The gold standard for assessing the adequacy of nutrient intake in pediatrics is that diet which promotes optimal growth and development. Thus, it is crucial that our methods for measuring these outcomes be valid, reliable, and widely accepted.
Oral nutrition is clearly a determinant of perhaps the most prevalent infectious disease in pediatrics: dental caries. Research in this field stresses the importance of oral fluoride intake in the prevention of caries, as well as the fact that current efforts at reducing milk-bottle tooth decay are inadequate.
Sonis A et al., Infant nutrition: implication for somatic growth, adult onset diseases, and oral health. Curr Opin Pediatr, 1997 Jun, 9:3, 289-97.
Neonatal screening
Many patients with cystic fibrosis are malnourished at the time of diagnosis. Whether newborn screening and early treatment may prevent the development of a nutritional deficiency is not known.
Compared the nutritional status of patients with cystic fibrosis identified by neonatal screening, or by standard diagnostic methods.
Evaluated nutritional status for up to 10 years by anthropometric and biochemical methods in 56 of the infants who received an early diagnosis and in 40 of the infants in whom the diagnosis was made by standard methods (the control group).
Pancreatic insufficiency was managed with nutritional interventions that included high-calorie diets, pancreatic-enzyme therapy, and fat-soluble vitamin supplements.
The diagnosis of cystic fibrosis was confirmed by a positive sweat test at a younger age in the early-diagnosis group than in the control group (mean age, 12 vs. 72 weeks). At the time of diagnosis, the early-diagnosis group had significantly higher height and weight percentiles and a higher head-circumference percentile (52nd, vs. 32nd in the control group).
The early-diagnosis group also had significantly higher anthropometric indexes during the follow-up period, especially the children with pancreatic insufficiency and those who were homozygous for the deltaF508 mutation.
Neonatal screening provides the opportunity to prevent malnutrition in infants with cystic fibrosis.
Farrell PM et al., Nutritional benefits of neonatal screening for cystic fibrosis. Wisconsin Cystic Fibrosis Neonatal Screening Study Group [see comments]. N Engl J Med, 1997 Oct 2, 337:14, 963-9.
Energy intake & CF
High energy intake
Higher fat and energy intakes confer a survival advantage in cystic fibrosis (CF). There is a need to develop effective nutrition programs that ensure optimal energy intake in CF.
Diets with a high percentage of energy derived from fat did not guarantee that individuals with CF met their energy requirements. Subjects with total fat intakes of 100 g per day or greater, however, achieved in excess of 110% recommended daily intake (RDI) for energy. Up to 47% of subjects consumed more pancreatic enzyme replacement capsules than shown to give maximum effectiveness.
Setting a 100 g daily fat target is a realistic way of ensuring high energy intakes in CF. Fat ready reckoners would identify the fat content of food and prescribe specific numbers of pancreatic enzyme replacement capsules to be consumed with each meal or food item.
Collins CE et al., Fat gram target to achieve high energy intake in cystic fibrosis. J Paediatr Child Health, 1997 Apr, 33:2, 142-7.
Diaper rash & CF
Diaper rash
Non-healing eruptions in the diaper area can be a sign of cystic fibrosis, but pancreatic enzyme replacement in these cystic fibrosis patients can quickly clear these eruptions. Non-healing eruptions can also be linked to zinc deficiency, fatty acid deficiency, and over-treatment of amino acid metabolism disorders. Some children and premature infants have an increased need for zinc. Those who have acrodermatitis enteropathica cannot absorb enough zinc. Essential fatty acid deficiency and biotinidase deficiency can cause skin eruptions that can be very similar to those seen in zinc-deficient children.
Bowser, Andrew: Nutritional Deficiency Tied to Nonhealing Diaper Eruptions, Family Practice News, January 1, 1998;37.
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