REVIEW PAPER
Nutritional status disorders in cystic fibrosis – dietary recommendations and supplementation. A review
1
Studenckie Koło Naukowe przy Zakładzie Dietetyki, Uniwersytet Medyczny we Wrocławiu
2
Zakład Dietetyki, Uniwersytet Medyczny we Wrocławiu
Med Og Nauk Zdr. 2017;23(2):115-121
KEYWORDS
TOPICS
ABSTRACT
Introduction:
Cystic fibrosis (CF) is a genetic and chronic disease affecting the functioning of many systems and organs in the body. From the respiratory system the disease is manifested by chronic cough, shortness of breath, bronchial obstruction, and also chronic infections with Pseudomonas aeruginosa and Staphylococcus aureus. Gastrointestinal disorders observed in CF include the exocrine pancreatic insufficiency, disorders of digestion and absorption, diarrhea and abdominal pain. In patients with CF, there also appear impaired liver function, fertility disorders, bone density disorders and deficiencies of vitamins and minerals. CF is also related with the occurrence of the nutritional status disturbances. As causes of the nutritional status disturbances, a number of factors may be distinguished which include: increased energy expenditure, exocrine pancreatic insufficiency, comorbidities, dietary mistakes or Pseudomonas aeruginosa infections. In more than a half of the patients with CF, malnutrition is noted, some of them are underweight, with improper body height; also, a reduced mid-arm circumference and reduced fat free mass are regularly observed. The shortages of fat-soluble vitamins are common. In supporting CF treatment the introduction of a high energy intake is recommended in the nutrition of the diet, as well high protein and high fat diets. In addition, dietary supplementation is recommended by the intake of proper doses of selected nutrients, such as: sodium, zinc, vitamins A, D, E and K. Considering the occurrence of pancreatic insufficiency, pancreatic enzymes supplementation is recommended. Dietary supplementation with probiotics should also be considered, and the use of high energy dietary supplements.
Cystic fibrosis (CF) is a genetic and chronic disease affecting the functioning of many systems and organs in the body. From the respiratory system the disease is manifested by chronic cough, shortness of breath, bronchial obstruction, and also chronic infections with Pseudomonas aeruginosa and Staphylococcus aureus. Gastrointestinal disorders observed in CF include the exocrine pancreatic insufficiency, disorders of digestion and absorption, diarrhea and abdominal pain. In patients with CF, there also appear impaired liver function, fertility disorders, bone density disorders and deficiencies of vitamins and minerals. CF is also related with the occurrence of the nutritional status disturbances. As causes of the nutritional status disturbances, a number of factors may be distinguished which include: increased energy expenditure, exocrine pancreatic insufficiency, comorbidities, dietary mistakes or Pseudomonas aeruginosa infections. In more than a half of the patients with CF, malnutrition is noted, some of them are underweight, with improper body height; also, a reduced mid-arm circumference and reduced fat free mass are regularly observed. The shortages of fat-soluble vitamins are common. In supporting CF treatment the introduction of a high energy intake is recommended in the nutrition of the diet, as well high protein and high fat diets. In addition, dietary supplementation is recommended by the intake of proper doses of selected nutrients, such as: sodium, zinc, vitamins A, D, E and K. Considering the occurrence of pancreatic insufficiency, pancreatic enzymes supplementation is recommended. Dietary supplementation with probiotics should also be considered, and the use of high energy dietary supplements.
REFERENCES (47)
1.
Augustynowicz-Kopeć E, Skorupa W. Diagnostyka mikrobiologiczna u chorych na mukowiscydozę. Pneumol Alergol Pol. 2014; 82: 327–329.
2.
Rusek D, Głąbski T. Nowe możliwości w terapii mukowiscydozy. Farm Pol. 2012; 68: 777–784.
3.
Turck D, Braegger CP, Colombo C. ESPEN-ESPHAGAN-ECFS gui¬delines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr. 2016; doi: 10.1016/j.clnu.2016.03.004.
4.
Słuszniak A, Kurtyka Z, Lemańska D. Badania przesiewowe noworodków w kierunku mukowiscydozy w Polsce południowo-wschodniej. Prz Lek. 2011; 68: 59–63.
5.
Semczuk K. Charakterystyka genotypowa i fenotypowa szczepów Staphylococcus aureus izolowanych od dzieci chorych na mukowiscy¬dozę. Wydawnictwo Instytut „Pomnik – Centrum Zdrowia Dziecka”, Warszawa 2013.
6.
Solomon M, Bozic M, Mascarenhas MR. Nutritional issues in cystic fibrosis. Clin Chest Med. 2015; 37: 97–107.
7.
Umławska W, Krzyżanowska M, Zielińska A et al. Effect of selected factors associated with the clinical corse of disease on nutritional status in children with cystic fibrosis. Adv Clin Exp Med. 2014; 23: 775–783.
8.
Hasan M, Lina F, Alaf M. Growth assessment and risk factors of malnutrition in children with cystic fibrosis. Saudi Med J. 2016; 37: 293–298.
9.
Stapleton D, Ash C, King S. Australasian clinical practise guideliness for nutrition in cystic fibrosis. 2006.
10.
Robinson NB, DiMango E. Prevalence of gastroesophageal reflux in cystic fibrosis and implications for lung disease. Ann Am Thorac Soc. 2014; 11: 964–968.
11.
Demeyer S, De Boeck K, Witters P et al. Beyond pancreatic insufficiency and liver disease in cystic fibrosis. Eur J Pediatr. 2016; doi: 10.1007/s00431–016–2719–5.
12.
Walkowiak J, Pogorzelski A, Sands D i wsp. Zasady rozpoznawania i leczenia mukowiscydozy. Zalecenia Polskiego Towarzystwa Muko¬wiscydozy 2009, Poznań–Warszawa–Rzeszów. Standardy Medyczne 2009; 6: 352–378.
13.
Kołodziej M, Wiśnowksa A. Osteoporoza w mukowiscydozie. Post Rehab. 2015; 29: 39–44.
14.
Sheikh S, Gemma S, Patel A. Factors associated with low bone mineral density in patients with cystic fibrosis. J Bone Miner Metab. 2015; 33: 180–185.
15.
Paccou J, Zeboulon N, Combescure C et al. The prevalence of osteo¬porosis, osteopenia, and fractures among adults with cystic fibrosis: a systematic review with meta-analysis. Calcif Tissue Int. 2010; 86: 1–7.
16.
Lisowska A, Walkowiak J. Intestinal bacterial overgrowth syndrome and intestinal inflammation in cystic fibrosis. Pediatr Pol. 2007; 82: 567–570.
17.
Smyth RL, Rayner O. Oral calorie supplements for cystic fibrosis. Cochrane Database Syst Rev. 2014, doi: 10.1002/14651858.CD000406.pub4.
18.
Kamińska B, Kaźmierska K, Szlagatys-Sidorkiewicz A i wsp. Zaburzenia stanu odżywienia u dzieci w przebiegu mukowiscydozy – przegląd piśmiennictwa. Forum Med Rodz. 2011; 2: 137–146.
19.
Brazowski J, Czerwionka-Szaflarska M. Zaburzenia wchłaniania w przewlekłych chorobach trzustki u dzieci. Pediatr Pol. 2010; 85: 633–636.
20.
Sands D, Umławska W, Zielińska A. A cross-sectional study of growth, nutritional status and body proportions in children and adolescents at a medical center specializing in the treatment of cystic fibrosis in Poland. Arch Med Sci. 2015; 11: 155–163.
21.
Chaves CR, Cunha AL, Costa AC et al. Nutritional status and body fat distribution in children and adolescentes with cystic fibrosis. Cien Saude Colet. 2015; 20: 3319–3328.
22.
Smith C, Winn A, Seddon P et al. A fat lot of good: balance and trends in fat intake in children with cystic fibrosis. J Cyst Fibros. 2012; 11: 154–157.
23.
Maqbool A, Schall JI, Gallagher PR et al. Relation between dietary fat intake type and serum fatty acid status in children with cystic fibrosis. J Pediatr Gatroenerol Nutr. 2012; 55: 605–611.
24.
Walkowiak J, Wilczyński M, Bolesławska I et al. Disturbances of polyunsaturated fatty acids profile do not occur among patients suffering from mucoviscidosis of mild clinical expression. Żyw Człow Metab. 2003; 30: 870–873.
25.
Umławska W, Krzyżanowska M, Zielińska A i wsp. Ocena stanu odżywienia oraz czynności płuc dzieci i młodzieży z mukowiscydozą. Pediatr Endocrinol. 2012; 18: 137–142.
26.
Tutak-Słupska M, Stępień-Jaszowska B, Staszak-Kowalska R i wsp.: Ocena stanu odżywienia i składu ciała pacjentów z mukowiscydozą. Pediatr Pol. 2012; 87: 146–153.
27.
Rana M, Wong-See D, Katz K. Fat-soluble vitamin deficiency in children and adolescents with cystic fibrosis. J Clin Pathol. 2014; 67: 605–608.
28.
Greer RM, Buntain HM, Lewindon PJ et al. Vitamin A levels on patients with CF are influenced by the inflammatory response. J Cyst Fibros. 2004; 3: 143–149.
29.
Woestenenk JW, Broos N, Stellato RK i wsp. Vitamin A intake and serum retinol levels in children and adolescents with cystic fibrosis. Clin Nutr. 2016; 35: 654–659.
30.
Chełchowska M, Sands D, Mielus M et al. The effect of a diet based on the specific nutritional needs of infants with cystic fibrosis on the con¬centration of vitamin A and E in blood. Pediatr Pol. 2007; 82: 631–634.
31.
Chesdachai S, Tanqpricha V. Treatment of vitamin D deficiency in cystic fibrosis. J Steroid Biochem Mol Biol. 2015; doi: 10.1016/j.jsbmb.2015.09.013.
32.
Sexauer WP, Hadeh A, Ohman-Strickland PA et al. Vitamin D deficiency is associated with pulmonary dysfunction in cystic fibrosis. J Cyst Fibros. 2015; 14: 497–506.
33.
Okebukola PO, Kansra S, Barrett J. Vitamin E supplementation in people with cystic fibrosis. Cochrane Database Syst Rev. 2014; doi: 10.1002/14651858.CD009422.pub2.
34.
Krzyżanowska P, Pogorzelski A, Skorupa W et al. Exogenous and endogenous determinants of vitamin K status in cystic fibrosis. Sci Rep. 2015; doi: 10.1038/srep12000.
35.
Sands D, Umławska W, Zielińska A. Clinical research. A cross-sectional study of growth, nutritional status and body proportions in children and adolescents at a medical center specializing in the treatment of cystic fibrosis in Poland. Arch Med Sci. 2015; 11: 155–163.
36.
Szabla A, Skorupa W, Milewska M et al. Nutritional status of adults with cystic fibrosis – current methods of assessment. Pol Med J. 2015; 39: 333–336.
37.
Borowitz D, Robinson KA, Rosenfeld M, Davis SD, Sabadosa Ka et al. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009; 9: 323–329.
38.
Wilschanski M. Nutrition in cystic fibrosis. World Rev Nutr Diet. 2015; 113: 244–249.
39.
Schindler T, Michel S, Wilson AWM. Nutrition management of cystic fibrosis in the 21st century. Nutr Clin Pract. 2015; 30: 488–500.
40.
Borowitz D, Baker RD, Stallings V. Consenus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenerol Nutr. 2002; 35: 246–259.
41.
Bertolaso C, Groleau V, Schall JI et al. Fat-soluble vitamins in cystic fibrosis and pancreatic insufficiency: efficacy of a nutrition intervention. J Pediatr Gastroenerol Nutr. 2014; 58: 443–448.
42.
Graham-Maar RC, Schall JI, Stettler N et al. Elevated vitamin A intake and serum retinol in preadolescent children with cystic fibrosis. Am J Clin Nutr. 2006; 84: 174–182.
43.
Hall WB, Sparks AA, Aris RM. Vitamin D deficiency in cystic fibrosis. Int J Endocrinol. 2010, doi: 2010:218691.
44.
Huang SH, Schall JI, Zemel BS et al. Vitamin E status in children with cystic fibrosis and pancreatic insufficiency. J Pediatr. 2006; 148: 556–559.
45.
Dougherty KA, Schall JI, Stallings VA. Suboptimal vitamin K status despite supplementation in children and young adults with cystic fibrosis. Am J Clin Nutr. 2010; 92: 660–667.
46.
Bruzzese E, Raia V, Spaqnuolo MI et al. Effect of Lactobacillus GG supplementation on pulmonary exacerbations in patients with cystic fibrosis: pilot study. Clin Nutr. 2007; 26: 322–328.
47.
Weiss B, Bujanover Y, Yahav Y et al. Probiotic supplementation affects pulmonary exacerbations in patients with cystic fibrosis: a pilot study. Pediatr Pulmonol. 2010; 45: 536–540.
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