transplantologiyaТрансплантологияTransplantologiya. The Russian Journal of Transplantation2074-05062542-0909IPO Association of Transplantologists10.23873/2074-0506-2017-9-2-125-136transplantologiya-167Research ArticleОБЗОРНЫЕ СТАТЬИ И ЛЕКЦИИREVIEW ARTICLES AND LECTURESСтатус питания больных муковисцидозом до и после трансплантации легкихNutritional status in patients with cystic fi brosis before and after lung transplantationХубутияМ. Ш.KhubutiyaM. Sh.

академик РАН, профессор, президент НИИ скорой помощи им. Н.В. Склифосовского, Москва, Россия

Acad. of RAS, Professor, President of N.V. Sklifosovsky Research Institute for Emergency Medicine, Moscow, Russia

sklifos@inbox.ruИльинскийМ. Е.Il'inskiyM. E.РыкА. А.RykA. A.ЛященкоЮ. Н.LyashchenkoYu. N.ГБУЗ «НИИ скорой помощи им. Н.В. Склифосовского ДЗМ»РоссияN.V. Sklifosovsky Research Institute for Emergency MedicineRussian Federation20172406201792125136Copyright © Хубутия М.Ш., Ильинский М.Е., Рык А.А., Лященко Ю.Н., 20172017Хубутия М.Ш., Ильинский М.Е., Рык А.А., Лященко Ю.Н.Khubutiya M.S., Il'inskiy M.E., Ryk A.A., Lyashchenko Y.N.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.jtransplantologiya.ru/jour/article/view/167

В обзоре освещены ключевые особенности статуса питания больных муковисцидозом, современные подходы к мониторингу и коррекции нарушений алиментарного статуса пациентов до и после трансплантации легких (ТЛ).

This review focuses on the nutritional status of patients with cystic fibrosis, systematic approach to monitoring and nutritional intervention in undernourished patients, and the maintenance of nutritional status before/after lung transplantation.

муковисцидозстатус питаниятрансплантация легкихcystic fibrosisnutritional statuslung transplantationReferencesCohen-Cymberknoh M., Shoseyov D., Kerem E. Managing cystic fibrosis: strategies that increase life expectancy and improve quality of life. Am. J. Respir. Crit. Care Med. 2011;183(11):1463–1471. PMID:21330455 DOI:10.1164/rccm.201009-1478CICohen-Cymberknoh M., Shoseyov D., Kerem E. Managing cystic fibrosis: strategies that increase life expectancy and improve quality of life. Am J Respir Crit Care Med. 2011;183(11):1463– 1471. PMID:21330455 DOI:10.1164/rccm.201009-1478CILi L., Somerset S. Digestive system dysfunction in cystic fibrosis: challenges for nutrition therapy. Dig. Liver Dis. 2014;46(10):865–874. PMID:25053610 DOI:10.1016/j.dld.2014.06.011Li L., Somerset S. Digestive system dysfunction in cystic fibrosis: challenges for nutrition therapy. Dig Liver Dis. 2014;46(10):865–874. PMID:25053610 DOI:10.1016/j.dld.2014.06.011Culhane S., George C., Pearo B., Spoede E. Malnutrition in cystic fibrosis: a review. Nutr. Clin. Pract. 2013;28(6):676–683. PMID:24170579 DOI:10.1177/0884533613507086Culhane S., George C., Pearo B., Spoede E. Malnutrition in cystic fibrosis: a review. Nutr Clin Pract. 2013;28(6):676–683. PMID:24170579 DOI:10.1177/0884533613507086Corey M., McLaughlin F.J., Williams M., Levison H. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J. Clin. Epidemiol. 1988;41(6):583–591. PMID:3260274Corey M., McLaughlin F.J., Williams M., Levison H. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol. 1988;41(6):583–591. PMID:3260274Stallings V.A., Stark L.J., Robinson K.A. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J. Am. Diet. Assoc. 2008;108(5):832–839. PMID:18442507 DOI:10.1016/j.jada.2008.02.020Stallings V.A., Stark L.J., Robinson K.A. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008;108(5):832–839. PMID:18442507 DOI:10.1016/j.jada.2008.02.020Петрова Н.В., Гинтер Е.К. Молекулярно-генетические аспекты муковисцидоза. В кн.: под ред. Капранова Н.И., Каширской Н.Ю. Муковисцидоз. М.: МЕДПРАКТИКА-М, 2014: 37–80.Petrova N.V., Ginter E.K. Moleculargenetic aspects of cystic fibrosis. In: Kapranov N.I., Kashirskaya N.Yu., eds. Cystic Fibrosis. Moscow: MEDPRAKTIKA- M Publ., 2014. 37–80. (In Russian).Andersen D.H., Dorothy H. Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathological study. Am. J. Dis. Child. 1938;56(2):344–399. DOI:10.1001/archpedi.1938.01980140114013Andersen D.H., Dorothy H. Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathological study. Am J Dis Child. 1938;56(2):344– 399. DOI:10.1001/archpedi.1938.01980140114013Stephenson A.L., Mannik L.A., Walsh S. et al. Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: a population-based cohort study. Am. J. Clin. Nutr. 2013;97(4):872–877.Stephenson A.L., Mannik L.A., Walsh S., et al. Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: a population-based cohort study. Am J Clin Nutr. 2013;97(4):872–877.Амелина Е.Л., Гембицкая Т.Е., Черменская А.Г. Особенности муковисцидоза взрослых. В кн.: под ред. Капранова Н.И., Каширской Н.Ю. Муковисцидоз. М.: МЕДПРАКТИКА-М, 2014: 548–575.Amelina E.L., Gembitskaya T.E., Chermenskaya A.G. Features of cystic fibrosis in adults. In: Kapranov N.I., Kashir skaya N.Yu., eds. Cystic Fibrosis. Moscow: MEDPRAKTIKA-M Publ., 2014. 548– 575. (In Russian).Robinson P.J., Smith A.L., Sly P.D. Duodenal pH in cystic fibrosis and its relationship to fat malabsorption. Dig. Dis. Sci. 1990;35(10):1299–1304. PM-ID:2120019Robinson P.J., Smith A.L., Sly P.D. Duodenal pH in cystic fibrosis and its relationship to fat malabsorption. Dig Dis Sci. 1990;35(10):1299–1304. PMID:2120019Perano S., Rayner C.K., Couper J. et al. Cystic fibrosis related diabetes – a new perspective on the optimal management of postprandial glycemia. J. Diabetes Complicat. 2014;28(6):904–911. PMID:25060530 DOI:10.1016/j.jdiacomp.2014.06.012Perano S., Rayner C.K., Couper J., et al. Cystic fibrosis related diabetes – a new perspective on the optimal management of postprandial glycemia. J Diabetes Complicat. 2014;28(6):904–911. PMID: 25060530 DOI:10.1016/j.jdiacomp.2014.06.012Debray D., Kelly D., Houwen R. et al. Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease. J. Cyst. Fibros. 2011;10(Suppl 2):S29–S36. PMID:21658639 DOI:10.1016/S1569-1993(11)60006-4Debray D., Kelly D., Houwen R., et al. Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease. J Cyst Fibros. 2011;10(Suppl 2):S29–S36. PMID:21658639 DOI:10.1016/S1569-1993(11)60006-4Каширская Н.Ю. Поражение поджелудочной железы, изменения со стороны гепатобилиарной системы у больных муковисцидозом. В кн.: под ред. Капранова Н.И., Каширской Н.Ю. Муковисцидоз. М.: МЕДПРАКТИКА-М, 2014: 295–345.Kashirskaya N.Yu. Pancreatic lesions, changes in the hepatobiliary system in patients with cystic fibrosis. In: Kapranov N.I., Kashirskaya N.Yu., eds. Cystic Fibrosis. Moscow: MEDPRAKTIKA-M Publ., 2014. 295–345. (In Russian).Moran A., Dunitz J., Nathan B. et al. Cystic fibrosisrelated diabetes: current trends in prevalence, incidence, and mortality. Diabetes Care. 2009;32(9):1626– 1631. PMID:19542209 DOI:10.2337/dc09-0586Moran A., Dunitz J., Nathan B., et al. Cystic fibrosisrelated diabetes: current trends in prevalence, incidence, and mortality. Diabetes Care. 2009;32(9):1626–1631. PMID:19542209 DOI:10.2337/dc09-0586Waugh N., Royle P., Craigie I. et al. Screening for cystic fibrosis-related diabetes: a systematic review. Health Technol. Assess. 2012;16(24):iii–iv,1–179. PMID:22572153 DOI:10.3310/hta16240Waugh N., Royle P., Craigie I., et al. Screening for cystic fibrosis-related diabetes: a systematic review. Health Technol Assess. 2012;16(24):iii-iv,1–179. PMID:22572153 DOI:10.3310/hta16240Moran A., Pillay K., Becker D.J. ISPAD Clinical Practice Consensus Guidelines 2014. Management of cystic fibrosis related diabetes in children and adolescents. Pediatr. Diabetes. 2014;15(Suppl 20):65–76. PMID:25182308 DOI:10.1111/pedi.12178Moran A., Pillay K., Becker D.J. ISPAD Clinical Practice Consensus Guidelines 2014. Management of cystic fibrosis related diabetes in children and adolescents. Pediatr Diabetes. 2014;15(Suppl 20):65–76. PMID:25182308 DOI:10.1111/pedi.12178Moran A., Brunzell C., Cohen R.C. et al. Clinical care guidelines for cystic fibrosis-related diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care. 2010;33(12):2697–2708. PMID:21115772 PMCID:PMC2992215 DOI:10.2337/dc10-1768Moran A., Brunzell C., Cohen R.C., et al. Clinical care guidelines for cystic fibrosis-related diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care. 2010;33(12):2697–2708. PMID:21115772 PMCID:PMC2992215 DOI:10.2337/dc10-1768Colombo C., Battezzati P.M., Crosignani A. et al. Liver disease in cystic fibrosis: a prospective study on incidence, risk factors, and outcome. Hepatology. 2002;36(6):1374– 1382. PMID:12447862 DOI:10.1053/jhep.2002.37136Colombo C., Battezzati P.M., Crosignani A., et al. Liver disease in cystic fibrosis: a prospective study on incidence, risk factors, and outcome. Hepatology. 2002;36(6):1374–1382. PMID:12447862 DOI:10.1053/jhep.2002.37136Lindblad A., Glaumann H., Strandvik B. Natural history of liver disease in cystic fibrosis. Hepatology. 1999;30(5):1151– 1158. PMID:10534335 DOI:10.1002/hep.510300527Lindblad A., Glaumann H., Strandvik B. Natural history of liver disease in cystic fibrosis. Hepatology. 1999;30(5):1151–1158. PMID:10534335 DOI:10.1002/hep.510300527Van Biervliet S., Van Biervliet J.P., Robberecht E., Christophe A. Fatty acid composition of serum phospholipids in cystic fibrosis (CF) patients with or without CF related liver disease. Clin. Chem. Lab. Med. 2010;48(12):1751– 1755. PMID:20961201 DOI:10.1515/CCLM.2010.336Van Biervliet S., Van Biervliet J.P., Robberecht E., Christophe A. Fatty acid composition of serum phospholipids in cystic fibrosis (CF) patients with or without CF related liver disease. Clin Chem Lab Med. 2010;48(12):1751–1755. PMID:20961201 DOI:10.1515/CCLM.2010.336Conway S.P., Morton A.M., Oldroyd B. et al. Osteoporosis and osteopenia in adults and adolescents with cystic fibrosis: prevalence and associated factors. Thorax. 2000;55(9):798– 804. PMID:10950902Conway S.P., Morton A.M., Oldroyd B., et al. Osteoporosis and osteopenia in adults and adolescents with cystic fibrosis: prevalence and associated factors. Thorax. 2000;55(9):798–804. PMID:10950902Gronowitz E., Lorentzon M., Ohlsson C. et al. Docosahexaenoic acid is associated with endosteal circumference in long bones in young males with cystic fibrosis. Br. J. Nutr. 2008;99(1):160– 167. PMID:17697399 DOI:10.1017/S000711450780105XGronowitz E., Lorentzon M., Ohlsson C., et al. Docosahexaenoic acid is associated with endosteal circumference in long bones in young males with cystic fibrosis. Br J Nutr. 2008;99(1):160–167. PMID:17697399 DOI:10.1017/S000711450780105XYen E.H., Quinton H., Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J. Pediatr. 2013;162(3):530– 535. PMID:23062247 DOI:10.1016/j.jpeds.2012.08.040Yen E.H., Quinton H., Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr. 2013;162(3):530–535. PMID:23062247 DOI:10.1016/j.jpeds.2012.08.040Kerem E., Viviani L., Zolin A. et al. Factors associated with FEV1 decline in cystic fibrosis: analysis of the ECFS Patient Registry. Eur. Respir J. 2014;43(1):125–133. PMID:23598952 DOI:10.1183/09031936.00166412Kerem E., Viviani L., Zolin A., et al. Factors associated with FEV1 decline in cystic fibrosis: analysis of the ECFS Patient Registry. Eur Respir J. 2014;43(1):125–133. PMID:23598952 DOI:10.1183/09031936.00166412Sinaasappel M., Stern M., Littlewood J. et al. Nutrition in patients with cystic fibrosis: a European Consensus. J. Cyst. Fibros. 2002;1(2):51–75. PM-ID:15463811Sinaasappel M., Stern M., Littlewood J., et al. Nutrition in patients with cystic fibrosis: a European Consensus. J Cyst Fibros. 2002;1(2):51–75. PMID:15463811Smyth A.R., Bell S.C., Bojcin S. et al. European cystic fibrosis Society standards of care: best practice guidelines. J. Cyst. Fibros. 2014;13(Suppl 1):S23– S42. PMID:24856775 DOI:10.1016/j.jcf.2014.03.010Smyth A.R., Bell S.C., Bojcin S., et al. European cystic fibrosis Society standards of care: best practice guidelines. J Cyst Fibros. 2014;13(Suppl 1):S23–S42. PMID:24856775 DOI:10.1016/j.jcf.2014.03.010Turck D., Braegger C.P., Colombo C. et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin. Nutr. 2016;35(3):557–577. PMID:27068495 DOI:10.1016/j.clnu.2016.03.004Turck D., Braegger C.P., Colombo C., et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr. 2016;35(3):557–577. PMID:27068495 DOI:10.1016/j.clnu.2016.03.004Thomson M.A., Bucolo S., Quirk P., Shepherd R.W. Measured versus predicted resting energy expenditure in infants: a need for reappraisal. J. Pediatr. 1995;126(1):21–27. PMID:7815217Thomson M.A., Bucolo S., Quirk P., Shepherd R.W. Measured versus predicted resting energy expenditure in infants: a need for reappraisal. J Pediatr. 1995;126(1):21–27. PMID:7815217Thomson M.A., Wilmott R.W., Wainwright C. et al. Resting energy expenditure, pulmonary inflammation, and genotype in the early course of cystic fibrosis. J. Pediatr. 1996;129(3):367–373. PMID:8804325Thomson M.A., Wilmott R.W., Wainwright C., et al. Resting energy expenditure, pulmonary inflammation, and genotype in the early course of cystic fibrosis. J Pediatr. 1996;129(3):367–373. PMID:8804325Wood L.G., Gibson P.G., Garg M.L. Circulating markers to assess nutritional therapy in cystic fibrosis. Clin. Chim. Acta. 2005;353(1–2):13–29. PMID:15698587 DOI:10.1016/j.cccn.2004.11.002Wood L.G., Gibson P.G., Garg M.L. Circulating markers to assess nutritional therapy in cystic fibrosis. Clin Chim Acta. 2005;353(1-2):13–29. PMID:15698587 DOI:10.1016/j.cccn.2004.11.002Cystic Fibrosis Trust. Nutritional management of cystic fibrosis. London, UK, 2002.Cystic Fibrosis Trust. Nutritional management of cystic fibrosis. London, UK, 2002.Powers S.W., Mitchell M.J., Patton S.R. et al. Mealtime behaviors in families of infants and toddlers with cystic fibrosis. J. Cyst. Fibros. 2005;4(3):175– 182. PMID:15982934 DOI:10.1016/j.jcf.2005.05.015Powers S.W., Mitchell M.J., Patton S.R., et al. Mealtime behaviors in families of infants and toddlers with cystic fibrosis. J Cyst Fibros. 2005;4(3):175– 182. PMID:15982934 DOI:10.1016/j.jcf.2005.05.015Gaskin K. J. Nutritional care in children with cystic fibrosis: are our patients becoming better? Eur. J. Clin. Nutr. 2013;67(5):558–564. PMID:23462946 DOI:10.1038/ejcn.2013.20Gaskin K.J. Nutritional care in children with cystic fibrosis: are our patients becoming better? Eur J Clin Nutr. 2013;67(5):558–564. PMID:23462946 DOI:10.1038/ejcn.2013.20Olveira G., Olveira C., Acosta E. et al. Fatty acid supplements improve respiratory, inflammatory and nutritional parameters in adults with cystic fibrosis. Arch. Bronconeumol. 2010;46(2):70– 77. PMID:20045240 DOI:10.1016/j.arbres.2009.11.001Olveira G., Olveira C., Acosta E., et al. Fatty acid supplements improve respiratory, inflammatory and nutritional parameters in adults with cystic fibrosis. Arch Bronconeumol. 2010;46(2):70–77. PMID:20045240 DOI:10.1016/j.arbres.2009.11.001Keen C., Olin A.C., Eriksson S. et al. Supplementation with fatty acids influences the airway nitric oxide and inflammatory markers in patients with cystic fibrosis. J. Pediatr. Gastroenterol. Nutr. 2010;50(5):537–544. PMID:20639712 DOI:10.1097/MPG.0b013e3181b47967Keen C., Olin A.C., Eriksson S., et al. Supplementation with fatty acids influences the airway nitric oxide and inflammatory markers in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2010;50(5):537–544. PMID:20639712 DOI:10.1097/MPG.0b013e3181b47967Oliver C., Watson H. Omega-3 fatty acids for cystic fibrosis. Cochrane Database Syst. Rev. 2013;11:CD002201. PMID:24282091 DOI:10.1002/14651858.CD002201.pub4Oliver C., Watson H. Omega-3 fatty acids for cystic fibrosis. Cochrane Database Syst Rev. 2013;11:CD002201. PMID:24282091 DOI:10.1002/14651858.CD002201.pub4Sermet-Gaudelus I., Bianchi M.L., Garabédian M. et al. European cystic fibrosis bone mineralisation guidelines. J. Cyst. Fibros. 2011;10(Suppl 2):S16–S23. PMID:21658635 DOI:10.1016/S1569-1993(11)60004-0Sermet-Gaudelus I., Bianchi M.L., Garabédian M., et al. European cystic fibrosis bone mineralisation guidelines. J Cyst Fibros. 2011;10(Suppl 2):S16–S23. PMID:21658635 DOI:10.1016/S1569-1993(11)60004-0Cystic Fibrosis Trust. Bone mineralisation in cystic fibrosis. London, UK, 2007.Cystic Fibrosis Trust. Bone mineralisation in cystic fibrosis. London, UK, 2007.Chinuck R., Dewar J., Baldwin D.R., Hendron E. Appetite stimulants for people with cystic fibrosis. Cochrane Database Syst Rev. 2014;7:CD008190. PMID:25064192 DOI:10.1002/14651858.CD008190.pub2Chinuck R., Dewar J., Baldwin D.R., Hendron E. Appetite stimulants for people with cystic fibrosis. Cochrane Database Syst Rev. 2014;7:CD008190. PMID:25064192 DOI:10.1002/14651858.CD008190.pub2Bruzzese E., Raia V., Gaudiello G. et al. Intestinal inflammation is a frequent feature of cystic fibrosis and is reduced by probiotic administration. Aliment. Pharmacol. Ther. 2004;20(7):813–819. PMID:15379842 DOI:10.1111/j.1365-2036.2004.02174.xBruzzese E., Raia V., Gaudiello G., et al. Intestinal inflammation is a frequent feature of cystic fibrosis and is reduced by probiotic administration. Aliment Pharmacol Ther. 2004;20(7):813–819. PMID:15379842 DOI:10.1111/j.1365-2036.2004.02174.xBruzzese E., Raia V., Spagnuolo M.I. et al. Effect of Lactobacillus GG supplementation on pulmonary exacerbations in patients with cystic fibrosis: a pilot study. Clin. Nutr. 2007;26(3):322–328. PMID:17360077 DOI:10.1016/j.clnu.2007.01.004Bruzzese E., Raia V., Spagnuolo M.I., et al. Effect of Lactobacillus GG supplementation on pulmonary exacerbations in patients with cystic fibrosis: a pilot study. Clin Nutr. 2007;26(3):322–328. PMID:17360077 DOI:10.1016/j.clnu.2007.01.004Kindstedt-Arfwidson K., Strandvik B. Food intake in patients with cystic fibrosis on an ordinary diet. Scand. J. Gastroenterol. Suppl. 1988;143:160–162. PMID:3164504Kindstedt-Arfwidson K., Strandvik B. Food intake in patients with cystic fibrosis on an ordinary diet. Scand J Gastroenterol. Suppl. 1988;143:160–162. PMID: 3164504Smith C., Winn A., Seddon P., Ranganathan S. A fat lot of good: balance and trends in fat intake in children with cystic fibrosis. J. Cyst. Fibros. 2012;11(2):154–157. PMID:22119390 DOI:10.1016/j.jcf.2011.10.007Smith C., Winn A., Seddon P., Ranganathan S. A fat lot of good: balance and trends in fat intake in children with cystic fibrosis. J Cyst Fibros. 2012;11(2):154–157. PMID:22119390 DOI:10.1016/j.jcf.2011.10.007Woestenenk J.W., Castelijns S.J., van der Ent C.K., Houwen R.H. Dietary intake in children and adolescents with cystic fibrosis. Clin Nutr. 2014;33(3):528– 532. PMID: 23920501 DOI: 10.1016/j.clnu.2013.07.011Woestenenk J.W., Castelijns S.J., van der Ent C.K., Houwen R.H. Dietary intake in children and adolescents with cystic fibrosis. Clin Nutr. 2014;33(3):528–532. PMID:23920501 DOI:10.1016/j.clnu.2013.07.011Cystic Fibrosis Foundation Patient Registry. Annual Data 2012. Bethesda, Maryland, 2013.Cystic Fibrosis Foundation Patient Registry. Annual Data 2012. Bethesda, Maryland, 2013.Steinkamp G., Demmelmair H., Rühl-Bagheri I. et al. Energy supplements rich in linoleic acid improve body weight and essential fatty acid status of cystic fibrosis patients. J. Pediatr. Gastroenterol. Nutr. 2000;31(4):418–423. PMID:11045840Steinkamp G., Demmelmair H., Rühl-Bagheri I., et al. Energy supplements rich in linoleic acid improve body weight and essential fatty acid status of cystic fibrosis patients. J Pediatr Gastroenterol Nutr. 2000;31(4):418–423. PMID:11045840Rettammel A.L., Marcus M.S., Farrell P.M. et al. Oral supplementation with a high-fat, high-energy product improves nutritional status and alters serum li pids in patients with cystic fibrosis. J. Am. Diet. Assoc. 1995;95(4):454–459. PMID:7699188 DOI: 10.1016/S0002-8223(95)00121-2Rettammel A.L., Marcus M.S., Farrell P.M., et al. Oral supplementation with a high-fat, high-energy product improves nutritional status and alters serum lipids in patients with cystic fibrosis. J Am Diet Assoc. 1995;95(4):454–459. PMID:7699188 DOI:10.1016/S0002-8223(95)00121-2Alison J.A., Donnelly P.M., Lennon M. et al. The effect of a comprehensive, intensive inpatient treatment program on lung function and exercise capacity in patients with cystic fibrosis. Phys. Ther. 1994;74(6):583–591. PMID:8197244Alison J.A., Donnelly P.M., Lennon M., et al. The effect of a comprehensive, intensive inpatient treatment program on lung function and exercise capacity in patients with cystic fibrosis. Phys Ther. 1994;74(6):583–591. PMID:8197244Heijerman H.G. Chronic obstructive lung disease and respiratory muscle function: the role of nutrition and exercise training in cystic fibrosis. Respir. Med. 1993;87(Suppl. B):49–51. PMID:8234970Heijerman H.G. Chronic obstructive lung disease and respiratory muscle function: the role of nutrition and exercise training in cystic fibrosis. Respir Med. 1993;87(Suppl B):49–51. PMID:8234970Williams S.G., Ashworth F., McAlweenie A. et al. Percutaneous endoscopic gastrostomy feeding in patients with cystic fibrosis. Gut. 1999;44(1):87–90. PMID:9862831Williams S.G., Ashworth F., McAlweenie A., et al. Percutaneous endoscopic gastrostomy feeding in patients with cystic fibrosis. Gut. 1999;44(1):87–90. PMID:9862831Rosenfeld M., Casey S., Pepe M., Ramsey B.W. Nutritional effects of longterm gastrostomy feedings in children with cystic fibrosis. J. Am. Diet. Assoc. 1999;99(2): 191– 194. PMID:9972186 DOI:10.1016/S0002-8223(99)00046-2Rosenfeld M., Casey S., Pepe M., Ramsey B.W. Nutritional effects of longterm gastrostomy feedings in children with cystic fibrosis. J Am Diet Assoc. 1999;99(2):191–194. PMID:9972186 DOI:10.1016/S0002-8223(99)00046-2Allen E.D., Mick A.B., Nicol J., McCoy K.S. Prolonged parenteral nutrition for cystic fibrosis patients. Nutr. Clin. Pract.1995;10(2):73–79. PMID:7731428 DOI:10.1177/011542659501000273Allen E.D., Mick A.B., Nicol J., McCoy K.S. Prolonged parenteral nutrition for cystic fibrosis patients. Nutr Clin Pract. 1995;10(2):73–79. PMID:7731428 DOI:10.1177/011542659501000273Авдеев С.Н. Трансплантация легких при муковисцидозе. В кн.: под ред. Капранова Н.И., Каширской Н.Ю. Муковисцидоз. М.: МЕДПРАКТИКА-М, 2014: 595–611.Avdeev S.N. Lung transplantation in cystic fibrosis. In: Kapranov N.I., Kashirskaya N.Yu., eds. Cystic Fibrosis. Moscow: MEDPRAKTIKA-M Publ., 2014. 595–611. (In Russian).Spahr J.E., Love R.B., Francois M. et al. Lung transplantation for cystic fibrosis: current concepts and one center's experience . J. Cyst. Fibros. 2007;6(5):334–350. PMID:17418647 DOI:10.1016/j.jcf.2006.12.010Spahr J.E., Love R.B., Francois M., et al. Lung transplantation for cystic fibrosis: current concepts and one center's experience. J Cyst Fibros. 2007;6(5):334–350. PMID:17418647 DOI:10.1016/j.jcf.2006.12.010Stehlik J., Edwards L.B., Kucheryavaya A.Y. et al. The Registry of the International Society for Heart and Lung transplantation: 29th adult lung and heart-lung transplant report – 2012. J. Heart Lung. Transplant. 2012;31(10):1073–1086. PMID:22975095DOI:10.1016/j.healun.2012.08.002Stehlik J., Edwards L.B., Kucheryavaya A.Y., et al. The Registry of the International Society for Heart and Lung transplantation: 29th adult lung and heart- lung transplant report – 2012. J Heart Lung Transplant. 2012;31(10):1073–1086. PMID:22975095 DOI:10.1016/j.healun.2012.08.002Lederer D.J., Wilt J.S., D'Ovidio F. et al. Obesity and underweight are associated with an increased risk of death after lung transplantation. Am. J. Respir. Crit. Care Med. 2009;180(9):887– 895. PMID:19608717 DOI:10.1164/rccm.200903-0425OCLederer D.J., Wilt J.S., D'Ovidio F., et al. Obesity and underweight are associated with an increased risk of death after lung transplantation. Am J Respir Crit Care Med. 2009;180(9):887–895. PMID:19608717 DOI:10.1164/rccm.200903-0425OCРегистр больных муковисцидозом в Российской Федерации. 2011 г. Пульмонология. 2014; Прил. Режим доступа: http://mukoviscidoz.org/doc/registr/Registr_end_2011.pdfThe register of patients with cystic fibrosis in the Russian Federation. 2011. Pulmonology. 2014; Suppl. Available at: http://mukoviscidoz.org/doc/registr/Registr_end_2011.pdfEgan J.J., Woodcock A.A., Webb A.K. Management of cystic fibrosis before and after lung transplantation. J. R. Soc. Med.1997;90(Suppl 31):47–58. PMID:9204012Egan J.J., Woodcock A.A., Webb A.K. Management of cystic fibrosis before and after lung transplantation. J R Soc Med. 1997;90(Suppl 31):47–58. PMID:9204012Elborn J.S., Jagoe T., Shale D.J. Metabolic and respiratory consequences of a glucose load in hypoxic patients with cystic fibrosis. Ulster Med. 1992;61(2):188– 192. PMID:1481313Elborn J.S., Jagoe T., Shale D.J. Metabolic and respiratory consequences of a glucose load in hypoxic patients with cystic fibrosis. Ulster Med. 1992;61(2):188– 192. PMID:1481313Hirche T.O., Knoop C., Hebestreit H. et al. Practical Guidelines: Lung Transplantation in Patients with Cystic Fibrosis. Pulm. Med. 2014;2014:621342. PMID:24800072 DOI:10.1155/2014/621342Hirche T.O., Knoop C., Hebestreit H., et al. Practical Guidelines: Lung Transplantation in Patients with Cystic Fibrosis. Pulm Med. 2014;2014:621342. PMID:24800072 DOI:10.1155/2014/621342Tynan C., Hasse J.M. Current nutrition practices in adult lung transplantation. Nutr. Clin. Pract. 2004;19(6):587–596. PMID:16215158 DOI:10.1177/0115426504019006587Tynan C., Hasse J.M. Current nutrition practices in adult lung transplantation. Nutr Clin Pract. 2004;19(6):587–596. PMID:16215158 DOI:10.1177/0115426504019006587Kalnins D., Wilschanski M. Maintenance of nutritional status in patients with cystic fibrosis: new and emerging therapies. Drug. Des. Devel. Ther. 2012;6:151–161. PMID:22787388 DOI:10.2147/DDDT.S9258Kalnins D., Wilschanski M. Maintenance of nutritional status in patients with cystic fibrosis: new and emerging therapies. Drug Des Devel Ther. 2012;6:151–161. PMID:22787388 DOI:10.2147/DDDT.S9258Ho T., Gupta S., Brotherwood M. et al. Increased serum vitamin A and e levels after lung transplantation. Transplantation. 2011;92(5):601–606. PMID:21841542 DOI:10.1097/TP.0b013e31822790e3Ho T., Gupta S., Brotherwood M., et al. Increased serum vitamin A and e levels after lung transplantation. Transplantation. 2011;92(5):601–606. PMID:21841542 DOI:10.1097/TP.0b013e31822790e3Stephenson A., Brotherwood M., Robert R. et al. Increased vitamin A and E levels in adult cystic fibrosis patients after lung transplantation. Transplantation. 2005;79(5):613– 615. PMID:15753854Stephenson A., Brotherwood M., Ro bert R., et al. Increased vitamin A and E levels in adult cystic fibrosis patients after lung transplantation. Transplantation. 2005;79(5):613–615. PMID:15753854Hadjiliadis D., Madill J., Chaparro C. et al. Incidence and prevalence of diabetes mellitus in patients with cystic fibrosis undergoing lung transplantation before and after lung transplantation. Clinical Transplantation. 2005;19(6):773– 778. PMID:16313324 DOI:10.1111/j.1399-0012.2005.00420.xHadjiliadis D., Madill J., Chaparro C., et al. Incidence and prevalence of diabetes mellitus in patients with cystic fibrosis undergoing lung transplantation before and after lung transplantation. Clinical Transplantation. 2005;19(6):773– 778. PMID:16313324 DOI:10.1111/j.1399-0012.2005.00420.xGottlieb J., Ballmann M., von Mallinckrodt C. et al. Lung transplantation in cystic fibrosis - a position paper. Pneumologie. 2009;63(8):451–460. PMID:19670104 DOI:10.1055/s-0029-1214821Gottlieb J., Ballmann M., von Mallinckrodt C., et al. Lung transplantation in cystic fibrosis – a position paper. Pneumologie. 2009;63(8):451–460. PMID:19670104 DOI:10.1055/s-0029-1214821Gilljam M., Chaparro C., Tullis E. et al. GI complications after lung transplantation in patients with cystic fibrosis. Chest. 2003;123(1):37–41. PMID:12527600Gilljam M., Chaparro C., Tullis E., et al. GI complications after lung transplantation in patients with cystic fibrosis. Chest. 2003;123(1):37–41. PMID:12527600Atkins B.Z., Petersen R.P., Daneshmand M.A. et al. Impact of oropharyngeal dysphagia on long-term outcomes of lung transplantation. Ann. Thorac. Surg. 2010;90(5):1622–1628. PMID:20971276 DOI:10.1016/j.athoracsur.2010.06.089Atkins B.Z., Petersen R.P., Daneshmand M.A., et al. Impact of oropharyngeal dysphagia on long-term outcomes of lung transplantation. Ann Thorac Surg. 2010;90(5):1622–1628. PMID:20971276 DOI:10.1016/j.athoracsur.2010.06.089Dellon E.S., Morgan D.R., Mohanty S.P. et al. High incidence of gastric bezoars in cystic fibrosis patients after lung transplantation. Transplantation. 2006;81(8):1141–1146. PMID:16641599 DOI:10.1097/01.tp.0000205813.54136.85Dellon E.S., Morgan D.R., Mohanty S.P., et al. High incidence of gastric bezoars in cystic fibrosis patients after lung transplantation. Transplantation. 2006;81(8):1141–1146. PMID:16641599 DOI:10.1097/01.tp.0000205813.54136.85Mendez B.M., Davis C.S., Weber C. et al. Gastroesophageal reflux disease in lung transplant patients with cystic fibrosis. Am. J. Surg. 2012;204(5):e21– e26. PMID:22921151 DOI:10.1016/j.amjsurg.2012.07.019Mendez B.M., Davis C.S., Weber C., et al. Gastroesophageal reflux disease in lung transplant patients with cystic fibrosis. Am J Surg. 2012;204(5):e21– e26. PMID:22921151 DOI:10.1016/j.amjsurg.2012.07.019Quon B.S., Mayer-Hamblett N., Aitken M.L., Goss C.H. Risk of post lung transplant renal dysfunction in adults with cystic fibrosis. Chest. 2012;142(1):185– 191. PMID:22222189 DOI:10.1378/chest.11-1926Quon B.S., Mayer-Hamblett N., Aitken M.L., Goss C.H. Risk of post lung transplant renal dysfunction in adults with cystic fibrosis. Chest. 2012;142(1):185– 191. PMID:22222189 DOI:10.1378/chest.11-1926Outbreak of Salmonella serotype Javinia infections. Orlando, Florida, June 2002. MMWR Morb Mortal Wkly Rep. 2002;51(31):683–684. PMID:12233909Outbreak of Salmonella serotype Javinia infections. Orlando, Florida, June 2002. MMWR Morb Mortal Wkly Rep. 2002;51(31):683–684. PMID:12233909Goulet V., Hebert M., Hedberg C. et al. Incidence of listeriosis and relate d mortality among groups at risk of acquiring listeriosis. Clin. Infect. Dis. 2012;54(5):652–660. PMID:22157172 DOI:10.1093/cid/cir902Goulet V., Hebert M., Hedberg C., et al. Incidence of listeriosis and relate d mortality among groups at risk of acquiring listeriosis. Clin Infect Dis. 2012;54(5):652–660. PMID:22157172 DOI:10.1093/cid/cir902Mathur S., Hornblower E., Levy R.D. Exercise training before and after lung transplantation. Phys. Sportsmed. 2009;37(3):78–87. PMID:20048531 DOI:10.3810/psm.2009.10.1732Mathur S., Hornblower E., Levy R.D. Exercise training before and after lung transplantation. Phys Sportsmed. 2009;37(3):78–87. PMID:20048531 DOI:10.3810/psm.2009.10.1732

The authors declare that there are no conflicts of interest present.