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Journal articles on the topic 'Cystic fibrosis Patients Victoria Nutrition'

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Author: Grafiati

Published: 10 December 2022

Last updated: 28 January 2023

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1

Olson, Diane L., and W. Frederick Schwenk. "Nutrition for Patients With Cystic Fibrosis." Nutrition in Clinical Practice 19, no. 6 (December 2004): 575–80. http://dx.doi.org/10.1177/0115426504019006575.

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2

Allen, Elizabeth D., Anne B. Mick, Jennie Nicol, and Karen S. Mccoy. "Prolonged Parenteral Nutrition for Cystic Fibrosis Patients." Nutrition in Clinical Practice 10, no. 2 (April 1995): 73–79. http://dx.doi.org/10.1177/011542659501000273.

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3

Ramirez, I., A. Filbrun, and S. Z. Nasr. "257 Improving nutrition for pediatric cystic fibrosis patients." Journal of Cystic Fibrosis 11 (June 2012): S122. http://dx.doi.org/10.1016/s1569-1993(12)60426-3.

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4

Kusuma Yana, I. Gusti Agung Ari. "Cystic Fibrosis: Review." Jurnal Sains dan Kesehatan 3, no. 1 (February 28, 2021): 79–87. http://dx.doi.org/10.25026/jsk.v3i1.231.

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Cystic fibrosis (CF) is a reversed autosomal genetic disease that originates from some white or caucasian races. This condition is caused by mutations in the CFTR gene, especially the CFTR Phe508del. If CFTR damage or error occurs then normal function will be disrupted and have a clinical impact on other organs or multiorgan. Complications such as the pancreas, liver, digestive tract, bone, genital, and respiratory tract that can cause most deaths in patients with cystic fibrosis. Malabsorption, inflammation, infection and lung obstruction are signs of cystic fibrosis. Therapy for cystic fibrosis is limited to the improvement of the airway mokus, recovery of infections maintained with antibiotics, improve physical health and nutrition of patients

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5

Brownell, Jefferson N., Hillary Bashaw, and Virginia A. Stallings. "Growth and Nutrition in Cystic Fibrosis." Seminars in Respiratory and Critical Care Medicine 40, no. 06 (October 28, 2019): 775–91. http://dx.doi.org/10.1055/s-0039-1696726.

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AbstractOptimal nutrition support has been integral in the management of cystic fibrosis (CF) since the disease was initially described. Nutritional status has a clear relationship with disease outcomes, and malnutrition in CF is typically a result of chronic negative energy balance secondary to malabsorption. As the mechanisms underlying the pathology of CF and its implications on nutrient absorption and energy expenditure have been elucidated, nutrition support has become increasingly sophisticated. Comprehensive nutrition monitoring and treatment guidelines from professional and advocacy organizations have unified the approach to nutrition optimization around the world. Newborn screening allows for early nutrition intervention and improvement in short- and long-term growth and other clinical outcomes. The nutrition support goal in CF care includes achieving optimal nutritional status to support growth and pubertal development in children, maintenance of optimal nutritional status in adult life, and optimizing fat soluble vitamin and essential fatty acid status. The mainstay of this approach is a high calorie, high-fat diet, exceeding age, and sex energy intake recommendations for healthy individuals. For patients with exocrine pancreatic insufficiency, enzyme replacement therapy is required to improve fat and calorie absorption. Enzyme dosing varies by age and dietary fat intake. Multiple potential impediments to absorption, including decreased motility, altered gut luminal bile salt and microbiota composition, and enteric inflammation must be considered. Fat soluble vitamin supplementation is required in patients with pancreatic insufficiency. In this report, nutrition support across the age and disease spectrum is discussed, with a focus on the relationships among nutritional status, growth, and disease outcomes.

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6

Kalnins, Daina, Peter R. Durie, and Paul Pencharz. "Nutritional management of cystic fibrosis patients." Current Opinion in Clinical Nutrition and Metabolic Care 10, no. 3 (May 2007): 348–54. http://dx.doi.org/10.1097/mco.0b013e3280a94f80.

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7

Winklhofer-Roob, B. M. "Antioxidant status in cystic fibrosis patients." American Journal of Clinical Nutrition 63, no. 1 (January 1, 1996): 138–39. http://dx.doi.org/10.1093/ajcn/63.1.138a.

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8

Luder, Elisabeth. "Nutritional care of patients with cystic fibrosis." Topics in Clinical Nutrition 6, no. 2 (April 1991): 39–50. http://dx.doi.org/10.1097/00008486-199103000-00007.

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9

Eisenblätter, J., K. Schlüter, F. Hellmond, A. Hofmann, and S. van Dullemen. "Nutrition cube: a nutrition counselling tool for patients with cystic fibrosis." Journal of Cystic Fibrosis 9 (June 2010): S89. http://dx.doi.org/10.1016/s1569-1993(10)60344-x.

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10

Schöni, M. H., and C. Casaulta-Aebischer. "Nutrition and lung function in cystic fibrosis patients: review." Clinical Nutrition 19, no. 2 (April 2000): 79–85. http://dx.doi.org/10.1054/clnu.1999.0080.

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11

Michel, Suzanne H., Asim Maqbool, Maria D. Hanna, and Maria Mascarenhas. "Nutrition Management of Pediatric Patients Who Have Cystic Fibrosis." Pediatric Clinics of North America 56, no. 5 (October 2009): 1123–41. http://dx.doi.org/10.1016/j.pcl.2009.06.008.

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12

Sinaasappel, M., M. Stern, J. Littlewood, S. Wolfe, G. Steinkamp, Harry G. M. Heijerman, E. Robberecht, and G. Döring. "Nutrition in patients with cystic fibrosis: a European Consensus." Journal of Cystic Fibrosis 1, no. 2 (June 2002): 51–75. http://dx.doi.org/10.1016/s1569-1993(02)00032-2.

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13

Holben, David H., and Robert W. Wilmott. "Enzyme replacement for patients with cystic fibrosis." Journal of the American Dietetic Association 93, no. 1 (January 1993): 14. http://dx.doi.org/10.1016/0002-8223(93)92117-g.

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14

Besore, B., S. Peterson, and K. Nowak. "190: Creation of a nutrition report for cystic fibrosis patients." Journal of Cystic Fibrosis 20 (November 2021): S93. http://dx.doi.org/10.1016/s1569-1993(21)01615-5.

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15

Borowitz, Drucy, Robert D. Baker, and Virginia Stallings. "Consensus Report on Nutrition for Pediatric Patients With Cystic Fibrosis." Journal of Pediatric Gastroenterology and Nutrition 35, no. 3 (September 2002): 246–59. http://dx.doi.org/10.1097/00005176-200209000-00004.

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16

Sheikh, Shahid, Jackie Quach, and Karen McCoy. "Nissen Fundoplication in Patients With Cystic Fibrosis. Impact on Nutrition." Chest 140, no. 4 (October 2011): 385A. http://dx.doi.org/10.1378/chest.1114003.

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17

O’Brien, Catherine E., Heather Harden, and Gulnur Com. "A Survey of Nutrition Practices for Patients With Cystic Fibrosis." Nutrition in Clinical Practice 28, no. 2 (December 6, 2012): 237–41. http://dx.doi.org/10.1177/0884533612466113.

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18

Michel, Suzanne H., and Donna H. Mueller. "Practical approaches to nutrition care of patients with cystic fibrosis." Topics in Clinical Nutrition 2, no. 1 (January 1987): 10–20. http://dx.doi.org/10.1097/00008486-198701000-00004.

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19

Michel, Suzanne H., and Donna H. Mueller. "Practical approaches to nutrition care of patients with cystic fibrosis." Topics in Clinical Nutrition 4, no. 4 (October 1989): 46–55. http://dx.doi.org/10.1097/00008486-198910000-00009.

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20

O'Loughlin, E., D. Forbes, H. Parsons, B. Scott, D. Cooper, and G. Gall. "Nutritional rehabilitation of malnourished patients with cystic fibrosis." American Journal of Clinical Nutrition 43, no. 5 (May 1, 1986): 732–37. http://dx.doi.org/10.1093/ajcn/43.5.732.

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21

McCabe, Helen. "Cystic fibrosis—what nutritional knowledge do patients have?" Journal of Human Nutrition and Dietetics 9, no. 6 (December 1996): 479–86. http://dx.doi.org/10.1046/j.1365-277x.1996.00482.x.

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22

Prinz, Nicole, Julia Wosniok, Doris Staab, Manfred Ballmann, Christian Dopfer, Nicole Regenfuß, Josef Rosenecker, Dirk Schramm, Reinhard Holl W., and Lutz Nährlich. "Glucose Tolerance in Patients with Cystic Fibrosis – Results from the German Cystic Fibrosis Registry." Klinische Pädiatrie 232, no. 04 (March 16, 2020): 210–16. http://dx.doi.org/10.1055/a-1117-3771.

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Abstract Background Oral glucose tolerance (OGT) deteriorates progressively in cystic fibrosis (CF). Clinical registries provide a unique basis to study real-world data. Patients & methods OGT tests (OGTTs) documented in the German CF-registry in 2016 were classified according WHO, modified by ADA: normal glucose tolerance (NGT), indeterminate glycaemia (INDET), impaired fasting glucose (IFG), impaired glucose tolerance (IGT), IFG+IGT, diabetes mellitus (DM). To study the association with lung function, multivariable regression adjusted for age, sex, and CFTR mutation was performed. Results Overall, OGTT screening was done in 35% of CF patients ≧10 years. Of the 996 patients (46.4% females; median age (IQR): 19 (14–27) years) with evaluable OGTTs, 56.2% had either NGT or INDET, whereas 34% had a pre-diabetic OGTT (IFG; IGT; IFG+IGT) and 9.8% a diabetic OGTT. 7 patients had glucose tolerance abnormalities <10 years. DM was more common in females or patients with F508del homozygote mutation, whereas IFG was more frequent in males (all p<0.05). Nearly 75% of patients after transplantation and about half with enteral/parental nutrition and/or steroid use had either a pre-diabetic or diabetic glucose tolerance. In the adjusted model, age (p<0.001) and OGTT category (p=0.013) had both a significant impact on %FEV1. Conclusion Our data of the German CF-registry highlights incidence of glucose tolerance abnormalities in second decade of life in CF patients. However, it also underlines the need for improvement of the documentation and/or performance of OGTT screening in real-world CF care.

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23

Ulyanova, L. V., V. S. Ledneva, N. S. Burdina, M. I. Talykova, A. S. Ivannikova, and V. V. Kolomackaya. "Study of the effectiveness of supplementary feeding in patients with inherited enzyme deficiency." Proceedings of the Voronezh State University of Engineering Technologies 81, no. 2 (November 1, 2019): 239–44. http://dx.doi.org/10.20914/2310-1202-2019-2-239-244.

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The successful provision of optimal nutrition in children with serious diseases depends on the right diet, as well as the addition of specialized mixtures for its correction. The aim of the study was to study the effectiveness of supplemental nutrition in hereditary fermentopathy (cystic fibrosis) in children. The work included a three-year follow-up of 69 children aged 3 to 15 years suffering from cystic fibrosis. Patients were divided into two groups: 37 patients with cystic fibrosis group 1, receiving a modified version of treatment with additional enteral nutrition and 32 patients with cystic fibrosis group 2, receiving traditional treatment. The study conducted a comparative analysis of changes in trophic status in patients using nutritional support mixture produced in the Russian Federation – "Nutrien-standard"in diet therapy. Laboratory, functional and anthropometric parameters were monitored once a month. A significant increase in the physical development of patients was obtained only in the second year of use in the diet of supplementary nutrition, p<0.05. At the 3rd year of treatment, the results of positive dynamics of nutritional status in both groups were confirmed, while the increase in body mass index in the main group was 6.7%, and in the control group-only 1% (p<0.05). It is noted that the use of this mixture allows to achieve positive dynamics of nutritional status in 27% of patients and in 73% of cases to completely eliminate it. The obtained results prove the expediency of additional use of "Nutrient-standard" mixture in the complex therapy of patients with hereditary pancreatic enzyme deficiency, cystic fibrosis

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24

Fok, Jenny, Neil E. Brown, Peter Zuberbuhler, Joan Tabak, and Mei Tom. "Low Bone Mineral Density in Cystic Fibrosis Patients." Canadian Journal of Dietetic Practice and Research 63, no. 4 (December 2002): 192–97. http://dx.doi.org/10.3148/63.4.2002.192.

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The goals of this practice-based, observational study were to describe the prevalence of low bone mineral density in patients at the Edmonton Cystic Fibrosis Centre, and to determine if body mass index and previous systemic corticosteroid use of over one month's duration were predictors of low bone mineral density. One hundred and thirteen pediatric and adult patients were studied. Bone mineral density of the lumbar spine region was measured using dual-energy X-ray absorptiometry. A total of 42.5% of patients had a bone mineral density Z-score of less than –1 standard deviation. Low bone mineral density was apparent at nine to 12 years of age, and was most evident in the 20- to 34-year-old group. All but one patient under age 20 with a Z-score of less than –2.5 also had a body mass index below the fifth percentile. A low Z-score was also associated with previous systemic corticosteroid use of over one month's duration (relative risk 1.81, p=0.003). We conclude that low bone mineral density is common in cystic fibrosis patients. Low body mass index percentiles may be used to identify children and adolescents at risk of low bone mineral density. These patients may benefit from aggressive nutrition therapy. Systemic corticosteroid use should be assessed carefully, as it is a risk factor for low bone mineral density.

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25

Bridges, M. "Micronutrient Management Algorithms for Adult Cystic Fibrosis Patients." Journal of the Academy of Nutrition and Dietetics 117, no. 10 (October 2017): A127. http://dx.doi.org/10.1016/j.jand.2017.08.027.

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26

Freswick, Peter N., Elizabeth K. Reid, and Maria R. Mascarenhas. "Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis." Nutrients 14, no. 7 (March 23, 2022): 1341. http://dx.doi.org/10.3390/nu14071341.

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While typically considered a pulmonary disease, cystic fibrosis patients develop significant nutritional complications and comorbidities, especially those who are pancreatic insufficient. Clinicians must have a high suspicion for cystic fibrosis among patients with clinical symptoms of pancreatic insufficiency, and pancreatic enzymatic replacement therapy (PERT) must be urgently initiated. PERT presents a myriad of considerations for patients and their supporting dieticians and clinicians, including types of administration, therapy failures, and complications.

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27

Van Biervliet, S., S. Vande Velde, J. P. Van Biervliet, and E. Robberecht. "The Effect of Zinc Supplements in Cystic Fibrosis Patients." Annals of Nutrition and Metabolism 52, no. 2 (2008): 152–56. http://dx.doi.org/10.1159/000129650.

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28

Ellis, J. A., S. A. Bond, and S. A. Wootton. "Energy and protein intakes of patients with cystic fibrosis." Journal of Human Nutrition and Dietetics 5, no. 6 (December 1992): 333–42. http://dx.doi.org/10.1111/j.1365-277x.1992.tb00173.x.

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29

Simonova, O. I., N. V. Lyabina, I. Sokolov, T. E. Borovik, E. A. Roslavtseva, T. V. Bushueva, I. V. Shirokova, and V. A. Kerimova. "Nutrition for children with cystic fibrosis and carbohydrate metabolism disorders." Voprosy praktičeskoj pediatrii 17, no. 1 (2022): 199–206. http://dx.doi.org/10.20953/1817-7646-2022-1-199-206.

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In children with chronic respiratory diseases, especially in cystic fibrosis (CF), poor nutritional status against the background of the underlying disease significantly affects their quality of life and disease prognosis. A special group of children with CF consists of patients who were diagnosed with carbohydrate metabolism disorders or with so-called cystic fibrosis-related diabetes. Early diagnosis of carbohydrate metabolism disorders in prediabetes and then treatment of this comorbid condition in CF is relevant and complex and requires a scientific and practical solution. Diet therapy contributes significantly to successful treatment of children with CF. Patients with CF and carbohydrate metabolism disorders especially need additional specialized therapeutic diet with high-calorie and low-glycemic food products. In our study, we obtained positive values of anthropometric measurements and body composition (based on bioelectrical impedance analysis), as well as positive dynamics of capillary blood glucose levels on the background of short-term intake of a specialized food product “Resource Diabet Plus”. Key words: cystic fibrosis, children, carbohydrate metabolism disorders, cystic fibrosis-related diabetes, diabetes mellitus, nutritional status, enteral feeding, Resource Diabet Plus

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30

Mariotti Zani, Elena, Roberto Grandinetti, Daniela Cunico, Lisa Torelli, Valentina Fainardi, Giovanna Pisi, and Susanna Esposito. "Nutritional Care in Children with Cystic Fibrosis." Nutrients 15, no. 3 (January 17, 2023): 479. http://dx.doi.org/10.3390/nu15030479.

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Patients with cystic fibrosis (CF) are prone to malnutrition and growth failure, mostly due to malabsorption caused by the derangement in the chloride transport across epithelial surfaces. Thus, optimal nutritional care and support should be an integral part of the management of the disease, with the aim of ameliorating clinical outcomes and life expectancy. In this report, we analyzed the nutrition support across the different ages, in patients with CF, with a focus on the relationships with growth, nutritional status, disease outcomes and the use of the CF transmembrane conductance regulator (CFTR) modulators. The nutrition support goal in CF care should begin as early as possible after diagnosis and include the achievement of an optimal nutritional status to support the growth stages and puberty development in children, that will further support the maintenance of an optimal nutritional status in adult life. The cornerstone of nutrition in patients with CF is a high calorie, high-fat diet, in conjunction with a better control of malabsorption due to pancreatic enzyme replacement therapy, and attention to the adequate supplementation of fat-soluble vitamins. When the oral caloric intake is not enough for reaching the anthropometric nutritional goals, supplemental enteral feeding should be initiated to improve growth and the nutritional status. In the last decade, the therapeutic possibilities towards CF have grown in a consistent way. The positive effects of CFTR modulators on nutritional status mainly consist in the improvement in weight gain and BMI, both in children and adults, and in an amelioration in terms of the pulmonary function and reduction of exacerbations. Several challenges need to be overcome with the development of new drugs, to transform CF from a fatal disease to a treatable chronic disease with specialized multidisciplinary care.

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31

Kurilina, T. V., T. V. Marushko, A. O. Pysariev, N. P. Hliadielova, T. V. Taranenko, and I. F. Lazarenko. "Modern view on the nutrition of children with cystic fibrosis." Modern pediatrics. Ukraine, no. 3(123) (April 30, 2022): 38–46. http://dx.doi.org/10.15574/sp.2022.123.38.

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Cystic fibrosis (CF) is an autosomal recessive, life-shortening disease in which generalized exocrine gland involvement occurs with predominantly pancreatic insufficiency, airway obstruction, and progressive lung involvement. Due to pancreatic insufficiency, malabsorption and malnutrition are observed in patients. The close attributive association of CF with nutritional status and adverse clinical outcomes determines a thorough assessment of the nutritional status, individual management and monitoring of all children with CF. Attainment of optimal physical development and adequate nutrition in patients with CF is currently possible through early diagnosis and aggressive nutritional medical therapy. Medical dietary interventions are aimed at ensuring normal growth and preventing of malnutrition, thus will upgrade the prognosis and quality of life of pediatric patients with CF. The reasons for the development of malnutrition in CF, specific screening tools for monitoring the growth of ill children, criteria for stratifying children into nutritional risk groups are reviewed in the lection. The procedure for calculating energy needs, taking into account activity, the severity of the disease and pancreatic insufficiency is presented. Attention is drawn to the ratio of macronutrients and the need to consume energy-dense and nutrient-rich foods. The prescription of medical nutritional therapy with the inclusion of special foods for medical purposes (clinical nutrition) to meet increased energy requirements and avoid the consumption of energy-dense but nutrient-poor foods also is discussed. It is meaningful to involve into a multidisciplinary team of a psychologist who can on a regular basis correct eating behavior and promote compliance with nutritional recommendations in order to achieve optimal physical development of patients. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors. Key words: cystic fibrosis, children, malnutrition, medical nutrition therapy.

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32

Lands, L. C., M. Iskandar, N. Beaudoin, B. Meehan, N. Dauletbaev, and Y. Berthiuame. "Dietary Supplementation with Pressurized Whey in Patients with Cystic Fibrosis." Journal of Medicinal Food 13, no. 1 (February 2010): 77–82. http://dx.doi.org/10.1089/jmf.2008.0326.

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33

HOLBEN, DAVID H., ANNE M. SMITH, and ROBERT W. WILMOTT. "Aminoglycosides Lower Serum Magnesium Concentrations in Patients with Cystic Fibrosis." Journal of the American Dietetic Association 95, no. 10 (October 1995): 1152–54. http://dx.doi.org/10.1016/s0002-8223(95)00310-x.

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34

Tam-Chung, T. C., G. Hubert, D. Mager, J. Lu, and S. Yoo. "Micronutrient Monitoring in Adult Cystic Fibrosis Patients Undergoing Lung Transplantation." Journal of the American Dietetic Association 108, no. 9 (September 2008): A37. http://dx.doi.org/10.1016/j.jada.2008.06.406.

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35

Marcason, Wendy. "What are the calorie requirements for patients with cystic fibrosis?" Journal of the American Dietetic Association 105, no. 4 (April 2005): 656. http://dx.doi.org/10.1016/j.jada.2005.02.040.

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36

Adde, Fabíola V., Joaquim C. Rodrigues, and Ary L. Cardoso. "Nutritional follow-up of cystic fibrosis patients: the role of nutrition education." Jornal de Pediatria 80, no. 6 (November 1, 2004): 475–82. http://dx.doi.org/10.2223/1261.

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37

Adde, Fabíola V., Joaquim C. Rodrigues, and Ary L. Cardoso. "Nutritional follow-up of cystic fibrosis patients: the role of nutrition education." Jornal de Pediatria 80, no. 6 (December 1, 2004): 475–82. http://dx.doi.org/10.2223/jped.1261.

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38

FERNANDES, J., C. M. F. KNEEPKENS, C. M. A. BIJLEVELD, and R. J. VONK. "General Aspects of Nutrition, Absorption and Metabolism in Patients with Cystic Fibrosis." Acta Paediatrica 74, s317 (March 1985): 5–8. http://dx.doi.org/10.1111/j.1651-2227.1985.tb14927.x.

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39

Souza dos Santos Simon, Miriam Isabel, Gabriele Carra Forte, Juliane da Silva Pereira, Elenara da Fonseca Andrade Procianoy, and Michele Drehmer. "Validation of a Nutrition Screening Tool for Pediatric Patients with Cystic Fibrosis." Journal of the Academy of Nutrition and Dietetics 116, no. 5 (May 2016): 813–18. http://dx.doi.org/10.1016/j.jand.2016.01.012.

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40

Di Lullo, Antonella M., Paola Iacotucci, Marika Comegna, Felice Amato, Pasquale Dolce, Giuseppe Castaldo, Elena Cantone, Vincenzo Carnovale, and Maurizio Iengo. "Cystic Fibrosis: The Sense of Smell." American Journal of Rhinology & Allergy 34, no. 1 (August 20, 2019): 35–42. http://dx.doi.org/10.1177/1945892419870450.

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Background Cystic fibrosis (CF) is a multisystem disease that involves the upper airways with chronic rhinosinusitis (CRS) causing nasal congestion, rhinorrhea, mouth breathing, facial pain, and olfactory dysfunction. Twelve percent to 71% of CF patients report smelling alterations with an impact on nutrition and quality of life. Objectives The goal was to study olfaction performance in CF patients with CRS that worsens quality of life. Methods A total of 121 subjects were enrolled in this study. Seventy-one had CF and underwent ear, nose, and throat evaluation with nasal endoscopy, sinonasal outcome test 22 (SNOT-22), visual analog scale (VAS), and “Sniffin’ Sticks.” Fifty subjects were age-matched with healthy controls. Results All 71 CF patients were affected by CRS; 59 of 71 (83.1%) had CRS without nasal polyps and 12 of 71 (16.9%) had CRS with early nasal polyps. None of the 50 controls had CRS. Total SNOTT-22 mean values in the 71 CF patients were 38.10 ± 21.08 points. If considering only the 59 CF patients without nasal polyps, the SNOTT-22 mean value was 36.76 ± 21.52 points. Moreover, based on the VAS scores, the degree of nasal symptoms was classified as mild for facial pain, smell alteration, nasal discharge, and sneezing and resulted in moderate symptoms for nasal blockage and headache. Among the CF patients, 55 of 71 (76.5%) declared to be normosmic, while the smelling ability assessed by “Sniffin’ Sticks” showed that only 4 of 71 (5.63%) were normosmic, 58 (81.69%) were hyposmic, and 9 (12.68%) were anosmic. In the controls, 41(82%) were normosmic, 9 (18%) were hyposmic, and none were reported to be anosmic ( P < .001). Conclusions We confirm that most CF patients have a relevant olfactory impairment, although only a low percentage declares such alteration. A careful evaluation with simple and rapid tests helps to select the patients who may benefit from specific therapies.

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41

McCLOSKEY, M., A. O. B. REDMOND, S. PYPER, C. McCABE, K. R. WESTERTERP, and J. S. ELBORN. "Total energy expenditure in stable patients with cystic fibrosis." Clinical Nutrition 20, no. 3 (June 2001): 235–41. http://dx.doi.org/10.1054/clnu.2001.0389.

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42

Brei, Christina, Annette Simon, Michael B. Krawinkel, and Lutz Naehrlich. "Individualized vitamin A supplementation for patients with cystic fibrosis." Clinical Nutrition 32, no. 5 (October 2013): 805–10. http://dx.doi.org/10.1016/j.clnu.2013.01.009.

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43

Coriati, Adèle, Évelyne Labrèche, Marjolaine Mailhot, Hortensia Mircescu, Yves Berthiaume, Annick Lavoie, and Rémi Rabasa-Lhoret. "Vitamin D3 supplementation among adult patients with cystic fibrosis." Clinical Nutrition 36, no. 6 (December 2017): 1580–85. http://dx.doi.org/10.1016/j.clnu.2016.10.002.

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44

Roulet, M., V. Spicher, and Y. Schutz. "Total energy expenditure in free-living cystic fibrosis patients." Clinical Nutrition 10 (January 1991): 8. http://dx.doi.org/10.1016/0261-5614(91)90143-z.

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45

Manner, T., L. Guida, D. P. Katz, J. Askanazi, E. Schlotzer, S. Wiese, B. Skeie, and P. Fürst. "Parenteral fish-oil administration in patients with cystic fibrosis." Clinical Nutrition 11 (January 1992): 40. http://dx.doi.org/10.1016/0261-5614(92)90191-r.

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46

Franklin Dos Santos, Thais, Andrea Rabassa, Oscar Aljure, and Reine Zbeidy. "Perioperative Management and Preemptive ECMO Cannulation of a Parturient with Cystic Fibrosis Undergoing Cesarean Delivery." Case Reports in Anesthesiology 2020 (December 4, 2020): 1–5. http://dx.doi.org/10.1155/2020/8814729.

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Physiologic changes of pregnancy and cystic fibrosis pathology provide a unique set of circumstances. Pulmonary disease accounts for over 90% of the morbidity and mortality of patients with cystic fibrosis. These abnormalities create anesthetic challenges due to multiple organ systems being affected including the respiratory, gastrointestinal, cardiovascular, and genitourinary tracts, where patients present with chronic respiratory failure, pancreatic insufficiency, poor nutrition, and cardiac manifestations. We present the perianesthetic management of a parturient with cystic fibrosis who successfully underwent preterm cesarean delivery under neuraxial anesthesia with preemptive bilateral femoral venous sheaths placed for potential extracorporeal membrane oxygenation (ECMO) initiation.

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Esposito, Susanna, Ilaria Testa, Elena Mariotti Zani, Daniela Cunico, Lisa Torelli, Roberto Grandinetti, Valentina Fainardi, Giovanna Pisi, and Nicola Principi. "Probiotics Administration in Cystic Fibrosis: What Is the Evidence?" Nutrients 14, no. 15 (July 30, 2022): 3160. http://dx.doi.org/10.3390/nu14153160.

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In the last 20 years, gut microbiota in patients with cystic fibrosis (CF) has become an object of interest. It was shown that these patients had gut dysbiosis and this could explain not only the intestinal manifestations of the disease but also part of those involving the respiratory tract. The acquisition of previously unknown information about the importance of some bacteria, i.e., those partially or totally disappeared in the gut of CF patients, in the regulation of the activity and function of the gut and the lung was the base to suggest the use of probiotics in CF patients. The main aim of this paper is to discuss the biological basis for probiotic administration to CF patients and which results could be expected. Literature analysis showed that CF intestinal dysbiosis depends on the same genetic mutations that condition the clinical picture of the diseases and is aggravated by a series of therapeutic interventions, such as dietary modifications, the use of antibiotics, and the administration of antacids. All this translates into a significant worsening of the structure and function of organs, including the lung and intestine, already deeply penalized by the genetic alterations of CF. Probiotics can intervene on dysbiosis, reducing the negative effects derived from it. However, the available data cannot be considered sufficient to indicate that these bacteria are essential elements of CF therapy. Further studies that take into account the still unsolved aspects on how to use probiotics are absolutely necessary.

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Skypala, Ashworth, Hodson, Leonard, Knox, Hiller, Wolfe, et al. "Oral nutritional supplements promote significant weight gain in cystic fibrosis patients." Journal of Human Nutrition and Dietetics 11, no. 2 (April 1998): 95–104. http://dx.doi.org/10.1046/j.1365-277x.1998.00091.x.

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Lark, Robert K., Gayle E. Lester, David A. Ontjes, Angelia D. Blackwood, Bruce W. Hollis, Margaret M. Hensler, and Robert M. Aris. "Diminished and erratic absorption of ergocalciferol in adult cystic fibrosis patients." American Journal of Clinical Nutrition 73, no. 3 (March 1, 2001): 602–6. http://dx.doi.org/10.1093/ajcn/73.3.602.

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Nicolo, Michele, Kathleen W. Stratton, Wanda Rooney, and Joseph Boullata. "Pancreatic Enzyme Replacement Therapy for Enterally Fed Patients With Cystic Fibrosis." Nutrition in Clinical Practice 28, no. 4 (June 10, 2013): 485–89. http://dx.doi.org/10.1177/0884533613491786.

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