Brazilian Guidelines for Nutrition in Cystic Fibrosis

Corresponding author: Lenycia de Cassya Lopes Neri Avenida Dr. Enéas Carvalho de Aguiar, 647 - Cerqueira César Zip code: 05403-000 - São Paulo, SP, Brazil Phone: (55 11) 2661-8524 E-mail:
lenycia@gmail.com

AUTHORS´ CONTRIBUTION

Lenycia de Cassya Lopes Neri: article conception, planning and organization of working groups, training coordinators on bibliographic search, scientific writing, review of all topics, translation review and submission of the final article. Miriam Isabel Souza dos Santos Simon, Valéria Laguna Salomão Ambrósio, Eliana Barbosa, Monique Ferreira Garcia, Juliana Ferreira Mauri, Renata Rodrigues Guirau, Mirella Aparecida Neves, Carolina de Azevedo Pedrosa Cunha and Marcelo Coelho Nogueira: planning, scientific writing, grammar review and translation review. Anna Carolina Di Creddo Alves, Jocemara Gurmini, Maria de Fatima Servidoni and Matias Epifanio: scientific writing, grammar review and translation review. Rodrigo Athanazio: supervision and technical support in planning, organizing and writing.

Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.

Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.

Hospital das Clínicas, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brazil.

Hospital Infantil Joana de Gusmão, Florianópolis, SC, Brazil.

Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.

Universidade Estadual de Campinas, Campinas, SP, Brazil.

Hospital Pequeno Príncipe, Curitiba, PR, Brazil.

Hospital da Criança de Brasília José Alencar, Brasília, DF, Brazil.

Hospital Infantil João Paulo II, Belo Horizonte, MG, Brazil.

Instituto do Coração (InCor), Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.

Pontifícia Universidade Católica do Rio Grande do Sul, Porto Alegre, RS, Brazil.

Nutritional status	Infants <2 years*	Children and adolescents 2 to 18 years^†	Adults
Optimal	W/H ≥p50 W/A ≥p50 H/A ≥p50 (z-score ≥0)	BMI p50-p85 (z-score 0 – z-score +1) 2-5 years W/H ≥p10 (z-score ≥-1.28)	Women: BMI 22-25kg/m² Men: BMI 23-25kg/m²
Eutrophic	W/H p25-p50 (z-score 0.67 – z-score 0)	BMI p25-p50 (z-score 0.67 – z-score 0) 2-5 years W/H ≥p10 With no recent loss of weight	Women: BMI 20-22kg/m² Men: BMI 20-23kg/m² With no recent non-intentional weight loss
Nutritional risk	W/H p10-p25 (z-score 1.28 – z-score 0.67)	BMI p10-p25 Weight loss or plateau in 2 to 4 months	BMI 18.5-20kg/m² and/or ≥5% non-intentional weight loss over the last 2 months
Malnutrition	W/H <p10 (z-score <-1.28) H/A <p5 and/or drop of 2 percentiles in growth	BMI < p10 (z-score <-1.28) and/or drop of 2 percentiles in weight and inflection of growth	BMI <18.5kg/m² On-going weight loss (>5%)
Overweight		BMI p85-p97 (z-score +1 – z-score +2)	BMI 25-30kg/m²
Obesity		BMI >97 (>z-score +2)	BMI >30kg/m²

Data*	Start and first week (weekly frequency)	Up to 3 weeks (weekly frequency)	Up to 8 weeks (monthly frequency)	After months (monthly frequency)
Weight of infants	7	3	8	2
Weight of children	3	2	2	1
Sodium and potassium	7	3	4	2
Ionic calcium	3	3	2	1
Phosphorus	3	3	2	1
Magnesium	3	3	2	1
Complete blood count	2	1	2	1
Urea and creatinine	3	2	4	1
Blood glucose	28	14	30	1
Glycosuria	14	7	4	1
Alanine aminotransferase	1	1	2	1
Gamma glutamyl transferase	1	1	2	1
Bilirubin	1	1	2	1
Triglyceride	7	2	4	1
Pre-albumin	At the start and after 5-7 days
Albumin	Monthly	Monthly	1	1

Nutrient	Immediate postoperative period	Late postoperative period
Calories	130%-150% of baseline energy expenditure	Sufficient for maintaining healthy weight
Proteins	1.5-2.0g/kg/day	0.8-0.9g/kg/day
Carbohydrates	50%-70% of non-protein calories	Avoid excessive food
Fats	30%-50% of the total calorie value	Monitor the concentration of cholesterol and triglycerides
Liquids	Restrict only if there is hyponatremia or edema	Restrict only if there is hyponatremia or edema
Electrolytes	Monitor levels of potassium, magnesium, phosphorus, and sodium. Supplement sodium if necessary	Monitor levels of potassium, magnesium, phosphorus, and sodium. Supplement sodium if necessary
Vitamins and minerals	Supplementation of vitamins and minerals as per the RDA; consider the post-transplant complications; monitor fat-soluble vitamins and supplement, if necessary; supplement calcium and vitamin D	Supplementation of vitamins and minerals as per the RDA; consider the post-transplant complications; monitor fat-soluble vitamins and supplement, if necessary; supplement calcium and vitamin D

Etiology	Medication	Treatment	Risk of non treatment
Obesity	Corticoids	Physical activity, healthy eating habits, and changes in lifestyle	Development of diabetes mellitus, hypertension, hyperlipidemia, and increased cardiovascular risk
Diabetes mellitus	Corticoids and cyclosporine	Insulin therapy	Poor healing, retinopathy, neuropathy, vascular disease, kidney disease, graft failure
Hypertension	Corticoids	Normal salt intake diet (5g salt/day) and antihypertensive medication	Vascular disease, kidney disease, and graft failure
Osteoporosis	Corticoids	Adequate consumption of calcium, supplementation of calcium and vitamin D, monitor bone density, parathyroid hormones, vitamin D metabolism, calcium, and levels of blood magnesium	Joint diseases, inactivity
Hyperlipidemia	Cyclosporine and corticoids	Diet with adequate saturated fat and cholesterol content, physical activity, maintenance of a healthy weight, and lipid-lowering medication	Peripheral vascular disease, coronary artery disease, and graft failure
Hyperkalemia	Cyclosporine and tacrolimus	Diet adequate in potassium	Cardiac arrhythmia

Age	Supplementation
Infants (up to 12 months)	2,000-4,000U lipase/120mL of formula or estimated consumption of maternal milk and approximately 2,000U of lipase/g dietary fat in foods
Children aged 1 to 4 years	2,000-4,000U lipase/g of dietary fat, increasing the dose as is necessary (maximum dose of 10,000 lipaseU/kg per day)
Children aged >4 years and adults	Consider starting at 500U lipase/kg/meal, titrating up to a maximum dose of: - 1,000-2,500U lipase/kg/meal OR - 10,000U lipase/kg/day OR - 2,000-4,000U lipase/g of dietary fat obtained from all meals, snacks, and beverages containing fat

[TFN1] * Consider W/H preferably to BMI/A in infants; ^† in all nutritional classifications of children and adolescents, the H/A indicator should be considered based on genetic potential (H/A ≥genetic potential).^(4,15)

[TFN2] W: weight; H: height; A: age; BMI: body mass index.

Vitamin A	<1 year: 4,000IU
	>1 year: 4,000IU-10,000IU
	Adults: 4,000IU-10,000IU
Vitamin D	<1 year: 400IU
	>1 year: 400IU-800IU
	Adults: 800IU-2,000IU
Vitamin E	<1 year: 50IU/day
	>1 year: 100IU-400IU/day
	Adults: 100IU-400IU/day
Vitamin K	<1 year: 0.3mg-1.0mg/day
Vitamin K	>1 year: 1mg-10mg/day