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Pediatrics International (2014) 56, 89–94
doi: 10.1111/ped.12214
Original Article
Prevalence of malnutrition and obesity among cystic fibrosis patients Paraskevi Panagopoulou, Maria Fotoulaki, Aristidis Nikolaou* and Sanda Nousia-Arvanitakis Fourth Pediatric Department, Aristotle University of Thessaloniki, Papageorgiou General Hospital, Thessaloniki, Greece Abstract
Background: Optimal nutritional status (NS) in cystic fibrosis (CF) is associated with better lung function and increased overall survival. This study estimated the prevalence of malnutrition and obesity among CF patients in a tertiary center. Methods: In a cross-sectional study of 68 CF patients (33 female; 37 children/adolescents) weight, height, body composition, respiratory function (% of the predicted forced expiratory volume in 1 s; FEV1%pred) and serum lipids were measured; body mass index (BMI), BMI standard deviation score (BMI-SDS) and BMI percentiles were calculated; Pseudomonas colonization, pancreatic insufficiency, diabetes mellitus (CFDM), liver disease (CFLD) and genotype were recorded; NS was classified according to the 2005 Cystic Fibrosis Foundation (CFF) criteria. Frequency distributions and associations between anthropometric and clinical parameters (univariate/multivariate) were calculated. Results: Mean age (±SD) was 19.81 ± 8.98 years. Regarding NS: 22.1% were malnourished, 13.2% overweight/obese and 29.4% had optimal NS. Pancreatic function (PF), Pseudomonas colonization, CFDM, CFLD and genotype differed significantly among the three groups. FEV1%pred was significantly higher among overweight/obese patients and correlated positively with anthropometric characteristics as well as serum cholesterol and negatively with age. BMI-SDS was associated with PF, FEV1%pred and CFDM. Among overweight/obese patients 89.9% had adequate PF and 66.7% carried mutations other than F508del. No patient had any traits of metabolic syndrome. Conclusions: Despite appropriate management only one-third of the present patients had optimal NS. One-fourth were malnourished and a significant percentage were overweight/obese. The latter were mostly carriers of mutations other than F508del and had better pulmonary function. CF patients require intensive monitoring for both malnutrition and overweight/obesity.
Key words body composition, cystic fibrosis, obesity, malnutrition, nutritional status.
Cystic fibrosis (CF) is the most common hereditary lethal disease among Caucasian people, with an incidence of 1 case per 2500 live births.1 It is caused by mutations in the gene encoding the protein cystic fibrosis transmembrane conductance regulator (CFTR), which functions as a chloride channel. According to the Cystic Fibrosis Mutation Database more than 1900 mutations have been described to date.2 F508del accounts for 70% of the cases worldwide3 and for 52–54% of CF cases in Greece.4 The mutation disrupts the function of chloride channels leading to production of thick mucous secretions in several glands and organs. As a result, the parenchyma of the pancreas is destroyed, leading to pancreatic insufficiency (PI) and subsequent malabsorption syndrome and malnutrition.5 The presence of thick mucus in the lungs leads to airway obstruction and colonization with Pseudomonas aeruginosa (or other Pseudomonas spp.) and/or Staphylococcus aureus, causing bronchiectasis and
Correspondence: Maria Fotoulaki, MD, 41 Alexandrou Michailidi, 54641, Thessaloniki, Greece. Email:
[email protected] *Present address: General Hospital of Athens for Chest Diseases, “Sotiria”, Athens, Greece. Received 7 February 2012; revised 27 September 2012; accepted 7 August 2013.
© 2013 The Authors Pediatrics International © 2013 Japan Pediatric Society
respiratory failure, which is the major cause of death for CF patients.6 CF is also related to conditions such as diabetes mellitus (CFDM) and liver disease (CFLD; e.g. focal/nodular biliary cirrhosis).7,8 All the aforementioned conditions in conjunction with factors such as genotype affect nutritional status (NS), which in turn influences the clinical course of CF.9,10 NS has been established as an important predictor of survival because it is associated with respiratory function and overall morbidity and mortality.11,12 Therefore, nutritional support with pancreatic enzyme replacement treatment (PERT) and adequate caloric and vitamin supplementation has become one of the major pillars of CF management, together with treatment with bronchodilators, mucolytics, antibiotics13 and continuous and vigorous physiotherapy. Regular monitoring of NS is an indispensable part of CF follow up,14 and malnutrition is frequently used as a red flag for imminent clinical deterioration requiring immediate intervention. At the same time some researchers have noted an increased prevalence of overweight/obese among CF patients.15–17 The aim of the present study was to determine the prevalence of both malnutrition and overweight/obesity among CF patients in a specialized center in Greece, and to correlate this with anthropometric, clinical and genetic characteristics.
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Methods
Disease characteristics
This was an observational, cross-sectional study carried out between 1 January 2007 and 30 June 2007. Patients were recruited among those followed at the Cystic Fibrosis Outpatient Clinic of a University Hospital in Northern Greece. All CF patients who are regularly followed in the Clinic gave informed consent to participate. Exclusion criteria were: age
