M Rafeey [1] , MD P Pourabdollahi [2] ,PhD SJ Ghaemmaghami [3] , MSc M Samsamy [4] , BSc Pourhossein D [5] , MD, A Nasiri [6] , BSc

 �

  Received: 5 May, 2009 Accepted: 29 July, 2009

 Abstract

 Background & Aims : C ystic fibrosis is the most common life threatening genetic disorder amongst Caucasians. Infants and toddlers with cystic fibrosis are at risk for poor growth. Controlled behavioral assessment studies have not focused on this population. Good nutrition is essential for normal growth and development. The aim of this study was to determine the usual pattern of intake and adherence enzyme replacement therapy in children with cystic fibrosis and pancreatic insufficiency.

  Materials & Methods: In this case control study 34 children (3 months to 11 years) with cystic fibrosis were covered in gastroenterology clinic of Tabriz Children Hospital (2007-2008). Anthropometrical measurements of weight-height, middle upper arm muscle environment and their nutritional status using the food frequency questionnaire were collected. The results were compared with 34 healthy children of the same age and gender.

  Results: Thirty four patients with the age range of 71.38±61.85 were evaluated. There was a significant difference only in weight/height (P=0.000) and weight/age value (P=0.04), but other anthropometric data were not statistically significant. 85.3% of the patients received nutritional supplements and 70.6% had enzyme replacement. In average food intake significant differences between cystic fibrosis patients and normal children were in spaghetti, egg, ice cream and liver. Only %47.1 of the patients’ parents received nutrition counseling.

  Conclusion: Malnutrition and poor growth is important for cystic fibrosis patients. P roper treatment and adoption of a standardized approach to nutritional assessment leads to significant improvement in nutritional outcomes of cystic fibrosis patients, demonstrating that systematic changes in clinical practice can improve clinical outcomes .

  Keyword : Cystic fibrosis, Child, nutrition, Enzyme replacement, Growth

  Address : Children Health Research Center, Tabriz University of Medical Sciences, Tabriz, Iran

 Tel: (+98) 9141146982

  Email : mrafeey@yahoo.com

  [1] Associate Professor of Pediatric Gastroenterology, Department of Pediatrics, and Liver and Gastrointestinal Diseases Research Center, Children Health Research Center , Tabriz University of Medical Sciences, Tabriz, Iran (Corresponding Author)

  [2] Assistant Professor of Diet Therapy Department, Tabriz University of Medical Sciences, Tabriz, Iran

  [3] Instructor of Food Science Department, Tabriz University of Medical Sciences, Tabriz, Iran

  [4] BSc in Nutrition Science, Tabriz University of Medical Sciences, Tabriz, Iran

  [5] Resident in Pediatrics, Tabriz University of Medical Sciences, Tabriz, Iran

  [6] BSc in Nursing , Tabriz University of Medical Sciences, Tabriz, Iran