Review
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World J Gastroenterol. Dec 14, 2013; 19(46): 8552-8561
Published online Dec 14, 2013. doi: 10.3748/wjg.v19.i46.8552
Pathophysiology of cystic fibrosis and drugs used in associated digestive tract diseases
Adriana Haack, Giselle Gonçalves Aragão, Maria Rita Carvalho Garbi Novaes
Adriana Haack, Giselle Gonçalves Aragão, Maria Rita Carvalho Garbi Novaes, Department of Health, Universidade Paulista (UNIP), Brasília 70381701, Brazil
Author contributions: Haack A worked on the design and planning, in the acquisition, analysis and interpretation of data, in writing and critical review; Aragon GG contributed to the manuscript writing and interpreting data; Novaes MRCG worked on the design and planning, data interpretation, the writing and the final revision.
Correspondence to: Adriana Haack, Professor of Nutrition, Department of Health, Universidade Paulista (UNIP), SHIGS 715 Block A House 87, Brasília Federal District, Brasília 70381701, Brazil. adrianahaack@hotmail.com
Telephone: +55-61-34432636 Fax: +55-61-32445302
Received: August 28, 2013
Revised: September 28, 2013
Accepted: October 13, 2013
Published online: December 14, 2013
Processing time: 114 Days and 0.4 Hours
Abstract

Cystic fibrosis (CF) causes chronic infections in the respiratory tract and alters the digestive tract. This paper reviews the most important aspects of drug treatment and changes in the digestive tract of patients with CF. This is a review of the literature, emphasizing the discoveries made within the last 15 years by analyzing scientific papers published in journals indexed in the Scientific Electronic Library Online, Sciences Information, United States National Library of Medicine and Medical Literature Analysis and Retrieval System Online databases, both in English and Portuguese, using the key words: cystic fibrosis, medication, therapeutic, absorption, digestion. Randomized, observational, experimental, and epidemiological clinical studies were selected, among others, with statistical significance of 5%. This review evaluates the changes found in the digestive tract of CF patients including pancreatic insufficiency, constipation and liver diseases. Changes in nutritional status are also described. Clinical treatment, nutritional supplementation and drug management were classified in this review as essential to the quality of life of CF patients, and became available through public policies for monitoring and treating CF. The information gathered on CF and a multi professional approach to the disease is essential in the treatment of these patients.

Keywords: Cystic fibrosis; Medication; Therapeutic; Absorption; Digestion

Core tip: Cystic fibrosis (CF) has been studied in Brazil and in many other countries. Digestive manifestations may significantly compromise the nutritional status of CF patients, leading to numerous symptoms. Supplementation with enzymes, vitamins and nutrients is usually necessary. When infections are present, antibiotics are necessary, and these infections are often multisystemic, involving the digestive tract. The pharmaceutical assistance included in public policies, especially those which are financed, and the constant incentive to study the digestive manifestations in CF patients are essential, as without them, there would be infinite clinical changes which would compromise patient survival.