Pathophysiology of cystic fibrosis and drugs used in associated digestive tract diseases

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.

Impact of clinical and laboratory weight gain in a cohort of patients with cystic fibrosis diagnosed by newborn screening factors

“…11 Como resultado, esteatorreia, dor abdominal, deterioração de órgãos,perda de peso, tornando menor a expectativa de vida. 12 A terapia de reposição enzimática é bem segura e fácil de ser tolerada, inclusive em crianças menores de 2 anos de idade. 11 Contudo, apesar do uso regular das enzimas pancreáticas, encontrou-se influência negativa no ganho de peso ao longo dos anos neste estudo.…”

Association between hematological profile and serum 25-hydroxyvitamin D levels and FokI polymorphism in individuals with cystic fibrosis

“…Cystic Fibrosis (CF) is a fatal autosomal recessive disease that is more common in Caucasians and caused by mutations in the CF Transmembrane Conductance Regulator (CFTR) gene. The dysfunction in this channel leads to changes in the viscosity, volume, and concentration of salt in the body fluids, leading to the formation of thick secretions, which prevent the hydration of the airways and create a favorable environment for bacterial colonization, chronic inflammation, and eventually respiratory insufficiency [1][2][3].…”

“…In Brazil, the incidence is approximately 1/7000. However, it is subjected to regional variations [1].…”

“…Fat loss through feces carries the risk of liposoluble vitamin deficiency, including vitamin D. In addition, these patients have increased energy expenditure due to the nature of the disease and the frequency and severity of infections and chronic pulmonary inflammation. Thus, the importance of nutrition in these patients is reinforced [1][2][3]5].…”

“…The discovery that most of the body tissues and cells contain vitamin D receptors and that several of these tissues have the enzymatic property to convert the primary form of vitamin D in the circulation to its active form has provided new insights about the function of this steroid hormone [10]. In addition, the concentration of 1,25-hydroxyvitamin D (1,25[OH]D) is a hundred times greater in the bone marrow than in the plasma. The clinical consequences of its deficit have been a topic of interest due to the new insights about the biological functions of vitamin D, and the hypotheses that there is an association between vitamin D and hemoglobin levels and that this phenomenon may be influenced by polymorphisms in the Vitamin D Receptor gene (VDR) have been reinforced [11,12].…”