Pathophysiology of Cystic Fibrosis PDF

Added on - 24 Apr 2021

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Running Head: CYSTIC FIBROSISCystic FibrosisName of StudentInstitution affiliation
CYSTIC FIBROSIS2Table of ContentsIntroduction......................................................................................................................................3Normal physiology of cystic fibrosis...............................................................................................3Pathophysiology of cystic fibrosis (CF)..........................................................................................4Gastrointestinal disease................................................................................................................4Pancreatic disease........................................................................................................................5Hepatobiliary Disease..................................................................................................................6Lungs............................................................................................................................................7Pharmacological treatments of CF...................................................................................................8CFTR Correctors and Potentiators; Orkambi..............................................................................8Contraindications......................................................................................................................8Mode of Action........................................................................................................................8Routes of administration..........................................................................................................9Pancreatic enzymes......................................................................................................................9The mechanism of action.........................................................................................................9Pharmacokinetics of the drug...................................................................................................9Routes of administration........................................................................................................10Contraindications....................................................................................................................11Antibiotics..................................................................................................................................11Mode of Action......................................................................................................................11Route of administration..........................................................................................................12Pharmacokinetics....................................................................................................................12Contraindications....................................................................................................................12Conclusion.....................................................................................................................................13
CYSTIC FIBROSIS3IntroductionThis is a genetic disease commonly found among the Caucasians. However, it is rare inother races. The gene in cystic fibrosis has 230 alleles on chromosome seven. Cystic FibrosisTransmembrane Conductance Regulator (CFTR) is the protein encoded by the gene. The proteinfunctions as an ion channels. A mutation of this gene causes cystic fibrosis. The mutations affectthe CTFR protein in many ways thus leading to a dysfunctional CTFR within the apicalmembrane in epithelial cells. The inability to transport ions leads to accumulation of mucus inthe epithelia of organs such as gastrointestinal tract, lungs, sweat glands and the hepatobiliarysystem (Jane, 2010).Normal physiology of cystic fibrosisCystic Fibrosis Transmembrane Conductance Regulator is the most vital chloride channelin the epithelial of tissues that performs functions such as water volume absorption andabsorption of salts in lungs and sweat ducts. Moreover, it is found in the pancreas and lungswhere it secretes water. All the processes above involve transport of chloride ions. Therefore,cystic fibrosis interrupts ion transport, which leads to several clinical effects (Jane, 2010).
CYSTIC FIBROSIS4Figure 1:The CTFR has two blocks of six trans-membrane domains and two nucleotide-binding domains. Furthermore, it has a regulatory domain that has phosphorylation sites for PKAand PKC. NBD1 participates in the formation of ion channels (Becq, 2010).The disease manifestation in CF is changeable. The manifestation can occur in the childstage or later stages of life. The basic symptoms are chronic diarrhea, malnutrition, and chroniccough. Furthermore, CF affects many body organs (Jane, 2010).Pathophysiology of cystic fibrosis (CF).CF affects secretory granules and intracellular organs, each organ has a unique clinicalresponse. In addition to that, defects in the respiratory tract lead to high mortality in 90% ofpatients.CF causes micro vascular and macro vascular complications caused by the deteriorationof lungs (Adriana, Aragao & Rita, 2013).Gastrointestinal disease.Gastrointestinal symptoms include vomiting, nausea, indigestion, and malnutrition. Inaddition to that, other symptoms are esophageal reflux, cholelithiasis, and distal intestinesyndrome. Despite conventional treatments, some patients continue to have complicatedgastrointestinal problems such as chronic inflammation (Adriana, Aragao & Rita, 2013).It is reported that eosinophilic esophasitis (EoE) can be presented in patients aged 4, 12,and 15. Furthermore, EoE has been under appreciated due to symptom overlap with otherdisorders of the intestines such as the gastro-esophageal reflux. (Adriana, Aragao & Rita, 2013).The entire process of digestion is affected by CF thus leading to malabsoption ofnutrients, gastrointestinal problems, and malnutrition. Other signs include abdominal pains,fibronising colonopathy and bond obstruction syndrome. The main cause of abdominal pain is
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