cystic Fibrosis Assignment PDF
Added on 2021-04-17
8 Pages1489 Words158 Views
1Cystic FibrosisStudent NameCourse NameInstitutional AffiliationWord Count: 10001
21.The epithelial disease is cystic fibrosis. Cystic fibrosis is a genetic progressive illnesses that causes over production of mucous in the lining of the epithelial cells of the affected glands such as lungs in addition reproductive system.The build up of the stick mucus in the affected glands can lead up to subsequent infections that are chronic.2.3 key features of the disease that make it epithelial in origin Fibrocystic disease is as a result of the mutation of the CFTR protein. The gene-product is a integument bound cAMP-controlled chloride conduit which, is proposedto control the other cell integument ion channels(1). Cystic fibrosis is mainly aninherited disorder when an offspring inherits the CFTR gene -which is recessive innature- and may result in a mutation. The disease affects the animal tissue ion and water transport which is primary incells found in systems such as respiration, gastrointestinal, hepatobiliary andreproductive as well as sweat glands (2). The CFTR lacks the amino acidphenylalanine resulting in chloride and sodium ions accumulation within the cellshence, drawing a lot of fluids in to the cells leading to dehydration. Therefore, itaffects the exocrine system or glands which are responsible for the mucus-secretingand sweat glands in the respiratory and digestive systems. Build up in mucous production in the epithelial lining of cells such as lungs wherethe mucus is sticky and thick. The consequential effect is the build up of germs in theareas of infections such as bacteria in addition fungal infections that can be chronicin persons living with CF. Bacteria can include Staphylococcus aureus orPseudomonas aeruginosa (3). 3.Describe the molecular pathophysiology of the epithelial disease 2
3The pathophysiology of cystic fibrosis contains one underlying condition which is the mutationof the CFTR gene that produces the CFTR protein responsible for production of ion and watertransport through cell membranes. CFTR is an acronym meaning Fibrocystic transmembranemediator regulator. There are several categories of cystic fibrosis reliant on the area of attack (3).They include respiratory system, hematopoietic system, gastrointestinal system, endocrine, sweatand reproductive systems. 1.Respiratory system and Sweat Glands The molecules involved in respiratory cystic fibrosis includes asialoGM1 units which are theattachments for bacterial respirational organism which increase the binding of P. aeruginosabesides S. aureus(3). As such, the decline in quantity of CFTR leads to an increase inbacteria biding which colonize the airways (2). Hence, it is typical for patients with cysticfibrosis to have decreased points of interleukin-10 which is a cytokine that has inflammatorycharacteristics in the lungs (4). The lowering of the interleukin-10 predisposes the persons tosevere lung infections such as increased thick mucus secretions on their lung epitheliallinings. 2.Hematopoietic System Patients lack iron leading to anemia. In most cases, the pathophysiology of the cysticfibrosis in hematopoietic system is the advancement in hypertrophied also convolutedbronchial arteries which result in long-lasting inflammation (5). 3.Gastrointestinal System Characterized by the inability of the pancreatic system to reduce capacity in production ofenzymes (6). The result is the pancreas excretes a lot of mucus which blocks the pancreaticducts then the capacity of enzymes secreted converts smaller (5). 4.Endocrine System The glycated hemoglobin (Hemoglobin A1C) but, tests are limited to such possibility. Inaddition, the primary problem is linked to insulin deficit due to obstacle of pancreatic duct. 3
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