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Cystic fibrosis

الكلية كلية التمريض     القسم قسم التخصصات التمريضية     المرحلة 3
أستاذ المادة عبد المهدي عبد الرضا حسن الشحماني       11/07/2018 08:31:47
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Right lower lung: Chronic cystic fibrosis destroys the lung tissue of the lung, causing the patient to die from respiratory damage.

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Of recessive physical chromosomal disorders
Specialty Medical Genetics
Classification and external resources
TD-10 E84
TD-9 277.0
Human Mendelian inheritance 219700
3347 Diseases
Medline Plus
E Medication ped / 535
NFM D003550
Amyloid drugs, acetylcysteine
[Edit]
Modification

Cystic Fibrosis is a genetic inherited disease that causes an impotence in the functioning of the external glands of secretion, which greatly affects multiple functions in the body. It significantly affects the lungs, and a lower proportion on the pancreas, liver and intestines, where it favors the accumulation of thick and sticky layer of mucus on those organs. It is one of the most common chronic pulmonary diseases in children and young people, resulting in a genetic disorder leading to death. The cause of death is mostly due to pneumonia caused by pseudo-staphylococcus aureus.

Cystic fibrosis occurs because of a mutation in the gene that encodes the membrane-mediated intracranial receptor protein (CFTR). This protein participates in the passage of chloride ion through the cell membranes, and there is a deficiency in the production of sweat, digestive juices and mucus. The disease develops in the case of both alleles. [1] More than 1,500 mutations have been described. Most of these mutations are small deletions or minute mutations. Less than 1% of them are due to mutations in chromosomal rearrangement.

Cystic fibrosis affects many organs and organs, causing the production of secretive glands of abnormal and thick substances. The main cause of the disease and death of lung disease caused the death of 95% of people infected with it, due to frequent infections caused by obstruction of the airways because of the secretion of mucus is very thick. Other members of the affected pancreas and testicular males. [2] [3] [4] ]

The symptoms of cystic fibrosis depend on the age of the individual, and the extent of the effect of the disease on multiple devices. Before treatment, the effects of cystic fibrosis include breathing, digestion, and sexual reproduction. Other symptoms persist with continued growth, onset of lung disease, and complications of impaired absorption of vitamins and nutrients by the gastrointestinal tract. In addition to the difficulty of enrichment.


Cystic fibrosis Cystic fibrosis is a cure for cystic fibrosis, and many cystic fibrosis patients die in their thirties [5] of lung failure. However, there are drugs to slow the progression of the disease and treat its symptoms.


Contents [lighter]
1 hereditary cystic fibrosis 2 diagnosis 3 complications 4 treatment 5 lung transplantation 6 gene cystic fibrosis 7 sources
Hereditary cystic fibrosis [edit] Cystic fibrosis is a non-sexual recessive characteristic. The disease is the result of mutations in the gene known as CFTR and located on chromosome 7. The most common mutation in the world in cystic fibrosis patients is the F508 delta. Today there are more than 600 known mutation in this gene. [6]

Diagnosis People with cystic fibrosis lose their weight, suffer from persistent cough, and chest blisters. Nearly 10% of newborns, who have the disease with an obstruction known as epilepsy, require urgent surgery. Doctors can diagnose the disease by testing sweat, which tests excess salt in sweat, because of the high level of sodium chloride (NaCl) in the sweat of the infected. Prenatal disease can be diagnosed by genetic testing, or early childhood through a sweat test. In genetic testing, mutations are found in the gene known as CFTR on chromosome 7.

Complications Cystic fibrosis patients suffer from malnutrition, because mucus blocks the pancreas canal, preventing the natural flow of digestive enzymes that break down food inside the intestine. They also block air passages in the lungs and become vulnerable to rapid infections. Poor growth in children appears to be unable to increase weight or height compared to other children of the same age. The causes of failure to grow are multifaceted, including: chronic lung inflammation, poor gastrointestinal absorption of nutrients, and increased need for thermal units due to chronic illness.

Treatment People with cystic fibrosis had previously died in their childhood. Today, the chance to survive until adulthood is 75%. Modern therapies aim to make the air passages in the lungs as clean as possible using physiotherapy. Preventive measures can be taken against chest compressions, or treatment, to reduce damage to the lung. Since the 1980s, it has been possible to cultivate the heart and lungs of some adult children and young adults whose lungs are severely damaged.

Lung transplantation Lung transplantation often becomes necessary for people with cystic fibrosis when the lung is functioning and the ability to exercise is reduced. Although one lung can be transplanted into other diseases, the lungs must be changed in people with cystic fibrosis Because the other lung may contain bacteria that can hurt the lung transplant.

The gene responsible for cystic fibrosis is a CFTR gene, which is found in the long arm of chromosome number 7 and consists of 27 translated cats. A different mutation is spread throughout 2009


المادة المعروضة اعلاه هي مدخل الى المحاضرة المرفوعة بواسطة استاذ(ة) المادة . وقد تبدو لك غير متكاملة . حيث يضع استاذ المادة في بعض الاحيان فقط الجزء الاول من المحاضرة من اجل الاطلاع على ما ستقوم بتحميله لاحقا . في نظام التعليم الالكتروني نوفر هذه الخدمة لكي نبقيك على اطلاع حول محتوى الملف الذي ستقوم بتحميله .