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Cystic Fibrosis

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  CLASS ROOM TEACHING CYSTIC FIBROSIS INTRODUCTION : Cystic fibrosis is the most common fatal autosomal recessive disease among the Caucasian population .a person must inherit a defect copy of the CF gene (one from each parent ) to have CF .although CF was once considered a fatal childhood disease , approximately 37% of people living with the disease are 18 years of age or older. Researchers have documented survival past age 76 years This multisystem genetic disease is usually diagnosed in infancy or early childhood but may be diagnosed later in life . respiratory symptoms are frequently the major manifestation of CF when it is diagnosed later in life . ETIOLOGY : Genetic predisposition and disorder in gene mutation . ANATOMY & PHYSIOLOGY :- Upper respiratory tract: Nose    –   The nose is composed of an external & an internal portion. The external portion protrudes from the face & is supported by the nasal bones & cartilage. The nose serves as a passageway for air to pass to & from the lungs. It filters impurities & humidifies & warms the air as it is inhaled. Para nasal sinuses-   The Para nasal sinuses include four pairs of bony cavities that are lined with nasal mucosa & ciliated pseudo stratified columnar epithelium. Turbinate bones    –   The turbinate bones, or conches, are adapted by shape &  position to increase the mucous membrane surface of the nasal passages & to obstruct the air flowing through them slightly. Pharynx, tonsils, & adenoids    –   The pharynx or throat is a tube like structure that connect the nasal & oral cavities to the larynx..  Larynx    –   The larynx or voice organ is a cartilaginous epithelium lined structure. Lower respiratory tract: Lungs    –    The lungs are  paired elastic structures enclose in the thoracic cage, which is an airtight chamber with distensible walls ventilation requires movement of the walls of the thoracic cage & of its floor, the diaphragm. There are two lungs one lying on each side of the midline of the thoracic cavity they are cone shaped & have an apex, base, costal surface & midline surface. Apex :- This is rounded & rises into the root of the neck about 25 mm above the level of the middle third of clavicle. It lies close to the first rib & the blood vessels & nerve in the root of the neck Base :- This is concave & semi lunar in shape & rice on the thoracic surface on the diaphragm. Costal surface :- The surface is convex & lies against the costal cartilages, the ribs & the intercostals muscles.  Medial surface :- This surface is concave & has a roughly triangular shaped area, called hilum at the level of the 5 th , 6 th  & 7 th  thoracic vertebrae. Associated structure of lungs :- The area between the lungs in the mediastinum. It is also occupied by the heart, great vessels, trachea, right & left bronchi esophagus, lymph nodes, lymph vessels & nerve. The right lungs is divided into three distinct lobes superior, meddle & inferior. The left lung is smaller because the heart occupies space left of the midline. If is divided into two lobes superior & inferior. Functions of the respiratory system    –    the function of respiratory system are given below:    Oxygen transport  –   oxygen is supplied to, & carbon dioxide is removed from, cells by way of the circulation blood. Cells are in close contact with capillaries, whose thin walls permit easy passage or exchange of oxygen & carbon dioxide.    Respiration  –   after these tissue capillary exchanges, blood enters the systemic veins & travels to the pulmonary circulation.    Ventilation  –   during inspiration, air flows from the environment into the trachea, bronchioles, & alveoli. PATHOPHYSIOLOGY : ↓  Cystic fibrosis is caused by mutations in the cystic fibrosis transmembrane conductance regulator protein , which is a chloride channel found in all exocrine tissues. ↓  Chloride transport problems lead to thick , viscous secretions in the lungs , pancreas , liver, intestine ,and reproductive tract as well as increased salt content in sweat gland secretions .  CLINICAL MANIFESTATIONS : The pulmonary manifestations of CF includes  ; 1.   Productive cough 2.   Wheezing 3.   Hyperinflation of the lung fields on chest x- ray , 4.   Chronic respiratory inflammation and infection are caused by impaired mucus clearance, 5.   Colonization of the airways with pathogenic bacteria usually occurs early in life ,Staphylococcus Aureus and H. Influenza are common organisms during early childhood ,as the disease progresses , Pseudomonas Aeruginosa is ultimately isolated from the sputum of most patients . 6.   Upper respiratory manifestations of the disease include sinusitis and nasal polyps , Non-pulmonary clinical manifestations include ; Gastrointestinal problems are ;    Pancreatic insufficiency    Recurrent abdominal pain    Biliary cirrhosis    Vitamin deficiencies    Recurrent pancreatitis    Weight loss Other ; CF related diabetes ,    Genitourinary problems like male and female infertility,    Clubbing of the digits ( fingers $ toes ). ASSESSMENT AND DIAGNOSTIC FINDING : The diagnosis of is suspected in patients with typical clinical features of CF ;once other diseases have been excluded . Elevated sweat chloride concentration , repeated sweat chloride values of greater than 60meq/l distinguish most people with CF from those with other obstructive diseases . The only acceptable procedure for sweat testing is the quantitative Pilocarpine Iontophoresis sweat test .it should be performed in a laboratory that frequently does this test .
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