CardioResp Pathologies Flashcards
(91 cards)
What is Cystic Fibrosis?
- Inherited autosomal recessive disease -defect on chromosome 7.
- Impacting protein CFTR responsible for Ion Transport
- Affecting several organs
- duct obstruction with mucus – impairment in the transport of chloride ions + levels of sodium and water in the cell - leading to inflammation and tissue scaring.
What does CFTP Stand for?
Cystic Fibrosis Transmembrane Regulating Protein
What happens in CFTR defect related respiratory disease?
- Concentrated fluid in the lungs
- Viscous secretions in airways
- Mucus not cleared - predisposes patient to disease and infection
What happens in CFTR defect related Pancreatic insufficiency?
- Abnormal ion transport leads to dehydration of pancreatic secretions
- Stagnation in pancreatic duct
What are some complications of CFTR Defect?
- High sodium sweat- Prevention of sodium absorption
- Biliary disease
reduction in water movement results in concentrated bile which can damage walls of lumen
-Infertility
Absence of Vas Deferens
-Cirrhosis of the Liver
Abnormality in ion transport - Gastrointestinal Disease
Intraluminal water deficiency - all of which related to a lack of Na +, Cl- or water diffusion problem
Give a Brief overview on the Prevalence of CF-
- 1 in 15 Caucasians carries the gene
- 2 carriers = 1 in 4 of affected baby and 1 in 2 of a carrier
- Affecting more than 10,400 people
- 1 in 2,500 babies born with CF
- Claiming 2 lives a week
Name the 3 ways in which CF is diagnosed
- Genetic testing
- Heal Prick Test - Babies leading to early diagnosis
- Sweat test - higher concentrations of chloride in sweat
Give the Symptoms of CF at birth-
- 10% babies = bowel obstruction - Meconium lleus
- Meconium - thick black substance
- Surgery often needed to remove this
- Jaundice
Give the Symptoms of the lungs in CF
- Persistent Cough
- Coughing fits
- Inflammation
- SOB
- SOB after exercise
- Chest and Lung infections
- Cross Infection
- Impaired diaphragm due to enlarged liver
Give the Symptoms of Digestive System in CF
- Large smelly stools
- Malnutrition/ poor weight/stunted growth
Give the general symptoms of CF
- Diabetes
- Sinusitis
- Arthritis
- Infertility
- Liver failure
- Urinary Incontinence
- Muscle weakness
- Kyphosis of the Spine
- Delayed Puberty
State the Medication used in CF treatment
- Bronchodilators
- Hypertonic saline nebs
- Antibiotics
- Steroids
- Vaccinations
- Creon
- DNAse
State the management strategies for CF
- Dietary advice
- Education
- Exercise
- Lung Transplant
- Psychological support
Give the ways in which Physio is used in the treatment of Cf
- PD- Postural Drainage
- Percussion,vibs/shakes
- ACBT-Active cycle of breathing techniques
- AD- Assisted Drainage
- Adjuncts
- Mobilisation /Exercise
- Suction - ITU
Outline the Prognosis for CF
- No cure or Prevention
- 1/2 will live past 40 - some to 47
- Lung complications are normally the cause of death
- Quality of life depends on specific protein mutation.
What is Bronchiectasis?
- Permanent abnormal dilation in one or more of the lungs Bronchi
- Extra mucus in airways
- Prone to infection
- Similar symptoms to COPD but does not always show as airflow obstruction.
Give a brief overview of the Pathogenesis of Bronchiectasis-
1 - Impaired mucocilliary clearance = accumulation of secretions
2- leading to infection by bacteria
3-leading to increased mucus production , impaired cilliary performance and inflammatory response
4-= tissue damage
5-leading to dilated Bronchi , loss of ciliated epithelium and impaired mucocilliary clearance
VISCIOUS CYCLE
What causes Bronchiectasis?
- Idiopathic
- Infection
- CF
- Immunodeficiency
- Cilliary dysfunction
- Inflammation
- Aspiration/obstruction
- ABPA
Give the Clinical features of Bronchiectasis
- Cough
- Chronic sputum production
- 75% dysponea and wheeze
- 50% chest pain
- 1/3 = chronic sinusitis and nasal polyps
- recurrent exacerbations are common
- 50% = haemoptysis
Give the ways in which Bronchiectasis is diagnosed
- chest xray
- HRCT
- Blood and Sputum
- PFTs
State the 3 types of Bronchiectasis
- Saccular - large bronchi = large and balloon-like
- Cylindrical- medium sized bronchi - symmetrically dilated
- Varicose - constrictions and dilations deform the bronchi
State the management strategies for Bronchiectasis
- Physio
- Antibiotics
- Bronchodilators
- Steriods
- Nasal spray
- Vaccinations
- Surgery
What is the definition for pneumonia?
- Inflammatory condition of the lung leading to abnormal alveolar filling with consolidation and exudation
Give a brief overview of the pathology behind Pneumonia
- Infection ,chemical or aspiration irritant.
- Acute inflammation results in the migration of neutrophils out of capillaries and into airspaces
- these cells phagocytose and release antimicrobial enzymes and inhibitors
- leading to more inflammation and subsequently more oedema