CardioResp Pathologies Flashcards
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
What is the first stage of pneumonia?
- Congestion
- First 24 hrs
- Vascular engorgement
- Intra-alveolar fluid
- Numerous bacteria
- Lung is heavy, boggy and red
What is the second stage of pneumonia?
Red Hepatization
- 2-3 days
- Exudation
- red blood cells , leukocytes and fibrin filling the alveolar spaces
- affected are is red and firm and airless
- Liver like consistency
What is Third stage of Pneumonia?
Grey hepatization
- 4-6 days
- Progressive disintegration of red blood cells and the persistence of fibrin exudate
What is the forth stage of Pneumonia ?
- Resolution
- > 6 days
- Exudate = progressive digestion
- resulting in debris that is later reabsorbed , ingested by macrophages or coughed up
Give the causes of Pneumonia
- Bacteria
- Fungi
- Varis
- Paracites
- Chemical
- Aspiration
- Inhalation
What does CAP Pneumonia stand for?
Community Acquired Pneumonia
what does HCAP Pneumonia stand for?
Health care acquired Pneumonia
What does HAP Pneumonia stand for?
Hospital acquired pneumonia
What does VAP Pneumonia stand for?
Ventilator acquired pneumonia
Give the common symptoms of pneumonia
- Fever
- Malaise
- Muscle ache/fatigue
- Coughing
- Tactile fremitus on palpation
- Dyspnoea
- Pleuritic or chest pain
- Loss of appetite
- Rapid heartbeat
Give the less common symptoms of Pneumonia
- Coughing up blood
- Fatigue
- Nausea/vomiting
- Diarrhoea
- Wheezing
- Confusion
What complications can arise from Pneumonia?
- Lung abscess
- Pleural effusions
- Empyema - Infection/pus in between the neural cavity
- Septic shock