Resp - Conditions Flashcards
CF - Genetics
Autosomal Recessive
Mutation in CFTR gene
Chromosome 7
Most common mutation: F508del
CF - Complications
- Bronchiectasis
- Pancreatic Insufficiency
- Diabetes
- Malnutrition +/- supplemental feeding
- CF Liver Disease
- Osteopenia/Osteoporosis
- Men: Infertile, Women: Subfertile
- Constipation/Risk of obstruction
- Stones (biliary and renal)
- Pneumothoraces
CF - Diagnosis
- Screened for in Neonatal Heel Prick Test (Guthrie Test)
- Chloride sweat test
- Genetic testing
CF - Management
Medical:
- Mucolytics (DNAase, hypertonic saline nebs)
- Prophylactic Abx (Azithromycin, neb. cholemycin)
- Recurrent prolonged courses IV abx (may need Portacath)
- Pancreatic enzymes (Creon) and Fat-Soluble Vitamins
- Insulin (if diabetes)
- CFTR modulators (Ivacaftor) if amenable mutation present
MDT:
- Chest physio for mucous clearance
- Nutritional Support
- Psychological Support
CF - Microbiology
- S. Aureus colonisation most common in childhood
- Pseudomonas common in adulthood
- Burkholderia Cepacia = poor prognosis and contraindicates transplant
SCLC - Paraneoplastic Phenomena
- SIADH
- Ectopic ACTH secretion
- LEMS (VGCC-autoantbodies)
- Dermatomyositis
- Neurological syndromes (Anti-Hu)
NSCLC - Paraneoplastic Phenomena
- PTHrP secretion (Squamous cell)
- HPOA (Squamous + Adeno)
- Carcinoid (BP-NETs)
PFT Thresholds for Lung Surgery
ppoFEV1 and ppoTLCO >40% predicted (= low risk)
Exercise testing: walk >400m
old guideline:
- lobectomy = FEV1>1.5L
- pneumonectomy = FEV1>2L
- CP exercise testing =
VO2 max >15ml/kg/minonn
Basal fibrosis - DDx
I-SHARD
- IPF
- Systemic Sclerosis
- Hypersensitivity Pneumonitis (EAA)
- Asbestosis
- RA
- Drugs (amiodarone, MTX, nitrofurantoin, bleomycin, busulphan)
Apical Fibrosis - DDx
CHARTS
- Coal Miner’s Lung
- Histiocytosis
- Ankylosing Spondylitis, ABPA
- Radiation
- TB
- Sarcoidosis, Silicosis
