PACES Station 1 Flashcards
Causes of Cirrhosis
Alcohol
Viral: HepB/C
Autoimmune: PBC, PSC, Autoimmune hepatitis
Metabolic: NASH, Haemochromatosis, alpha1antitrypsin, wilsons, CF
Drugs: methotrexate, isoniazid, amiodarone, phenytoin
Features of alcohol misuse
Cachexia, tremor, parotid enlargement, dupuytrens, cerebellar syndrome, peripheral neuropathy, myopathy
Classification of Hepatic Encephalopathy
Grade 1: Insomnia, reversal of sleep
Grade 2: Lethargy, disorientation
Grade 3: Confusion, somnolence
Grade 4: Coma
Childs-Pugh Cirrhosis Severity and Prognosis
Bilirubin, ascites, encephalopathy, PT, albumin
Grade A 5-6: 90% 5 yr survival
Grade B 7-9: 80% 5 yr survival
Grade C >10: 33% 1 yr mortality
Strategy of Management of Cirrhosis
Treat underlying cause to stop or slow disease
Prevent superimposed liver damage
Prevent complications: surveillance, 6 monthly US and alphafetoprotein, endoscopy,
Transplant
Causes of Ascites
Cirrhosis Malignancy Heart Failure TB Pancreatitis
SAAG
Serum albumin-ascites gradient
>11g/L suggests transudate
<11g/L suggests exudate
Pathophysiology of ascites and oedema in cirrhosis
- Disruption of portal flow in liver causing fluid to accumulate in peritoneum
- Vasodilation of splanchnic circulation
Hepatorenal Syndrome
Systemic vasodilation causes progressive decrease in effective circulation volume leads to RAS activation which reduces renal blood flow
Causes of Hepatomegaly
Malignancy RHF Infection: viral hepatitis, toxoplasma, hydatid cyst Infiltration: amyloid, sarcoid Vascular: budd-chiari, sickle cell Polycystic liver
Benign Liver Tumours
Cavernous haemangiomas (women of child bearing age)
Hepatic adenoma
Focal nodular hyperplasia
Causes of Splenomegaly
Portal HTN Myelofibrosis Haematological malignancy - CML, lymphoma Infection Congestion Splenic vein thrombosis
Cytogenetics of CML
Philadelphia translocation chr 9:22
bcr-abl oncogene
tyrosine kinase activity
Causes of hyposplenism
Splenic infarction - vasculitic, sickle Splenic artery thrombosis Infiltrative condition - amyloid, sarcoid Coeliac Autoimmune disease
Haemochromatosis - genetics
Autosomal recessive
HFE gene on chr 6
Leads to raised transferrin saturations >50%
Complications of haemochromatosis
Liver failure HCC Diabetes Skin pigmentation Arthropathy Dilated or restrictive cardiomyopathy
PBC management
ADEK supplements Treat bone disease Treat high cholesterol Treat liver disease with urso Manage pruritus with cholesytramine but this inhibits absorption of urso Manage fatigue - naltrexone
How to present renal abdomen
- Current mode of RRT
- Previous modes of RRT
- Adequacy of RRT - uraemia and volume
- Complications of renal failure e.g. anaemia/bone disease
- Complications of immunosuppressive drugs
- Aetiology if possible
Problems with haemodialysis
Time Washout - removal of too much fluid, feel fatigued, cramps, low BP Bacteraemia - staph Bleeding - heparin Amyloidosis
Problems with peritoneal dialysis
Peritonitis
Diabetes - glucose in dialysate
Ultrafiltration failure - peritoneal sclerosis
Local hernia, infection, fluid leak
Manifestations of renal oseteodystrophy
Osteitis fibrosa - increase bone turnover due to secondary hyperparathyroidism, can cause bone pain and cysts (brown tumours)
Osteomalacia - decreased mineralisation of bone due to aluminium deposition
Definition of nephrotic syndrome
Proteinuria > 3g/24hr
Hypoalbumaemia <30g/L
Oedema
(thrombosis and hyperlipidaemia often seen)
Complications of nephrotic syndrome
Oedema - diuretics and salt restrict HTN - ACEi High cholesterol Thrombosis Infection - pneumovax and meningoccal vaccination
PCKD genetics
Autosomal dominant, most cases chr 16, some chr 4
Screen in over 20s with relative
Extra renal manifestations of PCKD
Cerebral aneurysms
Liver cysts
Splenic and pancreatic cysts
Mitral valve prolapse and aortic regurgitation
von-Hippel Lindau syndrome
Autosomal dominant condition on chr 3
Cerebellar, retinal and spinal haemangioblastomas
Cysts on liver, kidney, spleen
Increased risk of RCC (bilateral) and phaeochromocytoma
Clinical Features of Interstitial Lung Disease
Clubbing Features of rheumatoid disease Reduced expansion and TVF End inspiratory crackles Pulmonary HTN: raised JVP, parasternal heave, peripheral oedema
Causes of Interstitial Lung Disease
IPF/CFA Rheumatological: RA, SS, SLE, AS, Sj Eosinophilic: ABPA Inhaled: EAA, silicosis, asbestosis Drugs: amiodarone, nitrofurantoin, methotrexate, bleomycin Vasculitis: wegeners, C-S, Goodpastures Sarcoid Radiation
Respiratory Causes of Clubbing
Interstitial Lung Disease Ca Lung Mesothelioma Cystic fibrosis Bronchiectasis Lung abscess/empyema TB
Investigations for ILD
Bloods: FBC, ESR, Ig, ANA, ENA, ANCA, antiGBM, CK, RhF, Serum ACE
ABG - T1RF
CXR - bilateral reticulonodular infiltrates
PFTs - restrictive pattern + reduced gas transfer
HRCT
MRI
BAL/Biopsy
Apical Pulmonary Fibrosis
Inhaled agents: EAA, berylliosis, silicosis Radiation Sarcoidosis ABPA Ank SPond
Basal Pulmonary Fibrosis
IPF/CFA
Most rheum disease/CTD
Drugs
Complications of Pulmonary Fibrosis
Rest Failure Chest infection Pulmonary HTN Cor pulmonale Carcinoma of lung
Management of ILD
General measures: stop smoking, remove exposures, treat infection promptly, education
Immunosuppressive therapy - trial of steroids
Surgery - single or double lung transplant option
Clinical Features of COPD
Audible wheeze Nicotine staining Prolonged expiratory phase Tracheal tug Accessory muscles of Rs Hyper resonant Quiet breath sounds NOT clubbing
Definition of COPD
Umbrella term for obstructive airway disease including
- Chronic Bronchitis - cough for 3 months during 2 consecutive years leading to inflammation
- Emphysema - histological diagnosis of abnormal and permanently enlarged small airspaces leading to loss of elastic recoil
Spirometry in COPD
FEV1<80% predicted + FEV1/FVC: < 70% mild 30-50 moderate <30 severe Plus do reversibility testing
MRC Dyspnoea scale
- Only breathless during strenuous exercise
- SOB when hurrying or up slight incline
- Walks slower than contemporaries on flat
- Stops every 100m
- Too breathless to leave house
Management of Acute IECOPD
Controlled O2 Nebuliser bronchodilators Antibiotics Steroids Consider IV aminophyline NIV in T2RF with pH<7.35
Most common pathogens in IECOPD
Streptococcus pneumonia
Haemophilus influenzae
Moraxella catarrhalis
LTOT Criteria for COPD
Stopped smoking paO2 <7.3 when well OR 7.3-8 plus - secondary polycythaemia - pulmonary HTN - peripheral oedema - nocturnal hypoxaemia (sats <90% for >30% of time )
ABG taken when well on 2 occasions at least 3 weeks apart
Alpha 1 antitrypsin deficiency
Autosomal dominant chr 14
encodes alpha 1 antitrypsin which is enzyme that inhibits neutrophil elastase
Multiple phenotypes M (medium), S (slow), Z (very slow)
Management of COPD
General measures: stop smoking, immunise
Stepwise bronchodilators/antiinflammatory
Mucolytics
LTOT
Diuretics for cor pulmonale
Pulmonary rehab - MDT programme - education, nutrition, psychological support, physical training
Role of surgery in COPD
Bullectomy, lung volume reduction surgery, transplantation
Stepwise therapies in COPD
- SABA
- FEV1>50% add LAMA or LABA, FEV1<50% add LABA+ICS or LAMA
- Add in the other one
Always educate and use spacer
Clinical Features of Pleural Effusion
Reduced chest expansion,
Stony dull percussion note
Reduced TVF
Diminished breath sounds
Causes of Pleural Effusion
Exudate >30g/L:
Neoplasm: Ca lung, mesothelioma, lymphoma
CTD: Ra, SLE
Infection: pneumonia, TB
Sub-diaphragmatic: pancreatitis, abscess
Drugs: methotrexate, nitrofurantoin, bromocriptine
Other: asbestosis, sarcoid, dresslers, trauma, yellow nail syndrome
Transudate <30g/L CCF Constrictive pericarditis Low albumin Nephrotic syndrome Cirrhosis Dialysis Uraemia Hypothyroidism
What volume of pleural fluid can be detected on CXR?
180ml would show was loss of costophrenic angle
Pathophysiology of a Pleural Effusion
Inflammatory causes lead to altered permeability of membrane
Reduced oncotic pressure in low albumin
Increased hydrostatic pressure in CCF
Reduced lymphatic drainage in malignancy
Causes of low glucose in pleural fluid
NB also causes of low pH (assuming patient has normal ABG)
Malignancy Empyema TB RA Oesophageal rupture SLE
What is the significant of low pH in context of malignant pleural effusion (<7.3)?
More extensive pleural involvement
Higher yield on cytology
Decreased success of pleurodesis
Shorter life expectancy
Complications of pleural fluid drainage
Pneumothorax
Haemothorax
Hypovolaemia
Unilateral pulmonary oedema (reexpansion)
Indications for pleurodesis
Recurrent malignant effusion
Recurrent pneumothoraces
Clinical Features of Bronchiectasis
Productive cough (sputum pot) Inspiratory coarse creps which alter after coughing Clubbing Sometimes wheeze Often no features of pulmonary HTN
Causes of Bronchiectasis
Childhood resp infection: pertussis, measles, TB
Bronchial obstruction: foreign body, chronic aspiration, endobronchial tumour
Long standing fibrosis
Mucociliary clearance problem: CF, kartageners
Immunodeficiency: hypogammaglobulinaemia, AIDS
ABPA
Autoimmune disease: RA, IBD, Sjogrens
Congenital abnormality
Idiopathic
Pathophysiology of Bronchiectasis
Abnormal bronchial wall dilatation, destruction and transmural inflammation caused by destruction of muscular and elastic components of bronchial wall leading to inflammation, oedema and scarring
Impaired clearance of secretions leads to colonisation and infection causing further damage
Complications of Bronchiectasis
Pneumonia Pneumothorax Empyema Collapse Metastatic cerebral abscess Respiration failure Pulmonary HTN Amyloidosis
Anatomical distribution of Bronchiectasis and aetiology
Infection - lower lobes
ABPA & TB - upper lobes
Obstruction - right middle lobe
Diagnosis of Bronchiectasis
HRCT - signet ring sign - bronchial diameter greater than adjacent vessel
Spirometry - may be normal, may have obstructive pattern, may be restrictive in advanced disease
Most common pathogens in bronchiectasis
Staphylococcus aureus Haemophilus influenzae Pseudomonas Streptococcus pneumoniae Klebsiella Aspergillus
Cystic Fibrosis
Autosomal recessive, defect on chr7
CFTR Chloride channel abnormality leads to thick mucus and impaired ciliary clearance. Also causes meconium ileum, gallstones, bile salt malabsorption, arthropathy and fertility problems in men
ABPA
Allergic bronchopulmonary aspergillosis
Hypersensitivity to aspergillus in patients with asthma or CF. Excess mucus production and ciliary clearance. Immune complex deposition incites type III reaction to damage bronchial wall leading to bronchiectasis
General management of bronchiectasis
General measures: smoking cessation, immunisations, nutrition, MDT, LTOT if needed
Antibiotics including nebuliser tobramycin in CF
Postural drainage and chest physio - flutter devices
Bronchodilators
Steroids
Surgical resection if localised, transplant in CF
Clinical Features of Ca Lung
Cachectic Productive cough Clubbing Palpable LNs Dull percussion, reduced TVF (may have effusion) Bronchial breathing above dullness Pan coast tumour - reduced sensation C8-T1, wasting of muscles, ipsilateral horners (ptosis, miosis, anhydrosis) Deviated trachea Radiation burns
Classification of bronchial Ca
Small cell - 20%, smokers Non small cell including: - adenocarcinoma: non-smokers, peripheral - bronchoalveolar: develops at multisite - squamous: cavitation, smokers - large cell: undifferentiated
Risk factors for Lung Ca
Smoking ILD Radon Asbestos Arsenic Coal tar Radiation
TNM classification of Lung Ca
Tis - in situ T1 <3cm T2 >3cm, invasion of pleura/main bronchus T3 extra pulmonary extension T4 Extensive extra pulmonary extension
N1 ipsilateral intrapulmonary nodule
N2 ipsilateral mediastinal LN
N3 contralateral nodule or scalene/supraclavicular nodule
M0 - none, M1 mets
Paraneoplastic Lung Ca in SCLC
Endocrine:
-SIADH ,Cushings, Hypercalcaemia, hypoglycaemia
Neurological:
- Lambert-Eaton myasthenia - presynaptic Ca channel Abs
- Cerebellar degeneration - antiYo
- Sensory neuropathy - anti Hu
MSK
- dermatomyositis
- clubbing
- osteoarthropathy
Skin
- acanthuses nigricans
- ichthyosis
Pancoast Tumour
Apical lung tumour erodes into lower part of brachial plexus (C8-T1) and cervical sympathetics
Ipsilateral wasting of small muscles of hand with dermatomal sensory loss
Ipsilateral horners
General Management of Lung Ca
MDT!!! surgery, chemotherapy, radiotherapy Analgesia Anxiolytics Stents in palliative Pleurodesis Psychological support
Clinical Features Lobectomy/Pneumonectomy
Deviated trachea Thoracotomy scar Reduced chest expansion Dull percussion Absent breath sounds/bronchial breathing above Clubbing - indicates bronchiectasis or malignancy or ILD Palpable LNs - malignancy Deformity of chest wall
Indications for Lung Lobectomy
Bronchiectasis with uncontrolled symptoms Malignancy Solitary pulmonary nodule CF TB
Indications for Pneumonectomy
Bronchiectasis
Malignancy
TB
Clinical Features of Old TB
Apical fibrosis
Scars from thoracoplasty/pneumonectomy/lobectomy
Phrenic nerve crush - left supraclavicular scar and raised hemidiaphragm
Patients at risk of TB
Immigrant population Immunocompromised Elderly Alcoholics Malnutrition Homeless
Complications of TB
Pneumothorax Pleural effusion Empyema Collapse Tubulointerstitial nephritis ARDS TB meningitis Miliary TB Fibrosis Bronchiectasis
Interferon Gamma Testing in TB
Allows to distinguish between active TB and someone who has previously had BCG and thus positive tuberculin skin test
Indications for chemoprophylaxis in TB
All children under 5 in close contact
Patients with previous hx of TB or radiological evidence and starting on immunosuppressive therapy
Clinical Features of Lung Consolidation
Productive cough
trachea pulled to affected side
Increased vocal resonance, coarse creps
Underlying cause: features of malignancy, pneumonia, infarction (PE)
Typical Pathogens in CAP
Streptococcus pneumoniae
Haemophilus influenzae
Staphylococcus aureus
Atypical Pathogens in CAP
Mycoplasma
Legionella
Chlaymdia
Coxiella
Pathogens in HAP
Gram negs: klebsiella, pseudomonas, e.coli
Staph
Anaerobes
Fungi
Mycoplasma pneumonia
occurs in epidemics every 3-4 years children and young adults prolonged prodromal phase Neurological manifestations: meningitis, encephalitis, GBS, transverse myelitis Cardiac: myocarditis, pericarditis Rheum: arthralgia, myalgia, myositis Haem: thrombocytopenia, DIC GI: D&V, hepatitis, pancreatitis Derm: erythema nodosum, S-JS Renal: glomerulonephritis
ECG features of PE
sinus tachycardia Tall R in V1 Right ventricular strain RBBB TWI V1-V3 S1Q3T3
Secondary Causes of Pneumothorax
Resp: COPD, CF, Asthma, ILD, TB, Malignancy, Sarcoid
CTD: Marfans, Ehlers-Danlos
Lung cysts: Neurofibromas, tuberous sclerosis
Iatrogenic: pleural aspiration, biopsy, pacemaker insertion, CPR
Trauma
Clinical Signs of Tension Pneumothorax
Tracheal deviation, away
Mediastinal shift - displaced apex
Raised JVP
Management of Spontaneous Primary Pneumothorax
If <2cm on CXR repeat in 7-10days, return if develop breathlessness
More than >2cm aspirate and if unsuccessful consider chest drain
Role of chest drain suction
Added after 48 hrs if persistent air leak or failure of pneumothorax to re-expand
Management of Secondary Pneumothoraces
Admit all
If <1cm and asymptomatic can observe
If <50, <2cm and asymptomatic can aspirate
All others require drain
Main predictor of re-expansion pulmonary oedema
greater time the lung has been collapsed the greater the risk
Clinical Features of Rheumatoid Lung
Inspiratory clicks
Symmetrical deforming arthropathy of hands
Clubbing
Steroid purpura
Fine end inspiratory crackles at bases extending to mid zone, do not alter with coughing
May be some bronchiectatic changes also
Respiratory Manifestations of Rheumatoid Arthritis
Pleural effusions Basal fibrosis: RA, gold, methotrexate Pneumonitis Pulmonary nodules Bronchiectasis Caplans syndrome
Pulmonary nodules in RA
histologically similar to subcutaneous nodules
In pleura or lung parenchyma
often do not compromise function but can lead to bronchopulmonary fistula or infection
Features of RA pleural effusion
Exudate
High protein and LDH
Low glucose and complement
High rheumatoid factor
Caplans syndrome
pneumoconiosis in patient with RA associated with coal miners
Causes of Lung Collapse
Obstructive
-malignancy, extrinsic compression, TB, mucus plus, foreign body
Non-obstructive
- adhesive atelectasis: PE, radiation, ARDS, pneumonia
- Relaxation atelectasis: pleural effusion, pneumothorax
- Rounded atelectasis: asbestos
Clinical Features of SVC obstruction
Upper limb and face oedema
Plethoric
Cyanosed
Conjunctival suffusion
Venous angiomata on undersurface of tongue
Prominent and dilated veins over upper limbs, neck and anterior chest
Firm raised venous pressure
Look for signs of radiation marks/effusions to suggest underlying malignancy
Causes of stridor
Inspiratory (mostly): Acute epiglottis Croup Vocal chord dysfunction Foreign body Trauma Neck surgery
Expiratory: Foreign body Bronchial Ca Stricture secondary to TB, sarcoid Lymphadenopathy
