PACES 3 Flashcards

1
Q

Purpose of NIHSS and MRS score

A

Both are disability scores to see whether pt have symptoms severe enough to benefit from intervention

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2
Q

DD of cerebellar syndrome

A

Ipsilateral
- stroke
- SOL
- demyelination (MS)

Bilateral
- alcohol cerebellar syndrome
- drug (antiepileptics, phenytoin)
- genetic (spinocerebellar ataxia, friedrech ataxia)
- MSA
- paraneoplastic
- infectious (VZV Cerebellitis)

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3
Q

DD Parkinsonism

A

Normal dopamine
- drug induced
- vascular Parkinsonism
- metabolic like alcohol
- Wilson
- NPH

Dopamine reduced
- idiopathic PD
- lewy body dementia
- MSA
- PSP

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4
Q

Name 5 MS mimics

A

Vasculitis - SLE, sjogren, behcet, sarcoidosis
Vascular - antiphospholipid, stroke, Fabry
Mitochondrial - MELAS
Infection - Lyme, HIV encephalitis, syphillis, PML
Metabolic - b12 deficiency

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5
Q

MRI finding in MS

A

Periventricular white matter lesions

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6
Q

Investigation for MS

A

Visual evoked potential
CSF unmatched oligoclonal band in serum VS CSF
MRI spine and brain showing new T2 lesion 30 days after clinical onset or a second clinical episode after a CIS (clinical isolated syndrome)
Bloods to exclude MS mimic - ESR, ANA, ANCA, dsDNA, ENA, antiphospholipid for AI cause + B12 level, treponemal serology etc

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7
Q

MS vs Neuromyelitis optica 3 differences

A

NMO is more severe
NMO has more extensive spinal cord lesion normally extending over 3 vertebral segments
NMO has anti aquaporin 4 antibodies

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8
Q

Rating scale for disability in MS and significance

A

Expanded disability status scale (EDSS)
Help guide disease modifying therapy use e.g. max EDSS 6.5 for initiating beta interferon or glatiramer acetate

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9
Q

Criteria for starting DMT in MS

A

Relapsing remitting MS with active disease defined by 2 significant relapses in last 2 years, 1 disabling relapse in last year or active MRI scan with new lesion that has developed over the last year

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10
Q

Common reasons for stopping Beta interferon in MS?

A

Flu like sx, autoimmune hepatitis, depression, treatment failure due to development of neutralising antibodies

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11
Q

Miller Fischer syndrome cause, 3 features and diagnosis

A

Autoimmune, post infectious
Ophthalmoplegia, ataxia, loss of lower limb reflexes
Anti GQ1b ganglioside antibody

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12
Q

Inflammatory myelitis

Predominantly sensory or motor symptoms?

A

Sensory

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13
Q

Cord compression

Predominantly sensory or motor symptoms?

A

Motor

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14
Q

Loss of pain and temp sensation spinal cord causes?

A

Syringomyelia
Anterior cord infarction

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15
Q

Loss of fine touch and proprioception sensation spinal cord causes?

A

B12 deficiency
HIV associated vacuolar myelopathy
Neurosyphillis
Friedreich ataxia

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16
Q

Marked spasticity out of keeping with degree of weakness causes

A

MND
Hereditary spastic paraparesis
Tropical spastic paraparesis

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17
Q

Early bladder involvement in predominantly motor syndrome

A

Compressive myelopathy

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18
Q

Dysarthria DD

A

Bulbar (LMN) - MG, MND, GBS, myopathy

Pseudobulbar (UMN) - brainstem stroke, MND, MS

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19
Q

Mixed UMN and LMN DD

A

Dual pathology - cervical spondylopathy + peripheral neuropathy - check glove and stocking neuropathy and finger prick signs

Cervical spondylomyelopathy from cervical spine degeneration - has sensory level, UMN below lesion and LMN at level of lesion

MND

Syringomyelia - cape like distribution pain and temp loss, normal sensation in lower limb. Wasting and weakness hand muscle. Brisk lower limb reflexes and extensor plantar.

Cauda equina

Subacute combined degeneration of cord - loss of proprioception (sensory ataxia) hence Romberg test positive, brisk knee jerk, absent ankle jerk. Extensor plantar. Mainly painful sensory neuropathy and lower limb weakness. Check for signs of pernicious anaemia

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20
Q

DD for absent ankle jerk and extensor plantar

A

MND
Fredreich ataxia
Subacute combined degeneration of cord
Syringomyelia
Tumour involving conus and cauda
Neurosyphillis

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21
Q

Name 10 causes of splenomegaly

A

Infective - malaria, EBV, CMV, leishmaniasis, salmonella
Infiltrative - SLE, amyloidosis, sarcoidosis, hereditary spherocytosis
Inflammation
Haematological - CLL, CML, myelofibrosis, lymphoma
Rheumatological - adult onset stills disease, felty syndrome
Liver disease

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22
Q

Causes of ascites

A

Cirrhosis
Cancer
CCF

CKD
Pancreatitis
Chylous ascites
Hypoalbuminaemia
Peritoneal dialysis associated ascites
IVC obstruction
Pericarditis
Budd chiari
Portal vein thrombus
Advanced hypothyroidism

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23
Q

Liver transplant criteria

A

UKMELD SCORE
KINGS COLLEGE CRITERIA - paracetamol overdose

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24
Q

Immunosuppressant clinical signs

A

Tacrolimus - fine tremor, hair loss
Ciclosporin - gum hypertrophy, renal toxicity, hypertension, hirsutism
Steroid - thin skin, Cushingnoid, bruises, cataract, infection, diabetes, hypertension

Skin cancer

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25
Q

Liver transplant complications (name 5)

A
  1. Infection
  2. Graft failure
  3. Graft rejection
  4. Graft leak
  5. Haemorrhage
  6. Disease relapse
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26
Q

Indication for liver transplant (name 5)

A

Refractory ascites despite TIPSS
HCC
Acute liver failure
HRS
intractable pruritus
Recurrent cholangitis
Polycystic liver disease

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27
Q

Contraindication for liver transplant

A
  1. Ongoing alcohol use
  2. Metastatic liver cancer
  3. Age >65
  4. IVDU
  5. Significant psychiatric problem
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28
Q

Name 5 most common causes of ESRD

A

Diabetes
HTN
Chronic glomerulonephritis
ADPKD
Obstructive uropathy
Unexplained CKD
Chronic pyelonephritis

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29
Q

Immunosuppressant for kidney transplant name 4 classes

A

Calcineurin inhibitor - tacrolimus, ciclosporin
Antiproliferative agent - mycophenolate mofetil
mTOR inhibitor - sirolimus
Steroids

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30
Q

Complications from immunosuppressant name 6

A
  1. Cancer (SCC, kaposi sarcoma, lymphoma, cervical)
  2. Diabetes
  3. Hypertension
  4. Hyperlipidaemia,
  5. CMV infection
  6. BK virus infection
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31
Q

Causes of bronchiectasis (name 4 categories)

A

Congenital
- CF
- Kartageners

Infective
- TB, measles, diphtheria

Immune overactivity
- RA, ABPA, IBD

Immune underactivity
- Hypogammaglobinaemia
- CVID

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32
Q

Hereditary sphereocytosis inheritance and chromosome affected

A

Autosomal dominant
Chromosome 8

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33
Q

Investigation for hereditary spherocytosis

A
  1. FBC, blood smear, blood film
  2. EMA binding test or osmotic fragility test
  3. Haptoglobin, reticulocyte, coombs test
  4. Split bilirubin
  5. USS abdomen for spleen and gallstones
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34
Q

Hereditary spherocytosis complication

A
  1. Aplastic crisis
  2. Anaemia
  3. Gallstones
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35
Q

Hereditary spherocytosis management

A

Vaccination for encapsulated organism - meningococcal, pneumococcal
Folic acid
Splenectomy
Blood transfusion

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36
Q

Criteria for simultaneous kidney and liver transplant

A

ESRD + poorly controlled diabetes (usually type 1)

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37
Q

Reason for simultaneous kidney and pancreas transplant

A
  1. Prevent diabetic nephropathy
  2. Better QOL with reduced dialysis
  3. Better long term outcome than isolated kidney transplant
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38
Q

Name 5 causes of chronic pancreatitis

A

Genetic - PRSS1, SPINK1, CFTR
Autoimmune - IgG4
Alcohol
Gallstones
Trauma

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39
Q

Indications for aortic valve replacement in aortic stenosis

A
  1. Symptomatic severe AS (angina, syncope, dyspnoea)
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40
Q

Signs of severe AS clinically

A

Slow rising pulse
Symptomatic
Quiet S2
LVH
Decompensation

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41
Q

Signs of severe AS on echo

A

Valve area <1cm2
Peak gradient >60mmhg or mean gradient >40mmhg

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42
Q

DD for aortic stenosis (name 4)

A

HOCM
Aortic sclerosis
Mitral regurgitation
Post infarction VSD

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43
Q

Criteria for surgery for aortic stenosis

A

All symptomatic
Asymptomatic + LVEF <45%, LVH, VT, or sx on exertion

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44
Q

Criteria for TAVI

A

Age >75
Frail
Not fit for surgery

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45
Q

Contraindications to TAVI

A

Bad CAD

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46
Q

Complications of TAVI (name 4)

A

Pacemaker
Vascular access complication
Annular rupture
CAD if block coronary artery

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47
Q

Aortic regurgitation criteria for surgery

A

All moderate to severe symptomatic
Asymptomatic + LV dilatation, aortic root dilatation or RF >50%

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48
Q

Mitral regurgitation causes (name 5)

A

Age related degeneration
Mitral valve prolapse
Infection (IE, Rheumatic)
Papillary muscle rupture
Connective tissue disorder

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49
Q

Criteria for MV repair in mitral regurg

A

Asymptomatic + EF 30-50% + ESLV dimension >40mm

Chronic severe MR + new AF or PH

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50
Q

Mitral stenosis causes name 3

A

Acquired
- rheumatic
- degenerative calcification
- infective endocarditis

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51
Q

Signs of severe mitral stenosis

A

Malar flush
AF
HF
pHTN
Loud S1

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52
Q

Chronic severe MR + new AF or PH

A

True

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53
Q

Mitral stenosis causes (name 3)

A

Acquired:
- rheumatic
- degenerative calcification
- infective endocarditis

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54
Q

Signs of severe mitral stenosis on echo

A

Valve area <1cm2, gradient >10mmhg

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55
Q

Pulmonary stenosis causes (name 5)

A

Congenital:
- Noonan’s
- Turner’s
- William
- Tetralogy of fallot (PS, VSD, overriding aorta, RVH)
Acquired:
- Carcinoid

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56
Q

Causes of peripheral neuropathy

A

Metabolic:
- Diabetes
- Hypothyroidism
- Uraemia
- Vit B1, B6, B12 deficiency
Toxic:
- Chemotherapy
- Antibiotics
- Alcohol - sensory predominant
Inflammatory:
- CIDP
- Sarcoidosis
- RA
- HIV
- Lyme
Neoplastic:
- lung cancer
- multiple myeloma or MGUS

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57
Q

Investigation for peripheral neuropathy

A

FBC - macrocytic anaemia
u&E - for urea
LFT
Vit B12
ESR
Serum electrophoresis
Hba1c

NCS - find out if demyelinating or axonal, also find out if length dependent in nature which is more likely peripheral neuropathy

EMG

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58
Q

Charcot Marie tooth is demyelinating or axonal?

A

Type 1 = demyelinating
Type 2 = axonal

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59
Q

Management of Charcot Marie tooth

A
  1. No disease modifying therapy available
  2. Multidisciplinary team
  3. Orthotics
  4. OT to help home set up
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60
Q

Charcot Marie tooth features

A

Wasting of thenar/hypothenar eminences
Distal lower limb wasting
Peripheral sensorimotor neuropathy

61
Q

Cerebellar ataxia management

A

MDT
- physio exercise
- OT home adaptions and mobility aids

Lifestyle advice
- look at occupational risk factors
- look at meds to see if affect cerebellum or dizziness
- advice reduce alcohol

62
Q

Differential for pure motor weakness

A

MND
ALS
KENNEDY DISEASE (LMN)
Multifocal neuropathy with conduction block (LMN)
Spinal muscular atrophy (LMN)

63
Q

Investigation for MND

A

EMG - check for fasiculation, fibrillation in absence of sensory signs

If multifocal neuropathy with conduction block then will find demyelinating disease with conduction block

MRI spine

64
Q

MND features

A

Asymmetrical
Rapid
Fasiculation
Muscle wasting
Behavioural disturbance like frontal temporal dementia

65
Q

Management of MND

A

Riluzole small increase in survival
MDT
- Physio, OT
- SALT, to consider alternative feeding, assess swallowing, communication aid
Neuro
Screen weight appetite and swallowing
Screen respiratory function (early morning blood gas, FVC) as some may get resp weakness. Cough assist device
Screen cognitive function for FTD

66
Q

Name the types of myelopathy

A

Compressive:
- tumour
- disc herniation
Autoimmune or inflammatory:
- MS, NMO
- SLE
- Sarcoidosis
Infectious:
- HIV
- Varicella
- Lymes disease
- Syphillis
Nutritional:
- B12
Genetic:
- hereditary spastic paraparesis

67
Q

Dorsal column sensation?

A

Fine touch
Vibration
Proprioception

68
Q

Anterior column sensation?

A

Pain (pin prick)
Temperature

69
Q

MND subtypes

A

UMN features:
- Primary lateral sclerosis

Mixed
- ALS
- PRogressive bulbar palsy

LMN
- Progressive muscular Atrophy

70
Q

Kennedy disease (x-linked spinal bulbar muscular atrophy) features

A

Perioral fasiculation
LMN predominant
X linked, associated with androgen insensitivity
Slow progression

71
Q

Features of Cerebellar syndrome

A
  • head tremor
  • truncal ataxia
  • jumpy vision / oscillopsia
  • scanning dysarthria
  • nystagmus in all directions
72
Q

How to differentiate spinocerebellar syndrome from cerebellar syndrome?

A

Spinocerebellar syndrome has UMN signs like upgoing plantar and increased tone or reflexes

73
Q

How to differentiate pure cerebellar syndrome with alcohol associated cerebellar syndrome?

A

Alcohol related would present with some degree of peripheral neuropathy

74
Q

Name 6 types of muscular dystrophy

A

Duchenne (childhood) MD

Beckers (childhood) MD

Ocular pharyngeal MD

Myotonic MD

Facioscapulohumeral muscular dystrophy

Limb girdle muscular dystrophy (inherited) – shoulder and pelvic girdle – raised CK and EMG myopathic changes + positive genetic test

Spinal muscular atrophy – will have UMN signs

Spinal bulbar muscular atrophy (Kennedy’s disease) – perioral fasiculation + LMN disease only unlike MND

75
Q

Neurological findings for friedreich’s ataxia

A

motor, sensory, UMN, LMN and cerebellar signs

76
Q

Features of spastic paraparesis

A

increased tone, brisk reflexes, upgoing plantars, weakness and reduced sensation

77
Q

DD for spastic paraparesis/myelopathy

A

Hyperacute + backpain = compressive pathology, such as an intravertebral disc herniation

Hyperacute without back pain = vascular cause, such as a spinal stroke, could also be the cause; however, I would not expect the patient to present with such spasticity in the acute phase

Days = inflammatory (multiple sclerosis) or infective pathology (VZV)

Months = metabolic causes such as Vitamin B12 deficiency, copper deficiency other infective causes e.g. HIV. If the patient has travel history, things such as HTLV1 infection could also be considered

Years = slowly growing tumours, neurodegenerative conditions such as primary lateral sclerosis

Positive family history = hereditary spastic paraparesis

78
Q

Post polio syndrome neurological findings

A

isolated LMN signs + cramp, weakness

79
Q

Pes cavus differential

A

Long standing neuropathy e.g. charcot marie tooth, polio neuropathy, Friederich ataxia , muscular dystrophy

80
Q

Absent reflex neurological syndromes

A

LMN disease

Sensorineuropathy

81
Q

DD for LMN findings only

A

Hereditary

  • Spinal muscular atrophy
  • Hereditary motor neuropathies

Sporadic

  • MND

Immune mediated

  • CIDP
  • MND

Infectious

  • Post polio syndrome
82
Q

Name the causes of interstitial lung disease

A

Idiopathic
 Idiopathic fibrosis (most common)
 Sarcoidosis (2nd most common)
 Cryptogenic organising pneumonia
Connective tissue disorder
 SLE
 AS (apical)
 RA (upper zone)
 Dermatomyositis
 Systemic sclerosis
 Mixed CTD
Occupational
 Asbestosis
 Silicosis
Organic exposure
 Bird (most common EEA)
 Hay
 Mold
Medication
 Amiodarone
 Nitrofurantoin
 Methotrexate

83
Q

Name the signs pointing towards idiopathic pulmonary fibrosis instead of other types of pulmonary fibrosis

A

clubbing, age <45

84
Q

Name 5 investigation for suspected interstitial lung disease

A

Spirometry + transfer factor – if FVC <80% then need referral to tertiary care centre to consider antifibrotic therapy

Blood test for CTD (ANA, dsDNA, ANCA, rheumatoid factor)

ACE level if suspect sarcoidosis

Allergy testing may be performed for example avian precipitins if bird fancier’s lung suspected.

CXR, HRCT

Echo for pul HTN

Blood gas

bronchoscopy with BAL, transbronchial lung biopsy or surgical lujng biopsy if indicated

85
Q

How is diagnosis of ILD made?

A

History + spirometry + radiology

Biopsy not needed

Need ILD MDT for a decision about diagnosis and to understand the underlying aetiology

86
Q

Management of IPF?

A

Refer for consideration for lung transplant

Pulmonary rehab, supportive treatments where necessary such as oxygen.

Nintedanib or pirfenidone can be considered in patients with a forced vital capacity between 50-80% predicted to slow disease progression.

87
Q

Management of NSIP

A

Immunosuppressive treatment may be started for patients with moderate to severe disease e.g. oral or IV glucocorticoids and steroid sparing immunosuppressive agents such as azathioprine or mycophenolate mofetil although other agents may be trialled for refractory disease.

88
Q

When to use steroids for ILD?

A

If CT shows groundglass changes suggesting inflammatory component

89
Q

Management of bronchiectasis

A

Physiotherapy chest
Prophylactic abx – low dose azithromycin 2 times a week
Salbutamol inhalers if significant wheeze or breathlessness

90
Q

DD for double thoracotomy scar with normal breath sounds

A

o Bilateral apical pleurectomy
o Bullectomy
o Pulmonary TB surgical management (in older patient)

91
Q

Pulmonary DD for clubbing

A

o Interstitial lung disease
o Chronic suppurative lung disease, bronchiectasis CF, lung abscess

92
Q

DD for fine bibasal crackles

A

o CCF
o Interstitial fibrosis
o Pneumonia (but would clear with cough)

93
Q

DD for double lung transplant

A

CF (most common)
Bronchiectasis
Pulmonary hypertension
Idiopathic pulmonary fibrosis

94
Q

DD for single lung transplant

A

“Dry” lung conditions
o Pulmonary fibrosis (single lung transplant)
o COPD (single lung transplant)

95
Q

When to refer for lung transplant in ILD?

A

Usual interstitial pneumonia or fibrotic NSIP (non-specific usual interstitial pneumonia) who have no contraindications should be considered for referral regardless of their lung function.

Non-fibrotic/cellular NSIP should be referred if their forced vital capacity < 80% or their transfer factor is < 40%, or any oxygen requirement or symptomatic dyspnoea. This does not mean that these patients will be immediately listed, however they can complete a full and timely transplant assessment and be listed if shown to be rapidly deteriorating for example > 10% drop in FVC within six months

96
Q

Eligibility for lung transplant?

A

Meet 3 criteria

  1. They should have a > 50% risk death from lung disease within two years if transplant is not performed.
  2. They should have a > 80% likelihood of surviving at least 90 days post-transplant.
  3. They should have > 80% likelihood of a 5-year post-transplant
97
Q

Contraindication to lung transplant

A

o Malignancy within last 5 years
o High or low BMI
o Smoker
o Illicit drug
o Psychiatric condition that stop them from taking meds or coming to regular appointments
o Smoking or dependence

98
Q

Complications of lung transplant

A

o Hyperacute rejection (common)
o Opportunistic infection
o Bronchiolitis obliterans (chronic rejection and terminal event)
o Malignancy (post transplant lymphoproliferative disorder)

99
Q

Indications for video assisted thoracoscopy

A

o Wedge resection or segmentectomy of solidary pulmonary nodule
o Lung biopsy
o Lobectomy
o Decortication
o Bullectomy
o Tx for recurrent pneumothoraces

100
Q

Benefit of VATS

A

Small incision = less pain, less healing time, less hospital stay, less infection

101
Q

Indications for lobectomy

A

Lung ca
Aspergilloma
TB
Lung absecess

102
Q

Investigation for fitness for lobectomy

A

FEV1 >1.5L

o Full lung fuction test with transfer factor assessment
o Cardiopulmonary testing

103
Q

Investigation for fitness for pneumonectomy

A

FEV >2L

o Full lung fuction test with transfer factor assessment
o Cardiopulmonary testing

104
Q

DD for wheeze

A

o COPD
o Asthma
o Bronchiectasis
o Obliterative bronchiolitis (due to viral infection, certain pollutants or stem cell transplantation as GVHD or after lung transplant as GVHD)

105
Q

Inheritance and chromosome and gene for CF

A
  • Autosomal recessive, chromosome 7, CFTR gene
106
Q

Management of CF

A

 Dietician, OT, physio, SALT, psychologist, social workers
 Regular chest physio for postural drainage and to clear mucus
 Nebulised antimucolytics (recombinant DNAse and hypertonic saline)
 Nebulised antibiotics
 Azithromycin prophylaxis
 Creon tablets
 Nutritional supplement by PEG tube

o CFTR modulating therapies such as ivacaftor and lumacaftor/ivacaftor have resulted in a marked improvement in lung function and exacerbation frequency in eligible patients who carry specific mutations that respond to these medications

107
Q

Most common bacteria in CF in adults and children

A

pseudomona in adult
Staph aureus in children

108
Q

Bacteria in CF that will be contraindicated for lung transplantation?

A

o Burkholderia cenocepacia, mycobacterium abscessus

109
Q

Things to look out for on examination of someone with suspected pulmonary fibrosis

110
Q

Indications for pneumectomy

A

o NSCLC (PET scan if potentially radically treatable)
o Localised bronchiectasis
o malignant nodules
o Lung abscess, treated either with a lobectomy or a wedge resection
o Tuberculosis
o Lung trauma with significant damage to the lung

111
Q

Why pneumonectomy and not lobectomy

A
  1. Lung ca involving both the upper and lower lobe or that the tumour was particularly central and excision of just one lobe would not have been possible
  2. significant bronchiectasis on the left side requiring pneumonectomy
112
Q

Bronchiectasis investigation

A
  • FBC, U&E, LFT, CRP
  • HIV, immunoglobulin, pneumococcal serology
  • Aspergillus precipitins
  • CF (for all age <40)
  • Sputum analysis (general, fungal and mycobacterium cultures)
  • Spirometry
  • CXR, HRCT
  • Referral to specialist centre for nasal biopsy if suspected primary ciliary dyskinesia
113
Q

Signs to look out for in suspected lung cancer

A
  • Clubbing
  • SVC obstruction
  • Radiotherapy tattoo
  • Hoarse voice (from recurrent laryngeal nerve compression)
  • Horner’s syndrome
  • Hypercalcaemia, SIADH, LEMS
  • Pulmonary osteoarthropathy or wasting of the small muscles of the hand

Also look for recurrence - cachexia, anaemia, neck lymphadenopathy, clubbing

114
Q

Investigation for suspected lung cancer

A
  • Bronchoscopy guided biopsy
  • Endobronchial USS biopsy (to sample mediastinal lymph nodes)
  • CT guided percutaneous biopsy
  • Lymph node biopsy
  • Spirometry to see fitness for surgery
115
Q

What is light criteria for exudate?

A

pleural fluid is exudative if one of the following applies:
 pleural fluid: serum ratio is > 0.5
 pleural fluid: serum LDH ratio is > 0.6
 pleural fluid is more than two-thirds limit of normal serum LDH
 a pH < 7.1 also suggests an exudate

116
Q

Reason for low glucose on pleural aspirate?

A

infection, malignancy and oesophageal rupture.

Particularly low glucose levels (< 1.6 mmol/L) are seen in effusions due to rheumatoid arthritis.

117
Q

Indication for drainage in pleural effusion?

A

If pH <7.2, frank pus, culture positive

118
Q

Management of pleural effusion in lung ca?

A

Pleural catheter long term

Pleurodesis (medically with talc or via thoracoscopy)

119
Q

Signs of yellow nail syndrome

A

Lymphodema (lower limb)
Thick dystrophic nail with fungal infection
Pleural effusion
Bronchiectasis

120
Q

Contraindication to renal transplant

A

Active or recent malignancy
Active vasculitis
Issue with donor matching
Deep seated infection

121
Q

Give 6 examples of congenital heart disease

A

pulmonary atresia
tricuspid atresia,
pulmonary stenosis
Eisenmenger syndrome
Ebstein’s anomaly
transposition of the great arteries
TOF

122
Q

Examples of scars visible in a fixed TOF

A

Lateral thoracotomy scar for a Blalock-Taussig Shunt, which is done by plumbing the left subclavian artery into the pulmonary artery, distal to the pulmonary stenosis to improve =blood flow to the lungs.

Midline sternotomy scar for repair of the ventricular septal defect or pulmonary valve replacement

123
Q

What is pneumonectomy space filled by?

A

Initially air then becomes filled with gelatinous material

124
Q

Causes of apical fibrosis

A

Berryliosis
Radiation
EEA
ABPA/AS
Silicosis/sarcoidosis
TB

125
Q

Causes of basal fibrosis

A

Radiation
UIP (RA)
Bronchiectasis
Aspiration
Asbestosis

126
Q

Complications of bronchiectasis

A

Corpulence
Secondary amyloidosis (dip urine for protein)
Massive haemoptysis (mycotic aneurysm)

127
Q

Side effect of TB drug

A

Rifampicin - orange urine and contact lens, hepatitis, reduced effectiveness of COCP

Isoniazid - peripheral neuropathy and hepatitis

Pyrazinamide - hepatitis

Ethanbutol - optic neuritis and hepatitis

128
Q

Causes of COPD

A

Smoking, industrial dust
A1AT deficiency

129
Q

Severity of COPD

A

Based on FEV1 vs predicted

Mild = >80% predicted
Moderate = 50-80%
Severe = 30-50%
Very severe = <30%

130
Q

LTOT criteria for COPD

A

PO2 < 7.3kPa on air
Evidence of decompensation
- corpulmonale
- secondary polycythaemia

131
Q

Benefit of LTOT in COPD

A

Improve average survival by 9 months

132
Q

How long to wear LTOT per day?

A

At least 16h a day

133
Q

Dystrophia myotonica inheritance and chromosome

A

Autosomal dominant

Type 1 = DMPK gene on chromosome 19
Type 2 = ZNF9 gene on chromosome 3

134
Q

Dystophia myotonica features

A

Cataract
Ptosis
Myotonia (slowly relaxing hand grip)
Frontal balding
Cardiomyopathy
Dysphagia
Muscle wasting
Areflexia

135
Q

DD ptosis

A

Unilateral
- Stroke
- Third nerve palsy
- Horner’s syndrome

Bilateral
- Myotonic dystrophy
- MG
- Congenital

136
Q

Features of tuberous sclerosis

A

Angiofibromata
Shagreen patch
Periungal fibroma
Ash leaf macules

Polycystic kidney disease
Renal angiomyolipomata
Seizures

137
Q

Causes of wasting of hand muscles

A

Anterior horn cell
- MND
- Syringomyelia
- Cervical cord compression
- Polio

Brachial plexus
- cervical rib
- pancoast tumour
- trauma

Peripheral nerve
- peripheral neuropathy
- combined median and ulnar nerve lesion

Muscle
- disuse atrophy like RA

138
Q

Features of neurofibromatosis

A

Cafe au lait spots 6 or more >15mm diamter
Lisch nodule (melanocytic haematoma of iris)
Interstitial lung disease
Enlarged palpable nerve

Type 1 = phaechromocytoma
Type 2 = bilateral acoustic neuroma and sensorineural deafness

139
Q

Causes of third nerve palsy (pupil sparing)

A

Medical causes
- Mononeuritis multiplex (DM)
Midbrain infarction e.g. Webers
Midbrain demyelination (MS)
Migraine

140
Q

Causes of complete third nerve palsy (dilated pupil)

A

Surgical causes
- Communicating artery aneurysm
- Cavernous sinus pathology (thrombosis, tumour, fistula)
- Cerebral uncus herniation

141
Q

Optic atrophy causes

A

Pressure e.g. tumour, glaucoma, pagets
Ataxia (friedreich)
LEbers
Dietary (b12)
Degenrative e.g. retinitis pigmentosa
Ischaemia (Central retinal artery occlusion)
Syphillis and other infections CMV, toxoplasmosis
Cyanide and other toxins e.g. alcohol, lead, tobacco
Sclerosis (MS)

142
Q

Age related macular degeneration features

A

Loss of vision

Wet = neovascularisation, drusen

Dry = non-neovascular, atrophic and non exudative

143
Q

Management of age related macular degeneration

A

Opthalmology referral
Wet AMD = intravitreal injections of anti VEGF

144
Q

Retinitis pigmentosa features

A

Gradual loss of peripheral vision

145
Q

Retinitis pigmentosa causes and associations

A
  1. Congenital (AR ingeritance)
  2. Post-inflammatory retinitis

Associations
- Friedreich ataxia
- Refsum disease (anosmia, iris atrophy)
- Kearns Sayre syndrome (ataxic, ptosis, opthalmoplegia, permanent pacemaker, deafness)

146
Q

Causes of tunnel vision

A

Papilledema
Glaucoma
Chorioretinitis
Migraine
Hysteria
Retinitis pigmentosa

147
Q

Causes of retinal artery occlusion

A
  1. Embolic e.g. AF, carotid plague rupture, cardiac mural thrombus - tx aspirin, anticogulation and endaterectomy
  2. GCA - tx high dose steroids
148
Q

Causes of retinal vein occlusion

A

Hypertensin
Hyperglycaemia
Hyperviscosity syndrome e.g. waldenstrom macroglobulinaemia or MM
Glaucoma