Cases for PACES Mushkies Flashcards
Causes of palmar erythema?
Birds have red palms and they like to CHiRPP
- Cirrhosis
- Hyperthyroidism
- Rheumatoid arthritis
- Pregnancy
- Polycythaemia
Causes of gynaecomastia?
A tall pubertal boy with Klinefelters and liver failure who is a drug addict with tiny testes and is brown and overactive
- Physiological = puberty and selinity
- Klinefelters
- Cirrhosis
- Drugs = spironolactone and digoxin
- Testicular trumour/orchidectomy
- Endocrine = Addisons and hyper/hypothyroidism
Complications of haemochromatosis?
- Endo = bronze diabetes, hypogonadism, testicular atrophy
- Cardiac = CCF
- Joints = arthropathy (pseudogout)
Causes of unilateral kidney enlargement?
- PCKD (other kidney not palpable or contralateral nephrectomy)
- RCC
- Simple cysts
- Hydronephrosis
Causes of bilateral kidney enlargement?
- PCKD
- Bilateral RCC
- Bilateral hydronephrosis
- Tuberous sclerosis
- Amyloidosis
ADPKD defn?
Progressive replacement of normal kidney tissue by cysts leading to renal enlargement and renal failure
How to tell if pt has had a kidney-pancreas transplantation?
Lower midline abdominal incision, with a palpable kidney in an iliac fossa WITHOUT any overlying scar
Success of renal transplantation?
- 90% 1 year graft survival
2. 50% 10 year graft survival (better with live-related donor grafts)
Single lung transplant patient features?
Unilateral fine crackles and contralateral thoracotomy scar with normal breath sounds
Bronchiectasis CXR?
Tramlines and ring shadows
Kartagener’s investigation?
Saccharine ciliary motility test (nares to taste buds in 30 minutes)
3 complications of bronchiectasis?
- Cor pulmonale
- Haemoptysis
- Amyloidosis
Indication for single lung transplant?
Dry lung conditions = COPD, pulmonary fibrosis
Indication for double lung transplant?
Wet lung conditions = CF, bronchiectasis, pulmonary HTN
Causes of COPD?
- Environmental = smoking and industrial dust exposure (apical disease)
- Genetic = a1-antitrypsin deficiency (basal disease)
How to assess severity of COPD?
GOLD classification: FEV1
- GOLD 1 = <80%
- GOLD 2 = 50-80
- GOLD 3 = 30-50%
- GOLD 4 = <30%
When to perform bullectomy for COPD?
If bullae >1L and compresses surrounding lung
Surgical COPD Mx?
- Bullectomy
- Endobronchial valve placement
- Lung reduction surgery
- Single lung transplant
COPD acute exacerbation in-hospital mortality?
15%
DDx of a wheezy chest?
- COPD
- Asthma
- GPA
- RHA
- Post lung transplant
Pleural effusion percussion?
Stony dull
Lung cancer workup?
- Diagnosis = CXR, Volume acquisition CT thorax
- Determine cell type = induced sputum cytology, biopsy by bronchoscopy/percutaneous needle
- Stage = CT/bronchoscopy/EBUS/mediastinoscopy/thoracoscopy/PET
- Lung FTs for operability assessment = Pneumonectomy C/I if FEV1 <1.2L
How many courses of chemo for SCLC?
6
Types of radiotherapy for NSCLC?
- Single fractionation = weekly
2. Hyper fractionation = daily for 10 days
Causes of false positive sweat test?
Hypothyroidism and Addisons
Physio for CF?
Postural drainage and active cycle breathing techniques
Double lung transplant for CF prognosis?
50% survival at 5 years
Poor prognosis for CF if what happens?
Infected with Burkholderia Cepacia
Urine dip for Pneumonia?
- Legionella antigens
- Pneumococcal antigens
- Haemoglobinuria = mycoplasma causes cold agglutinins –> haemolysis
Complications of Pneumonia?
- Septic shock and MOF
- Haemoptysis
- Para-pneumonic effusion/empyema
- Lung abscess (S. aureus, Klebsiella, Anaerobes)
DDx for ejection systolic murmur?
- All causes of AS
- Aortic sclerosis
- VSD
- HOCM
- High output clinical states e.g. pregnancy or anaemia
Associations of aortic stenosis?
- Coarctation of the aorta
2. Angiodysplasia
What is EuroScore?
European System for Cardiac Operative Risk Evaluation
Collapsing pulse aka?
Waterhammer pulse
Signs of severe aortic regurgitation?
- Collapsing pulse
- Pulmonary oedema
- S3
When to replace valve in chronic aortic regurgitation?
- Symptomatic AND/OR the following criteria are met:
a. Wide pulse pressure > 100mmHg
b. ECG changes - Echo = LV enlargement >5.5cm systolic diameter or EF <50%
Aortic regurgitation prognosis?
- Asymptomatic with EF >50% = 1% mortality at 5 years
2. Symptomatic w/ all 3 criteria present = 65% mortality at 3 years
Causes of mitral stenosis?
- Congenital = rare
2. Acquired = Rheumatic (commonest), Senile Degeneration, Endocarditis (mitral ‘plop’ and late diastolic murmur)
DDx of mitral stenosis?
- LA myxoma
2. Austin-flint murmur
Mitral stenosis prognosis?
- Latent asymptomatic phase = 15-20 years
2. NYHA > II = 50% mortality at 5 years
Mx of rheumatic fever?
Rest, high dose aspirin, penicillin
Secondary prevention of rheumatic fever?
Penicillin V for 5-10 years
Mitral valve prolapse association?
Tall young women, and connective tissue diseases
Midline sternotomy indications?
CABG, AVR, MVR
Lateral thoracotomy indications?
- MVR
- Coarctation repair
- Blalock–Taussig shunt
Subclavicular scar indications?
Pacemaker, ICD
Indications for ICD?
Primary prevention and secondary prevention
Primary prevention indications for ICD?
- Familial condition with high risk SCD = LQTS, ARVC, HCM, Brugada, complex congenital heart disease
- MI > 4 weeks AND
Primary prevention indication for MI > 4 weeks AND?
- LVEF < 35% AND non sustained VT AND positive EP study
2. LVEF < 30% AND QRSd >120ms
Secondary prevention indications for SCD?
- Cardiac arrest due to VF or VT
- Haemodynamically compromising VT
- VT with LVEF < 35%
Criteria for CRT?
- LVEF <35% and
- NYHA II-IV on optical medical therapy and
- Sinus rhythm and QRSd > 150ms
Kussmaul’s sign?
Paradoxical rise in JVP on inspiration, a sign of constrictive Pericarditis
Pulsus paradoxus?
A >10mmHg drop in systolic pulse in inspiration, a sign of constrictive pericarditis (not a paradox as it usually decreases by 2-3mmHg)
Causes of constrictive pericarditis?
5 Ts
- TB
- Trauma
- Tumour
- Therapy = radio
- Tissue disease = RhA, SLE
Mx of constrictive pericarditis?
- Diuretics and fluid restriction
2. Surgical = pericardial stripping
How to differentiate between restrictive cardiomyopathy from pericardial constriction?
Ventricular interdependence (filling of one ventricle decreases the size and filling of the other, a sign of constrictive pericarditis)
Pathophysiology of ventricular interdependence?
Thickened, fibrous capsule reduces ventricular filling and ‘insulates’ the heart from intrathoracic pressure changes during respiration
Types of ASD?
- Secundum = most common
2. Primum = associated with AVSD and cleft mitral valve, seen in Downs syndrome
Causes of VSD?
- Congenital = e.g TOF
2. Acquired = traumatic, post-operative or post-MI
What is a Blalock-Taussig shunt?
Anastamoses subclavian artery to pulmonary artery, and thus can partially correct right to left shunts
Coarctation of the aorta defn?
A congenital narrowing of the aortic arch that is usually distal to the left subclavian artery
Associations of coarctation?
- Cardiac = ASD, PDA, bicuspid aortic valve
2. Non-cardiac = Turner’s and berry aneurysms
PDA defn?
Continuity between the aorta and pulmonary trunk with a left to right shunt
RF for PDA?
Rubella
Coarctation Mx?
- Percutaneous = EVAR
- Surgical = Dacron patch aortoplasty
- Long-term anti-HTN
- Long term surveillance with MRA for aneurysms and recoarctation
Complications of PDA?
- Eisenmenger’s
2. Endocarditis
Mx of PDA?
Closed surgically or percutaneously
Clinical signs of HOCM?
- Jerky pulse character and double apical impulse
2. Ejection systolic murmur at LLSE that radiates throughout the praecordium
2 associations of HOCM?
- Friedrich’s ataxia
2. Myotonic Dystrophy
HOCM Mx?
- Conservative = avoidance of strenuous exercise and dehydration
- Medical = BB
- Interventional = ICD, Alcohol septal ablation
- Surgery = myomectomy, heart transplant
HOCM annual mortality rate?
2.5%
Dystrophia myotonica signs?
- Face = myopathic, wasting of facial muscles and SCM, bilateral ptosis, frontal balding, dysarthria (due to myotonia of pharynx and tongue)
- Hands = myotonia, wasting and weakness of distal muscles with areflexia, percussion myotonia
- Eye = cataracts
- Heart = CM, brady and tachyarrhythmias
- Endo = diabetes, testicular atrophy
- GI = dysphagia
Dystrophia myotonica cause?
Both AD, show genetic anticipation
- DM1 = CTG, DMPK gene on Chr 19
- DM2 = CCTG, ZNF9 gene, Chr 3
Dystrophia myotonica Dx?
- Clinical fx
- EMG = divebomber potentials
- Genetic testing
Dystrophia myotonica Dx?
Phenytoin may help myotonia
Dystrophia myotonica Px?
- Affected individuals die prematurely of respiratory and cardiac complications
- Weakness is a major problem = no treatment
- Advise against GA (high risk of resp/cardiac complications)
Causes of ptosis?
- Bilateral = myotonic dystrophy, myasthenia gravis, congenital
- Unilateral = Third nerve palsy, Horner’s syndrome
MS genetic factors?
HLA DR2, IL-2, Il-7
MS Dx?
Clinical features AND
- CSF = oligoclonal IgG bands
- MRI = periventricular white matter plaques
- VEPs = delayed velocity but normal amplitude
MS Mx?
- MDT
- Disease modifying = IFNb, Alemtuzumab (anti-CD-52), Natalizumab (anti-a4 integrin)
- Symptomatic = methylprednisolone during acute, ant-spasmodics e.g. baclofen, carbamazepine for neuropathic pain, laxatives and intermittent catheterisation/oxybutinin for bowel and bladder disturbance
MS prognosis?
Variable, majority will remain ambulant at 10 years
MS and pregnancy?
- Reduced relapse rate during pregnancy
- Increased risk of relapse in postpartum period
- Safe for foetus (possibly reduced LBW)
MS impairment, disability and handicap?
- Arm paralysis is the impairment
- Inability to write is the disability
- Subsequent inability to work as an accountant is the handicap
Stroke defn?
A rapid onset, focal neurological deficit due to a vascular lesion lasting >24 hours
Dominant parietal lobe cortical signs?
- Dysphasia = receptive, expressive or global
2. Gerstmann’s syndrome = Dysgraphia, dyslexia and dyscalculia + L-R disorientation + Finger agnosia
Non-dominant parietal lobe signs?
- Dressing and constructional apraxia
2. Spatial neglect
Most common brainstem vascular syndrome?
Lateral medullary syndrome, due to PICA occlusion, variable in its presentation
Common causes of spastic legs?
- MS
- Spinal cord compression
- Trauma
- MND
Rare causes of spastic legs?
- Beck’s syndrome
- Syringomyelia
- Hereditary spastic paraplegia
- SCDC
- Friedrich’s ataxia
Causes of cord compression?
- Infection = abscess or TB
- Malignancy
- Disc prolapse (above L1/L2)
- Trauma = vertebral fracture
Main clinical signs of syringomyelia?
- Dissociated sensory loss in upper limbs and chest
- Loss of reflexes in the upper limbs
- Weakness and wasting of small muscles of the hand
- Scars from painless burns
- Charcot joints = elbow and shoulder
Additional signs of syringomyelia?
- Pyramidal weakness in lower limbs with extensor plantars
2. Horners
Pathophysiology of Syringomyelia?
Caused by a progressively expanding fluid filled cavity within the cervical cord, typically spanning several levels, and extends ventrally, affecting:
- Decussating spinothalamic neurones producing segmental pain and temperature loss at the level of the syrinx
- Anterior horn cells producing segmental LMN weakness at the level of the weakness
- Corticospinal tract, producing UMN weakness below the level of the weakness
Common causes of syringomyelia?
Arnold-Chiari malformation and spina bifida
Syringomyelia Ix?
Spinal MRI
Charcot joint defn?
A neuropathic arthropathy: painless deformity and destruction of a joint with new bone formation following repeated minor trauma secondary to loss of pain sensation
Most important causes of charcot joint?
- Foot and ankle = diabetes
- Hip and knee = tabes dorsalis
- Elbow and shoulder = syringomyelia
Mx of charcot joints?
Bisphosphonates
MND sensory examination?
Normal
Bulbar MND speech?
Donald Duck (nasal) speech due to palatal weakness
Pseudobulbar MND speech?
Hot Potato speech due to a spastic tongue
What involvement is spared in MND?
- Sensory
- Extra-ocular muscles
- Cerebellum
- Extra-pyramidal
MND defn?
A progressive disease of unknown aetiology characterised by axonal degeneration of the upper and lower motor neurones
MND Ix?
- Clinical Dx
- EMG = fasciculation
- MRI (brain and spine) = excludes the main DDx of cervical cord compression and myelopathy and brainstem lesions
MND Mx?
- MDT
- Supportive = PEG, NIPPV
- Riluzole (glutamate antagonist)
What is a fasciculation?
- Visible muscle twitching at rest
- Axonal loss results in the surviving axons recruiting and innervating more myofibrils than usual, resulting in larger motor units
- Seen commonly in MND and syringomyelia
Causes of generalised wasting of hand muscles?
- Anterior horn cell = MND, syringomyelia, cervical cord compression, polio
- Brachial plexus = cervical rib, Pancoasts tumour, trauma
- Peripheral nerve = peripheral neuropathy, combined median and ulnar nerve lesions
- Muscle = disuse atrophy e.g. RhA
Inverted champagne bottle appearance description?
Wasting of distal lower limb muscles with preservation of the thigh muscle bulk
Causes of mononeuritis multiplex?
- DM
- CTD e.g. SLE and RhA
- Vasculitis e.g. PAN and eGPA
- Infection e.g. HIV
- Malignancy
Friedrich’s Ataxia Neuro Fx?
- Young adult in wheelchair or ataxic gait
- Pes cavus
- Bilateral cerebellar ataxia
- Leg wasting with absent reflexes and extensor plantars
- Posterior column signs (loss of vibration and joint position)
Friedrich’s Ataxia other Fx?
- Kyphoscoliosis
- Optic atrophy
- High arched palate
- Sensorineural deafness
- HOCM
- DM
Causes of extensor plantars with absent knee jerks?
MAST CC
- MND
- Ataxia, Friedrich’s
- SCDC
- Taboparesis
- Combined upper and lower pathology e.g. cervical spondylosis with peripheral neuropathy
- Conus medullaris lesions
Causes of bilateral extra-ocular palsies?
- MG
- Miller-fisher variant of GBS
- Graves
Relationship between TS and ADPKD?
The genes for them both are contiguous on Chromosome 16, hence some mutations lead to both conditions
What was TS previously known as?
EPILOIA = Epilepsy, low intelligence, adenoma sebaceum
NF features?
- Cutaneous neurofibromas = 2 or more
- Cafe au lait patches = >5, >15mm
- Axillary freckling
- Lisch nodules = melanocytic hamartomas of the iris
- BP = HTN associated with phaeo and RAS
- Lung = fibrosis
- Neuropathy with enlarged palpable nerves
NF complications?
- Epilepsy
- Sarcomatous change
- Scoliosis
- Mental retardation
Causes of enlarged nerves and peripheral neuropathy?
- NF
- Leprosy
- Amyloidosis
- Acromegaly
- Refsum’s disease
Horner’s pupil?
PEAS
- Ptosis
- Enophthalmos
- Anhydrosis
- Small pupil (miosis)
Holmes Adie pupil?
- Moderately dilated pupil that has a poor response to light and a sluggish response to accomodation (you may have to wait)!
- May have absent or diminished ankle jerks
- Benign condition that is more common in females, reassure pt that nothing is wrong
Argyll Robertson pupil?
Small irregular pupils that accommodate but dont react to light, with an atrophied and depigmented iris
Foster Kennedy syndrome?
Papilloedema in one eye due to raised ICP and optic atrophy in the other due to direct compression by the tumour
Age-related macular degeneration types?
- Wet = neovascular and exudative
2. Dry = non-neovascular, non-exudative and atrophic
Macular changes of AMD?
- Drusen = extracellular material
- Geographic atrophy
- Fibrosis
- Neovascularisation (wet)
AMD RFs?
- Age
- White
- FHx
- Smoking
AMD Mx?
- Ophthalmology referral
- Wet AMD may be treated by intravitreal injections of anti-VEGF (though can increase cerebrovascular and cardiovascular risk)
AMD Px?
Majority of pts progress to blindness in the affected eye within 2 years of diagnosis
Retinitis pigmentosa defn?
Inherited form of retinal degeneration characterised by loss of photoreceptors
Retinitis pigmentosa causes?
- Congenital = AR, 15% due to rhodopsin pigment mutations
2. Acquired = post-inflammatory retinitis
Retinitis pigmentosa clinical features?
- White stick and braille book (registered blind)
2. Reduced peripheral field of vision (tunnel vision)
Retinitis pigmentosa on fundoscopy?
- Peripheral retina bone spicule pigmentation which follows the veins and spares the macula
- Optic atrophy due to neuronal loss
- Association = cataract (absent red reflex)
Retinitis pigmentosa prognosis?
- Progressive loss of vision due to retinal degeneration
- Begins with reduced night vision
- Most are registered blind at 40 y/o with central visual loss in the 7th decade
- No tx although Vitamin A may slow progression
Causes of tunnel vision?
- Papilloedema
- Glaucoma
- Migraine
- Choroidoretinitis
- Hysteria
Retinal artery occlusion on fundoscopy?
- Pale, milky fundus with thread-like arterioles
2. Cherry red macula (choroidal blood supply)
Retinal artery occlusion causes?
- Embolic = carotid plaque rupture or cardiac mural thrombus –> aspirin, anticoagulation, endarterectomy
- GCA = tender scalp and pulseless temporal arteries –> high dose steroid urgently, check ESR and arrange temporal artery biopsy to confirm diagnosis
Effect of retinal artery occlusion?
Optic atrophy and blind
Branch retinal artery occlusion field defect?
Opposite to the quadrant of affected retina
Retinal vein occlusion fundoscopy?
- Flame haemorrhages +++ radiating out from swollen disc
- Engorged tortuous veins
- Cotton wool spots
Causes of retinal vein occlusion?
4 Hs
- HTN
- Hyperglycaemia = DM
- High IOP = glaucoma
- Hyperviscosity = Waldenstrom’s macroglobulinaemia or myeloma
What is battery?
Actual physical contact or injury, direct or indirect, without consent
Migraine features mnemonic?
POUND
- Pulsating
- 4-72 hOurs
- Unilateral
- Nausea
- Disabling and may have aura
DVT calf confirmation?
Calf swelling 10cm below the tibial tuberosity, >3cm difference between legs
DVT complications?
Thrombophlebitis and PE
DKA precipitating factors?
4 Is
- Insulin forgotten
- Infection
- Infarction
- Injury
Legal considerations at end of life?
Lawyers need to write on a PAD
- Power of attorney
- Advanced directives or living wills
- DNAR and other treatments
Inflammatory condition Hx questions?
Skin, joints, eyes
AF types?
- Paroxysmal = <7 days, self terminating
- Persistent = >7 days, requires chemical or electrical cardioversion
- Permanent = >1 year or when no further attempts to restore sinus rhythm
Pulmonary vein isolation for AF?
- Reserved for refractory, symptomatic patients
- More successful in paroxysmal AF (>90% cure rate)
- Young = may limit progression to permanent AF
Bleeding risk for anticoagulation on AF score?
HASBLED, >=3 = avoid oral anticoagulation
- Hypertension
- Abnormal kidney/liver function
- Stroke
- Bleeding
- Labile INR
- Elderly
- Drugs (NSAIDs) and Alcohol (1 for each)
What should you do for AF pts at high risk for both embolic and bleeding complications?
Left atrial appendage occlusion to isolate the commonest source of thrombus in AF
AF prevalence?
8% of >80 year olds have AF
Crohns DDx?
- Infection = Yersinia, TB
- Inflammation = UC
- Malignancy = Lymphoma
UC DDx?
- Infection = Campylobacter
- Inflammation = CD
- Ischaemia
- Drugs
- Radiation
When to give metronidazole for IBD?
In Crohn’s with perianal infection, fistulae or small bowel bacterial overgrowth
UC surveillance?
3 yearly colonoscopy for pts with pancolitis >10 years, increasing in frequency with every decade from diagnosis
Fundoscopy features of hypertensive nephropathy?
- Silver wiring (increased reflectance from thickened arterioles)
- AV nipping (narrowing of veins as arterioles cross them)
- cotton wool spots and flame haemorrhages
- Papilloedema
There may also be hard exudates (macular star)
- Papilloedema
Mechanism of silver wiring?
Increased reflectance from thickened arteriole
Mechanism of AV nipping?
Narrowing of veins as arterioles cross them
Causes of HTN?
ERECP
- Essential = Age, obesity, salt, alcohol
- Renal = CKD, ADPKD, renovascular disease
- Endocrine = Conns, Cushings, Acromegaly, Phaeo
- Coarctation of the aorta
- Pregnancy = pre-eclampsia
Papilloedema fx?
Blurring of disc margins, elevation of disc, loss of venous pulsation, venous engorgement
Causes of papilloedema?
- Raised ICP
- Accelerated phase hypertension
- Central retinal vein occlusion
5 forms of psoriatic arthropathy?
- DIPJ involvement (similar to OA)
- Large joint mono/oligoarthritis
- Seronegative (similar to RhA)
- Sacroiliitis (similar to AS)
- Arthritis mutilans
4 psoriasis nail fx?
- Pitting
- Onycholysis
- Hyperkeratosis
- Discoloration
Psoriasis definition?
Epidermal hyperproliferation and accumulation of inflammatory cells
Psoriasis Mx?
- Topical = emollients, calcipotriol, coal tar, dithranol, hydrocortisone
- Phototherapy = UVB or PUVA
- Systemic = Cytotoxics (methotrexate and ciclosporine), anti-TNFa (adalimumab), retinoids
Main complication of psoriasis?
Erythroderma
Causes of koebner phenomenon?
- Psoriasis
- Vitiligo
- Lichen planus
- Viral warts
- Sarcoid
Eczema Mx?
- Avoid precipitants
- Topical = emollients, steroids, tacrolimus
- Antihistamines for pruritis
- Abx for secondary infection
- UV light therapy
- Prednisolone in severe cases
What leg ulcers are painless?
Venous and neuropathic
2 main complications of leg ulcers
- Infection = temperature, pus and cellulites
2. Malignant change = Marjolin’s ulcer
Causes of leg ulcer?
- Arterial
- Venous
- Neuropathic
- Infection = syphilis
- Inflammatory = RhA
- Malignancy = SCC
- Haem = SCA
- Tropical = cutaneous leishmaniasis
3 causes of neuropathic ulcers?
- DM
- Tabes dorsalis
- Syringomyelia
Mx of necrobiosis lipoidica diabeticorum?
- Topical steroid and support bandaging
2. Tight glycaemic control does not help
Causes of secondary hyperlipidaemia?
- Hypothyroidism
- Nephrotic syndrome
- Alcohol
- Cholestasis
Pathology of erythema nodosum?
Inflammation of the subcutaneous fat (panniculitis)
HSP discussion?
- Small vessel vasculitis with IgA and C3 deposition
- Normal or raised platelet count, distinguishing it from other forms of purpura
- Male children
- Mx = most spontaneously recover without treatment although steroids may help recovery and treat painful arthralgia
- Px = 90% full recovery although can recur
Glass eye and ascites?
Ocular melanoma
Pseudoxanthoma elasticum features?
- Skin = plucked chicken skin appearance
- Eyes = blue sclerae, retinal angioid streaks
- CVS = HTN, MVP
- Joints = hyperextensible
Pseudoxanthoma elasticum defn?
Autosomal recessive (80%, ABCC6 gene, Chr16) condition causing degenereative elastic fibres in the skin, blood vessels and eye, and leads to premature coronary artery disease
Ehlers Danlos inheritance?
AD
Ehlers Danlos pathophysiology?
Defect in collagen causing increased skin elasticity, no premature coronary artery disease
RhA hand scars?
- Wrist = carpal tunnel release
- Thumb = joint replacement
- Dorsum of hand = tendon transfer
3 RhA grips to test?
- Power = squeeze fingers
- Precision = pick up coins/do buttons
- Key = pretend to use this key
RhA prognosis?
- 5 years = 1/3rd unable to work
2. 10 years = 1/2 significant disability
Jacoud’s arthropathy?
A chronic non-erosive reversible joint disorder that may occur after repeated bouts of arthritis, commonly secondary to SLE
Cyclophosphamide s/e?
HHIT
- Haematological
- Haemorrhagic cystitis
- Infertility
- Teratogenicity
SLE prognosis?
90% survival at 10 years
Diffuse systemic sclerosis prognosis?
50% survival to 5 years (most deaths due to respiratory failure)
Most common complications of ankylosing spondylitis?
Anterior uveitis
Ankylosing spondylitis Mx?
- Physiotherapy
- Analgesia
- Anti-TNFa
Marfans genetics?
AD disorder on chromosome 15, due to mutation in fibrillin gene
Marfans Mx?
- Conservative = monitoring of aortic root size with annual TTE
- Medical = BB and ARB to slow aortic root dilatation
- Surgery = Pre-emptive aortic root surgery to prevent dissection and aortic rupture
- Screen family members
Homocystinuria fx?
- Mental retardation and downward lens dislocation
Gout radiograph fx?
‘Punched out’ periarticular changes
Diabetic eye on fundoscopy?
- Background = hard exudates, blot haemorrhages, microaneurysms
- Pre-proliferative = cotton wool spots and flame haemmorrhages
- Proliferative = Neovascularisation of the disc (NVD) and panretinal photocoagulation scars
- Maculopathy = macular oedema or hard exudates within one disc space of the fovea
Diabetic retinal screening?
- Annual screening for all pts with DM using retinal photography
- Refer to ophthalmology if pre-proliferative or changes near the macula
- Background retinopathy usually occurs 10-20 years after diabetes is diagnosed
Mx of diabetic retinopathy?
- Tight glycaemic control
2. Treat other RFs e.g. HTN
Photocoagulation indications?
- Maculopathy
2. Proliferative and pre-proliferative diabetic retinopathy
Panretinal photocoagulation MOA?
Prevents the ischaemic retinal cells secreting angiogenic factors causing neovascularisation, whilst focal photocoagulation targets problem vessels at risk of bleeding
Complications of pre-proliferative diabetic retinopathy?
- Vitreous haemorrhage (may require vitrectomy)
- Traction retinal detachment
- Neovascular glaucoma due to rubeosis iridis
Cataracts signs?
- Loss of the red reflex
- Cloudy lens
- May have RAPD
- Association = myotonic dystrophy
Cataracts causes?
- Congenital = Rubella, Turners
2. Acquired = Age, DM, Steroids, Radiation, Trauma
Cataracts Mx?
- Phacoemulsification with prosthetic lens implantation
2. Yttrium aluminium garnet (YAG) laser capsulotomy
Progression of eye signs in Graves?
NOSPECS
- No signs or symptoms
- Only lid lag/retraction
- Soft tissue involvement
- Proptosis
- Extra-ocular muscle involvement
- Chemosis
- Sight loss due to optic nerve compression and atrophy
Complications of hypothyroidism?
- Cardiac = pleural effusion, CCF
2. Neuro = Carpal tunnel syndrome, proximal myopathy, ataxia
Causes of hypothyrodism?
- AI = Hashimotos, atrophic
- Iatrogenic = post-thyroidectomy
- Iodine deficiency = Derbyshire neck
- Genetic = Pendred’s syndrome
- Dyshormonogenesis
Complications of acromegaly?
Acanthosis nigricans BP raised Carpal tunnel syndrome DM Enlarged organs Field defect = bitemporal hemianopia Goitre, GI malignancy HF, hirsute, hypopituitary IGF1 raised Joint arthropathy Kyphosis Lactation Myopathy = proximal
3 signs of active acromegaly?
Raised BP, Bitemporal hemianopia, DM
Acromegaly Mx?
- Medical = Octreotide (SS analogue), Cabergoline (DA agonist), Pegvisomant (GH receptor antagonist)
- Surgical = Trans-sphenoidal approach
- Radiotherapy = in non-surgical candidates
4 causes of macroglossia?
- Acromegaly
- Amyloidosis
- Hypothyroidism
- Down’s syndrome
Cause of pseudo-Cushings?
Ethanol excess
Cushings syndrome?
The physical signs of glucocorticoid excess
Cushings disease?
Glucocorticoid excess due to ACTH secreting pituitary adenoma
Medical mx for cushings?
Metyrapone (blocks cholesterol synthesis)
Nelson’s syndrome?
Bilateral adrenelectomy to treat Cushings disease causing massive release of ACTH –> hyperpigmentation and pituitary overgrwoth
Cushings Px?
Untreated –> 50% mortality at 5 years
Causes of proximal myopathy?
- Inherited = myotonic dystrophy, muscular dystrophy
- Endocrine = Cushings, HyperPTH, thyrotoxicosis, diabetic amyotrophy
- Inflammatory = polymyositis, RhA
- Metabolic = osteomalacia
- Malignancy = paraneoplastic, LEMS
- Drugs = alcohol, steroids
Addisons prognosis?
Normal life expectancy
HbSS Prognosis?
40-50 years old