PACES 1 Flashcards
Common causes of splenomegaly and investigations
Haem- CML, myelofibrosis, hereditary spherocytosis, haemolytic anaemia, ITP
Infective- malaria, visceral leishmaniasis, EBV, IE
Infiltrative- amyloidosis, Felty’s, SLE
Other- portal HTN, splenic vein thrombus, HIV
Investigations- FBC, blood film, thin/thick films, US, CT, LN biopsy
Hereditary spherocytosis- signs, inheritance, investigation, management
Signs- Splenomegaly, anaemia, jaundice, cholecystectomy.
Autosomal dominant
Ix- FBC, retics, EMA/osmotic fragility test, direct coombs test (to rule out AIHA)
Mx- folic acid, splenectomy, transfusions
Pulmonary fibrosis- signs, Ix, Mx, spirometry pattern
Signs- Clubbing, LTOT, cyanosis, fine creps, CT disease signs
Ix- CXR, HRCT, bloods (ANA, ENA, ANCA), spirometry, obs/ABG
Mx- pirfenidone, nintendanib, steroids, transplant, supportive
Spirometry- restrictive and reduced gas transfer
Causes of upper zone lung fibrosis
Coal workers pneumoconiosis, histiocytosis, ank spond, ABPA, radiation, TB, silicosis, sarcoidosis
Causes of lower zone lung fibrosis
RA, asbestosis, CT disease, IPF, other (drugs- MTX, nitro, amio)
ADPKD- types, signs, complications, management
Type 1 and 2- type 1 more rapidly progressive to ESRF
Signs- uni/bilateral ballotable kidneys, signs of RRT/lines, nephrectomy
Complications- HTN, berry aneurysms, MVP, cystic liver
Management- RRT, transplant, nephrectomy, control HTN (ACEi/ARB), genetic counselling, 5 yearly screening for berry aneurysm, 3L fluid per day, screen family
ABPA- what is it and who gets it? Ix? Mx?
Asthmatics, immunocompromised and CF patients- colonise aspergillus fungus in airways and unable to clear it they develop an immune response/hypersensitivity reaction
Ix- aspergillus RAST, IgE, eosinophils, CXR, HRCT
Mx- steroids, itraconazole
EAA/hypersensitivity pneumonitis- pathology, causes, Mx
Exposure to allergen causes hypersensitivity reaction- fever, cough, sob, wheeze
Causes- mushroom worker, bird fancier, malt worker, farmers lung
Mx- steroids and avoid trigger
Pneumonectomy- scar, signs, indications
Scar- lateral thoracotomy, drain scars
Signs- hemithorax fills with fluid, dull to percuss, no air entry, trachea deviated towards side of surgery, reduced chest expansion
Indications- cancer, TB, PTX, COPD, trauma, aspergilloma, abscess, CF
Lobectomy- scar, signs indications
Scar- lateral thoracotomy, drain scars
Signs- may be normal. Hyperexpansion of remaining lobes, reduced air entry over lobectomy area
Indications- cancer, TB, PTX, COPD, trauma, aspergilloma
FEV1 required for lobectomy and pneumonectomy
Pneumo >2L
Lobectomy >1.5L
Stigmata of chronic liver disease
Dupuytrens contracture, palmar erythema, clubbing, gynaecomastia, jaundice, spider naevi, caput medusae, ascites, hepatomegaly
Tuberous sclerosis- inheritance, complications
Autosomal dominant
Angiomyolipoma in kidneys, epilepsy, ash leaf/shagreen patches, hamartomas, cystic lung disease
Causes of CLD
ETOH, NAFLD, viral hepatitis, AI hepatitis, PBC, PSC, A1AT deficiency, Wilson’s, HCC, liver mets, haemochromotosis, right heart failure
What monitoring should be offered in CLD
6 monthly US and AFP for HCC, yearly OGD for varices
Causes of hepatomegaly
Malignancy- HCC, mets
CLD- ETOH, NAFLD, PBC, haemochromotosis
Infective- acute/chronic Hep, abscess, cyst
Infiltrative- amyloid, gauchers, sarcoid
Vascular- budd-chiari
Congestive- CCF, constrictive pericarditis
Budd-Chiari syndrome
Hepatic vein occlusion of unknown cause
Wilson’s disease - inheritance, pathology, Ix, complications, Mx
Autosomal recessive
Copper accumulation in liver, heart, brain, eyes joints
Ix- high serum cooper, low caeruloplasmin, genetic testing
Parkinsonism, cirrhosis, psychosis, kayser-fleischer rings
Penicillamine, transplant, low copper diet
Causes of hepatosplenomegaly
portal HTN
Haem- myelofibrosis, spherocytosis, AIHA, thalassaemia
Infection- malaria, visceral leishmaniasis, EBV, CMV
Infiltrative- sarcoid, amyloid
Thalassaemia- signs, Ix, Mx, complications
Signs- frontal bossing, hepato/splenomegaly, pallor, jaundice, cholecystectomy scar
Ix- haemoglobin electrophoresis
Mx- transfusion, folic acid, splenectomy, iron chelation
Complications- iron overload
What characterises bronchiectasis?
Abnormal dilation of bronchial walls due to loss of elasticity and muscular tone. Chronic inflammation,
retention of secretions, oedema, scarring and ulceration of bronchial wall. Recurrent infections cause further dilation resulting in a vicious cycle of impaired clearance, infection, and bronchial damage.
Causes of bronchiectasis
Childhood infections: pertussis, TB
Congenital: CF, kartageners
Hypo-immune: CVID, hypogammaglobulinaemia
Hyper-immune: ABPA
Rheum: SLE, IBD, RA, Sjogrens
Idiopathic
Where would you see VATS scars and give some indications
2-3 2cm port side scars on thorax
Indications: biopsy, removal of mass, pleurodesis
Signs of pulmonary HTN
RV heave, palpable P2, loud P2, TR (PSM), displaced apex beat
Signs of thoracoplasty
Posterior thoracotomy scar, tracheal deviation towards affected side, flattening of chest wall, reduced expansion, absent ribs, dull percussion, reduced breath sounds
Types of pleural effusion and causes
Transudate: CCF, nephrotic syndrome hypothyroid, Meig’s
Exudate: empyema, parapneumonic, TB, malignancy, SLE, RA, sarcoid, PE
Chylothorax
Haemothorax
HIV- common infections/complications
Candida, herpes zoster, PCP, TB, CMV, lymphoma, Kaposi’s sarcoma
Types of jaundice and causes
- Pre-hepatic: AIHA, sphero/elliptocytosis, Gilbert’s, transfusion incompatibility, SCD, thalassaemia
- Hepatic: paracetamol, ETOH, viral hepatitis, EBV, CMV, HCC, NASH, haemochromotosis, Wilson’s
- Post-hepatic: gallstones, biliary tract malignancy, PBC, PSC, pancreatitis
What are the clinical and echo markers of severe AS?
Clinical: lengthening of murmur, evidence of LV failure, quiet/absent S2, narrow pulse pressure
S4, thrill
Echo: area <1cm2, gradient >40mmHg
Where is a normal apex beat located?
5th ICS MCL
Causes of AR
Aortic dissection, IE, bicuspid AV, Marfans, syphilis, ank spond, SLE, RA
Indications for surgery in AR
Angina, CCF, EF <50%, aortic root diameter >50mm
Signs of an ASD
Parasternal heave and palpable thrill, fixed split S2, loud P2, ESM
Signs of Turner syndrome
Short stature, shield like chest, wide spaced nipples, webbed neck, high arched palate, coarctation of the aorta
Signs of coarctation of the aorta
Radio-radial/radio-femoral delay, thoracotomy scar, ESM over LSE radiating to back
Signs of HCM
AF, heaving apex beat, ESM at LSE (with no radiation and louder on valsalva), PSM of MR, signs of CCF
Causes of MR
Rheumatic heart disease, IE, IHD, functional MR due to LV dilatation, Marfans, SLE, Ehler’s danlos, chordae rupture post MI, trauma
Signs of mitral stenosis
AF, malar flush, elevated JVP, RV heave/palpable S2, opening snap, mid-diastolic rumbling murmur, signs of anticoagulation
Signs and associations of MVP
Displaced apex beat, AF, mid-systolic click, with late crescendo-decrescendo murmur
Causes: idiopathic, CT disease (marfans, EDS, SLE), ADPKD, myotonic dystrophy
Causes of pHTN
- Idiopathic/PAH
- Secondary to left heart disease
- Secondary to hypoxic lung disease
- CTEPH
- Other
Signs of TR
Distended JVP with giant V waves, RV heave, palpable P2, PSM at LSE, pulsatile hepatomegaly
Signs of PDA
wide pulse pressure, eisenmenger’s, continuous machinery murmur in left subclavicular region, palpable thrill, S2 may be obscured by murmur
Features of ToF
VSD, PS, over-riding aorta, RVH
Signs and causes of VSD
Downs syndrome, parasternal heave and thrill, PSM at LSE (very loud all over precordium)
Causes: congenital, Down’s, ToF, post MI, post surgery
CF- inheritance, signs, complications
Autosomal recessive
Short stature, clubbing, lines, bronchiectasis, diabetes
Diabetes, infertility, bronchiectasis, DIOS
4 causes of nephrotic syndrome
Minimal change disease, membranous glomerulonephritis, focal segmental glomerulosclerosis, mesangiocapillary glomerulonephritis
PD - signs
Asymmetric resting tremor, bradykinesia, show/shuffling/festinant gait, hypomimia, micrographia, autonomic signs, rigidity dysphonia
Types of PD
Idiopathic
PSP - vertical gaze palsy
MAS- autonomic and cerebellar symptoms
CBD - alien limb
Vascular - gait predominant
Drug induced
DLB- hallucinations and cognitive impairment
Mx of PD
Hold off if symptoms mild due to tolerance
1st levodopa and dopamine dexcarboxylase inhibitor (co-beneldopa, madopar)
COMT inhibitor
MAO-B inhibitor
Dopamine agonist
Amantadine
DBS
Indications for LTOT
pO2 <7.3 or pO2 <8 with secondary polycythaemia or right heart failure
Signs of COPD
Cyanosis, Co2 retention, LTOT, barrel shaped chest, tar staining, prolonged expiratory phase, cor pulmonale
Autoantibodies in CLD
PBC- anti mitochondrial
PSC - anti smooth muscle
AI hepatitis - anti LKM1, anti SM, ANA
NMO- signs, antibodies, how to differentiate
Transverse myelitis and optic neuritis (demyelination)
Symptoms more severe than MS but MRI changes less, no OCB
Anti aquaporin 4 ab’s
Signs of lateral medullary syndrome and vessel affected
Posterior inferior cerebellar artery
Ipsilateral horners, contralateral hemisensory loss (pain and temp), nystagmus, dysphagia, vertigo
Pyramidal weakness pattern
UL’s flexed
LL’s extended
UNILATERAL
Causes of RAPD
Optic nerve pathology
CRAO/CRVO, glaucoma, MS, optic neuropathy, tumour, retinal detachment
GCA until proven otherwise in 50+
Causes of cerebellar dysfunction
Paraneoplastic
Alcohol
Stroke
Tumour
MS
What is INO
Failure of adduction of affected eye with nystagmus on abduction of contralateral eye
TACS vs PACS vs lacunar stroke
TACS: contralateral homonomous hemianopia, contralateral hemiplegia, high cortical dysfunction
PACS: 2/3 above
Lacunar: pure hemimotor/sensory loss
CI’s to thrombolysis
ICH, SoL, seizure at onset, anticoagulation, uncontrolled HTN, LP within 1/52, surgery within 3/12, UGIB within 3/52, stroke within 3/12
Features of dermatomyositis and polymyositis
Dermatomyositis: Gottrons papules, heliotrope rash, elevated CK, legs affected more than arms
Polymyositis: progressive, symmetric, proximal weakness
Charcot-Marie-Tooth- signs, inheritance, Ix, Mx
5 different types of varying inheritance
Aka hereditary sensory motor neuropathy
Reduced sensation, wasting, weakness.
Pes cavus, inverted champagne bottle legs, footdrop, hammertoe
Ix: NCS
Mx: AFO, PT/OT
Causes of proximal myopathy
ETOH, drugs (statins, steroids), dermato/polymyositis, muscular dystrophy, MG/LEMS, Cushings, hypothroid, acromegaly
Causes of ptosis
3rd nerve palsy
Myotonic dystrophy
MG
Horner’s
Congenital
Age
SoL
Triad and ab’s in Miller-Fisher
Anti gq1b abs
Triad- areflexia, ataxia, ophthalmoplegia
Causes of nystagmus
Cerebellar disease, BPPV, posterior stroke, Wernicke’s, MS, stroke, tumour, trauma, drugs
Top reasons for liver transplantation
Cirrhosis, acute hepatitis (paracetamol, infectious), HCC
Top reasons for renal transplantation
HTN, Diabetes, ADPKD, chronic glomerulonephritis
Haemochromotosis- inheritance, signs, investigations, management
HFE gene, autosomal recessive
Diabetes, bronzed, slate-grey, venesection scars, hepatomegaly, signs of CLD, arthralgia, cardiomyopathy
Ferritin >200 females and >300 males, elevated t sats
Regular venesection, desferrioxamine, transplant, family screening, ETOH avoidance
UMN pattern, unilateral weakness
Tumour, stroke, hemisection, brain abscess
UMN pattern, bilateral, pure motor
Hereditary spastic paraparesis
UMN pattern, bilateral, mixed motor and sensory
MS, SCD, syringomyelia, transverse myelitis, Freidrich’s ataxia, cervical spondylosis
LMN pattern, pure motor, bilateral, proximal
Statins, steroids, hypothyroid, MG, LEMS, dermato/polymyositis, muscular dystrophy, MND, spinomuscular atrophy
LMN pattern, pure motor, bilateral, distal
Myotonic dystrophy, MND, polio
LMN pattern, pure motor, bilateral, asymmetric
Mononeuritis multiplex
LMN pattern, mixed motor and sensory, bilateral, proximal
CIDP
LMN pattern, mixed motor and sensory, bilateral, distal
Charcot-Marie-Tooth, diabetic peripheral neuropathy, alcohol related peripheral neuropathy, GBS, CIDP, paraneoplastic, cauda equina
LMN pattern, mixed motor and sensory, asymmetric
Radiculopathy, plexopathy, vasculitis, compression neuropathy, chemo
UMN signs
UMN: spasticity, hyperreflexia, clonus, positive Babinski. No fasciculations, mild wasting
LMN signs
LMN: flaccid, hyporeflexic / arreflexic, absent Babinski. Severe wasting with fasciculations
Dysphonia vs dysphasia vs dysarthia
Dysphasia - language disorder, inability to understand or generate speech
Dysarthia - speech disorder, articulation and pronunciation
Dysphonia - inability to properly formulate sound from the larynx
Causes of brainstem pathology
Vascular (ischaemic / haemorrhagic stroke)
Inflammatory (multiple sclerosis, acute disseminated encephalomyelitis)
Brainstem encephalitis (Bickerstaff’s encephalitis)
Central pontine myelinolysis
Trauma
Neoplastic (brainstem gliomas / ependymomas)
Arnold-Chiari malformation
Syringobulbia
Signs and causes of CNIII palsy
Ptosis, dilated pupil, down and out eye
Causes: posterior communicating artery aneurysm, DM, GCA, SoL, MS, cavernous sinus thrombosis, surgery, SLE, PAN
Signs and causes of CNVI palsy
Esotropia (permanent adduction of affected eye), paralysis of lateral gaze with diplopia
Causes: ischaemia, trauma, MS, SoL, stroke, sarcoid, GCA, raised ICP (false lateralising sign)
Signs and causes of CNVII palsy
Weakness of muscles of facial expression (forehead sparing if UMN lesion), hyperacusis
Causes: stroke, SOL, MS, lyme disease, sarcoid, syphilis, Bell’s palsy, Ramsay-Hunt (HSV- ear vesicles)
Signs and causes of cavernous sinus syndrome
Signs: painful ophthalmoplegia, orbital congestion, orbital chemosis, periorbital oedema, proptosis, ipsilateral Horner’s syndrome, ipsilateral loss of facial sensation
Causes:
Vascular: carotid artery aneurysm, carotid-cavernous fistulas, cavernous sinus thrombosis
Infections: sinuses
Tumours: nasopharyngeal carcinoma, craniopharyngioma, pituitary adenoma, mets, Inflammatory: sarcoidosis, GPA
Bulbar palsy- what is it, signs, causes
Disorder of lower cranial nerves (7-12)
Signs: nasal speech, slurred words, fasciculating and weak tongue, absent jaw jerk, reduced gag reflex, drooling, dysphagia
Causes: MND, myotonic dystrophy, myasthenia gravis, MSA, syringobulbia, poliomyelitis, leptomeningeal disease, radiation damage, nasopharyngeal carcinoma
Signs and causes of Horner’s syndrome
Signs: partial ptosis (overcome by upgaze and not fatigable), miosis, anhidrosis
Causes: stroke, syringomyelia, pancoast tumour, thyroid mass, cavernous sinus thrombus, ICA dissection,
Pathology, Signs of Freidrichs ataxia + inheritance
Damage to cerebellum, spinal cord and peripheral nerves. Causes mixed UMN/LMN signs
Autosomal recessive
Signs:
Progressive cerebellar ataxia
Hyporeflexia / arreflexia
Distal weakness, initially involving the lower limbs
Dorsal column loss, initially distal
Upgoing plantars
Dysphagia
Reduced visual acuity
Sensorineural hearing loss
Kyphoscoliosis
Pes cavus
Urinary incontinence
Cardiomyopathy
Diabetes mellitus
Signs of spinocerebellar ataxia + inheritance
Autosomal dominant
Signs:
Usually presents in third or fourth decade
Progressive cerebellar ataxia
Dysarthria, bulbar palsy
Increased tone
Hyperreflexia
Extensor plantar responses
May have peripheral neuropathy causing fasciculations and wasting
3 types of ataxia
- Cerebellar
- Sensory - loss of proprioception, romberg’s +ve, stomping gait
- Vestibular - vertigo, N&V
Describe sensory ataxia, signs and give causes
Loss of proprioception, near-normal coordination when the movement is visually observed by the patient, but marked worsening of coordination when the eyes are shut, pseudoathetosis, stomping gait, romberg’s positive
Causes
Peripheral neuropathy- alcohol, diabetes, nutritional deficiency
DCML disruption- tabes dorsalis, SCD, posterior column demyelination
What is pseudoathetosis and what is the cause
Sensory ataxia
Random finger movements seen on outstretched hands with eyes closed