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Showing posts with label MRCP notes. Show all posts

Pulmonary Hypertension

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Pulmonary hypertension is defined as a mean pulmonary artery pressure at cardiac catheterisation of  25mmHg or more.

Presentation tends to be progressive breathlessness, and as the condition worsens exertional dizziness and syncope.  Oedema and ascites are late signs.
On examination you might find a loud P2, a systolic murmur of TR, raised JVP, oedema or ascites.


It is classified into 5 groups (NB not all subsets of each of the groups are listed below, just the main ones):

1.  Pulmonary arterial hypertension
  • idiopathic (=IPAH)
  • heritable - including gene BMPR2
  • drug/toxin induced - eg fenfluramine, amphetamines, possibly interferon alpha and beta
  • CTD
  • HIV infection
  • portal HTN
  • schistosomiasis
  • pulmonary veno-occlusive disease

2.  Pulmonary hypertension due to left heart dysfunction

3.  Pulmonary hypertension due to lung disease/chronic hypoxia 

4.  Chronic thromboembolic pulmonary hypertension (CTEPH)
  • between 0.5 and 4% of patients develop CTEPH after PE

5.  Pulmonary hypertension with unclear multifactorial mechansms
  • haematological disorders - chronic haemolytic anaemia
  • sarcoidosis

Prevalence is high in:
  • systemic sclerosis (7-12%)
  • congenital heart disease (up to 10%)
  • portal hypertension (up to 6%)
  • HIV (0.5%)

 Investigations include: cardiac echo, CXR (cardiomegaly and prominent pulmonary vasculature), BNP, and the definitive investigation is right heart catheterisation.  Investigations would also be undertaken to look for the cause.

Treatment:
  • phosphodiesterase-5 inhibitors such as sildenafil or tadalafil
  • endothelin receptor antagonists such as bosentan
  • prostanoid infusions such as epoprostenol (avoid stopping as this can cause death)
  • possibly pulmonary endarerectomy in CTEPH
  • possibly high dose calcium channel blockade in IPAH



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Horner's Syndrome

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Horner's syndrome is the classic triad of:
  • ptosis
  • miosis
  • anhydrosis

Causes include:
  • stroke
  • lateral medullary syndrome (Wallenberg syndrome)
  • syrinx
  • carotid dissection or aneurysm
  • MS
  • Pancost tumour
  • trauma


Treatment depends on the cause.


Topical cocaine can be used to confirm Horner's syndrome; normal pupils dilate in response to topical cocaine while in Horner's they do not.


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Bronchiectasis

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Bronchiectasis describes persistent or recurrent bronchial sepsis related to irreversibly damaged and dilated bronchi (BTS definition)

It presents as:
  • chronic cough with large volumes of sputum
  • possibly haemoptysis

Clinically look for finger clubbing.
 
Causes of bronchiectasis include:
  • infective: post severe pneumonia, pertussis, TB
  • inflammatory: post gastric aspiration
  • congential: CF, Kartageners
  • immune-mediated
Conditions associated with bronchiectasis include:

Investigations:
  • serum immunoglobulins and electrophoresis
  • IgE to aspergillus fumigatus and aspergillus precipitins
  • CF investigations - sweat chloride and CFTR genetic mutation analysis
  • cilary investigation - saccharin test
  • bronchoscopy to exclude proximal obstruction
  • baseline CXR
  • HRCT - look for 'signet ring' = dilated bronchi larger than adjacent vascular bundle
  • sputum culture
  • lung function tests

Treatment:
  • physiotherapy - airway clearance techniques
  • bronchodilators if any obstruction
  • antibiotics for exacerbations
    • first line: amoxicillin, or clarithromycin if penicillin allergic
    • ciprofloxacin for those colonised with pseudomonas 
  • long-term antibiotics if 3 or more exacerbations per year; nebulised antibiotics should be considered
  • lung resection may be considered if localised disease and symptoms not controlled with medical management.

Complications
  • massive haemoptysis
  • cor pulmonale

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Interstitial Lung Disease

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Interstitial lung disease describes a group of lung diseases which affect the lung interstitium (= the tissue of the alveolar wall between the capillary endothelium and the alveolar epithelium) and share similar clinical and radiological features.

Diffuse parenchymal lung disease is synonymous with interstitial lung disease.


In 2002 the European Respiratory Society and American Thoracic Society produced the following classification of interstitial lung diseases:

  • ILD of known cause or association
    • Environmental - eg bird exposure
    • Occupational - eg silicosis, asbestosis
    • Drug-associated - eg amiodarone, methotrexate, cyclophosphamide, nitrofurantoin
  • ILD of unknown cause 
    • Idiopathic interstitial pnuemonias
      • Idiopathic pulmonary fibrosis (previously known as crytogenic fibrosing alveolitis)
      • IIP other than IPF eg crytogenic organising pneumonia,
    • ILD associated with connective tissue diseases eg RA, scleroderma, polymyositis
    • Granulomatous ILD eg sarcoidosis
    • Other forms, eg histiocytosis X, eosinophilic pneumonias


Presentation tends to be:
  • progressive breathlessness with exercise
  • persistent non-productive cough


Investigations:
  • CXR - may be normal; may show increased reticular markings, ground glass abnormalities or nodular opacities
  • HRCT 
    • honeycombing - pulmonary fibrosis
    • ground glass changes - hypersensitivity pneumonitis
    • distribution of changes can also help diagnosis
  • lung function tests 
    • most commonly show a restrictive pattern with decreased lung volume and diffusing capacity for carbon monoxide
    • however patterns can vary
  • bronchoscopy
    • can help exclude infections
    • transbronchial biopsies - may diagnose sarcoidosis/hypersensitivity pnuemonitis
  • surgical biopsy
    • most commonly via VATS (video-assisted thoracic surgery)


Management
  • pulmonary rehabilition
  • smoking cessation
  • ? oxygen therapy
  • details depend on the type of interstitial lung disease, for example:
    • idiopathic pulmonary fibrosis - ?  N-acetylecysteine
    • sarcoidosis - ?prednisolone if pulmonary function badly affected
    • hypersensitivity pneumonitis - avoidance of allergen


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Alcoholic hepatitis

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Alcoholic hepatitis is a clinical syndrome characterised by recent onset of jaundice and/or ascites in a patient with ongoing alcohol misuse.

The predominant cause of alcoholic hepatitis is alcoholic steatohepatitis, which is diagnosed histologically by the presence of steatosis, hepatocyte ballooning and inflammatory infiltrate with PMNs.

Presentation may include:
  • progressive jaundice
  • ascites
  • fever 
  • weight loss
  • hepatolomegaly
  • anorexia
  • nausea and vomiting
  • abdominal pain

Investigations:
  • LFTs
    • AST typically 2-6x upper limit of normal
    • AST/ALT ratio >2
    • bilirubin frequently raised
  • clotting
  • FBC
  • screen for HBV, HCV, HIV
  • abdominal US to exclude other causes of jaundice
  • consider transjugular liver biopsy

Several scoring systems exist to assess prognosis (Maddrey's discriminant function, MELD, ABIC, Glasgow).  Maddrey's is the commonest:

Maddrey discriminant factor = (4.6 x prolongation of PT) + serum bilirubin (mg/dl) 
(NB in UK bili is given in micromol/l so needs to be divided by 17)

>32 indicates poor outcome.

Management of high-risk patients:
  • prednisolone 40mg OD 
    • or pentoxifylline (a phosphodiesterase inhibitor and TNF alpha inhibitor) 400mg TDS if patient septic
  • ? enteral feeding
  • ? n-acetylcysteine in patients with severe disease receiving steroids
  • careful monitoring of renal function  
  • careful watching for infection and prompt treatment if present

Complications
  • GI bleeds requiring transfusion occur in around 20%
  • encephalopathy occurs in around 20%
  • hepatorenal syndrome


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Sturge-Weber Syndrome

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Sturge-Weber Syndrome is the association of a port-wine haemangioma in the trigeminal nerve distribution area (predominantly the opthalmic branch) with a vascular malformation of the brain.

Possible associated features include:
  • glaucoma
  • epilepsy
  • mental retardation
  • hemiparesis
Management may include neurosurgery.


However, it is worth noting than only around 3% of patients with a facial port-wine stain have Sturge-Weber syndrome.

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Ankle-brachial pressure index

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The ankle-brachial pressure index is calculated by:

ABPI = (ankle systolic BP)/(brachial systolic BP)


Interpretation of results is not entirely straightforward as various consensus papers give different cut -offs for the definitions of 'moderate' and 'severe'.  One set of definitions is:
  • >1.4: calcification may be present
  • >1.0 probably no arterial disease
  • 0.81-1.0 no significant arterial disease
  • 0.5-0.8 moderate disease
  • <0.5 severe disease
  • <0.3 critical ischaemia

It is worth noting that an ABPI <0.9 or of >1.4 is an independent risk factor for cardiovascular disease.

ABPI may be non-diagnostic in patients with heavy arterial calcification, such as:
  • diabetics (5-10% of diabetic patients cannot have their occlusive ankle pressure measured)
  • the elderly
  • renal failure

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Primary Sclerosing Cholangitis

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Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease, likely autoimmune.

It most commonly affects men (3:1) and mean age of diagnosis is 40.

At presentation up to 55% of patients are asymptomatic.  Possible symptoms include:
  • pruritis
  • fatigue
  • abdominal pain
  • jaundice
Investigations:
  • LFTs - raised ALP, mild to moderate increases in AST/ALT
  • Autoantibodies
    • ANCA positive in 80% - but not specific
    • ANA - 20-60%, again not specific
    • SMA - 20-60%, not specific
    • (AMA is rarely positive - far more associated with PBC)
  • MRCP - gold standard
  • ERCP
    • shows strictures of biliary tree with characteristic 'beaded' appearence
  • Liver biopsy
    • not necessarily helpful
    • may reveal concentric periductal fibrosis (='onion skinning')

Complications
  • cholangiocarcinoma - up to 30%
  • colorectal cancer

Associations:
  • 80% of patients with PSC have ulcerative colitis (although only 4% of patients with UC have PSC)
  • 7-50% of patients with PSC also have autoimmune hepatitis
Treatment:
  • ursodeoxycholic acid
  • colestyramine or rifampicin for pruritus
  • liver transplant
    • but up to 40% recurrence of PSC in organ
    • median time from diagnosis to transplant needed: 18 years
  • ERCP to stent strictures
  • (yearly colonoscopy if UC)

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Acute promyelocytic leukaemia

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Acute promyelocytic leukaemia is the M3 subtype of AML

Features include:
  • translocation t(15;17) that produces the PML-RARα gene
  • bundles of auer rods (=faggots) 

It predisposes to DIC, but as long as this is treated its prognosis is good with treatment with all trans retinoic acid or arsenic trioxide.

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Progressive multi-focal leucoencephalopathy

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Progressive multi-focal leucoencephalopathy (PML) is a demyelinating disease of the central nervous system caused by the JC virus.  Despite many people being infected by the JC virus PML is very rare, generally only occurring in association with conditions such as:
  • HIV
  • CLL
  • patients on natalizumab

Presentations include:
  • motor weakness
  • impaired vision
  • mental changes
  • speech abnormalities
  • seizures

Investigations
  • CT: subcortical hypodensities
  • MRI: hyperintense lesions on T2-weighted images, hypointense on T1-weighted images
  • CSF: often normal; do PCR on CSF for JC virus

Management:
  • in patients with HIV: start HAART
  • in patients on natalizumab: stop natalizumab and initiate plasmapheresis

Prognosis: generally very poor. 


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Angioid Retinal Streaks

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Angioid retinal streaks are caused by breaks in Bruch's membrane.

Conditions associated with angioid retinal streaks include:

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Management of Atrial Fibrillation

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Atrial Fibrillation is the commonest sustained cardiac arrhythmia, occurring in 1-2% of the population.  Its incidence increases with age: <0.5% of the population are affected aged 40-50 but up to 15% are affected by the age of 80.

This article focuses on the management of AF, looking at:
    A) Management of permanent/persistent AF
    B) Management of paroxysmal AF 


A) Management of permanent/persistent atrial fibrillation

This requires consideration of 2 questions:
       1) rhythm control or rate control?
       2) to anticoagulate or not?

So to consider question 1:
  • Rhythm control?
    • NICE suggests rhythm-control first in:
      • younger patients
      • those with symptoms
      • presenting for the first time with lone AF
      • those with AF secondary to a treated or correct precipitant
      • with congestive heart failure
    • Treatment options are:
      • DC cardioversion
      • Chemical cardioversion 
        • amiodarone, sotalol or flecanide (the latter only in a structurally normal heart)
  • Rate control?
    • NICE recommends trying rate-control first in:
      • over 65s
      • patients with coronary artery disease
      • in those with contraindications to antiarrhythmic drugs
      • in patients who are unsuitable for cardioversion
        • left atrium >5.5cm
        • mitral stenosis
        • contraindications to anticoagulation
        • longstanding AF (>12 months)
        • multiple failed attempts at cardioversion 
    • Treatment options are:
      • beta blocker
      • rate-limiting calcium antagonist
      • digoxin

And then there is question 2: to anticoagulate or not:
  • You can stratify risk using either the NICE stroke risk stratification (2006) or the CHA2DS2-VASc score (2010):
    • Using the Nice Stroke Risk stratification:
      • Define the 'risk' in the patient:
        • High Risk
          • Previous ischaemic stroke/TIA/thromboembolic event
          • age >/= 75 with hypertension, diabetes or vascular disease
          • clinical evidence of valve disease, heart failure or LV dysfunction
        • Medium Risk
          •  age >/=65 with no high risk factors or age <75 with hypertension, diabetes or vascular disease
        • Low Risk
          • age <65 with no high or moderate risk factors
      • Unless contraindications, anticoagulate all high risk patients with warfarin (target INR 2.5) and all low risk patients with aspirin.
      • Consider whether to use warfarin or aspirin in moderate risk patients on an individual basis
      • NB: 2012 European Society of Cardiology guidelines suggest that use of aspirin monotherapy should be avoided now there are 'novel oral anticoagulants'
    • Using the CHA2DS2-VASc score
      • Depends on points
        • Congestive heart failure/LV dysfunction: 1 point
        • Hypertension: 1 point
        • Age >/= 75: 2 points
        • Diabetes: 1 point
        • Stroke/TIA/thromboembolism: 2 points
        • Vascular disease (= MI, PVD, aortic plaque): 1 point
        • Age 65-74: 1 point
        • Sex catagory: if female, 1 point
      • Any score of 1 or more warrants anticoagulation
  • The world of AF anticoagulation has recently got far more complex, as in addition to warfarin and aspirin you must now consider dabigatran and rivaroxaban:
    • Dabigatran (= oral direct thrombin inhibitor)
      • can be considered instead of warfarin in patients with nonvalvular AF who have one or more of the following risk factors:
        • previous stroke/TIA/systemic embolism
        • LVEF <40%
        • HF NYHA class 2 or above
        • age 75 or older
        • age 65 or older with diabetes, hypertension or coronary artery disease
      • The trial that proved non-inferiority compared to warfarin was the RE-LY trial
    • Rivaroxaban (mechanism of action: directly inhibits factor Xa) 
      • can be considered instead of warfarin in patients with nonvalvular AF who have one or more of the following risk factors:
        • previous stroke/TIA
        • hypertension
        • congestive cardiac failure
        • age 75 or older
        • diabetes mellitus 
      • The trial that proved non-inferiority compared to warfarin was the ROCKET-AF trial

DC Cardioversion
  • Anticoagulation:
    • Before cardioversion, patients should be on warfarin with an INR of 2-3 for at least 3 weeks.
    • After successful cardioversion, they should remain on warfarin with an INR of 2-3 for at least 4 weeks.
    • If cardioversion cannot be postponed for 3 weeks, give heparin beforehand and warfarin for at least 4 weeks afterwards
    • If the duration of AF is confirmed as less than 48 hrs no anticoagulation pre-cardioversion is needed.
  • If previous DCCV was unsuccesful, give amiodarone or sotalol for at least 4 weeks prior to subsquent cardioversion attempt
  • If no cause for the AF identified, consider medications post cardioversion to help maintain sinus rhythm:
    • beta blocker first line
    • if beta blocker fails:
      • sotalol or flecainide if heart structurally normal
      • amiodarone if not structurally normal

 B)  Management of paroxysmal AF

Again, this requires consideration of 2 questions:

1) Pill in pocket therapy or 'normal' ongoing treatment?
2) To anticoagulate or not?

To answer question 1:
  • 'Pill in pocket' therapy is used if:
    • Patient has no LV dysfunction, valvular heart disease or IHD
    • Patient have infrequent symptomatic episodes
    • Patient has a SBP >100 and resting HR >70
    • Patient is able to understand how to use this approach
  • If all of the above criteria are not fullfilled:
    • give standard beta blocker
    • if this fails
      • try sotalol in CAD
      • amiodarone in LV dysfunction
      • flecanide in a structurally normal heart
    • If sotalol/flecanide fails, amiodarone can be trialled

The method of determining whether to anticoagulate or not is exactly the same as in permanent AF.


 Other points
  • Post-TIA, give warfarin as soon as possible once bleed/infarct has been excluded
  • Post stroke, start warfarin 2 weeks afterwards as long as no bleed/large infarct on CT



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IgA Nephropathy

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IgA nephropathy, also known as Berger's disease, is the commonest glomerulonephritis.

The classical patient is a young man with haematuria after an upper respiratory tract infection.

Overall micro or macrohaematuria is the presenting feature in around 40% of cases.

Histologically there is mesangial hyperplasia and on immunofluorescence there is IgA and C3 deposition.

It is often a subclinical condition, but amongst those for whom it becomes clinically apparent there is around 20% progression to end stage renal failure at 20 years.

A poorer prognosis is associated with
  • male gender (the majority of sufferers are male)
  • proteinuria
  • obesity 

Associated conditions include

Management
  • Angiotensin 2 inhibitor or ACE-i
  • if this fails, ?immunosupression
  • in renal failure consider transplant, although IgA nephropathy recurs in the majority of grafts

 Henoch Schonlein Purpura is sometimes regarded as the systemic manifestation of IgA nephropathy.

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Membranous glomerulonephritis

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Membranous glomerulonephritis is usually regarded as the commonest cause of nephrotic syndrome in the western world (although focal segmental glomerulonephritis is sometimes now regarded to have overtaken it.)

It is characterised by a thickened basement membrane on microscopy and IgG and C3 deposits on immunofluorescence. 

It may be primary or secondary to:
  • drugs
    • captopril
    • NSAIDs
    • penicillamine
    • gold
    • clopidogrel
  • mercury
  • infections
    • HBV
    • HCV
    • malaria
    • schistosomiasis
    • TB
  • cancer
  • autoimmune conditions

Treatment
  • management depends on the risk of progression
    • low risk patients are managed conservatively (BP control and ACE-i)
    • higher risk patients are given corticosteroids and cyclophosphamide

Prognosis
  • a third remit spontaneously
  • the remainder either develop persistent proteinuria or progress to chronic renal failure


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Focal segmental glomerulonephritis

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Focal segmental glomerulonephritis is the commonest cause of nephrotic syndrome in afro-american adults.

Histologically there are focal areas of sclerosis and on immunofluorescence there is IgM and C3 deposition.

Focal segmental glomerulonephritis may be primary or secondary to:
  • reflux nephropathy
  • obesity
  • Alport's syndrome
  • sickle cell disease
  • HIV
  • heroin use
  • IgA nephropathy

Treatment is:
  • corticosteroids
  • if steroids fail, ? ciclosporin
  • if all fails, kidney transplant
    • but note that focal segmental glomerulonephritis can recur in the transplanted kidney

Prognosis
  • around a third of patients respond to steroid treatment and recover
  • around 50% progress to end-stage renal failure



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Contrast radiology

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There are 3 main risks associated with using contrast:

1) Allergic reactions
  • Reactions occur in 0.15% of cases.  These are classified as 'severe' in just 0.04% of cases.
  • Allergic reactions are 6 to 10 times more common in asthmatics; non ionic low or iso-osmolar agents should be used for these patients.

2) Contrast nephropathy
  • This is defined as an impairment in renal function (an increase in serum creatinine by more than 25% or 44micromol/l) within 3 days of administration of IV contrast in the absence of an alternative aetiology
  • Risk of contrast nephropathy is increased in patients with:
    • eGFR <60
    • dehydration
    • CCF
    • gout
    • age >70
    • concurrent use of nephrotoxic drugs, eg NSAIDs or gentamycin
  • The risk of contrast nephropathy can be reduced by:
    • using the smallest possible dose of contrast
    • ensuring the patient is well hydrated before and after the procedure either orally or, if the contrast is to be given arterially or at high dose, by IV fluids
    • there is currently insufficient evidence to support the use of n-acetyl cysteine in prevention of contrast-induced nephropathy
  • There is no need for metformin to be stopped routinely for contrast investigations as long as the eGFR is >60.  If it is below this level, stopping for 48 hrs before and after the investigation should be considered.

3) Extravasation
  • Conservative management is sufficient in most cases - elevate limb and apply ice packs.
  • Plastic surgeon input may occassionally be required.

Other points:
  • Contrast can be used in breastfeeding mums with no special precautions.
  • Contrast cannot be used in patients who are hyperthyroid.
  • There is a specific risk of a delayed skin rash if contrast is given to patients on interleukin-2 therapy.




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Budd Chiari Syndrome

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Budd-Chiari syndrome is a group of disorders characterised by hepatic venous outflow obstruction.  The obstruction may occur anywhere from the hepatic venules to the right atrium.

 As a result of this obstruction there is increased hepatic sinusoidal pressure, leading to:
  • portal hypertension
  • liver congestion
  • decreased liver perfusion

 For clinical disease to occur, 2 veins must be blocked. Clinical symptoms/signs include:
  • RUQ pain
  • ascites
  • hepatomegaly

Causes of Budd-Chiari syndrome:

 Investigation
  • doppler ultrasound - assessment of hepatic venous flow - sensitivity/specificity >85% - first line
  • MR - second line
  • CT

 Management
  • medical
    • anticoagulant therapy
    • manage ascites - spironolactone
  • surgical
    • portosystemic shunt - typically a transjugular intrahepatic portosystemic shunt (TIPS)
    • liver transplant

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Status epilepticus

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Status epilepticus is a prolonged seizure, usually defined as >30 minutes.

Over half of patients presenting with status epilepticus do not have known epilepsy.

The main acute causes in adults are:
  • stroke
  • hypoxia
  • metabolic derangement
  • alcohol intoxication or withdraw
In children, the leading cause is infection.

Management:
  • secure airway and resuscitate
  • give oxygen
  • anti-epileptics:
    • pre-hospital: diazepam 10-20mg PR or midazolam 10mg buccally
    • first line in hospital: lorazepam (or diazepam) IV
      • terminates around 75-80% of seizures
    • if benzo unsuccessful: phenytoin infusion 15-18mg/kg at a rate of 50mg/minute 
      • will terminate around 50% of resistant seizures
  • if any suggestion of alcohol abuse or impaired nutrition give 50mls of 50% glucose and/or 250mg IV thiamine
  • if status is refractory (ie not resolved 60-90 minutes after initial anti-epileptic) anaesthetise using propofol, midazolam or thiopental sodium.

Prognosis: mortality of 10-20% rising to nearly 50% in refractory status epilepticus. 


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Yaws

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Yaws is a tropical disease of the skin caused by treponema pallidum subspecies pertunue.

It is spread by direct skin contact with an infected person or insect bite.

Yaws presents as:
  • an initial papule/papilloma that may persist for 3-6 months
  • if untreated, a late stage with multiple lesions, disfigurement of nose and bones and palmar/plantar hyperkeratosis.

Diagnosis is by dark light microscopy, rapid plasma reagin (RPR) or VDRL.  Note that it and syphilis give the same positives due to the close relationship of the causative organism.

Treatment is a single IM injection of benzylpenicillin.

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McArdle's disease

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McArdle's disease is a type V glycogen storage disease caused by a deficiency of glycogen phosphorylase in muscle.  It is characterised by:
  • muscle cramps on exertion
  • dark urine due to myoglobin
  • absence of normal rise in serum lactate on exertion

Ingestion of sucrose during exercise may help improve symptoms.

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Secret collector of interesting anonymised ECGs. Fan of the Bath Photomarathon. Lover of cream teas. [Sarah Hudson] (Your Picture)