Abdominal Flashcards
Management of coeliac disease?
Patient education
Primary management is the avoidance of gluten (wheat, rye and barley-containing foods)
Dietician input
Gluten-free food prescriptions
Vitamin supplements if needed
NB: around 30% of patients with coeliac disease have hyposplenism, consider influenza, meningococcal and pneumococcal vaccines
How do we diagnose coeliac disease?
Must enquire if the patient is on a diet containing gluten or not.
Gluten-free diet can make TTG normal.
If patient is on a gluten-containing diet and coeliac disease is suspected, the gold standard diagnostic test is an OGD with biopsies from the D2 region.
Looking for subtotal or total villous atrophy.
Investigations for coeliac disease?
Bedside: obs, history, urine dip and MC+S
Bloods: FBC (anaemia), haematinics (cause of anaemia), WCC, CRP (infection), UE, LFTs
Specific to coeliac: anti-TTG antibody test and screen for other AI conditions (TFTs)
Imaging: OGD and biopsies
Differential diagnosis of coeliac disease?
Inflammatory bowel disease
Infective cause
Other autoimmune diseases such as thyroid disease
What clinical findings would you expect in a patient with coeliac disease?
Often a normal abdominal examination.
Important negatives include:
- lack of signs of anaemia
- no lymphadenopathy
- no signs of cachexia
Investigations for suspected IBD?
Bedside: obs, stool culture for microscopy, culture and c. diff toxin, faecal calprotectin
Bloods: FBC, B12 and folate (malabsorption), UEs, CRP, LFTs (liver abnormalities as an extra-intestinal manifestation of iBD or PBC / PSC / AIH)
Imaging: AXR (rule out toxic megacolon), sigmoidoscopy / colonoscopy + biopsy for histological confirmation, MRI entercolysis if Crohn’s is suspected (to detect small bowel strictures)
What are the differences between Crohn’s and UC?
Crohn’s has skip lesions, UC is continous
Crohn’s is transmural, UC is superficial
Crohn’s affects the whole GI tract, whereas UC affects the large bowel (predilection for rectum)
Smoking increases your Crohn’s risk, whereas it decreases your UC risk
In Crohn’s, fistulae and stenosis is common, whereas it is rare in UC
What are some emergency indications for surgery in IBD?
Toxic megacolon
Haemorrhage
Perforation
What screening tests should be considered prior to starting biologic therapy for IBD?
History and exam (previous TB)
Hepatitis serology
HIV test
CXR (TB)
T-spot test for TB
Name some biologics used in IBD.
UC: infliximab and golimumab
Crohn’s: infliximab and adalimumab
(Vedolizumab is used for both CD and UC in patients who fail first-line treatment)
What extra-intestinal manifestations of IBD can occur?
Skin: erythema nodosum, pyoderma gangrenosum, aphthous ulceration
Joints: seronegative arthritides
Eye: uveitis, scleritis / episcleritis
HPB: PSC in UC, cholangiocarcinoma
Renal: oxalate stones
Name some surgeries that are commonly performed in cases of IBD.
Subtotal colectomy with end ileostomy
End ileostomy with mucous fistula (usually emergency)
Proctocolectomy and end ileostomy
Proctocolectomy with ileo-anal pouch reconstruction
What stomas might be seen in a patient with IBD?
Stoma in RIF: likely ileostomy, contents are semi-liquid and stoma is spouted
Stoma in LIF: likely a colostomy, solid contents, flush to skin
Also look for other scars associated with prior abdominal surgery (e.g. mucous fistula scar)
Ask patient to cough to assess for a parastomal hernia
Management of IBD?
Medical:
Steroids (in acute-severe disease, IV hydrocortisone)
5-ASA (standard of care to maintain remission)
Disease-modifying agents: azathioprine, MTX, mercaptopurine
Biological agents: for patients who have 2 or more flares on 5-ASA requiring steroids
Surgical:
For fistulae, strictures, failure to respond to medical therapy, haemorrhage, perforation, toxic megacolon
Other:
Nutritional support (elemental and low-residue diet)
Psychological support
Tell me about the drainage of a transplanted pancreas.
Traditionally, they were drained into the bladder so lipase could be measured in patient’s urine to assess for rejection, however increased incidence of UTIs.
More recently, transplanted pancreases are attached to the small bowel and drained via the bowel.
What different types of renal transplant are available?
Live donors: altrustic or related to the patient
Cadaveric donors: after circulatory or neurological death
What is the benefit of a simultaneous kidney pancreas transplant (SPK)?
A combined kidney and pancreas transplant intends to cure both the patient’s ESRF and diabetes.
This means the patient can be free of haemodialysis and insulin therapy.
Reduces risk of vascular complications of diabetes too.
Type 1 diabetics typically receive combined transplantation, but more recently have been offering them to T2 diabetics who meet criteria and are insulin-dependent.
What are the different causes of jaundice?
Can be divided into:
Pre-hepatic: haemolytic anaemia, drugs, Gilbert’s syndrome
Hepatic: viral hepatitis, alcohol, NAFLD, autoimmune hepatitis, biliary malignancy
Post-hepatic: gallstones, CBD stricture, extra-hepatic malignancy, pancreatitis
Management of ascites?
Diagnostic or therapeutic ascitic tap - calculate SAAG, send for cytology and culture
Limit salt intake to 5 - 6.5 g/day
Spironolactone
Give albumin
Consider TIPSS in refractory ascites
Treat SBP with IV antibiotics (ciprofloxacin)
Acute management of upper GI bleed?
Use BSG’s acute upper GI bleeding bundle:
A to E assessment, stabilise patient, IV crystalloid
Transfuse if Hb <70 g/L
Calculate Glasgow-Blatchford score (consider discharge if 0 - 1)
If suspected variceal bleed, give terlipressin + antibiotics
OGD within 24h
If haemodynamically unstable, major haemorrhage protocol +/- critical care review
Define ‘acute liver failure’
Loss of liver function in days or weeks (<6 mo)
In a patient WITHOUT pre-existing liver disease
Acute liver injury, encephalopathy and raised INR
Investigations in a patient with chronic liver disease?
Bedside: full history including PMH, drug / alcohol / FH / travel history
Bloods: FBC, UEs, LFTs including GGT, coagulation screen, BBV screen, ferritin, immunoglobulins, caeruloplasmin, autoantibody screen
Imaging: USS abdomen, CT +/- biopsy. Test for fibrosis with fibroscan (usually as OP)
Which autoantibodies are tested for on an autoantibody screen in the context of CLD?
ANA
AMA
Smooth muscle
LKM antibody
Tumour markers (AFP for HCC)
What autoantibodies are associated with PBC?
Positive anti-mitochondrial antibody.
Raised IgM level.
What are the causes of CLD?
Most common 3 causes are:
1. Alcohol
2. NAFLD
3. Viral hepatitis
Other important causes are:
- Autoimmune: AI hepatitis, PBC, PSC
- Haemochromatosis, Wilson’s, A1AT deficiency
- HHT
- Drug causes
Why do we calculate the SAAG in ascites and what does it tell us?
The SAAG (serum ascites albumin gradient) allows us to differentiate between portal hypertensive and non-portal hypertensive causes of ascites.
Portal hypertensive causes have a SAAG >11 g/L:
- CCF
- Portal vein thrombosis / Budd-Chiari
- Cirrhosis
Non-portal hypertensive causes have a SAAG <11 g/L:
- TB
- Malignancy
- Pancreatitis
- Nephrotic syndrome
How is PBC managed?
Treatment of choice - ursodeoxycholic acid
Smoking cessation
Liver transplantation
How do patients with PBC present?
Fatigue and pruritus are most common
Can progress to CLD + cirrhosis
There is a link with HCC
What are the features of hepatic decompensation?
‘JAVEC’
Jaundice
Ascites
Variceal haemorrhage
Encephalopathy
Coagulopathy
Name some absolute contraindications to liver transplantation?
Severe irreversible pulmonary disease
Severe extrahepatic disease with high mortality
Ongoing alcohol / drug use
Active or previous extra-hepatic malignancy
Indications for liver transplant
Acute liver failure: paracetamol, hepatitis (viral or ischaemic)
Chronic liver failure:
- Alcoholic liver disease
- NAFLD
- Chronic viral hepatitis
- Autoimmune: PSC, PBC, AIH
- Genetic: Wilson’s, haemochromatosis, A1AT deficiency
Why is the use of haemodialysis preferable to PD in a patient with polycystic kidney disease?
The bulk of PKD and the fluid required in the peritoneal cavity can cause discomfort to patients.
Having PD increases the risk of cyst infection, which can lead to a number of complications.
What are the potential complications of dialysis?
HD: dialysis washout, infection, bleeding, B2 microglobulin amyloidosis, psychological
PD: peritonitis, diabetes, local complications (catheter site infections, hernias)
Why might a patient have a tunneled line rather than an AV fistula?
Tunnelled (Tesio) lines are usually a temporising measure whilst waiting for an AV fistula to mature.
Can also be created as emergency access for haemodialysis.
Tunnelled lines are prone to infection, have a shorter lifespan and lower blood flow rates, so dialysis takes longer.
What are the differences between haemodialysis and peritoneal dialysis?
HD:
- Takes place via a fistula / tunneled line
- 3x a week, for 4 hours each time
PD:
- Takes place via a catheter leading into the abdominal cavity
- Toxins diffuse across the peritoneum into the diasylate fluid
- Fluid is either removed as the patient sleeps (automatic PD) or at various points through the day (continuous ambulatory PD)
What are the most common causes of ESRD?
Diabetes
Hypertension
ADPKD
Glomerulonephritis
TIP: try to work out WHY the patient has developed ESRD
What investigations would you request for a patient with AKI / CKD of unknown cause?
Bedside: urine dip (blood / protein)
Bloods: FBC / LFT / UE / clotting / WCC and CRP, PSA (obstructive uropathy), ANA, ANCA, anti-GBM, complement, myeloma screen, immunoglobulins, blood cultures
Imaging: CXR (infection, pulmonary haemorrhage), renal USS (obstruction / size / cysts), biopsy if cause remains unclear
Management of CKD / ESRF
MDT approach
Address underlying cause / reversible causes (diabetes, GN, HTN)
BP control with ACEi / ARB
Treatment of complications: renal osteodystrophy, anaemia, secondary / tertiary hyperparathyroidism
Early dialysis / transplant planning - ‘predialysis clinic’ with doctors / nurses / dieticians / psychologists to start working patient up for transplant
What are some barriers / contraindications to renal transplant?
Donor matching
Active / recent malignancy
Ongoing deep-seated infection
Active vasculitis
Severe obesity due to technical difficulty
In terms of renal function, define ESRF?
A patient who has an eGFR of less than 15ml per minute.
When would you start working a patient up for renal transplantation?
As they approach ESRF, but before they require dialysis.
When they receive transplantation is based on the availability of donors.
Transplanting patients before they end up on dialysis carries a better prognosis.
What clinical signs would you expect if your patient has had an SPK?
Lower midline abdominal incision, with palpable kidney in iliac fossa but no overlying scar.
Evidence of previous diabetes.
Patients often younger, transplanted in their 30s-40s.
What problems can follow renal transplantation?
Rejection: acute / chronic
Infection: pneumocystis / CMV / BK virus
Skin malignancies
Graft dysfunction
Hypertension and high lipids - CVD
Immunosuppression side effects / toxicity
Recurrence of disease
List some side effects of long-term immunosuppression.
Infection / malignancy
Steroids: skin thinning, easy bruising, Cushing’s syndrome
Ciclosporin: gingival hyperplasia, hirsuitism, renal impairment
Tacrolimus: tremor and diabetes
What are the signs of a failing renal transplant?
Declining renal function
Proteinuria
Tenderness over graft site
Fluid overload
When presenting a renal transplant patient, which 3 things are important to state?
- Underlying reason for renal failure
- Current RRT modality
- Complications of past / current treatment
If you suspect haemochromatosis, which investigations would you like to perform?
Ferritin levels (raised)
Transferrin saturation (raised)
To make diagnosis, need HFE gene testing
List some causes of gum hypertrophy.
Drugs: ciclosporin, phenytoin, nifedipine
Scurvy
Acute myelomonocytic leukaemia
Pregnancy
Familial
If you saw jaundice in the context of a liver transplant patient, what would you be thinking of?
This raises the question of graft dysfunction or extrahepatic causes
Need to do a conjugated and unconjugated bilirubin
How are patients selected for liver transplant?
MDT approach
Patient needs to have significant liver dysfunction
Certain criteria and guidelines which are used to predict liver prognosis (UK End Stage Liver Disease Model)
What are the top 3 reasons for needing liver transplantation?
Cirrhosis (haemochromatosis, NAFLD, AI liver disease, viral hepatitis)
Acute hepatic failure (hep A, B, paracetamol)
Hepatic malignancy (HCC)
What are the survival rates of patients with a liver transplant?
80% 1-year survival
70% 5-year survival
What signs / symptoms may a patient with ADPKD present with?
Hypertension
Signs of renal failure
Proteinuria / haematuria
Extrarenal manifestations of ADPKD such as liver cysts, pancreas, spleen, epididymis, thyroid
Loin pain: due to renal haemorrhage, calculi or infection
What is the inheritance modality of ADPKD?
Autosomal dominant condition with 2 main associated genetic mutations.
80% of patients - mutation on chromosome 16
15% of patients - mutation on chromosome 4
The remaining 5% have no detectable genetic abnormality
PKD 2 is associated with less severe disease with later onset. Fewer cysts and later progression to renal failure.
Important positive and negative signs when presenting a patient with suspected ADPKD?
Positive signs: bilateral ballotable masses in the flanks, which I can get above.
Negative signs: no evidence of kidney disease. The patient is euvolaemic and has no evidence of previous or current RRT.
What are the extrarenal manifestations of ADPKD?
Hypertension
Cysts in liver, pancreas, epididymis
Colonic diverticulae
Cardiac valve disease
Abdominal / inguinal herniae
Most significant = IC Berry aneurysms which may present with intracranial or subarachnoid haemorrhage. Rupture is associated with uncontrolled HTN
What are the indications for nephrectomy in a patient with ADPKD?
In general, should be avoided and the old kidney is usually left in situ.
However, may be required in cases of:
- To make room for a transplanted kidney (volume in abdomen)
- RCC
- Chronic pain
- Chronic infection
- Significant haematuria
What is the management of ADPKD?
Management is multi-modal, and implemented by the MDT
- BP control with ACEi’s
- Control of hyperlipidaemia (as CKD = major risk of IHD)
- Tolvaptan may be of use earlier in the disease
- May eventually require RRT or transplant
- High fluid, low-salt diet
Differentials for unilateral kidney enlargement?
PKD
Renal cell carcinoma
Renal cysts
Hydronephrosis
Differentials for bilateral kidney enlargement?
PKD
Bilateral RCC
Bilateral hydronephrosis
Tuberous sclerosis
Amyloidosis
Indications for splenectomy?
Rupture (trauma)
Haematological (ITP and hereditary spherocytosis)
What forms part of the splenectomy workup?
Vaccination: 2 weeks prior against encapsulated bacteria - pneumococcal, flu, meningococcal
Lifelong penicillin
Medic alert bracelet
Causes of massive splenomegaly?
Massive = >8cm ‘MMM’
CML
Myelofibrosis
Malaria / visceral leishmaniasis
Causes of moderate splenomegaly? (4 - 8 cm)
Myelo / lymphoproliferative disorder
Gaucher’s disease
Amyloidosis
Causes of an enlarged splenic tip? (<4 cm enlargement)
Myelo / lymphoproliferative disorders
Portal hypertension
Infection (EBV, IE)
Haemolytic anaemia
What clinical findings would you expect in a patient who has had a splenectomy?
Scar in LUQ / midline
Laparascopic scars ?cholecystectomy (in severe cases of hereditary spherocytosis)
Pallor (anaemia)
Jaundice
Tell me about hereditary spherocytosis.
Autosomal dominant pattern of inheritance
Results from genetic alteration of 1 of the 5 genes that encode the RBC membrane proteins
This leads to a spherocyte instead of the usual biconcave shape of a RBC
This subsequently leads to haemolysis
How does hereditary spherocytosis usually present?
A triad of:
- anaemia
- jaundice
- splenomegaly
Can also present through screening programmes of 1st degree relatives
Can present with neonatal jaundice or in adolescence / adulthood with anaemia and lethargy
What are the complications of hereditary spherocytosis?
Most concerning = aplastic crisis (usually due to infection)
Anaemia - treat with blood transfusions
Gallstones - treated with cholecystectomy
How would you go about investigating and diagnosing hereditary spherocytosis?
FBC with reticulocyte count (raised) and MCH
Blood smear: looking for spherocytes and haemolysis
Haemolysis screen: LDH (raised), haptaglobin (reduced), split bilirubin levels
Coombs’ test + EMA binding test (if not available, osmotic fragility test) - this is to exclude immune-mediated haemolysis
Please give some indications for a splenectomy.
Tumours (primary and secondary)
Trauma - splenic rupture
Sickle cell anaemia
Splenic artery aneurysm
Erythrocyte disorders: hereditary spherocytosis, hereditary elliptocytosis, AIHA
Platelet disorders: ITP
How would you differentiate a spleen from a kidney on examination?
Palpable splenic notch
Not ballotable
Unable to get above
Moves inferomedially with respiration
What investigations would you request for a patient with unknown cause of splenomegaly?
NB: should be targeted to the most likely underlying cause.
Bedside: obs, travel history
Bloods: FBC / UE / LFT / LDH / blood film / AI screen / blood cultures / viral serology / HIV test
Imaging: CXR (bihilar lymphadenopathy), CT-TAP (malignancy), echo (IE)
Other: LN biopsy, bone marrow aspirate + trephine
Management of hereditary haemochromatosis?
Regular venesection (1 unit/week), until iron deficient, then 1 unit 3-4 x a month
Avoid alcohol
HCC surveillance
What is the prognosis of hereditary haemochromatosis?
200x increased risk of HCC if cirrhotic
Reduced life expectancy if cirrhotic
Normal life expectancy without cirrhosis and with treatment
Inheritance of hereditary haemochromatosis?
Autosomal recessive inheritance
HFE gene mutation, on chromosome 6 - regulates gut iron absorption
Prevalence of 1:300
Males affected at younger age than females as not protected by menses
Investigations for suspected hereditary haemochromatosis?
Haematinics: increased ferritin, increased transferrin saturation
Blood glucose - DM
Liver biopsy
Genotyping
ECG, CXR, echo - CCF
Liver USS, alpha fetoprotein (HCC)
Clinical features in a patient with hereditary haemochromatosis?
Signs:
- slate-grey pigmentation
- stigmata of CLD
- hepatomegaly
Scars:
- venesection
- liver biopsy
- joint replacement
- rooftop incision (liver transplant)
Evidence of complications:
- ‘bronze diabetes’
- testicular atrophy
- cardiac: CCF
- joints: pseudogout
What complications occur with liver cirrosis?
Variceal haemorrhage
Encephalopathy
SBP
How do you classify liver cirrhosis?
Child-Pugh classification system:
- A: 5-6, 100% 1y survival
- B: 7-9, 81% 1y survival
- C: 10-15, 45% 1y survival
NB Child Pugh - Cirrhosis
What investigations would you request in a patient with suspected liver cirrhosis?
Bloods: liver screen
- autoantibodies + immunoglobulins
- hepatitis B and C serology
- ferritin
- caeruloplasmin (Wilson’s)
- A1AT levels
- AFP (HCC)
INR / coag, albumin (synthetic function)
Imaging: ERCP to exclude PSC, abdominal USS / fibroscan
Biopsy: diagnose fibrosis / stage
Causes of hepatomegaly?
‘CCC III’
Cirrhosis
Cancer (secondary, mets)
CCF
Infective (hepatitis B and C)
Immune (PSC, PBC, AI hepatitis)
Infiltrative (amyloidosis, myeloproliferative)
What clinical signs are noted as evidence of liver decompensation?
‘AAA’
Ascites
Asterixis
Altered consciousness (encephalopathy)
Which antibodies are important in liver disease?
PBC: anti-mitochondrial abs (M2 subtype) in 80%
PSC: ANA, anti smooth-muscle
AI hepatitis: anti-LKM
What prognostication scores are used to determine the benefit of steroids in alcoholic hepatitis?
Patient admitted with alcoholic hepatitis:
- Assessment of disease severity with Maddrey’s Discriminant Function score. If >=32, trial of prednisolone 40mg / day.
- Assess treatment response at day 7 with Lille score. If <0.45, continue treatment for 28 days. If Lille score >=0.45, stop treatment and assess for early liver transplantation in highly selected patients.
What are the genes in APKD?
AD.
Polycystin 1 on Chromosome 16
Polycystin 2 on Chromosome 4.
4 is less severe and presents later. Some patients with a positive family history do not have an identified mutation. A small percent develop a sporadic mutation that is then passed on in an AD pattern.
Complications of APKD?
Renal:
1. Pain
2. Haematuria
3. Stones
4. CKD
5. Infection
Extra-renal
1. HTN
2. Berry aneurysms
3. Liver/pancreatic cysts
4. MV prolapse, AR
APKD - indications for nephrectomy?
Recurrent infection
Uncontrolled bleeding
Suspected RCC
Uncontrolled pain
Extension into transplanted kidney
To make room for a transplanted kidney
What are the causes of renal osteodystrophy in CKD?
Low Vitamin D leading to osteomalacia
High phosphate
Low calcium due to both above
Secondary hyperparathyroidism (leading to bone cysts - hyperparathyroid bone disease)
What are the principles of managing CKD?
Treat the underlying cause
HTN (ACEi)
CV risk (QRISK)
Volume overload
Hyperkalemia
Hyperphosphatemia (diet, non-calcium phosphate binders like Serelamer)
Renal osteodystrophy (Address hyperphos, Vit D, consider parathyroidectomy)
Anaemia (replete iron, then give EPO)
What are the indications for RRT?
Refer when GFR <30
Uraemic complications (pericarditis, encephalopathy, bleeding)
Refractory acidosis
Refractory fluid overload
Refractory hyperkalaemia
What are the complications of haemodialysis?
Dialysis washout - hypotension, fatigue, cramps, chest pain, headaches
Infection
Bleeding (heparin as anticoagulant)
Amyloidosis
Decreased bone turnover
What are the complications of peritoneal dialysis
Peritonitis
Peritoneal sclerosis
DM due to systemic absorption of glucose
Local complications: hernias, fluid leakage, catheter exit site infection,
Decreased bone turnover
What are some of the complications of immunosuppression in renal transplant?
Infection
Skin lesions - BCC, SCC, melanoma
Cyclosporin (calcineurin inhibitor) - hirsuitism, gum hypertrophy, hypertension
Steroids - purpura, diabetes, HTN, cushingoid features
Tacrolimus (calcineurin inhibitor) - DM, tremor (especially in toxicity)
Tell me about alpha 1 anti-trypsin disease
Variable phenotype based on amount of residual AIAT produced
Lack of protease inhibitor A1AT
PI MM - normal
PI MZ - carrier
PI SS - 50% normal AIAT
PI ZZ - 10% normal AIAT (usually the ones that manifest disease)
Lungs: lower lobe pan-acinar emphysema
Liver: cirrhosis and HCC in adults; cholestasis in children
Ix: AIAT levels, spirometry
Rx: AIAT infusion, supportive, volume reduction surgery
How do you grade liver cirrhosis?
Child Pugh Score
based on
1. Excretion (bilirubin and ammonia)
2. Synthesis (albumin, PT)
3. Portal (portal hypertension and ascites)
Pathophysiology of ascites in cirrhosis?
Disruption of portal blood flow within liver that leads to splanchnic vasoDILATION
Self-propogating as this reduces renal blood flow that actives the RAS system
Net result is sodium and water retention that propagates the ascites and oedema
Hepatomegaly causes
Liver problem
Cancer
Benign lesions
Heart - CHF
Infection
Infiltration - amyloid etc.
Vascular - Budd-Chiari, Sickle
Cysts
Causes of Splenomegaly
In F iltration
- Malignant (AML, lymphoma, myelofibrosis)
- Benign (amyloid, sarcoid, gylcogen storage, Gaucher’s (Ashkenazi Jew), thyrotoxicosis)
F unctional
- Increased removal of blood cells (thalasemia, spherocytes, sickle cell)
- Infective (viral, malaria, bacterial, fungal, parasitic, infectious mononucleosis, Brucellosis, endocarditis)
- Disordered immune regulation (RA, Felty, SLE, sarcoid)
F low
- Cirrhosis
- Vascular problem - hepatic/portal vein obstruction
F*cking MASSIVE
- CML
- Myelofibrosis
- Gaucher’s storage
- Malaria
- Kala Azar
Investigations
- FBC with film
- CT Neck TAP
- Bone marrow with trephine
- LN biopsy
- Sickle screen, x3 thick/thin films
What are the autoantibodies in PBC?
anti-mitochondiral antibodies
Raised IgM
What is the pathophsiology of PBC?
Autoimmune destruction of inter-lobar bile ducts leading to progressive cholestasis and eventually cirrhosis
Is PBC associated with other auto-immune conditions?
Sjogren’s
RA
Systemic Sclerosis
Thyroid disease
Treatment for PBC?
Ursodeoxycholic acid (improves prognosis)
Liver transplant
Initial investigations for CLD?
Bedside: Full history (including travel, drug use, alcohol, and family history)
Bloods: (UE FBC Liver profile, coagulation)
Autoimmune screen (Anti-LKM, AntiSM, Anti-mitochondrial, immunoglobulins)
Iron studies, caeruloplasmin, alpha 1 antitripsin levels, AFP
Imaging: Liver USS +/- biopsy
Cross sectional imaging if indicated
Define acute liver failure
Severe impairment of liver function with hepatic encephalopathy occurs within 8 weeks of onset of symptoms and no underlying chronic liver disease.
Management of varices and variceal bleeding
Stabilise airway
Correct clotting
Transfuse
Terlipressin
Antibiotics
Urgent OGD for banding (calculate GBS)
Sengstaken-Blakemore if uncontrollable haemorrhage
TIPPS if above fail
What is the Rockall score?
Estimates mortality and re-bleed risk
Modified Rockall - pre-endoscopy (age, comorbidities, haemodynamics)
Rockall (Complete)
1. Age
2. Haemodyanimics
3. Co-morbidities
4. Diagnosis
5. Signs of recent/ongoing haemorrhage
What scoring criteria is used to assess liver transplant eligibility?
UKELD (UK model of end-stage liver disease) score
Predicts 1 year mortality
>=49 = survival advantage from transplant
UKELD score is based on:
1. INR
2. Cr
3. Bilirubin
4. Sodium
What are the causes of acute liver failure?
Paracetamol
Alcohol
Viral (Hep A,B,CMV)
Drugs (valproate, isoniazid, nitrofurantoin, co-amox, sulfonamides)
Metabolic: Wilson’s, Reye’s
Vascular: Ischaemic hepatitis, Budd-Chiari
Pregnancy - Fatty liver of pregnancy, HELLP
Variceal prophylaxis?
Propanolol
Endoscopic variceal band ligation
TIPPS
What are the indications for liver transplant?
Acute Fulminant Liver failure including paracetamol and alcohol overdose
HCC
Cirrhosis (ALD, NASH, AI liver disease, Haemachromotosis, Wilson’s, A1AT)
Other: Diuretic resistant ascites, chronic hep enceph, refractory pruritis, hepato-pulmonary syndrome, polycystic liver disease, recurrent cholangitis)
What are the contra-indications for liver transplant?
Absolute
1. Untreated HIV
2. Other disease with >50% mortality at 5 years
3. Irreversible lung disease
4. Ongoing alcohol/drug misuse
5. Current or previous other cancer (HCC eligible)
Relative
1. Smoking
2. Poor social support
3. Extensive prev abdominal surgery
4. BMI >40
5. Poor clinical attendance
Diagnosis of Hereditary Spherocytosis?
Can be clinical
EMA binding test and the cryohaemolysis test to confirm
How do you prognosticate acute alcoholic hepatitis?
Madrey Discriminant Function for alcoholic hepatitis
1. PT
2. Total biliruin
Glasgow Alcoholic Hepatitis Score
1. Age
2. Bil
3. Urea
4. INR
5. WCC
What are the indications for super-urgent transplant in paracetamol poisoning?
pH <7.25 24 hours after poisoning
PT > 100 or INR >6.5
SCr >300
Grade 3-4 encephalopathy
What causes a high SAAG?
CCF
Cirrhosis
Budd Chiari
What causes a low SAAG?
TB
Malignancy
Pancreatitis
SBP
What are the causes of ascites?
Liver Cirrhosis
Vascular: Budd Chiari, CCF, constrictive pericarditis
Low albumin: nephrotic syndrome
Peritoneal disease: Meig syndrome, infectious, malignancy
Misc: Pancreatic leak, chylous leak, peritoneal dialysis, advanced hypothyroidism
How does Hereditary Haemachromatosis usually present?
Screening
Raised ferritin
Arthralgia, lethargy, sexual dysfunction
DM, cardiomyopathy, bronze pigmentation of skin
How do you diagnose hereditary haemochromatosis?
Raised ferritin (200 mcg/L in females, 300 mcg/L in males)
Raised transferrin saturation (>40% in females, and 50% in males)
Positive HFE gene defect (note there is variable penetrance)
Treatment for hereditary spherocytosis?
Folic acid (to keep up with RBC turnover)
Bood transfusions
Splenectomy (will need vaccination for encapsulated organisms particularly meningococcal, pneumococcal, and influenza, and prophylactic antibiotics)
Cholecystectomy
How do you diagnose coeliac disease?
Anti-TTG.
If equivocal - OGD + D2 biopsy looking for total or sub-total villous atrophy
How do you manage coeliac disease?
Wheat, rye, barley all have gluten.
Need dietitian consult
Prescription of gluten-free foods
Treat hyposplenism (30% of people with coeliac): vaccinations and prophylactic abx
What potential reasons might a patient have a surgical scar in both iliac fossa?
SPK transplant
One failed renal transplant
What is your differential for gynaecomastia?
Gynaecomastia is due to imbalance in androgen:oestrogen ratio. It can be physiological
Liver problem (reduced androgens)
HypERthyroidism
Syndromic (Klienfelters)
HCG secreting testicular cancers
Drugs (spironolactone, digoxin, finasteride, cimetidine)
What is a TIPSS?
Transvenous intrahepatic porto-systemic shunt that diverts blood from portal to systemic system to relieve refractory portal hypertension. 10% will subsequently become encephalopathic.
What is hepatorenal/hepatopulmonary syndrome?
Due to splanchnic vasodilation = underfilling of kidneys
Type 1
Rapidly progressive and very poor prognosis
Type 2
Slowly progressive but poor prognosis
Rx: Terlipressin, albumin, TIPSS, transplant
What are the causes of chronic pancreatitis?
Alcohol
Gallstones
Hypercalcaemia
Hypertriglyceridaemia
Smoking
ERCP
Pancreatitic divisium
Pancreatic cancer
Idiopathic
Genetic (PRSS1, CFTR, SPNK1)
What are the complications of chronic pancreatitis?
Pancreatogenic diabetes
Pancreatic ductal strictures
BIliary obstruction
Duodenal obstruction
Pseudocysts
Pancreatic malignancy
What are the extra-intestinal manifestations of Crohn’s disease?
Skin: pyoderma, erythema nodosum
Joints - arthritis
Eyes - episcleritis/uveitis
Gallstones
PSC (more common in UC)
What are the key differences in the treatment of Crohn’s and UC?
5-ASA are less effective in Crohn’s
5-ASA used as maintenance in UC (oral + topical); azathioprine / mercaptopurine can be added if >2 flares
Azathioprine / Mercaptopurine (need to check TPMT levels first) are used as maintenance in Crohn’s
What side effects do you know of with immunosuppression?
All - infection due to myelosupression
Tac - tremor, impaired glucose tolerance, HTN, lymphoma, skin cancer
Cic - less potent than Tac, lymphoma, skin cancer
MMF - rarely associated with GI bleeding and lung fibrosis
What are common indications for a splenectomy?
Haematological: haemolytic anaemia, thalasemia, haem malignancy, myeloproliferative disorders, ITP
Trauma
Infectious complications (hydatid, malaria)
Congestive disorders
Metabolic storage disorders
What is a specific sign of ALCOHOLIC liver disease?
Parotid swelling
Dupuytren’s contracture (can be deforming of tendons and lead to fixed flexion/extension of digits!)
End of the bed signs of CLD?
Cachecia and Loss of tricep bulk
Palmar erythema
Dupuytrens
Asterixis
Spider naevi
Gynaecomastia
Loss of central chest hair
How is hereditary haemochromatosis inherited?
HH is inherited in an autosomal recessive pattern.
It is usually the result of an abnormality in the HFE gene, which is located on chromosome 6.
How does hereditary haemochromatosis usually present?
Can present via screening programmes of affected first-degree relatives.
It can also present in asymptomatic patients with a raised ferritin.
Patients may present with arthralgia, sexual dysfunction, lethargy.
Later complications include: diabetes, cardiomyopathy, and bronzed pigmentation to the skin.
Describe an ideal screening programme for hereditary haemochromatosis?
Screening should be undertaken in patients with first-degree relatives with HH.
Baseline ferritin: looking for levels >200 in women and >300 in men.
Transferrin saturation of >40% in women, and >50% in men.
Screen for the HFE gene defect.
Impt to remember there is variable penetrance with this gene, so not everyone with the defect will demonstrate a disease phenotype.
What monitoring tests would you conduct once you establish that a patient has hereditary haemochromatosis?
Investigate potential complications of HH. These include:
- Regular HbA1C, looking for diabetes
- 6-monthly USS liver in the context of cirrhosis, with measurement of AFP (HCC)
- Baseline echo looking for features of cardiomyopathy
What arthralgia features are to be expected in a patient with hereditary haemochromatosis?
HH-associated arthralgia typically presents in the metacarpophalangeal joints.
This causes characteristic squaring of these joints, and can be differentiated from OA on an x-ray due to the presence of chondrocalcinosis.
Features and complications of hereditary haemochromatosis?
Asymptomatic with raised iron levels
May present with fatigue, arthralgia, sexual dysfunction
Pseudogout, chondrocalcinosis and chronic arthropathy
Late features
Liver cirrhosis, hepatomegaly, HCC
T1DM
Bronze pigmentation to skin
Cardiomyopathy
Name some causes of pancreatitis.
The most common causes:
- gallstones
- alcohol-related
Others:
- trauma
- secondary to ERCP
- hypertriglyceridaemia
- hypercalcaemia
- drugs (immunosuppressives)
Are there any genetic causes of pancreatitis?
PRSS1: autosomal dominant hereditary pancreatitis
CFTR: autosomal recessive
SPINK1: more complex genetics
What are the possible complications of pancreatitis?
Complications can be divided into acute and chronic.
Acute:
- sepsis
- ARDS
- death
Chronic:
- chronic pancreatitis
- portal vein thrombosis
- splenic vein thrombosis
- pseudocyst formation - can causes sepsis or have compressive effects which causes biliary / pancreatic obstruction
How is a pancreatic pseudocyst best drained?
They are best drained using an endoscopic approach.
Can use an AXIOS stent - typically performed 6 weeks after initial presentation.
What is the typical presentation of pancreatitis?
Longstanding, chronic pain with intermittent / regular flare-ups.
Often pain presents in epigastric region with radiation to the back. Relieved by sitting up and leaning forwards.
Treatment of chronic pancreatitis?
Avoiding causes of pancreatitis and analgesia.
Stop smoking and alcohol.
If any signs of malabsorption, consider starting Creon (pancreatic enzyme replacement) and PPI.
Vitamin D + magnesium replacement.
What are the clinical indicators of pancreatic exocrine insufficiency?
Steatorrhoea
Weight loss
Vitamin D deficiency
Hypomagnesaemia
Low faecal elastase
What are the main investigations for malaria?
3 lots of thick and thin blood films.
Quick spot test for malaria.
Importantly, I would seek advice from the local ID service.
What investigations would you consider in a case of splenomegaly with unknown cause?
Bloods: INR, FBC, UE, LFT, CRP, blood film, autoimmune screen, HIV test.
Imaging: USS abdomen, CT-TAP looking for lymphadenopathy if concerned about lymphoma.
Other: bone marrow test and trephine (for evidence of bone marrow infiltration in context of malignancy).
What cause of splenomegaly would you consider in a young, otherwise well patient?
Thyrotoxicosis. Suspect this in cases with no other relavant abdominal signs and no clinical anaemia.
Causes of splenomegaly?
Can be subdivided into 3 main categories:
- Infiltration: malignant or benign - lymphoma, amyloid, sarcoid, Gaucher’s, thyrotoxicosis.
- Increased function: HS, thalassaemia, early sickle cell anaemia, chronic malaria and Kala-azar. EBV.
- Abnormal blood flow: cirrhosis, hepatic or portal vein thrombosis.
Causes of massive splenomegaly: CMV, myelofibrosis, kala-azar, malaria.
If a patient with ascites has a temperature - what investigations should be performed?
Concerns about SBP. Need to do an ascitic tap.
- Would be looking for a raised corrected neutrophil count (levels >250 are concerning for SBP)
- Gram stain and culture
- Broad spectrum abx
What is an OGD looking for in the context of liver cirrhosis?
Varices
Portal hypertensive gastropathy
GAVE syndrome (gastric antral vascular ectasia)
What is a ‘non-invasive liver screen’ made up of?
LFTs and AST, INR, FBC
AST, GGT, AFP
Ferritin
TTG, immunoglobulins, AI profile and cholesterol
Hep B core antibody and surface antigen, Hep A and C antibodies
Caeruloplasmin and A1AT (if suspicious family history)
How would you clinically assess for decompensated liver disease?
Decomp liver disease presents as asterixis, encephalopathy and ascites.
How would you test for encephalopathy in decompensated liver disease, clinically?
Test for asterixis
Assess for constructional dyspraxia: ask patient to draw a five-pointed star
What prognostic scores exist for cirrhosis?
Most commonly used in the UK is the Child-Pugh score which looks at the presence of ascites, albumin, encephalopathy, INR and bilirubin.
The MELD score is another score that can be used to assess cirrhosis prognosis.
How much albumin cover is needed when draining ascites?
1 bag of albumin for every 2 - 2.5 litres of ascites drained.
What are the potential causes of gynaecomastia?
Alcoholic liver disease
Klinefelter syndrome
Spironolactone use
Causes of liver cirrhosis?
Most common in UK are alcohol, NAFLD and hepatitis.
Other causes include:
* Hereditary haemochromatosis
* Autoimmune hepatitis
* PSC
* PBC
* Wilson’s disease
* A1AT deficiency
* Cardiac failure
* Constrictive pericarditis
* Methotrexate
Multisystem consequences of alcohol abuse?
Cardiac problems: dilated cardiomyopathy and HTN
GI problems: pancreatitis, peptic ulcers, GI malignancy
Neuro: cerebellar atrophy, Wernicke encephalopathy and Korsakoff’s syndrome
