Abdo Flashcards
What are the causes of chronic liver disease?
3 commonest: Alcohol, hep B/C and nonalcoholic hepatic steatosis
Others:
Autoimmune: PBC
CHronic hepatitis
Metabolic: haemochromatosis
What are the complications of CLD?
Portal HTN Haemorrhage secondary to varices Ascites SBP Hepatic encephalopathy Hepatorenal syndrome Hepatopulmonary syndrome
WHat are the criteria for grading encephalopathy?
West-Haven criteria
0: clinically normal, small changes to memory
1: Mild confusion, euphoria, depression, short attention, impaired mental tasks
2: Drowsy, lethargy, mild disorientation, inappropriate behaviour
3: Somnolent but rousable with voice, grossly confused
4: comatose
How do you grade cirrhosis?
Modified Child-Pugh system
Looks at bilirubin, albumin, INR, ascites, encephalopathy
How do you classify jaundice?
Pre-hepatic: autoimmune haemolytic anaemia, malaria, SCD
Hepatic: acute viral hepatitis, paracetamol, alcohol
Post-hepatic: gallstones, malignancy
What do you know about hepatorenal syndrome?
Inadequate hepatic breakdown of vasoactive substances leading to excessive renal vasoconstriction. Type 1 develops rapidly and type 2 slowly
Difficult to reverse without hepatic transplantation, diagnosis of exclusion
How do you manage ascites in association with CLD?
No added salt diet Spironolactone Loop diuretics next Therapeutic drainage Radiological procedures such as TIPS but increase risk of encephalopathy Liver transplant
WHat are the causes of ascites
Common: Cirrhosis with portal hypertension Malignancy CCF Nephrotic syndrome
Uncommon: Budd-chiari syndrome Portal vein thrombosis Constrictive pericarditis Malabsorption syndormes Peritoneal mesothelioma TB peritonitis Myoxedema Ovarian disease
What causes ascites formation in CLD?
Relative renal hypoperfusion causes increased renin from juxtaglomerular cells
Renin activates aldosterone
Deficient hepatic metabolism reduces aldosterone and ADH breakdown
Hypoalbuminaemia decreases oncotic pressure
The combination of salt and water retention from hyperaldosteronism and high ADH with high portal pressures causes nelt ultrafiltration of fluid into abdo cavity
How do you distinguish between transudate and exudate in ascites?
SAAG
<11g/L= exudate
>11g/L= transudate
What are the causes of hepatomegaly?
Top 3 causes:
- CCF
- Malignancy
- Lymphoma
Other: malaria, hepatitis, leishmania, sarcoid
What scoring systems may help in evaluation of a patietn presenting with alcoholic hepatitis?
Maddreys discriminant function test
Mayo end stage liver disease score
Glasgow alcoholic hepatitis score
How would you manage a patient with acute alcoholic hepatitis?
Supportive: alcohol abstinence, nutrition adequate, treatment of any infection
Maddreys discrimenant function score >32 indicates treatment with steroids
What are the histological features of alcoholic liver disease?
Hepatic steatosis: accumulation of fat in liver cells (reversible)
Alcoholic hepatitis: acute inflammation and hepatocyte necrosis (reversible)
Hepatic cirrhosis- fibrosis of liver tissue
What clotting factor abnormalities may be assoc with amyloidosis?
Loss of clotting factors IX and X. Also infiltration of amyloid protein contributes to vascular fragility so significant risk of bleeding with percutaneous liver biopsy
What are the common and uncommon causes of isolated splenomegaly?
Common:
- Chronic malaria
- Kala-azar
- Schistosmiasis
- lymphoproliferative disease
Uncommon:
- Feltys disease
- chronic haemolytic anaemia
- IE
- left sided portal hypertension
What is the characteristic chromosomal abnormality in CML?
Philidelphia chromosome
Translocation (9;22)
BCR-ABL protein
Treat imatinib
What is feltys syndrome?
Triad of RA, splenomegaly and neutropenia
COmplications are recurrent infection, hypersplenism- anaemia and thrombocytopenia, skin hyperpigmentation, cutaneous ulceration
Why might the platelet count be reduced in alcoholic liver disease?
Splenomegaly assoc with portal hypertension results in platelet sequestation and thrombocytopenia
Direct toxic effect of alcohol on production
Folate def may contribute
How would you differentiate a renal and splenic mass?
Characeteristics of splenic mass are:
- Dull to percussion
- Not ballotable
- Palpable splenic notch
- Palpating finger cannot get above a splenic mass
What are the common causes of hepatosplenomegaly?
- infective
- myelo/lymphoproliferative diseases
- cirrhosis with portal hypertension
Less common: Wilsons, haemochromatosis, glycogen storage diseases
Worldwide what would be the most common cause of hepatosplenomegaly?
Malaria
Visceral leishmania
Schistosomiasis
What is meant by pre-sinusoidal portal hypertension?
In schistosomiasis it is causes by S.Mansoni and S.Japonicum, the eggs become trapped in portal traps where they cause granulomatous reaction and subsequent fibrosis. Synthetic function of liver maintained
What is the genetic basis of Wilsons disease?
Mutation of adenosine triphosphatase 7b (ATP7B) gene on chromosome 13
AR
Gene is key in transport of copper into secretory pathway for incoperation into copper containing enzymes.
Levels of copper containing enzymes like caerulopasmin decrease
Urinary copper is high
What are the clinical manifestations of Wilsons disease?
GI: chronic active hepatitis, fulminant hepatitis, chronic liver disease
Neuro: tremor, parkinsonism, dysdiadokokinesis, chorea, dystonias, chroeoathetosis, ataxia, epilepsy
Psychiatric: Psycosis, personality change, intellectual impairment
Opthalmological: Kayser Fleisher rings in Descemets membrane, sunflower cataracts
MSK: osteopenia, arthropathy, chondrocalcinosis
Renal: Fanconi syndrome, nephrocalcinosis
Cardiac: cardiomyopathy
Haematological: haemolytic anaemia
What are the biochemical features of Wilsons disease and what are the limitations of these tests?
Low serum caeruloplasmin
High urinary copper
Both copper and caeruloplasmin are acute phase reactants so should be interpreted in context
How might somebody present with PBC?
Asymptomatic
Intense pruritis and lethargy
RUQ pain
easy bruising
Female, age 40-50yrs
What signs would you look for in PBC?
Scratch marks Jaundice Orbital xanthelesmata and skin xanthomas Hepatosplenomegaly signs of CLD Sings of other autoimmune conditions eg systemic sclerosis and autoimmune thyroiditis
What is the natural history of PBC?
Anti mitchondrial antibodies are directed against mitochondrial enzyme complex M2.
AMA is positive in 90%
Disease progression is so slow that often not affected during lifespan but once symptomatic unlikely to survive >2yrs
What treatment options are currently available for PBC?
General measures- alcohol avoidance, fat soluble vitamins ADEK
Ursodeoxycholic acid: reduces cholestasis and improves liver function tests however has little effect on symtpoms/prognosis
Cholestyramide- for itching
Liver transplant
What are the early features of haemochromatosis?
Fatigue
Arthralgia
Sexual dysfunction
What are the later features and complications of haemochromatosis?
Hepatic cirrhosis- chronic liver disease- hepatomegaly
T1DM
Bronze skin discolouration (you can tell because there is no tan line!)
Cardiomyopathy- often dilated with arrythmias
Chondrocalcinosis
What is the typical genetic abnormality found in haemochromatosis?
AR
One of the most common heritable genetic conditions in northern europe- prevalence 1/200
Most common allelle is C282Y on HFE gene
Can you name a cause of accelerated iron accumulation in patients with primary haemochromatosis?
Alcohol may accelerate it
What are the difficulties screening for haemochromatosis?
Serum ferritin, iron and Saturation may be increased in other causes of cirrhosis particularly alcohol and hep c
Phenotypically women have less severe disease due to menstrual losses
More than one mutation in HFE gene and mutations inn other genes so a negative screen does not rule it out.
How would you treat somebody with primary haemochromatosis?
MDT input Avoid alcohol and iron supplements Weekly phlebotomy to reduce iron stores Desferrioxamine can be used Liver transplant in those with advanced cirrhosis
How might somebody that has had a liver transplant present?
Complication of immunosuppression
- Infectious disease- typical or atypical
- Malignancy- BCC, SCC, bowel
- metabolic complications- post transplant diabetes
- CV complications- IHD, CVD, acute rejection
Transplant dysfunction- rejection or recurrent disease
Surgical complications in early post op period: biliary leak or hepatic/portal vein thrombosis
What are the causes of end stage liver disease?
Alcohol abuse
Viral hepatitis
Autoimmune (PBC, autoimmune heptaitis)
Cryptogenic cirrhosis
What is the common cause of liver transplantation?
Alcohol
Is a liver transplant ever indicated for cirrhosis from chronic hepatitis C
Yes it is now an accepted indication
What are the criteria for referral to a liver unit for transplantation?
The Kings College hospital criteria are divided into:
paracetamol induced
- arterial lactate >3,5 after 4hrs resuscitation
- Or pH <7.3 or arterial lactate >3.0 12hrs after resuscitation
- Or all 3 of: INR >6.5, Cr >300, encephalopathy (grade 3 or 4)
Non-paracetamol induced
- Arterial lactate >3.5 4hrs after resuscitation
- INR >6.5
- Or any 3 of: INR >3.5, age <10yrs or >40yrs, bili >300, jaundice >7 days, aetiology being drug reaction
What immunosuppressive drug regimens are currently in use for liver transplant?
Steroids in combination with a calcineurin inhibitor such as tacrolimus or ciclosporin and an antiproliferative agent such as mycophenolate mofetil
What other forms of liver support are available?
MARS system (Molecular adsorbents recirculation system)- 2 circuits- 1st exposed to albumin to bind toxins and ammonia and 2nd the albumin is removed ECLAD (extracorporeal liver assist devices)
Heterotopic liver transplant- implantation of a lobe of allogenic liver
What are the extra abdominal features of IBD?
Clubbing
Uveitus
Large and small joint arthropathies and sacroilitis
Skin disease- pyoderma gangrenosum, erythema nodusum
Peripheral oedema from hypoalbuminaemia
Cushiongoid from steroid therapy
What are the differences between Crohns and UC?
Crohns
- skip lesions
- any part of bowel- mouth to anus
- transmural (full thickness) inflammation
- Granulomas
UC
- continuous
- Colon+ backwash ileitis
- superficial inflammation
- crypt abcesses
How might a patient with IBD present to emergency department?
- bloody diarrhoea worsening over several days
- de novo presentation may be difficult to distinguish from bacterial gastroenteritis
What are the criteria for grading severity of IBD?
Truelove and Witts criteria
- Mild: < 4 stools per day, no systemic disturbance, normal ESR
- Moderate: > 4 stools per day with minimal systemic disturbance
- Severe: >6 stools per day with blood and evidence of systemic disturbance evidenced by fever, tachycardia, anaemia or ESR >30
How would you induce remission in UC and Crohns?
Crohns
- steroids (budesonide if not tolerated)
+ azathioprine/mercaptopurine
+ inflixumab/adalimumab
UC
- aminosalicylates (topical if proctitis)
- Steroids if severe
+ ciclosporin
How would you maintain remission in UC and Crohns?
Crohns
- Azathioprine or mercaptopurine (only use methotrexate in those who needed it to induce remission)
UC
- Aminosalicylates (mesalazine, sulphasalazine)
+ azathioprine
What are the complications/extra-abdominal features of Crohns and UC?
Crohns
- erythema nodusum
- pyoderma gangrenosum
UC
- Primary sclerosing cholangitis
- toxic megacolon
- osteoporosis
- colon canacer
Causes of epigastric mass
Carcinoma of stomach or pancreas
Abdominal aortic aneurysm
Lymphoma
Caudate lobe of liver
Causes of right iliac fossa mass
Crohns disease Caecal carcinoma Ileocaecal mass including amoebic abcess, ileocaecal TB, appendicular mass Ovarian tumour Renal transplant
Causes of left iliac fossa mass
Carcinoma of sigmoid colon Diverticular mass Faecal mass Ovarian tumour Renal transplant
How is ADPKD inherited and how and when would you recommend screening?
AD
Mutation in PKD1 on chromosome 16 and PKD2 on chromosome 4
Screening with US but does not rule out if <30yrs
US criteria if positive FH
- <30yrs- at least 2 unilateral or bilateral renal cysts
- 30-59yrs- at least 2 cycts in each kidney
- >60yrs- at least 4 cysts in each kidney
Supported by pancreatic and hepatic cysts
if no FH
- Bilateral renal enlargement and cysts or
- Presence bilateral renal and hepatic cysts
- no manifestations of other disease
Name some extra renal manifestations of ADPKD
Cysts in other organs Intracranial berry aneurysms Polycythaemia (excess erythropoietin release) Hypertension (excess renin release) Cardiac valve disease (MV prolapse) Diverticular disease Aortic aneurysm Abdominal wall hernias
Name some organs that contain cysts in ADPKD
Kidneys
Liver
Pancreas
Arachnoid cysts
Name some causes of abdominal pain in patients with ADPKD?
Infected cyst
Haemorrhage into cyst
Some conditions occur in higher frequency in patients with ADPKD and can cause abdo pain:
- Diverticulitis/diverticular perforation
- Nephrolithiasis
- Strangulated/incarcerated hernia
- ruptured AAA
- Tuberous sclerosis complex
What signs do you look for in somebody with a renal transplant?
Oblique scar in iliac fossa + mass below
Signs of immunosuppression
- Increased skin pigmentation
- Multiple skin warts
- Premature aging of skin
- Skin malignancy or evidence of previous exicision
- Sun cream or hat by bed
- Fine tremor of ciclosporin toxicity or tacrolimus
- Gingival hypertrophy- ciclosporin
Signs of ESRF or RRT- peritoneal or haemodialysis
Evidence of aetiology of native kidney
- ADPKD
- Diabetic- BM stick marks, lipodystrophy from insulin injections
- Hearing aids- Alports syndrome or aminoglycoside induced nephropathy with ototoxicity
- Facial lipodystrophy assoc with mesangiocapillary GN type 2
- If no evidenc of underling cause then likely GN
Functional status of the transplant
- HTN
- Fluid overload
- Current evidence of dialysis
- phosphate binders by bed
What are the complications of chronic kidney disease?
Anaemia- EPO Hyperparathyroidism- initially secondary (hyperplasia of parathyroid due to low calcium and have low/normal calcium) then tertiary (hypercalcaemia) Hyperphosphataemia (due to insuffient removal of phosphate by kidneys, worsens hyperparathyroidism)- 1st line is calcium acetate as phosphate binder Renal bone disease Uncontrolled hypertension Uraemic encephalopathy, pericarditis Fluid overload Amyloidosis secondary to dialysis
What are the commonest causes of end stage renal failure?
Diabetes mellitus
ADPKD
Chronic Glomerulonephritis
Hypertension in Afro-Caribbean population
What are the complications of transplanting a patient with Alports syndrome?
Defect in type IV collage so a new transplant exposes them to antigens they have not encountered before. The subsequent antibody response against this antigen may result in some patients developing anti-glomerular basement membrane disease
Name some opportunistic infections assoc with renal transplantation
CMV
Pneumocystis jiroveci
EBV
BK virus ( common polyomavirus which lies dormant in renal tract and can cause ureteric stenosis and interstitial nephritis
JC virus ( causes progressive multifocal leucoencephalopathy)
Why are the kidneys transplanted into the pelvis?
Good blood supply
Space
Proximity to bladder for anastomosis
Easy access for biopsy/nephrostmy
What are the commonest causes of death in a patient with a renal transplant?
Cardiovascular: accelerated CVD is a feature of all patietns with renal disease
Infection due to immunosuppression is 2nd
What are some causes of a distended abdomen?
Fat Faeces Foetus Flatus and Fluid
Name the surface markings of aorta
Lies in epigastrum, just to left of midline and bifurcates at L4/5
What is peutz Jeghers syndrome?
Characeterised by the appearance of benign haemartomas in GI tract accompanied by mucocutaneous pigmented macules over hands, feet and lips.
Presents at screening, with intersussception or malignancy disease
What is the inheritance of Peutz-Jeghers syndrome?
AD
mutation in gene STK11 which is tumour suppressor gene
WHat are the complications of HHT?
Anaemia
Recurrent epistaxis
GI haemorrhage from telangiectasia and AVM in bowel
HIgh output cardiac failure as result of large AVM
SOB and haemoptysis as result of lung AVMs
Stroke and SAH- cerebral AVMs
What are the common sites of AVM in HHT?
Brain, lung, liver, bowel, spine
What is the genetic basis for HHT?
AD
Number of genes described
What is Heyde’s disease?
Association between aortic stenosis and colonic angiodysplasia
Mild form of von willebrands disease due to sheer stresses around aortic valve which increase the breakdown of large molecules of vWF resulting in consumptive deficiency
Often improves with AVR
What are the signs of kidney- pancreas transplant?
May have 2 scars in iliac fossa or midline scar
Look for signs of functioning pancreas- no finger prick marks or injection site marks
How is the pancreas drained when transplanted and what are the complications?
Old pancreatic transplants were bladder drained, urinary amylase could be used to monitor for early signs of graft pancreatitis . Route of drainage was complicated by reflux pancreatitis, persistant haematuria, recurrent UTIs, metabolic acidosis (bicarb loss)
Now most perform primary enteric drainage
Can you name some complications of post transplant immunosuppression?
Infections
Malignant disease- BCC, SCC, post transplant lymphoproliferative disorder
CVD
Chronic transplant injury- ciclosporin and tacrolimus nephrotoxic
Morphological changes- cushingiod, acne vulgaris
What are the indications for a liver and kidney transplant?
Usually hep B/C with assoc glomerulonephritis or cryoglobulinaemia
(not usually hepatorenal syndrome as with liver transplant the renal function is potentially reversible)
