Abdominal Flashcards
Causes of clubbing
C: cardiac. subacute infective endocarditis. cyanotic heart disease. atrial myxoma.
L: lungs. lung abscess. empyema.
U: ulcerative colitis.
B: biliary cirrhosis.
B: bronchogenic carcinoma (most commonly small cell)
I: idiopathic.
(N: not COPD)
G: gastrointestinal. malabsorption (e.g. coeliac disease) Crohn disease.
What is the mode of inheritance of Peutz-Jeghers syndrome?
Autosomal dominant
Caused by mutations in the STK11 which is a tumour suppressor gene.
Causes polyps + melanocytic macules.
What are the manifestations of Peutz-Jeghers syndrome?
Dark-coloured freckling on oral mucosa.
Polyps within the GI tract which may be complicated by bleeding, obstruction and chronic pain.
What is the mode of presentation of HHT (Osler-Weber-Rendu syndrome)
Recurrent epistaxis in childhood with red spots on lips / tongue / fingertips + FH of disease
Characterised by multiple telangiectasia.
The patient is at risk of haemorrhage from AV malformations, particularly pulmonary + cerebral.
What is the mode of inheritance of HHT? (Osler-Weber-Rendu syndrome)
Autosomal dominant.
More than 80% of cases are due to mutations in ENG or ACVRL1 genes.
Most common causes of CLD in the Western world?
Alcohol
Chronic viral hepatits B and C
NAFLD
Autoimmune hepatitis
Metabolic conditions: Wilson’s, haemochromatosis
What are the reversible causes of hepatic encephalopathy?
Alcohol, drugs, GI haemorrhage, infection, constipation
Name some complications of CLD.
Portal hypertension
Haemorrhage
Ascites
SBP
Hepatic encephalopathy
Hepatorenal syndrome
Hepatopulmonary syndrome
What do you understand by decompensated CLD? What are the factors which can lead to decompensated CLD?
The liver can compensate for a significant amount of hepatocyte injury, but can decompensate as a result of ongoing liver injury or additional stress.
Precipitating factors: alcohol intake, untreated chronic active viral hepatitis, GI bleed, large salt intake, sepsis, constipation, dehydration.
How does decompensated CLD present clinically?
Clinically, decompensated CLD presents with: ascites, encephalopathy, hepatorenal syndrome, coagulopathy, jaundice
How do you grade severity of hepatic encephalopathy?
West Haven criteria:
Grade 0: clinically normal but with small changes in memory
Grade 1: mild confusion, short attention span, disordered sleep
Grade 2: drowsiness, lethargy, mild disorientation
Grade 3: somnolent but rousable, grossly confused and disorientated
Grade 4: comatose, no response to verbal / painful stimuli
How do you grade severity of cirrhosis?
Modified Child-Pugh system - classes correspond to 1-year survivals
Class A (5-6 points) - 100% 1y survival
Class B (7-9 points) - 80% 1y survival
Class C (10-15 points) - 50% 1y survival
Parameters measured: encephalopathy stage, bilirubin, INR, ascites, albumin
How do you classify causes of jaundice?
Pre-hepatic: excessive breakdown of RBCs e.g., haemolytic anaemia, malaria, hereditary haemoglobinopathies
Hepatic: due to hepatocyte injury e.g., acute viral hepatitis, paracetamol, alcohol, hypoxic / ischaemic
Post-hepatic: due to obstruction of bile flow e.g., gallstone obstruction, pancreatic head malignancies
Tell me about hepatorenal syndrome.
Results from inadequate hepatic breakdown of vasoactive substances, leading to excessive renal vasoconstriction.
It can develop rapidly (type I) or slowly (type II).
Mimics pre-renal impairment.
As the kidneys attempt to conserve maximum salt and water in response to perceived hypovolaemia, the patient produces low volumes of highly concentrated urine that is low in sodium.
It is usually diagnosis of exclusion, having ruled out obstruction, sepsis and nephrotoxic meds.
How is ascites investigated?
The protein level of ascites is measured to determine whether ascites is of a transudative or exudative cause. The serum ascites albumin gradient (SAAG) is an accurate test for diagnosis of the cause.
SAAG >11g/l - transudate (cirrhosis, cardiac failure, nephrotic syndrome)
SAAG <11g/l - exudate (malignancy, pancreatitis, TB)
Would also measure: neutrophil count and microscopy / culture (SBP), amylase (pancreatitis), and cytology (malignancy).
What are the indications for liver transplant in an adult?
Acute: paracetamol poisoning, other drugs, acute hepatitis, EBV and CMV infections.
Chronic: alcoholic liver disease, PBC, PSC, chronic viral hepatitis, Wilson’s disease, Budd-Chiari syndrome, hepatic malignancy.
How do you manage ascites in association with CLD?
Low-salt diet, spironolactone, furosemide.
If these measures fail, can perform therapeutic paracentesis.
TIPSS may help to relieve portal hypertension.
Liver tx is a curative procedure for end-stage disease when medical management fails.
Causes of ascites?
Common: cirrhosis with portal HTN, malignancy, CCF, nephrotic syndrome
Uncommon: Budd-Chiari syndrome, portal vein thrombosis, constrictive pericarditis, malabsorption, TB peritonitis, myxoedema, ovarian diseases
How would you distinguish between transudative and exudative ascites in patients with low serum albumin?
By using the serum ascites albumin gradient (SAAG)
A low gradient <11g/L indicates loss of protein into the ascites - EXUDATE
A high gradient >11g/L indicates TRANSUDATE
What is the differential diagnosis of generalised lymphadenopathy?
Lymphoproliferative disease: CLL, ALL, Hodgkin’s and NH lymphoma
Viral disease: HIV, EBV, CMV
Other infections: TB, brucellosis and toxoplasmosis
Inflammatory disease: SLE, RA, sarcoidosis
How are Hodgkin’s and Non-Hodgkin’s lymphoma differentiated pathologically?
Through the presence of Reed-Sternberg cells in Hodgkin’s lymphoma. These are characteristic binucleate cells found on light microscopy of a biopsy.
What are ‘B symptoms’ in lymphoma and what are their significance?
These include weight loss, unexplained fever and night sweats.
B symptoms are included in the Ann Arbor staging classifications of NHL and HL, and indicate a poorer prognosis.
Tell me about CLL.
Monoclonal proliferation of lymphocytes.
Most commonly suspected from routine bloods, with a high WCC.
Staged by the Binet system, and cytogenetic testing can give info on how the disease is likely to progress, therefore guiding treatment.
Stage A disease can be managed with a ‘watch and wait’ approach, more advanced disease requires chemotherapy and MABs.
Complications of CLL include bone marrow failure, haemolytic anaemia, chest infections and acute transformation to diffuse large B-cell lymphoma.
Top 3 causes of hepatomegaly?
Congestive cardiac failure
Malignancy
Lymphoma
What scoring systems may help in the evaluation of a patient presenting with acute alcoholic hepatitis?
Maddrey’s discriminant function test to predict prognosis in alcoholic hepatitis.
The Mayo End Stage Liver Disease (MELD) score - predicts survival probability over 90 days.
The Glasgow alcoholic hepatitis score on day 1 has an overall accuracy of 81% when predicting 28-day outcome.
How would you manage a patient with acute alcoholic hepatitis?
Mainstay of treatment is supportive: abstinence from alcohol, adequate nutrition, treatment of infection.
Liver biopsy may be indicated to confirm the diagnosis.
Maddrey’s discriminant function score of >32 is an indication for treatment with steroids (40mg prednisolone).
What are the histological features of alcoholic liver disease?
Hepatic steatosis - accumulation of fat in liver cells
Alcoholic hepatitis - acute inflammation and hepatocyte necrosis
Hepatic cirrhosis - fibrosis of liver tissue
What clotting factor abnormalities may be associated with hepatic amyloidosis?
Hepatic amyloidosis is associated with the loss of clotting factors 9 and 10.
Infiltration with amyloid protein contributes to vascular fragility - significant risk of bleeding if percutaneous biopsy is performed.
What are the causes of massive splenomegaly?
‘MMM’
Myelofibrosis
CML
Malaria / Visceral leishmaniasis
What are the most common causes of isolated splenomegaly worldwide?
Chronic malaria
Kala-azar
Schistosomiasis
What is the characteristic chromosomal abnormality in CML and what signal transduction pathway is this associated with?
The Philadelphia chromosome is the hallmark of CML, found in 90% of cases.
Cytogenetically results from a chromosomal translocation t(9;22).
Results in an altered bcr-abl protein which increases tyrosine kinase activity.
This activates proteins which speed up cell division and inhibit DNA repair.
Tell me about Felty’s syndrome.
Felty’s syndrome is: splenomegaly, RA and neutropaenia.
Frequency of Felty’s increases with duration of RA.
People with this syndrome are at risk of infection due to neutropaenia.
Complications of Felty’s include: recurrent infection, hypersplenism causing anaemia and thrombocytopaenia, skin hyperpigmentation and cutaneous ulceration.
Why might the platelet count be reduced in alcoholic liver disease?
Splenomegaly associated with portal HTN results in platelet sequestration and thrombocytopaenia.
Direct toxic effect of alcohol on production + function of platelets.
Platelet count of <50 is an indication for platelet transfusion in the presence of bleeding, particularly from varices.
How would you differentiate between a splenic and a renal mass?
4 characteristics of a splenic mass (not present with renal mass) - CNPD ‘cannot percuss daintily’
Cannot get above it
Not ballotable
Palpable notch
Dull to percussion
What is the function of the spleen?
Removal of old/damaged RBCs
Storage of platelets
B- and T-lymphocyte-mediated immune function
What advice should be given to a patient undergoing a splenectomy?
Preoperative vaccinations: pneumococcal, influenza, meningococcus
Lifelong prophylactic penicillin
Annual influenza vaccine
Wear medic alert bracelet
Sick day rules
What are the indications for a splenectomy?
Hypersplenism (ITP)
Mass effect of spleen
Traumatic rupture
Haematological malignancies
Congenital haemolytic anaemias (hereditary spherocytosis)
Tell me about polycythaemia rubra vera.
Primary polycythaemia, due to a fault in the bone marrow.
Results in uncontrolled RBC proliferation, often due to a mutation in the JAK2 gene.
PRV usually presents with raised Hb, or with thrombosis, headache, sweating and itch.
Investigation is initially with an FBC, if PRV suspected, would test for JAK2 mutation.
Management is targeted at lowering thrombosis risk. Includes venesection, aspirin and myelosuppression. JAK2 inhibitors are under trial.
A major complication, alongside thrombosis, is transformation to AML.
What is the ddx of hepatosplenomegaly?
‘CMLVII’
Chronic liver disease with portal hypertension
Myeloproliferative disease: CML, AML
Lymphoproliferative disease: CLL, HL and NHL
Viral disease: EBV, CMV, HIV
Inflammatory disease: sarcoidosis
Infiltrative disease: amyloidosis, glycogen storage disease
What are the myeloproliferative disorders?
A group of conditions caused by abnormal myeloid stem cell proliferation in the bone marrow.
RBCs: PRV
WCCs: CML
Platelets: essential thrombocytopaenia
Fibroblasts: myelofibrosis
Tell me about amyloidosis.
A multisystem disease that results from extracellular deposition of abnormal proteins.
2 main types: Amyloid L and Amyloid A
AL mainly occurs on its own, can be seen alongside myeloma. Mainly dx on biopsy, can treat its complications (e.g., renal transplant).
AA mainly occurs in conjunction with other inflammatory disorders and infections (such as RA). Diagnosed by biopsy. Condition often improves with tx of underlying inflammatory disorder.
