Abdo & haem Flashcards
Causes of a single palpable kidney
Hydronephrosis
Compensatory renal hypertrophy if renal agenesis
Carcinoma
PKD with only one palpable kidney
Contraindications for renal transplant
Sepsis or active infection (AIDS with opportunistic infection, Active viral hepatitis)
Severe obesity
Uncontrolled IHD or extensive peripheral vascular disease
Malignancy not curative or expected remission for >5yrs
Uncontrolled vasculitis
Indications for nephrectomy in PKD?
1 - to allow renal transplant 2 - cyst haemorrhage / persistent haematuria 3 - cyst infection 4 - mass effects 5 - chronic pain
Causes of isolated hepatomegaly
Alcoholic liver disease
Malignancy - benign, malignant and mets
Heart failure
Infection
Haematological
haemachromatosis
What systems are affected in haemachromotosis
Liver - micro nodular cirrhosis
Testes - hypogonadism, erectile dysfunction
Heart - heart block, cardiomyopathy and heart failure
Pancreas - DM
Skin - slate grey appearance
Joints - pseudo gout
Liver biopsy in the presence of ascites must be done by which route
Transjugular
Complications of PBC
Progression to chronic liver disease and cirrhosis
Increased risk of malignancy, typically HCC
Criteria for liver transplant for chronic disease (Non-Kings)
Child Pugh B/C - BRAIN Bili elevated (3-5mg/dL) Refractory ascites Albumin <27 (synthetic hepatic failure) INR <1.7 - coagulopathy Encephalopathy
Ix for hereditary spherocytosis
EMA test - flow cytometry with eosin binding
Osmotic fragility test
Causes of haemolytic anaemia
Hereditary
H. spherocytosis and elliptocytosis
Thalassaemia
Sickle cell anaemia
Acquired
Non-immune - sepsis, malaria
MAHA - HUS, TTP, DIC
Immune - Warm (malignancy, SLE) and cold (mycoplasma, CLL)
Causes of a kyphoscoliosis
Idiopathic (commonest)
Congenital (fused vertebrae, hemivertebrae)
Paralytic - anterior poliomyelitis
Neuropathic - neurofibromatosis, spina bifida, Friedrich’s ataxia
Muscular - muscular dystrophy
How does PRV normally present
Hyperviscosity sx: headache, visual change, dizziness
Thrombotic: DVT, splenic artery thrombosis, stroke
Complications of HS
Anaemia
Aplastic crisis post infection (EBV - increases spleen size)
How does AD PKD present
1 - htn 2 - evidence of renal failure 3 - finding of CKD / proteinuria / haematuria 4 - extra-renal manifestations - MR, SAH 5 - familial screening
Complications of PRV
Progression - myelofibrosis or AML
Thrombosis - DVT, PE
Hyperviscosity
Ddx of RIF scar without a palpable mass
Ovarian tumour Asbcess - appendiceal / ileocaecal Crohn's resection Diverticular mass Caecal malignancy
How to present chronic liver disease
This patient has chronic liver disease, as evidenced by (peripheral findings) and hepatomegaly
Evidence of decompensation - jaundice, encephalopathy (liver flap), ascites
Evidence of portal htn - peripheral oedema, splenomegaly
Signs of the underlying cause - tattoos, diabetes
Nutritional status
Associations of AD PKD
Hepatomegaly (more common in AR PKD)
Intracranial aneurysms
Mitral valve prolapse
Haemolysis screen
LDH, haptoglobin, split bilirubin, Coomb’s test, blood film
Complications of liver transplant
Rejection - acute and chronic
Recurrence of disease - viral hepatitis, B-C syndrome, PBC
Related to immunosuppression - infection, malignancy and PTLPD, metabolic syndrome
Acute and chronic kidney disease - hepatorenal, drugs, DM, htn
Biliary complications - strictures and leaks
What are the treatments. for PKD in advance of ESRF
BP control
Increased fluid intake to suppress ADH release
Inhibition of RAAS with ACEi
minimal salt intake
Aim to minimise cardiovascular risk
Early treatment of UTI
What kind of haemolysis does hereditary spherocytosis cause
Extravascular haemolysis - splenomegaly, anaemia and jaundice
Indications for liver transplant (adults)
Acute
Paracetamol OD (see Kings criteria)
Trauma
Fulminant hepatic failure - viral hepatitis A/B
Non-acute Cirrhosis: Alcoholic liver disease (abstinent >6mths); NAFLD Viral hepatitis - B/C Autoimmune hepatitis Haemachromotosis Wilsons A1AT def.
Malignancy:
HCC
Liver metastasis where primary malignancy has been fully resected
Biliary disease:
PBC, PSC
Contraindications to receiving a renal transplant:
Sepsis or active infection
Active viral hepatitis
Unable to manage:
Mental incapacity
Poor prognosis
AIDS with opportunistic infection
Uncontrolled IHD or extensive peripheral vascular disease
Malignancy not curative or expected remission for >5yrs
Uncontrolled underlying cause
Uncontrolled vasculitis
Causes of bilateral enlarged kidneys
AD PKD Bilateral hydronephrosis Amyloidosis Tuberous sclerosis Von Hippel Lindau syndrome
Complications of immunosuppression
Infection - typical and atypical
Malignancy - skin and haematological
Specific SE of immunosuppressive drugs - steroids (Cushingoid), tacrolimus (tremor, nephrotoxicity), cyclosporin (gum hyperplasia)
Causes of hepatosplenomegaly
Haematological:
Myelo- / lymphs-proliferative disorders
CLL
Lymphoma
Hepatic:
Cirrhosis with portal htn
Infection:
EBV, CMV, viral hepatitis
Storage diseases:
Gauchers
amyloidosis
How does AD PKD present
1 - htn 2 - evidence of renal failure 3 - finding of CKD / proteinuria / haematuria 4 - extra-renal manifestations - MR, SAH 5 - familial screening
Complications of splenectomy
Surgical risk
Early post-op risk of VTE
Infection - strep, meningococcus, haemophilus, salmonella
Advise: Prior vaccination 2wks before procedure - men A and C, haemophilus, pneumococcus Annual flu vaccine 5yrly pneumococcal vaccination Penicillin prophylaxis Medic alert bracelet
Tx of PRV
Venesection
Cytoreductive tx if unable to tolerate: hydroxycarbamide, , anagrelide
Aspirin
Features of haemachromatosis
Chronic liver disease DM Arthropathy (pseudo gout) Cardiac features Hypopituitarism
Weekly venesection until Hb is <110, ferritin <10.
Desferrioxamine can be used if venesection not possible
Cardiac features of Marfans
Mitral valve prolapse
Aortic root dilatation
Aortic dilatation at any point along its length
Causes of a distal myopathy
Myotonic dystrophy
Inclusion body myositis
Complications of nephrotic syndrome
AKI and CKD
Thrombosis (urinary loss of antithrombin)
Hypercholesterolaemia
Immunodeficiency
What are the indications for a peg tube
Head and neck cancer - risk of cancer seeding
Neurological - risk of aspiration
Stroke, cerebal palsy, head injury
Neuromuscular conditions - MS, MND, Parkinson’s
Contraindications for liver transplant
Absolute Severe infection or sepsis AIDS infection Extrahepatic malignancy Advanced cardiopulmonary disease Ongoing alcohol or drug use Non-compliance
Relative HIV Severe chronic renal disease Extra-biliary sepsis Prev extensive biliary tract surgery Psychosocial issues
Causes of pain over renal graft in transplant pt?
Hyperacute or acute rejection
Abscess
Pyelonephritis
Causes of abdominal pain independent of renal transplant
Causes of jaundice post liver transplant
Graft dysfunction - look for other signs of decompensated liver disease
Extra-hepatic causes of jaundice - haemolysis, obstruction
What are the complications of peg tube insertion
Surgical risk - infection, failure, pain, scarring, perforation, leak. Wound complications
Persistent, but reduced risk of aspiration
Occlusion, displacement
Mx of hereditary spherocytosis
Folate
Blood transfusion as needed
Splenectomy with prior vaccination
How would you treat somebody with chronic liver disease
Tx the cause - antivirals, alcohol abstinence, reduce HbA1c, penicillamine, workup for transplant
Tx the complications - nutrition, laxatives
Causes of lymphadenopathy
What else will you examine for
Haematological - CLL, lymphoma,
Infection - EBV, HIV
Sarcoid
Assess for splenomegaly
Causes of ascites
Portal htn Peritoneal disease - TB Malignancy - ovarian and GI Meig's Pancreatitis Transudative - Low albumin, heart/liver/kidney failure, gut-losing enteropathy, B-C syndrome, constructive pericarditis
Complications of renal transplant
Surgical complications
Rejection - hyper acute, acute and chronic
Recurrence of underlying disease
Complications related to immunosuppression
Complications relating to original ESRF - tertiary hyper-PTHism
Ddx of RIF scar without a palpable mass
Ovarian tumour Asbcess - appendiceal / ileocaecal Crohn's resection Diverticular mass Caecal malignancy
How to investigate nephrotic syndrome
Observations
Urine - microscopy (casts) and PCR (quantify proteinuria)
Bloods - FBC, UE, LFT, HIV, viral hepatitis, Serum and urine electrophoresis (BJP), Ig, complement, ANCA, GBM, cholesterol, HbA1c
Renal biopsy
Ddx of RIF scar without a palpable mass
Ovarian tumour Asbcess - appendiceal / ileocaecal Crohn's resection Diverticular mass Caecal malignancy
Causes of nephrotic syndrome
Diabetic nephropathy Glomerulonephritis Amyloidosis Infection - hepatitis, HIV Drugs - NSAIDs Malignancy - myeloma Lupus nephritis
Sites of inappropriate EPO secretion
Renal, adrenal, hepatic, ovarian
Kings criteria for liver transplant
Kings criteria for paracetamol OD
Acidosis (pH <7.3)
OR all 3 of coagulopathy (PT > 100, INR >6.5), Creat >300, severe encephalopathy (Gr 3-4)
Non-paracetamol fulminant liver failure:
Coagulopathy - PT >100s (INR >6.5)
Or 3 of:
Age <11 or >40;
Bilirubin >300;
Coagulopathy - INR >3.5 (PT >50)
Disease - 2° to non-A or -E hepatitis or drugs;
Extended time period - Time from jaundice -> encephalopathy/coma >7 days (non-acute)
Indications for a renal transplant
Commonest causes:
Glomerulonephritis
Diabetic nephropathy
AD polycystic kidney disease
Signs of malnutrition / indications for creon in chronic pancreatitis
Steatorrhoea
Hypo-Ca/Mg
Low Hb, albumin, vit D
Low BMI
Measurements of faecal elastase
What mode of RRT is preferred in PKD? Why.
HD preferred over PD
PD increases risk of cyst infection
Also size of kidneys and presence of fluid can be uncomfortable inside the abdomen
Investigations for splenomegaly
Bloods - FBC with blood film (malaria, leukaemia), viral markers (EBV), bilirubin
Abdo USS
CT CAP for lymphadenopathy (leukaemia / lymphoma / myeloproliferative disease)
Bone marrow aspirate, trephine, LN biopsy
causes of ESRF
Diabetes
Hypertensive renal disease / vascular disease
Glomerulonephritis
Noonan syndrome
Inheritance
Features
Cardiac features
AD
Cubitus valgus (wide carrying angle at elbows), webbed neck, short stature, mild learning disabilities, widely spaced nipples, strabismus, ptosis, pacts excavatum
Pul stenosis, hypertrophic cardiomyopathy, septal defects
Causes of decompensation in chronic liver disease
GI bleed Sepsis Drugs - benzos Alcohol Constipation
Diseases where renal transplant can be a problem
Haemolytic uraemia syndrome - recurrence and cyclosporin worsens risk
Sickle cell - improved haematocrit increases the risk of subsequent crises
Systemic sclerosis - recurrence
FSGS - recurrence
Complications of coeliac disease
Malabsorption
Fe deficiency
Functional hyposplenism
Enteropathy-assocaited T-cell lymphoma (EATL) - duodenal
Features of Kartageners syndrome
Dextrocardia and situs inversus
Bronchiectasis
Sinusitis
Otitis media
Extra GI manifestations of IBD
Clubbing Arthritis - migratory oligoarthritis, sacroiliitis, myositis Eyes - uveitis Stomatitis PSC (UC>Crohns) Erythema nodosum / pyoderma gangrenous Osteoporosis Bile acid malabsorption
Mx of Marfans
Ongoing echocardiograms to track aortic root dilatation
Conservative
Lifestyle - avoid sudden or high stresses on aortic wall ie intense exercise
Medical
BP control - B-blockers or ARB
Surgical
Prophylactic aortic root graft repair if aortic root diameter >5cm
Which glomerulonephritides can cause nephrotic syndrome
1 - minimal change disease
2 - membranous GN (hepatitis, HIV, SLE, dermatomyositis, malignancy)
3 - mesangiocapillary GN (SLE, cryo, scleroderma)
4 - FSGS (HIV, drugs)
Causes of isolated splenomegaly
Haematological - myelo/lympho-proliferative disorders, CML
Infiltration - Myelofibrosis, sarcoid, amyloidosis, Lipid storage disease - Gauchers, thyrotoxicosis
Infection (immune hyperplasia ) - endocarditis, EBV, malaria, visceral leishmaniasis
Liver related - Cirrhosis with portal htn
Felty’s syndrome (RA)
Increased function - haemolytic anaemia (hereditary spherocytosis), early sickle cell, thalassaemia, nutritional anaemia
Causes of chronic liver disease (Cs and Is)
C - Cirrhosis (normally alcoholic)
C - congestion - CHF and B-C syndrome
C - Carcinoma
I - infiltration - amyloidosis, Wilsons
I - infection - viral hepatitis
I - Iron - haemachromatosis
I - immune - autoimmune hepatitis, PBC
Causes of ascites split by SAAG gradient
Low gradient (<11g/L) Peritoneal TB Peritoneal carcinomatosis Pancreatitis Bowel infarction Biliary ascites
High gradient (>11g/L) - portal htn Prehepatic - splenic / portal vein thrombosis Hepatic - Cirrhosis, metastatic liver disease, fibrosis Post hepatic - Cardiac (HF, constrictive pericarditis), B-C syndrome
How would you work up somebody presenting with chronic liver disease
Hx
Bloods - incl viral screen, autoantibodies (ANA, AMA, ANCA, anti-smooth muscle, anti-LKM), caeruloplasmin, ferritin, tumour markers (AFP)
Imaging - USS +/- CT
Biopsy
What is the treatment for PBC
Ursodeoxycholic acid - improves elimination of bile acid and can cause disease remission in 25%
How does AD PKD present
1 - htn 2 - evidence of renal failure 3 - finding of CKD / proteinuria / haematuria 4 - extra-renal manifestations - MR, SAH 5 - familial screening
Indications for splenectomy
Elective
Hypersplenism: Haemolytic anaemia (HS, HE), autoimmune haemolytic anaemia, Thalassaemia, ITP refractory to steroids
Associated with other resection - stomach malignancy
Vascular - Aneurysmal dilatation
Mass effect - myelofibrosis
Emergency
Traumatic rupture
Spontaneous rupture (EBV)
Management of hepatorenal syndrome
Type 1 - rapid onset
Terlipressin to constrict the splanchnic circulation.
20% HAS
Type 2 - slow onset
Treat underlying liver disease - TIPPS
Both supported with RRT (likely HD)
Ultimately if renal function does not improve they may need a liver and kidney transplant
Target Hb for venesection in haemochromatosis
Hb <110
