Abdo Paces Flashcards

1
Q

Causes of enlarged kidneys

A

ADPKD
Recessive form of PKD
Von Hippel Lindau
Tuberous Sclerosis
Hydronephrosis
Renal cell carcinoma (5%)

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2
Q

Genetics of PKD

A

Autosomal dominant:
PKD1: chromosome 16 in 80%- mean age to ESKD = 54
PKD2: chromosome 4 in 15% - mean age to ESKD = 74
Autosomal recessive:
Chromosome 6 - ESKD in early childhood

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3
Q

Clinical presentation on PKD

A

Abdominal mass or pain
Hypertension
UTI
Renal calculus
Haematuria
Intracranial bleed

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4
Q

Diagnostic criteria for ADPKD

A

In a patient known to be at 50% risk because of FHx, dx is suggested by:
- 2 cysts, either unilateral or bilateral at <30 yrs
- 2 cysts in each kidney in patients aged 30-59yrs
- 4 cysts in each kidney in patients >60yrs
- dx supported by hepatic or pancreatic cysts

In sporadic cases:
- bilateral renal enlargement and cysts, or the presence of multiple bilateral renal and hepatic cysts
- no manifestations suggesting an alternative renal cystic disease

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5
Q

Management of PKD

A
  • management of cardiovascular risk factors
    -BP control and RAAS blockade
  • tolvaptan (ADH receptor antagonist) limits cyst development
  • may require nephrectomy to remove problematic cysts and reduce mass effects
  • renal transplant and dialysis
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6
Q

Extra renal manifestations of PKD

A
  • hypertension
  • liver cysts (70%)
  • pancreatic cysts (10%)
  • berry aneurysms (5%)
  • diverticular disease
  • mitral valve prolapse
  • aortic incompetence
  • hepatic fibrosis
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7
Q

Pathophysiology of PBC

A

Autoimmune condition
Progressive inflammation and destruction of small intrahepatic ducts.
Antimitochondrial antibodies (AMA) is hallmark feature
Strongly associated with RA, sjorgens, CREST

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8
Q

Presentation of PBC

A

Incidental finding of raised ALP
Pruritus
Cholestatic jaundice
Diarrhoea
Lethargy
Xanthelasmata
Skin pigmentation
Clubbing
Hepatosplenomegaly
GI bleed
Osteoporosis

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9
Q

Diagnosis of PBC

A

AMA antibodies present in 95% of cases
Raised ALP
Raised IgM
May require liver biopsy for confirmation

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10
Q

Management of PBC

A

Cholestyramine for pruritis
Ursodeoxycholic acid
Obeyicholic acid either as mono therapy or combination with urso if not tolerated or response (reduces circulating bile acid)
Liver transplant

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11
Q

Complications of PBC

A

Hepatic cancer
Cirrhosis
Liver failure
Osteoporosis
Vitamin deficiency

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12
Q

Causes of hepatomegaly

A

Cirrhosis
Carcinoma
Congestive cardiac failure
Hepatitis B or C
PBC, PSC, AIH
Amyloid
Myeloproliferative disorder

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13
Q

Causes of cirrhosis

A

Alcohol
NAFLD
Hepatitis C and B
Autoimmune hepatitis
PBC
PSC
Wilson’s
Haemachromatosis
a1 antitripsin disease
HHT
Medications

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14
Q

Liver screen bloods

A

Autoimmune antibodies (ANA, AMA, SMA, LKM)
Immunoglobulins
Hepatitis b and c serology
Ferritin
Caeruloplasmin
a1 antitrypsin
AFP

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15
Q

Presenting a chronic liver disease patient

A
  1. Peripheral stigmata of liver disease
  2. Hepatomegaly
  3. Evidence of portal hypertension
  4. Evidence of decompensation
  5. Evidence of cause of liver disease
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16
Q

Classification of cirrhosis

A

Bilirubin, albumin, INR, ascites, encephalopathy
A= 5-6
B= 7-9
C= 10-15

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17
Q

Causes of palmer erythema

A

Cirrhosis
Hyperthyroidism
RA
Pregnancy
Polycythaemia

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18
Q

Causes of gynaecomastia

A

Puberty, old age
Kleinfelters syndrome
Cirrhosis
Drugs - spironolactone or digoxin
Testicular tumour/orchidectomy
Hyper/hypothyroidism
Addisons

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19
Q

Autoantibodies in PSC

A

ANA
anti- smooth muscle may be positive

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20
Q

Autoantibodies in autoimmune hepatitis

A

Anti smooth muscle
Anti LKM1
ANA

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21
Q

Causes of chronic renal failure

A

Diabetic nephropathy (34%)
Hypertension (29%)
Glomerulonephritis (14%)
PKD (14%)
Chronic pyelonephritis (10%)
Obstructive/reflux nephropathy

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22
Q

Advantages of transplant over continued dialysis

A

Improved quality of life
Increase patient survival rate
Cost effective in the long term

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23
Q

Renal diseases where there are a high risk of reoccurrence in transplanted kidney

A

Focal segmental glomerular sclerosis
Amyloidosiss
IgA nephropathy
HUS

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24
Q

Technical considerations for renal transplant

A

Atheromatous iliac vessels
Bladder dysfunction
Bleeding or infection on PKD cysts
Large and bulky kidneys
Uncontrolled hypertension
Renal calculi
Persistent anti- glomerular antibodies

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25
Methods of transplant rejection
Hyper acute - due to presence of recipient antibodies against the donor kidney - requires removal of transplant kidney Acute cell mediated rejection - mononuclear cell infiltration occurs in the interstitial and subsequently in vessel walls - treated with high dose steroids Acute antibody mediated rejection - at least 3/4 of graft dysfunction, histological evidence of tissue injury, positive staining for C4d, presence of donor specific antibodies - poor prognosis, treated with plasmapheresis and IVIg Chronic rejection
26
Kidney donor work up
-Cross match and screening of transmissible disease - MRA and isotope renography
27
MOA and side effects of ciclosporin
Calcineurin inhibitor Inhibits production of IL-2 and TNF-a by binding to ciclophilin protein and inhibiting calcineurin SE: nephrotoxic, hyperkalaemia, hypomagnesaemia, gingival hypertrophy, hyperlipidaemia, glucose intolerance, hypertension
28
MOA and side effects of tacrolimus
Calcineurin inhibitor Inhibits production of IL-2 by helper T cells, by binding Calcineurin to tacrolimus binding protein SE: nephrotoxicity, neurotoxicity ( tremor), glucose intolerance, prolonged QT
29
MOA and SE of mycophenolate mofetil
A prodrug. The active compound is mycophenolic acid which inhibits the enzyme required for guanosine synthesis which results in impaired b and T cell proliferation. SE: nausea, diarrhoea, leukopenia, anaemia, thrombocytopenia
30
MOA and side effects of azathioprine
Anti-metabolite to inhibit DNA and RNA synthesis SE: leukopenia, thrombocytopenia, GI disturbance, cholestasis, alopecia
31
MOA and SE of steroids
Reduce IL-3, IL-6 and TNF-a production and inhibit T cell activation. SE: glucose intolerance, oseoporosis, AVN, cataracts, cushiongoid appearance, infections, poor wound healing
32
MOA and SE of sirolimus
Inhibition of TOR (a regulatory kinase) SE: hyperkalaemia, hypomagnesaemia, hyperlipidaemia, leukopenia, anaemia, impaired wound healing, joint pain
33
Complications of immunosuppression
- New onset diabetes after transplantation - Hypertension - Hyperlipidaemia - SCC of the skin - malignancy related to viral infection eg. HPV and cervical cancer, lymphoma and EBV, or Kaposi sarcoma and HHV-8 - CMV due to reactivation of endogenous disease or transmission from a donor - BK infection
34
Prognostic factors of renal transplant
- primary diagnosis - previous graft failure - episodes of rejection - kidney total ischaemic time - donor factors eg. Age
35
CI to renal transplant
Active malignancy ( must be cancer free for 2 years) Active infection Advanced atheromatous disease is a relative contraindication
36
Calculating SAAG
Serum albumin - ascitic fluid albumin
37
Causes of increased SAAG
Cirrhosis Alcoholic hepatitis Schistosomiasis Fulminant hepatic failure Budd Chiari Portal vein obstruction Cardiac disease SBP
38
Causes of normal or low SAAG
Nephrotic syndrome Protein losing enteropathy Peritoneal carcinomatosis Tuberculous peritonitis Pancreatic duct leak Biliary ascites
39
Treatment of ascites
- fluid restriction - low salt intake - diuretic therapy - paracentesis - prophylactic abx
40
Indications for dialysis in CKD
Uraemic symptoms Uncontrollable fluid overload EGFR of 5-7
41
Indications for dialysis in AKI
Severe metabolic acidosis pH <7.1 Hyperkalaemia refractory to treatment Refractory fluid overload Signs of uraemia Certain toxins
42
Complications of peritoneal dialysis
Bacterial peritonitis Ultrafiltration failure Encapsulating peritoneal sclerosis Hernias Fluid leak, eg through diaphragm causing pleural effusion Malnutrition
43
Complications of haemodialysis
Hypotension Infection, at access point or bacteraemia Venous stenosis or occlusion Air embolus Anaphylactic reaction to sterilising agent
44
Long term complications of dialysis
Vascular disease Cardiac valve calcification - particularly aortic Dialysis related amyloid due to a build up of beta2 microglobulin Pseudogout and gout
45
Causes of splenomegaly
1. Infiltration - lymphoma, amyloidosis, sarcoidosis, Gaucher’s disease, thyrotoxicosis 2. Increased function - spherocytosis, thalassaemia, sickle cell anaemia, infections (chronic malaria, visceral leishmaniasis, glandular fever, brucellosis, RA with feltys, SLE, sarcoidosis 3. Abnormal blood flow - cirrhosis with portal hypertension, hepatic or portal vein obstruction
46
Causes of massive splenomegaly
CML Myelofibrosis Gauchers storage disease Chronic malaria Kala- azar (visceral leishmaniasis)
47
Causes of moderate splenomegaly
Myelo/lymphoproliferative disorders Infiltration eg. Amyloidosis
48
Causes of mild splenomegaly
Myelo/lymphoproliferative disorders Portal hypertension Infections eg. EBV, IE, infective hepatitis Haemolytic anaemia
49
Investigations in patients with splenomegaly
US abdo FBC + film CT CAP Bone marrow aspirate and trephine Lymph node biopsy Thick and thin films x 3 for malaria Viral serology
50
Indications for splenectomy
Rupture (trauma) Haematological (ITP or hereditary spherocytosis)
51
Splenectomy work up
Vaccination (ideally 2 weeks prior to protect against encapsulated bacteria) - pneumococcus - meningococcus - haemophilus influenza Prophylactic penicillin life long Medic alert bracelet
52
Reasons for liver transplant
Cirrhosis - alcohol, NAFLD, Haemachromatosis, Wilson’s Acute hepatic failure - hepatitis A and B, paracetamol overdose Hepatocellular carcinoma
53
What scoring system is used for deciding to list chronic liver disease patients for transplant
Model for End Stage Liver Disease (MELD) Comprised of INR, creatinine, bilirubin, sodium Score > or = 49
54
Complications of liver transplant
Initially: - haemorrhage, graft failure, infection, vascular and biliary complications, rejection Delayed complications: - immunosuppression side effects, disease relapse, chronic graft rejection
55
Clinical signs of haemochromatosis
Increased skin pigmentation (slate grey colour) Stigmata of chronic liver disease Hepatomegaly Venesection scars Liver biopsy scar Swelling and squaring off of MCP joints Joint replacement scars CCF Evidence of diabetes monitoring (Bronze diabetes) Hypogonadism and testicular atrophy
56
Inheritance of haemochromatosis
Autosomal recessive on chromosome 6 Variable penetrance HFE gene mutation: regulator of gut iron absorption Males affected earlier then females due to menstrual iron losses
57
How May Haemachromatosis present
Fatigue Arthritis Chronic liver disease Screening
58
Investigations for Haemachromatosis
Dx: Increased serum ferritin Increased transferrin saturation Liver biopsy used to assess severity of cirrhosis Genotyping Additional: BM/ HbA1c ECG, CXR, Echo Us liver, afp Joint X-rays: chondrocalcinosos
59
Treatment of haemochromatosis
Regular venesection (1unit/week) until ferritin 20-30mg/L and transferritin saturation <50% Then maintenance venesection 1 unit 3-4 times/ year based on ferritin and transferritin levels Avoid alcohol Surveillance for HCC
60
Screening for haemochromatosis
1st degree relative >20 yrs old: Ferritin > 300 in men and >200 in women Transferrin saturation > 50% in males and > 40% in females If positive: genotyping
61
Prognosis of haemochromatosis
200x increased risk of HCC if cirrhotic Reduced life expectancy if cirrhotic Normal life expectancy without cirrhosis + effective treatment
62
Pathogenesis of Haemachromatosis
Mutation in the HFE gene which causes increased intestinal iron absorption, leading to iron deposition in joints, liver, heart, pancreas, pituitary, adrenals and skin
63
Pathophysiology of hereditary spherocytosis
An inherited defect in the structural proteins of red blood cell membranes (usually dominant inheritance). Mutation in 1 of 5 genes. Abnormalities result in changes in red cell shape due to defects in skeletal proteins (alpha spectrin, beta spectrin, ankyrin, band 3 protein and protein 4.2). These red cells are more fragile and so go through haemolysis predominantly in the spleen
64
Clinical presentation of hereditary spherocytosis
Pallor, jaundice, splenomegaly, fatigue, RUQ pain (gall stones)
65
Inv/diagnosis in suspected hereditary spherocytosis
Low Hb, increased mean corpuscular Hb concentration, MCV low Reticulocyte count elevated Blood smear showing spherocytes Elevated unconjugated bilirubin DAT -ve EMA binding or Osmotic fragility test
66
Management of hereditary spherocytosis
Folic acid supplementation Splenectomy +/- cholecystectomy Pre op vaccination regime + post op prophylactic abx
67
Complications of hereditary spherocytosis
Aplastic crisis usually secondary to infection Anaemia Gall stones Bone marrow expansion Extramedullary haematopoeisis Post splenectomy sepsis Post splenectomy thrombosis
68
Aetiology of coeliac disease
Gluten peptides are found in barley, wheat and rye. There is an increased immunological response to ingested gluten. Predisposing factors include: association to HLA antigens, presence of HLA-DQ2 in 95%, the rest are DQ8, other autoimmune conditions.
69
Pathophysiology of coeliac disease
Gluten peptides are resistant to human proteases allowing them to persist in the small intestinal lumen. These peptides trigger immune activation in the intestinal submucosa mediated by T helper cells. Epithelium of small intestine is damaged causing loss of brush border, villous atrophy, crypt hyperplasia.
70
Associated conditions with coeliac disease
Dermatitis herpetiformis Down syndrome Selective immunoglobulin A deficiency Auto immune conditions
71
Diagnosis and inv of coeliac disease
FBC, iron studies, b12, folate, calcium IgA tTg levels EMA Small intestinal biopsy showing intraepithelial lymphocytes, villous atrophy, crypt hyperplasia
72
Where would you expect to palpate combined pancreatic and kidney transplant?
Pancreas: right iliac fossa Kidney: left illiac fossa
73
Prognosis of SPK transplantation vs. Isolated renal transplant
10 year prognosis similar >10 years spk has greater survival
74
Benefits of pancreatic transplant
Improved quality of life Improved glycaemic control Does not reverse established CVD but does slow progression Improved neuropathy Improved nephropathy The effects on retinopathy are unclear
75
Pathophysiology of alcohol dependency
Long term alcohol consumption down-regulates inhibitory neuronal GABA receptors, up regulates excitatory glutamate receptors and increases norepinephrine activity. Cessation of alcohol leaves the excitatory state unopposed.
76
Pathogenesis of alcohol liver disease
Alcohol metabolism requires alcohol dehydrogenase, this process reduces NAD to NADH. Excessive NADH increases fatty acid synthesis, promoting fatty infiltration.
77
Pathogenesis of alcoholic hepatitis
Acetaldehyde binds to macromolecules in hepatocytes eg. Enzymes and proteins. The immune system detects these complexes and neutrophil infiltration occurs. Hepatocyte necrosis and inflammation occurs.
78
Pathogenesis of cirrhosis
Alcohol directly affects stellate cells which transform into collagen- producing myofibroblast cells. There is loss of normal hepatic architecture with modular regeneration and fibrosis.
79
Causes of chronic pancreatitis
Alcohol Cystic fibrosis Smoking Hypercalcaemia/hyperlipidaemia Autoimmune disease Drugs eg. Azathioprine
80
Clinical presentation of chronic pancreatitis
Malabsorption and steatorrhoea Abdomen pain radiating to the back Pain may be relieved by leaving forward DM Erythema ab igne
81
Clinical presentation of acute pancreatitis
Severe epigastric pain radiating to back Worse after meals, improved on leaning forward N&V Tachycardia/hypotension/oliguria Cullens sign Grey-turners sign
82
Investigation of acute pancreatitis
AXR: sentinel loop of adynamic bowel adjacent to pancreas Raised amylase and lipase CT
83
Investigating chronic pancreatitis
Pancreatic calcification on AXR or CT ERCP: irregular dilatation and stricturing of the pancreatic ducts MRCP EUS Faecal elastase Oral glucose tolerance test
84
Management of chronic pancreatitis
Abstain from alcohol and smoking Creon + PPI Dietitian assessment Analgesia Diabetes monitoring Vit D replacement
85
Kings college criteria for transplant in paracetamol overdose
pH < 7.3 Or all of the following: - PT > 100 - creatinine > 300 - grade 3 or 4 encephalopathy
86
Kings college criteria for liver transplant in acute liver failure
PT > 100 Or any 3 of the following; Age <10 or > 40 Bili > 300 Coag- INR > 3.5 Duration (jaundice to HE > 7 days) Etiology- absence of hep A or B or presence of a drug reaction
87
What is the cause of Wilson’s disease?
Autosomal recessive defect in ATP7B on chromosome 13, resulting in reduced excretion of copper into the bile and subsequent accumulation in other organs including the liver and brain
88
Investigations for Wilson’s disease
Low caeruloplasmin levels High urinary copper levels
89
How is Wilson’s managed
Avoidance of high copper containing foods (shellfish, avocados) Alcohol cessation Copper chelation with penicillamine and zinc acetate
90
How can Wilson’s present
Acute or chronic liver failure Haemolysis Neurological involvement inc. movement disorders Psychiatric manifestations Cardiomyopathy Fanconi’s syndrome
91
What are the commonest causes of HCC
CLD particularly hepatitis b and c, alcoholism, hereditary haemochromatosis and PBC. In some countries there is a link with aflatoxins.
92
What primary sites metastasise to the liver?
Stomach, pancreas, colon, lung, breast, ovarian, melanoma
93
Evidence of pancreatic exocrine insufficiency
Fatty stools Weight loss Vit D deficiency Hypomagnesia Low faecal elastase