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PACES - Pastest bites. > Abdominal > Flashcards

Abdominal Flashcards

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1
Q

What is the inheritance of Polycystic kidney disease

A

Auto dominant, 80% have mutation on chr 16, 20% on chr 4. small % have no abnormality

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1
Q

How might someone with ADPKD present

A

with worsening renal function , renal failure , proteinuria or haematuria , pain , or extra renal cysts - in the pancreas or the liver

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2
Q

Phenotype of each Type of PKD

A

Type 2 , chr 4 has less cysts and less likely to progress to ESRF

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3
Q

Complications of PKD

A

1) Hypertension, 2) Cysts : liver pancreas or seminal vesicles 3) risk of cerebral aneurysms - SAH 4) colonic diverticulae.

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3
Q

Management of PKD

A

Multimodal management - good control of BP with ACE inhibitors, aggresive control of hyperlipidaemia , diet should be high fluid low salt. Early disease Vasopressin antagonists may be of use , Late disease may need RRT or Renal transplant

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4
Q

Indications of a nephrectomy of a PKD

A

Ideally Nephrectomy should be avoided. Indications could be however to make room for a transplant , progression to renal cell carcinoma, chronic pain or chronic infection , haematuria .

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5
Q

Do you always need to perform a nephrectomy before a transplant

A

On occasion it may be necessary to make room but otherwise it should not be needed.

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6
Q

Causes of ESRF

A

HTN , Diabetes, glomerulonephritis , ADPKD

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7
Q

When do you work up for Transplantation

A

Work up as they approach ESRF but before they require dialysis. WHen they receive transplatation is based on availability of donors. Prognosis is improved if it is done before they have ESRF

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8
Q

Define ESRF

A

Egfr < 15ml/min

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9
Q

Contraindications to transplantation

A

Donor Mismatching
Malignancy
Ongoing deep seated infection
Ongoing vasculitis
Severe obesity ( technical difficulty)

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10
Q

Immunosuppression Side effects / toxicity in Renal transplant

A

Tacrolimus - Tremor
Ciclosporin - Gingival hypertrophy, Hirsutism
Steroids - Fat redistribution , Bruising
General for all immunosuppression:
- Infection , Skin malignancy

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11
Q

What investigations would you request for PKD

A

BS: Urinalysis and BP
Bloods: Creat , electrolytes
Radio : USS KUB

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12
Q

Reasons for Cirrhosis

A

Viral hepatitis
Autoimmune conditions
Metabolic: wilson/ haemochromatosis
ETOH
NAFLD

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13
Q

Reasons for liver transplant

A

1) Cirrhosis
2) Paracetamol toxicity
3) HCC

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14
Q

Scoring systems for a liver transplant prognosis

A

UK end stage liver disease model

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15
Q

Haemochromatosis investigations

A

Ferritin levels, transferrin saturations, genetic testing of the HFE gene.

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16
Q

Haemochromatosis signs

A

Synovitis - typically metacarpophalyngeal arthralgia , squaring of the joints, costocaldrinosis on XRAY
Venesection
Chronic liver disease

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17
Q

Inheritance of haemorchromatosis

A

Hereditary recessive , Chromosome 6 , HFE gene

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18
Q

Presentation of haemochromatosis

A

Asymptomatic Ferritin
Severe disease: sexual dysfunction , lethargy , bronze pigmentation, diabetes, arthralgia

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19
Q

HFE screening programme

A

With first degree relatives, Baseline ferritin >200 Females, >300 Males combined with:
Transferrin saturation of >40% females >50 % males

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20
Q

Further investigations for HFE

A

Hba1c (urine dip glycosuria)
Plain films of joints - hook like osteophytes in the MCPJ
6 monthly USS of liver
HCC screen - AFP
Baseline ECHO : Cardiomyopathy
(liver biopsy for severity , not diagnosis)

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21
Q

Treatment of Haemochromatosis

A

Venesection 1/week until transferrin is below acceptable level.
Avoid alcohol

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22
Q

Complicatiations of HFE

A

Liver cirrhosis
Cardiomyopathy
Arthropathy
HCC
Anterior pituitary dysregulation.

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23
What does HFE gene code for
Iron absorption.
24
Hereditary spherocytosis how is it inherited
Autosomal dominant , Chromosome 8, defect in 1 in 5 genes , that code for proteins in the RBC
25
Clinical findings of Spherocytosis
Anaemia Jaundice Leg ulcer ? Cholecystectomy (slightly unusualfor HS) ? Splenectomy
26
Typical presentation of Hereditary spherocytosis
1) Screening programmes 2) Neonatal jaundice 3) Anaemia and lethargy 4) Anaemia , jaundice and splenomegaly
27
spherocytosis Complications
Aplastic crisis - due to an infection Anaemia - serial Blood transfusions Gallstones
28
Establish a spherocytosis diagnosis
FBC - reticulocyte count and MCH Blood smear - looking evidence of spherocytes or haemolysis Haemolysis screen - Haptoglobin (low) , LDH (high) , split bilirubin- unconjugated is high , Coombs test (eliminates immune mediated haemolysis), EMI binding test (eosin based fluorescent dye , the mean fluoscent is lower with HS) / osmotic fragility test
29
Treatment for Hereditary spherocytosis
Folic acid Serial blood transfusions Splenectomy Cholecystectomy
30
Prior to splenectomy a patient will need
regular vaccines : pneumococcal , influenza, haemophilus, lifelong penicllin. Alert bracelet.
31
Mechanism of haemolysis in spherocytosis
The gene defect cause the rbc to be spherocyte shape rather than biconcave meaning they are susceptible to haemolysis in the spleen ( hence splenectomy eliminates haemolysis )
32
Causes of isolated splenomegaly
malaria , CML , myelofibrosis , spherocytosis
33
Investigations for Coeliac disease
FBC - HAematinics TTG gene ( on gluten diet) OGD - with biopsy taken from D2
34
What type of renal transplantations do you know
Live or deceased donors. Cadaveric circulatory or neurological death
35
which is better live kidney or cadaveric combined
A live kindey's advantages : donor and recipient can be brought together, reducing warming ischaemic time. A Cadaveric combined has the ability to cure both the diabetes and the ESRF
35
What's the drainage of the transplanted pancreas
Transplanted pancreas are drained to the small bowel
36
Causes of liver cirrhosis
Viral hepatitis Alcohol hepatitis Non alcoholic steatohepaittis Wilson Haemochromatosis PBC PSC AI Alpha 1 antitrypsin Drugs : methotrexate
36
how do you assess for decompensated liver disease
encephalopathy asterixis ascites
37
How do you assess for encepholpathy
ask them to perform a 5 pointed start
38
How do you assess cirrhosis
Child Pugh (albumin , coagulopathy , bili rubin , encephalopathy)
39
How do you treat ascites
Fluid and na restriction Spironolacton ascites TIPs ( refractory to drainage)
40
What are the complications of TIPS
Coagulopathy 5-10% become encephalopathic post procedure
41
What are the differentials of gynaecomastia
Is due to an relative androgen deficiency compared to oestrogens Testicular atrophy Physiological at puberty Tumour Orchidectomy Klienfelters drugs; spironolactone, digoxin.
42
Complications of Cirrhosis
Portal hypertension : varices Ascites : heptorenal , hepatopulmonary syndrome coagulopathies: UGIB
43
Treatment for varices
Non selective b blockers Banding the presence of bleeding.
44
Complications of Alcohol use
Liver cirrhosis Polyneuropathy Werncike's encephalopathy Cardiomyopathy Pancreatitis
45
Causes of pancreatitis
Gallstones Ethanol Trauma Steroids Mumps Autoimmune Scorpion bite Hypertriglyceridaemia ERCP D
46
complications of pancreatitis
Acute and chronic. Acute is multisystem disorder leading to SIRS , respiratory failure , death Chronic complications : portal vein thrombosis , splenic vein thrombosis , pseudocyst
47
how do you drain a pancreatitic pseudocyst
endoscopic approach , axios stent , performed 6 weeks after pancreatitis
48
how does chronic pancreatitis present
chronic pain epigastric, radiating to back and sitting up is better grumbling gnawing pain
49
Mainstay of treatment of pancreatitis
avoid causes of pancreas inflammatoion : Alcohol and smoking avoidance , Creon , PPI
50
How would you investigate a patient with IBD
FBC - Anaemia Folate , B12 - malabsorption Urea and electrolyte CRP LFTs AXR - toxic megacolon OGD/ colonscopy- biopsies of the large bowel (consider that they may have had a colectomy) Faecal calprotectin faeces MCS to r/o other infections
51
Whats the differences between crohns disease and UC
UC - affects the large bowel predominantly , rectal and sigmoid colon, causes no anal problems, only affects the mucosa of the large bowel continuous , fibrosis of the large bwoel Crohns: affects any part of the GI tract, anal problems occur, transmural and fistulating, skip lesions
52
Treatment of UC
oral and rectal -5-aminosalicylic acid Oral steroids IV steroids >2 flares of UC requiring steroids then treatment escalation to Azathioprine or infliximab.
53
Differentials of causes of a liver transplant
Liver Cirrhosis Alcohol Hepatocellular carcinoma Acute Liver failure : paracetamol overdose or acute viral hepatitis
54
Complications of prednisolone
Thinning of the skin poor glycaemic control Easy bruising Weight gain hypertension cataracts
55
Complications of ciclosporin
Gum hypertrophy Hypertension Increased risk of Cancers.
56
Evidence of Liver transplant failure
Jaundice ascites Encephalopathy Portal hypertensions : Varices , caput medusae
57
Which patient's are eligible for a liver transplant
MDT decision Significant liver disease Prognosis following liver transplant UKESLD ( UK END STAGE LIVER DISEASE) is helpful for prognosis
58
Which signs of CLD persist post transplanation
Gynaecomastia Duputyren's contracture
59
Define acute liver failures
Multisystem disorder with severe acute impairment of liver function with encephalopathy that occurs within 8 weeks of onset of symptoms.
60
Variant syndromes that would mean people would be listed for liver transplant
Diuretic resistant ascites, chronic hepatitic encephalopathy , intractable pruritis , polycystic liver disease hepatoportopulmonary syndrome, chronic cholangitis
61
Contraindications to liver transplant
ongoing alcohol excess, psychiatric comorbidities , cardio or cerebrovascular disease, IV Drug use. Survival is worse for those >65 y.o
62
Criteria for Elective liver transplant
1) Chronic liver disease and have high UKELD score 49 or more (serum NA , creat, Bili and INR) 2) Severe acute alcohol hepatitis , - Madrey and Glasgow . ( Must have a 5 year survival of more than 50%)
63
Portopulmonary hypertension
Long acting prostatcylin or sildenafil
64
Paracetamol poisoining criteria for super urgent transfer.
pH < 7.25 despite fluid resuscitation , PT >400 INR >6.5 , + Creat >300 Grade III IV of encephalopathy
64
Complications of trasnpalnat
rejection ( early phase) immunsuppression : infection and malignancies , post transplant lymphoproliferative disorders metabolic syndrome: hyperglycae, hypertriglyceridaemia avoid live vaccines Biliary complications : leaks and strictures recurrence of dieass
65
Commonest causes of ESRF
Diabetes Hypertension Glomerulonephritides ADPKD
66
When do you start to think about performing a renal transplant
Approaching < 15 ml / min
67
Contraindcations to renal transplant
Donor matching issues, active or recent malignancy , deep seated infection , active vasculitis , Severe obesity

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