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PACES - Pastest bites. > Abdominal > Flashcards

Abdominal Flashcards

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
Q

What does HFE gene code for

A

Iron absorption.

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24
Q

Hereditary spherocytosis how is it inherited

A

Autosomal dominant , Chromosome 8, defect in 1 in 5 genes , that code for proteins in the RBC

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25
Q

Clinical findings of Spherocytosis

A

Anaemia
Jaundice
Leg ulcer
? Cholecystectomy (slightly unusualfor HS)
? Splenectomy

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26
Q

Typical presentation of Hereditary spherocytosis

A

1) Screening programmes
2) Neonatal jaundice
3) Anaemia and lethargy
4) Anaemia , jaundice and splenomegaly

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27
Q

spherocytosis Complications

A

Aplastic crisis - due to an infection
Anaemia - serial Blood transfusions
Gallstones

28
Q

Establish a spherocytosis diagnosis

A

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
Q

Treatment for Hereditary spherocytosis

A

Folic acid
Serial blood transfusions
Splenectomy
Cholecystectomy

30
Q

Prior to splenectomy a patient will need

A

regular vaccines : pneumococcal , influenza, haemophilus, lifelong penicllin. Alert bracelet.

31
Q

Mechanism of haemolysis in spherocytosis

A

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
Q

Causes of isolated splenomegaly

A

malaria , CML , myelofibrosis , spherocytosis

33
Q

Investigations for Coeliac disease

A

FBC -
HAematinics
TTG gene ( on gluten diet)
OGD - with biopsy taken from D2

34
Q

What type of renal transplantations do you know

A

Live or deceased donors. Cadaveric circulatory or neurological death

35
Q

which is better live kidney or cadaveric combined

A

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
Q

What’s the drainage of the transplanted pancreas

A

Transplanted pancreas are drained to the small bowel

36
Q

Causes of liver cirrhosis

A

Viral hepatitis
Alcohol hepatitis
Non alcoholic steatohepaittis
Wilson
Haemochromatosis
PBC PSC AI
Alpha 1 antitrypsin
Drugs : methotrexate

36
Q

how do you assess for decompensated liver disease

A

encephalopathy
asterixis
ascites

37
Q

How do you assess for encepholpathy

A

ask them to perform a 5 pointed start

38
Q

How do you assess cirrhosis

A

Child Pugh (albumin , coagulopathy , bili rubin , encephalopathy)

39
Q

How do you treat ascites

A

Fluid and na restriction
Spironolacton
ascites
TIPs ( refractory to drainage)

40
Q

What are the complications of TIPS

A

Coagulopathy
5-10% become encephalopathic post procedure

41
Q

What are the differentials of gynaecomastia

A

Is due to an relative androgen deficiency compared to oestrogens
Testicular atrophy
Physiological at puberty
Tumour
Orchidectomy
Klienfelters
drugs; spironolactone, digoxin.

42
Q

Complications of Cirrhosis

A

Portal hypertension : varices
Ascites : heptorenal , hepatopulmonary syndrome
coagulopathies: UGIB

43
Q

Treatment for varices

A

Non selective b blockers
Banding the presence of bleeding.

44
Q

Complications of Alcohol use

A

Liver cirrhosis
Polyneuropathy
Werncike’s encephalopathy
Cardiomyopathy
Pancreatitis

45
Q

Causes of pancreatitis

A

Gallstones
Ethanol
Trauma
Steroids
Mumps
Autoimmune
Scorpion bite
Hypertriglyceridaemia
ERCP
D

46
Q

complications of pancreatitis

A

Acute and chronic.
Acute is multisystem disorder leading to SIRS , respiratory failure , death
Chronic complications : portal vein thrombosis , splenic vein thrombosis , pseudocyst

47
Q

how do you drain a pancreatitic pseudocyst

A

endoscopic approach , axios stent , performed 6 weeks after pancreatitis

48
Q

how does chronic pancreatitis present

A

chronic pain
epigastric, radiating to back and sitting up is better
grumbling gnawing pain

49
Q

Mainstay of treatment of pancreatitis

A

avoid causes of pancreas inflammatoion : Alcohol and smoking avoidance , Creon , PPI

50
Q

How would you investigate a patient with IBD

A

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
Q

Whats the differences between crohns disease and UC

A

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
Q

Treatment of UC

A

oral and rectal -5-aminosalicylic acid
Oral steroids
IV steroids
>2 flares of UC requiring steroids then treatment escalation to Azathioprine or infliximab.

53
Q

Differentials of causes of a liver transplant

A

Liver Cirrhosis
Alcohol
Hepatocellular carcinoma
Acute Liver failure : paracetamol overdose or acute viral hepatitis

54
Q

Complications of prednisolone

A

Thinning of the skin
poor glycaemic control
Easy bruising
Weight gain
hypertension
cataracts

55
Q

Complications of ciclosporin

A

Gum hypertrophy
Hypertension
Increased risk of Cancers.

56
Q

Evidence of Liver transplant failure

A

Jaundice
ascites
Encephalopathy
Portal hypertensions : Varices , caput medusae

57
Q

Which patient’s are eligible for a liver transplant

A

MDT decision
Significant liver disease
Prognosis following liver transplant
UKESLD ( UK END STAGE LIVER DISEASE) is helpful for prognosis

58
Q

Which signs of CLD persist post transplanation

A

Gynaecomastia
Duputyren’s contracture

59
Q

Define acute liver failures

A

Multisystem disorder with severe acute impairment of liver function with encephalopathy that occurs within 8 weeks of onset of symptoms.

60
Q

Variant syndromes that would mean people would be listed for liver transplant

A

Diuretic resistant ascites, chronic hepatitic encephalopathy , intractable pruritis , polycystic liver disease hepatoportopulmonary syndrome, chronic cholangitis

61
Q

Contraindications to liver transplant

A

ongoing alcohol excess, psychiatric comorbidities , cardio or cerebrovascular disease, IV Drug use.

Survival is worse for those >65 y.o

62
Q

Criteria for Elective liver transplant

A

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
Q

Portopulmonary hypertension

A

Long acting prostatcylin or sildenafil

64
Q

Paracetamol poisoining criteria for super urgent transfer.

A

pH < 7.25 despite fluid resuscitation ,
PT >400
INR >6.5 ,
+ Creat >300
Grade III IV of encephalopathy

64
Q

Complications of trasnpalnat

A

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
Q

Commonest causes of ESRF

A

Diabetes
Hypertension
Glomerulonephritides
ADPKD

66
Q

When do you start to think about performing a renal transplant

A

Approaching < 15 ml / min

67
Q

Contraindcations to renal transplant

A

Donor matching issues, active or recent malignancy , deep seated infection , active vasculitis , Severe obesity

PACES - Pastest bites. (3 decks)

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