gastro - conditions overview Flashcards
what classically presents with dyspnoea, cirrhosis and cholestatic jaundice
which feature is more common in children and which is more common in adults?
what tests would you do to confirm the suspected diagnosis?
a1-antitrypsin deficiency
liver in children - commonest form of genetic liver disease in paeds
THE ELASTIC HAS ALL BEEN DESTROYED… THINK ACCELERATED COPD…
emphysema in adults
- serum a1AT levels decreased (nb - remember that a1at is part of the acute phase response - thus may be falsely high..)
- lung function testing - obstructive pattern, decreased fev1 (think the elastic has all been destroyed)
- liver biopsy - Periodic acid schiff stain positive
what is the function of lung a1-antitrypsin?
protect the lungs from neutrophil elastase - it is a serine protease inhibitor (made in the liver) that controls inflamm cascades
how do you manage a1at def?
what is the prognosis
smoking cessation (neut elastase inhibition usually aleviates damage caused by this - but no neutrophil elastase…)
prompt tx of lung infections
proph vacc for lung infections
liver transplant if decompensated cirrhosis
lung transplant
similar prog to copd if lung transplant
what might present early with tiredness / arthragia of 2nd/3rd MCP joints / knee pseudogout / decreased libido
and what would be the later stage presentation?
what organs may be involved?
Hereditrary haemochromatosis
later: slate-grey skin pigmentation / chronic liver disease / hepatomegaly / cirrhosis / cardiomyopathy
liver - cirrhosis
cardiac - c/megaly
kidneys - bronze dm - iron deposited
joints - iron deposited especially in working joints
hypogonadism - pituitary dysfunction because of iron deposition - less production of ACTH
what tests would you do to confirm a Dx of HH?
how would you manage it?
how do you monitor it?
what lifestyle recommendations?
anything else you need to do?
what prognosis?
LFTs raised ferritin raised (but obvs also raised in acute phase stuff) transferrin saturation raised CONFIRM Dx - HFE genotyping (gene most responsible for HH)
management:
venesect! between 0.5 and 2 units per 1/2 wks
once have venesected down to a nice low ferritin (iron stores lowered) - can just maintain with venesection about once a month
Desferrioxamine if intolerant of venesection
Monitoring:
- lfts
- FPglucose - monitoring for DM - as vnesection will throw the HBa1c off..
- if cirrhotic - screen for HCC with USS + AFP 6monthly
lifestyle:
- normal balanced diet - no raw seafood as may cont bacteria that thrive on the iron stores eg. listeria
- avoid alcohol
- ensure vitamins if they take any don’t contain Fe
SCREEN 1ST DEGREE RELATIVES BY GENETIC TESTING FOR HFE GENE - even if Asx and normal LFTs - until age of 30, when Fe deposition would have occured if it was going to….
prog:
normal LE if venesected and non-DM / non-cirrhotic
if cirrhosis - 1/4 get HCC
what does hepcidin do?
what is ferritin?
what is transferrin?
hepcidin reduces gut absorption of Fe and reduces haem iron recycling by Macs
ferritin - iron stores intracellular
transferrin - protein that transports Fe around the blood
where do liver tumours commonly originate?
90% are mets - breast / bronchus / GI tract
what Liver Ca presents early with jaundice…
cholangiocarcinoma. all other liver Cas only have jaundice later…
what Liver Ca presents early with jaundice…
cholangiocarcinoma. all other liver Cas only have jaundice later…
what condition do you suspect in someone presenting with fatigue, wt loss, ruq pain, ascites…
how would you screen for it in people with RFs?
HCC
AFP + ultrasound
what condiiton do you suspect in someone with fever, abdo pain, bili increased, and alp very raised + malaise
what are some causes of this condition?
cholangiocarcinoma
PSC (can screen with Ca19-9)
biliary cysts
HBV / HCV
what should you always exclude in diarrhoea + wt loss / anaemia?
coeliac
what is the management of an acute GI bleed?
- 2 large bore IV cannulae - take blood for LFTs, PT/APTT, group and save to enable crossmatching of blood
2.
what is the management of an acute GI bleed?
ABCDE approach - start by protecting the airway and giving high flow O2
Bedside:
1. 2 large bore IV cannulae - take blood for LFTs, PT/APTT, group and save to enable crossmatching of blood
2. give IV fluids (not saline if cirrhotic / varices…) to restore IV volume while waiting for crossmatched blood… - if still haemodynically deteriorating - give group O Rh-ve blood
3. urinary catheter - hourly UO
Tests:
4. CXR + ECG
5. ?call ICU for a CVP - monitor and guide fluid replacement…
6. transfuse if Hb<70g/L
7. correct clotting abnormalities - Vit K / FFP / platelets
8. ?Varices - GIVE TERLIPRESSIN + broad spectrum IV ABx cover…
9. pulse / BP / CVP monitoring
10. ARRANGE URGENT ENDOSCOPY - WHAT’S GOING ON….?
11. if endoscopic control fails - someone with experience could place a sengstaken-blakemore tube… to compress varices
what ant-emetics carry a dystonic and oculogyric crsis risk - and especially in who..?
anti-dopaminergics younger patients (especially early 20s..)