A Case, think ...

[Hour_Glass]

This is my case in final year examination ...i wanted to share it with you people

Case: a patient aged 19 years old presented with a history of 3 years of prolonged jaundice and pallor ...sometimes in morning he passes red urine. now he is complaining from abdominal pain ,hepatomegaly and ascitis ...ascitic fluid examination revealed protein level of 4.5 gm/dl ....imaging revealed caudate hypertrophy .his Hb electrophoresis is normal .

1.what is your diagnosis?
2.tests to confirm the diagnosis?
3.managment of this patient


Tell me what you are thinking of ....it is nice to discuss



thanx
Farah Baha

[asclepius]

I don't know where did they find such a case!
Is it paroxysmal nocturnal hemoglobinuria?
I don't know much about this. In fact, I don't know anything about it but I the name seems suitable for the occasional morning red urine. I don't know how to relate the other signs.

[EKRAM F.]

Hi Farah how u doing..
i am not in the 6th stage but i asked about it and here is what i got

1-the picture is suggestive 4 chronic active hepatitis
& this disease may b as a complication of a- hepatitis D infection b- autoimmune disease mainly but may present as acomplication of other diseases as SLE..
*the red urine in the morning is due 2 high bilirubin level(and the pt is jaundiced as u mentioned)and as u know the highest bilirubin conc. is usually in the morning
*and because of hepatomegally we exclude cirrhosis
*u may suspect in haemolytic uraemic syndrome (but this is with no red color urine)
also suspection of paroxysmal nocturnal haemoglobinurea (as asclepius said) is excluded cuz u have ascitis & jaundice ...

2- about the investigation a- liver function test AGOT and AGPT
bilirubin: direct & indirect , prothrombin time
b- ascitic cells: types and count
c- US check the portal system, eosophgeal varices
d- nuclear Ab , HCV Ab
and finally u may do liver biopsy

3-im not sure about the management
i hope this is useful
take care
ps.its anice idea 2 share the cases here, thanks farah 4 this .. i wonder if any one else have an interesting case 2 share with us...

[Hour_Glass]

Dear Asclepius and Ekram ,

this case is given as the hit of the exam among many other cases .
the true story is that they had a patient who presented to them in Sulaymania hospital with the mentioned findings and it took them one year to diagnose it !

Back to the case ......Asclepius you just answered in the same way i did which will give you 4-5 /6 which is good .

so here is the answer
1.the diagnosis is paroxysmal nocturnal hemoglobinurea (explains the occasional red urine) this PNH resulted in forming Budd Chiari syndrome which is thrombosis of hepatic vein which is by the way a very common complicaton of PNH (explains ascitis ,abdominal pain...and hepatomegaly).

2.Diagnositic tool .Ham's test to confirm PNH and ultrasound for abdomen to demonstrate the thrombosis .

3.management is anticoagulation, blood transufusion in severe cases and stem cell transplant .

it is difficult ..i know but after all we are learning from our mistakes

hope that helps
Farah Baha

[Dr.Leo1984]

from the first time i guess the case is related to dr. ziad jredeni lectures but i wasn't sure of that coz i am in the fifth stage and do not remember them...thnx for u alllllll

[hammodi]

thanks farah, and tell me, what about the caudate hypertrophy?

[dr.leo]

well this is a bizzarecase i was asked about thing lik it in my final practical exam for 6th stage this year
ask about spleen is there is splenomegaly or not??
u can put TB as 1 st ddx it work every where cauz we endemic
pallor is anaemia pictur suggest leukamia so ch .myl .leuk with auto immun haemolytic anaemia also work
infection hepatitis is ch hepat b or c asociated with secondory renal involv
auto immune hepatitis i think is good answer but wt the geneder of the pt??

invest depend on ddx lft rft pcr us gue ct abd many liver biopsy bm asp the last
mx depend on cause treat anaemia look 4 underlying cause

[dr.leo]

porphyria this case might be