Author Topic: Case (20)  (Read 4008 times)

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Offline Dr.Hersi

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Case (20)
« on: December 06, 2007, 10:49:20 AM »
A 28 year-old  male presented tto hospital with hypotension, vomiting, and coagulopathy. His past medical history was significant only for an episode of "hepatitis"  which he was treated with one "injection" and afterward was told he was "cured". He did not use tobacco, alcohol, or illicit drugs. Family history was significant only for hypertension and diabetes. He was taking no medications. The patient was admitted to the ICU.

 Ultrasound showed a nodular liver, splenomegaly, and ascites. Paracentesis was done which revealed an ascitic WBC of >11,000.

. Serum ceruloplasmin was quite low at 10,

CT revealed ascites, a shrunken liver, and possible portal vein collaterals.

On examination he was obtunded and jaundiced. . cardiopulmonary was normal ,. abdomen was distended with shifting dullness.

On the basis of this clinical presentation and labwork,

What is the diagnosis, ?

Tell one pathognomic  sing  ?

Tell one investigation to confirm the Diagnosis, ?

-Treatment  of this case , ?





Offline Kassim

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Re: Case (20)
« Reply #1 on: December 06, 2007, 03:38:49 PM »
What is the diagnosis?
I think it is Wilson's disease.

Tell one pathognomonic sing?
 Kayser-Fleischer rings (they are no longer considered pathognomonic of Wilson disease unless accompanied by other manifestations. They may also be observed in patients with chronic cholestatic disorders, such as partial biliary atresia, primary biliary cirrhosis, primary sclerosing cholangitis, and cryptogenic cirrhosis.)

Tell one investigation to confirm the Diagnosis?
In the absence of Kayser-Fleischer rings or neurologic abnormalities, a liver biopsy for quantitative copper determination is essential to establish the diagnosis of Wilson disease.
It may also be useful to confirm the diagnosis and to evaluate the response to chelation therapy.

Treatment  of this case ?
The mainstay of therapy for Wilson disease is pharmacologic treatment with chelating agents, (Penicillamine) if he cannot tolerate penicillamine then Trientine will be the alternative.  Since the initial presentation is hepatic, Zinc should be added.
He should generally avoid eating foods with a high copper content, such as liver, chocolate, nuts, mushrooms, legumes, and shellfish (especially lobster). Drinking water from atypical sources (eg, well water) should be analyzed for copper content and replaced with purified water if the copper content is greater than 0.2 parts per million.

Orthotopic liver transplantation is a potentially curative treatment of Wilson disease.
hope this right answer
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Offline Dr.Hersi

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Re: Case (20)
« Reply #2 on: December 08, 2007, 05:48:07 PM »
thanks dr kassim  your answers are correct, 

Offline Admin

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Re: Case (20)
« Reply #3 on: December 09, 2007, 12:16:02 AM »
Congratulations
The 15 points of this case goes to Kassim

Dr.Mahdi
"you never cure a patient, you treat pain often but you always comfort the patient."
www.somalidoc.com


 

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