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Dr. Andrew Rynne
MD
Dr. Andrew Rynne

Family Physician

Exp 50 years

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Suggest Treatment For Budd-Chiari Syndrome In Infants

hi, My son is 2 years old and is diagnosed of budd chairi syndrome a year ago. all is veins are patent now however his liver is enlarged (2.5 inches below the last rib) and he shows psudeo spleenomegaly. There is no ascites. Also he does not sit or walk on his own. He sits only if we make him sit and walks if we hold his hand however he is fond of climbing stairs. What should I do so that his liver goes back to normal and he achieves his milestones appropriately?
Fri, 11 Mar 2016
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Internal Medicine Specialist 's  Response
Thanks for asking at Healthcare Magic I read your question and I understand your concern.
Hepatomegaly with Pseudosplenomegaly are signs of Portal Hypertension. Hepatomegaly occurs because of liver congestion that leads to decrease synthesis which include Growth factors, albumin, some harmones, diuretics can also lead to weight loss.
I would like to suggest you that don't be much panic as inspite of this disorder he still can sit/walk with support. Give him good balanced diet.keep him in observation If liver size increase and splenomegaly also getting massive or developing ascites despite diuretics, then go for the following investigations
Do his liver biopsy if shows necrosis then straight away go for reducing portal hypertension to stop fibrosis either through Portocaval shunt surgery or Transjugular intrahepatic shunt. If biopsy shows extensive necrosis then liver transplant is best option.
Hope it helps best.
take his best care and of yourselves.
I will be glad for leading questions.
don't forget to rate this answer and close the discussion at Healthcare Magic.
Regards
Dr Ejaz
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Suggest Treatment For Budd-Chiari Syndrome In Infants

Thanks for asking at Healthcare Magic I read your question and I understand your concern. Hepatomegaly with Pseudosplenomegaly are signs of Portal Hypertension. Hepatomegaly occurs because of liver congestion that leads to decrease synthesis which include Growth factors, albumin, some harmones, diuretics can also lead to weight loss. I would like to suggest you that don t be much panic as inspite of this disorder he still can sit/walk with support. Give him good balanced diet.keep him in observation If liver size increase and splenomegaly also getting massive or developing ascites despite diuretics, then go for the following investigations Do his liver biopsy if shows necrosis then straight away go for reducing portal hypertension to stop fibrosis either through Portocaval shunt surgery or Transjugular intrahepatic shunt. If biopsy shows extensive necrosis then liver transplant is best option. Hope it helps best. take his best care and of yourselves. I will be glad for leading questions. don t forget to rate this answer and close the discussion at Healthcare Magic. Regards Dr Ejaz