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Brave Fight Against Propionic Acidemia: Liver Transplant Success

Created by:Dr.
Published:January 15, 2024
Last updated:
Views:5449+

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Reviewed by Dr. , MBBS, MD on .
Next review due: November 2026

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Medical References

This video content is based on current medical evidence and guidelines from authoritative sources:

  1. 1.
    World Health Organization (WHO) - Global Health GuidelinesView Source
  2. 2.
    Centers for Disease Control and Prevention (CDC) - Evidence-Based GuidelinesView Source
  3. 3.
    National Health Service (NHS) - Clinical StandardsView Source
  4. 4.
    Peer-Reviewed Medical Journals - Latest Research & Clinical Studies(The Lancet, JAMA, NEJM, BMJ)

Transcript

dis um for coor hello everyone I'm Dr rindra CV consultant Hilary and liver transplant surgeon at Nar H City Bangalore today I would like to share my um thoughts and our experiences of the team with the child with propionic Academia this was one of a challenging case where we identified the propionic acidemia uh nearly when the child was around less than 6 months age initially we advised the child for the liver transplantation but they took some much of a time to just just to make the decision to how to go about and when to go about and finally nearly when the child was around 1 year 2 months the they stepped forward for the living donor liver transplantation the father was the donor here and the child after evaluation she had a neurological issues also wherein the Milestones were not been completely reached she was not able to sit comfortably without support and uh she was irritable all the time and she was refusing to eat not putting on much of the weight so the challenges in this case were multifold with respect to the metabolic liver disorder itself though we go when we go through the literature the results are good enough it doesn't get translated in all the cases the initial pre-operative management and even the pre-operative management and the post-operative management was a challenging in this case how the child would respond in this particular situation was one of the situations which we try to handle and it came out very nicely wherein the transplantation was uneventful uh the surgery took around 10 to 12 hours and with not much of any blood loss and uh her liver was been explanted out and the father's liver was a left lateral segment which was transplanted to the child postoperatively she did completely fine she was shifted out of the ICU by around day six and she was started on uh initially with NG feeds and then subsequently orally initially we went about with the metabolic nutrient metan nutrient diet and then subsequently changed over to the normal diet the post-operative care was even um uh supported by the critical care team from the Pediatric team Dr SOS UT and Dr sapare Anil sapare and um uh subsequently the child is been now completely doing fine she is is on minimal imuno oppression and completely on a normal diet she has been able to sit comfortably now with support she's making her attempts to stand and even walk with support initially theological evaluation which was initially was made pre-operatively that the neurological squl was there with the propionic cadmia slowly I think over a period of time has been getting resulted and I hope very much hopeful the child will get back to normal SE and lead a normal life as other children so this is one of the success stories again with the Pediatric liver transplantations and the setup at narana Health City we do both had and pediatric liver transplantations and we do both living donor and the cavic liver transplantations with high success rates and it is a Under One Roof everything has been provided wherein multi-disciplinary team with the surgical medical management the hepatologist the surgical uh um team and the anesthetist and the radiologist and the intervention radiologist everyone has been there with all the setup has been [Music] there

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