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Pediatric Liver Disease

Pediatric Liver Disease. Alyssa Brzenski. Pediatric Liver Disease. Keely DA, Hartley. End Stage Liver Disease. Chronic Liver Disease. Acute Liver Disease. Biliary Atresia. Biliary Atresia. The Lancet. . The Statistics. Most common in southeast Asia 1: 15,000 born in the US

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Pediatric Liver Disease

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  1. Pediatric Liver Disease Alyssa Brzenski

  2. Pediatric Liver Disease Keely DA, Hartley. End Stage Liver Disease.

  3. Chronic Liver Disease

  4. Acute Liver Disease

  5. Biliary Atresia Biliary Atresia. The Lancet.

  6. The Statistics • Most common in southeast Asia • 1: 15,000 born in the US • 2.5 times more common in africanamerican mothers • Most common cause of pediatric liver transplant • 10-20% associated with congenital anomalies • Biliary atresia splenic malformation • Polysplenia (90%) • Situsinvertus (50%) • Vascular anomalies (70%) • Intestinal malrotation (60%) • Cardiac anomalies- VSD, ASD, HLHS (50%)

  7. Presentation Jaundice Pale Stools Dark Urine Failure to Thrive Coagulopathy

  8. Work up

  9. Kasai Procedure

  10. Anesthesia for a Kasai Routine Monitors +/- A-line 2 PIV Balanced Anesthetic +/- Epidural

  11. Prognosis Previously unpublished data, based on UK Biliary Atresia Registry (1999–2008).93 BASM=biliary atresia splenic malformation syndrome.

  12. Liver Transplants

  13. Statistics • 16,414- currently on the Liver Transplant waitlist (as of March 9, 2013) • 475 of which are children • 3 children listed at Radys • In 2011, there were 536 pediatric recipients in US • Within 2 years of being listed for a liver transplant, 20-30% of patients had not been transplanted

  14. LI 8.1 Pediatric patients waiting for a liver transplant

  15. LI 8.2 Distribution of pediatric patients waiting for a liver transplant

  16. LI 8.7 Pre-transplant mortality rates among pediatric patients wait-listed for a liver transplant, by age

  17. LI 8.8 Pediatric liver transplants, by donor type

  18. LI 8.12 Use of DCD donors in pediatric liver transplant recipients

  19. LI 8.18 Survival among pediatric liver transplant recipients, 2002–2006, by age

  20. Split Liver Transplant

  21. Anesthesia Considerations • Full stomach- RSI • Large bore Upper extremity IV access • 1 central line • Large bore PIV • Arterial Line

  22. Surgical Stages Yudkowitz FS, Chietero M. Anesthesia Issues in Pediatric Liver Transplants. Pediatric Transplant. 2005; 9; 666-72.

  23. Hepatic Artery Thrombosis Hematocrit less than 30 Attempt to avoid platelet transfusion Avoid hypercoagulable state Dextran used by some centers

  24. Sources Hartley JL, Davenport M, Kelly DA. Biliary Atresia. Lancet 2009; 374:1704-13. Scientific Registry of Transplant Recipients. 2011 Liver OPTN/SRTR annual report. http://www.ustransplant.org/ Hendrickson R et al. Pediatric Liver transplantation. Current OpinPediatr 2004; 16: 309-13. Hartley J and Kelly D. End stage liver failure. Paediatrics and Child Health 2009; 20: 30-5.

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