Online Aspen Webinar (Part 7) -Liver Organ Allocation

Below I’ve included a few slides and some notes from recent Aspen Webinars; my notes may have errors of omission or transcription.

Key Points:

  • The new allocation policy tries to make liver organ allocation more equitable in terms of disease acuity at time of transplantation and access to allografts
  • The changes, based on some preliminary data, appear to improve the likelihood of children receiving needed organs. Dr. Bondoc specifically cited the work of Dr. John Bucuvalas in pointing out some of the systemic ways that the previous system disadvantaged children.
    • Infants are at the greatest risk on the wait list.  Yet, successful transplantation in children could be beneficial for many decades
    • PELD underestimates mortality risk
    • 25% of pediatric donors have historically gone to adults

 

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Online Aspen Webinar (Part 6) -NAFLD and NASH

Aspen Online Webinar July  14-16, 2020

Below I’ve included some of my notes and slides.  There may be errors of omission or transcription.

What’s Hot? NAFLD and NASH Stavra Xanthakos

  • Fatty liver disease burden of NAFLD and NASH is increasing.  This increases the rate of cirrhosis, liver cancer and liver transplantation; the latter is being needed at younger ages
  • Explained that “Lean” (normal BMI) NAFLD is common
  • Diabetes is strongest risk factor for severe fatty liver disease (NASH or fibrosis). PNPLA3 is genetic risk factor for NAFLD risk.
  • Discussed treatment, particularly diet  and bariatric surgery.  Stated that some emerging treatments look promising.
  • In those with suspected NAFLD, Dr. Xanthokos recommends liver biopsy, if lifestyle therapy is ineffective, under specific circumstances: prior to bariatric surgery, in some cases to determine severity, and prior to instituting therapy (eg Vitamin E)

              

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Online Aspen Webinar (Part 5) -Biliary Atresia Diagnosis and Screening

Online Webinar –Annual Aspen Conference  —July 14, 2000

Below I’ve included a few slides and some notes; my notes may have errors of omission or transcription.

Biliary Atresia -Prompt Diagnosis and Screening Ronald Sokol

Key Points:

  • We have NOT improved age of diagnosis in biliary atresia in the past 30 years
  • Uniform screening of fractionated bilirubin has been effective in Texas:  Diagnostic Yield of Newborn Screening for Biliary Atresia Using Direct or Conjugated Bilirubin Measurements S Harpavat et al. JAMA 2020; 323: 1141-50
  • Pale stools are usually NOT due to biliary atresia but should prompt investigation (eg. fractionated bilirubin)
  • MMP-7 may improve diagnostic approach; unclear if MMP-7 performs well in all populations (eg. prematurity)
  • Outcome key factors: age at diagnosis (goal less than 30-45 days) and surgeon/center

 

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Online Aspen Webinar (Part 4) -How to Treat Hepatitis C in Children

Below I’ve included a few slides and some notes; my notes may have errors of omission or transcription.

How I Treat Children with Hepatitis C  William Balistreri

Key points:

  • The recommendations for pediatric hepatitis C infection have been rapidly-changing due to a large number of recent studies/new direct-acting antivirals.  There are many new treatment options (see HCVguidelines.org); currently available treatment regimens noted below
  • All children >3 years of age with HCV should be treated –high cure rates (91-100% SVR) and this leads to long-term improvements in health outcomes
  • Test for Hepatitis B before instituting DAA therapy
  • Universal screening has been recommended for all adults >18 years.  This omits the pediatric age group; however, if all pregnant women are screened, the majority of pediatric HCV infections could be identified

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Online Aspen Webinar (Part 3) -Primary Sclerosing Cholangitis

Below I’ve included a few slides and some notes; my notes may have errors of omission or transcription.

Primary Sclerosing Cholangitis: Beyond Anecdotal Medicine  Jim Squires

Key points:

  • MMP-7 is emerging as better biomarker than alk phos or GGT
  • Patients with PSC-IBD often have PUCAI scores which underestimates severity of IBD activity. Even PSC-IBD patients in “clinical remission” often have disease activity.
  • PSC-IBD phenotype includes pancolitis (often with rectal sparing and backwash ileitis
  • Long-term prognosis is associated with level of GGT values
  • Prognosis: ~70% have event-free survival at 5 years
  • Adult prognosis models are inadequate due to frequent differences between disease in children and disease in adults.  Adults also have more comorbidities: obestiy, smoking, alcohol and medications
  • SCOPE index is a useful prognostic model for children (scores of 3 or less indicate very low risk of disease progression over next 5 years)
  • Actigall is current first line treatment in children based on biochemical improvement (no long term proof of efficacy); vancomycin has only anecdotal evidence of effectiveness

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NY Times: Opening Schools Safely

NY Times: Opening Schools Won’t Be Easy, but Here’s How to Do It Safely

An excerpt:

  • First, schools cannot reopen safely when community transmission is high and climbing. In our view, schools should open only in places that have fewer than 75 confirmed cases per 100,000 people cumulatively over the previous seven days, and that have a test positivity rate below 5 percent
  • Second, schools should avoid high-risk activities. ..
  • Third, focus on the basics where risks are tolerable — that is at the medium level or lower on our chart. ..
  • [Fourth] Schools must adhere to public health measures and reduce density in classrooms and elsewhere on campus.

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Online Aspen Webinar (Part 2) -Abnormal Liver Enzymes in a Tween

What Do Abnormal Liver Enzymes Mean in a Tween William Balistreri

Below I’ve included a few slides and some notes; my notes may have errors of omission or transcription.

Key Points:

  • Provided updated normal reference data for ALT/AST along with patterns of abnormalities
  • Reviewed step-wise workup for teenagers with elevated ALT/AST, particularly fatty liver disease and drug-induced liver disease
  • Increasingly frequent cause of fatty liver disease: psychotropic medications
  • Discussed role/indications of liver biopsy. Liver biopsy is NOT practical option for all children with fatty liver disease and elevated liver enzymes
  • However, ALT values tend to underestimate severity of liver disease

 

 

Online Aspen Webinar (Part 1)

Online Webinar –Annual Aspen Conference  —July 14, 2000

Below I’ve included a few slides and some notes; my notes may have errors of omission or transcription.

Can We Skip Liver Biopsies in Infants with Cholestasis? Jim Squires

Key points:

  • Data suggest that cholestasis in infants needs to be defined as direct bilirubin/conjugated bilirubin >0.3 (if TB <5) or 10% if TB >5.
  • Identifying cholestasis is challenging as cholestasis occurs in ~1 in 2500 whereas jaundice occurs in 15% of all infants
  • Genetic testing (eg. Cholestasis Panel, or exome) needs to be moved up earlier in diagnostic algorithm, after ultrasound completed and after A1AT & biliary atresia considered

Related blog posts:


Not part of webinar:

More School Advice for Organ Transplant Recipients, Plus Another Benefit of the Influenza Vaccine

Link to PDF (from Pediatric Infectious Disease Society:

FAQs Regarding Return to School for Children after Solid Organ Transplant in the United States During the COVID-19 Pandemic

Some excerpts:

Are pediatric SOT recipients at higher risk for getting COVID-19 compared with other children?
Children of any age can get COVID-19, but they seem to have milder disease than adults. Pediatric SOT recipients do not seem to get COVID-19 more often than other children.

If infected with COVID-19, are pediatric SOT recipients at higher risk for developing severe disease or complications?

Based on experience with other viruses, and from reports of COVID-19 in adult SOT patients, there are a few things that may increase the risk of severe COVID-19. These include:
1) Having undergone transplantation in the last 3-6 months
2) Receiving high doses of immunosuppression (such as for treatment of rejection)
3) Having other medical problems such as diabetes, obesity, or certain lung conditions (refer to CDC website under Helpful Resources for more details)
It is not known if the above factors also put children with SOT at risk. In fact, of all the reports among pediatric SOT recipients with COVID-19 published so far, the majority have had mild symptoms and recovered.

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