Category Archives: Pediatric Gastroenterology Liver Disease

#NASPGHAN19 Intestinal Failure Session Part 1

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Here are some notes and a few slides from NASPGHAN’s plenary session.  There could be errors of transcription in my notes.

Benjamin Gold, NASPGHAN president and part of our GI group, GI Care For Kids, welcomed everyone to the meeting.

Link to NASPGHAN_Annual_Meeting_Program 2019

John Kerner  Potential Role of New Fat Emulsions

Key points:

  • Both SMOFlipid and Omegaven help prevent and/or treat parenteral nutrition associated cholestasis.
  • SMOFlipid is much less expensive (see slide below) -50 gm of SMOFlipid ~$5 compared to 10 gm of Omegaven at $35, thus omegaven costs more than 30 times SMOFlipid.
  • Though SMOFlipid is not FDA approved in children, it is being used widely and allows for increased calories compared to lipid minimization with intralipid and could improve neurocognitive outcomes.
  • SMOF dosing (listed below) with goal of 3 g/kg in preterm infants.
  • Resolution of cholestasis does not mean reversal of cirrhosis.  Thus, lipid emulsion intervention at earlier stage may be important.

Bram Raphael  Getting In Line: Towards a Clinical Practice Guideline For CVC Salvage

Key points:

  • Several infections are very difficult to clear, especially yeast, enterococcus, and pseudomonas
  • Salvaging central lines may obviate the need for multi-visceral transplant which carries a 5-year ~50% mortality rate
  • Cefepime provides good gram-negative coverage; consider meropenem in those with septic shock

Disclaimer: This blog, gutsandgrowth, assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. These blog posts are for educational purposes only. Specific dosing of medications (along with potential adverse effects) should be confirmed by prescribing physician.  Because of rapid advances in the medical sciences, the gutsandgrowth blog cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition.

#NASPGHAN19 Postgraduate Course (Part 5)

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Here are some selected slides and notes from this year’s NASPGHAN’s postrgraduate course.  There may be errors in omission or transcription on my part.

Link to the full NASPGHAN PG Syllabus 2019 (Borrowed with permission)

– Intestinal Inflammation Session

192 David T. Rubin, MD, University of Chicago Positioning the new IBD therapies: Merging experience with evidence

Some key points:

  • Ustekinumab escalation can increase response. Optimization in CD patients with loss of response led to recapture of response in 69% of patients
  • Tofacitinib –given black warning, will likely be used in more refractory patients
  • May be able retry a previous therapy (Chicago protocol in slide below)

As an aside, while Dr. Rubin is an excellent speaker, my view is that there are so many terrific pediatric IBD specialists, I would favor having a pediatric IBD specialist give this talk at our postgraduate course.  (Some might argue that adult IBD specialists would have more experience with emerging therapies.)

204 Anne Griffiths, MD, FRCPC, Hospital for Sick Children Immunosuppressive therapy in IBD: Can we de-escalate therapy?

  • High rate of relapse when biologic therapy is stopped.  Use of an immunomodulator may reduce the relapse rate when stopping an anti-TNF agent

215 Stacy Kahn, MD, Boston Children’s Hospital When it is not IBD … rare forms of intestinal inflammation

  • For patients with milder microscopic colitis, antidiarrheal agents can be given.  For more severe disease, budesonide is effective.

223 Edaire Cheng, MD, UT Southwestern Medical Center  Eosinophilic inflammation beyond the esophagus

 

Disclaimer: NASPGHAN/gutsandgrowth assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. The discussion, views, and recommendations as to medical procedures, choice of drugs and drug dosages herein are the sole responsibility of the authors. Because of rapid advances in the medical sciences, the Society cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure. Some of the slides reproduced in this syllabus contain animation in the power point version. This cannot be seen in the printed version.

#NASPGHAN19 Postgraduate Course (Part 2)

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Here are some selected slides and notes from this year’s NASPGHAN’s postrgraduate course. My notes from these lectures may contain errors of omission or transcription.

Link to the full NASPGHAN PG Syllabus 2019 (Borrowed with permission)

9:00 – 10:20 “Potpourri”

46 Alessio Fasano, MD, MassGeneral Hospital for Children  Celiac disease: Beyond diagnosis

  • Reviewed potential non-biopsy option for diagnosis if anti-TG2 >10 x normal. Pediatricians are not following recommendations –>many children placed on gluten-free diet at lower titer antibody-positivity.
  • Recommends checking Hepatitis B antibody because many children with celiac disease do not seroconvert.
  • TTG levels are good for diagnosis but not as helpful for monitoring after diagnosis.
  • Only 10 out of 1000 are true refractory, about 100 out of 1000 are exquisitely sensitive to gluten

56 Meghana Sathe, MD, UT Southwestern Medical Center The role of the gastroenterologist and hepatologist in Cystic Fibrosis (CF) care today

  • Fecal elastase monitoring useful for determining need for PERT.
  • Discussed CF liver involvement.  Multilobular cirrhosis, 7% of individuals, is most important liver disease in CF.
  • Modulator therapy can elevate liver enzymes and may need to hold if ALT >5 ULN or lower elevation if elevated bilirubin (see Stop Rules -Practical Advice on DILI)
  • DIOS -for partial obstruction, polyethylene glycol and/or gastrogastrin enemas could be used.
  • Consider treatment of SBBO as well which is frequent with CF.

67 Sonia Michail, MD, Children’s Hospital Los Angeles Update on C. difficile

The slide I liked the best was showing a change in microbiome after FMT which is not in syllabus.

82 Ed Hoffenberg, MD, Children’s Hospital Colorado  What the pediatric GI provider needs to know about cannabis

Disclaimer: NASPGHAN/gutsandgrowth assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. The discussion, views, and recommendations as to medical procedures, choice of drugs and drug dosages herein are the sole responsibility of the authors. Because of rapid advances in the medical sciences, the Society cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure. Some of the slides reproduced in this syllabus contain animation in the power point version. This cannot be seen in the printed version.

#NASPGHAN19 Liver Symposium (Part 4)

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Although I was unable to attend this year’s liver symposium at NASPGHAN19, I reviewed the lecture notes.  There is some terrific content.  Here are some of the slides (borrowed with permission from NASPGHAN).

Link to complete NASPGHAN Chronic Liver Disease Symposium 2019

SESSION IV – LIVER TRANSPLANT: PRE- AND POST-TRANSPLANT CONSIDERATIONS

Referring your patient for liver transplantation Shikha S. Sundaram, MD MSCI, FAASLD, Children’s Hospital Colorado

Where will we get our organs from in 2020?  Jean Emond MD, Columbia University Medical College (Slides not available in online handout)

What should I do if my liver transplant patient has elevated liver tests?  Udeme Ekong MD, Georgetown University Hospital

What is a “normal” childhood after liver transplantation? Estella Alonso MD, Ann and Robert H Lurie Children’s Hospital

Disclaimer: NASPGHAN/gutsandgrowth assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. The discussion, views, and recommendations as to medical procedures, choice of drugs and drug dosages herein are the sole responsibility of the authors. Because of rapid advances in the medical sciences, the Society cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure. Some of the slides reproduced in this syllabus contain animation in the power point version. This cannot be seen in the printed version.

#NASPGHAN19 Liver Symposium (Part 3)

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Although I was unable to attend this year’s liver symposium at NASPGHAN19, I reviewed the lecture notes.  There is some terrific content.  Here are some of the slides (borrowed with permission from NASPGHAN).

Link to complete NASPGHAN Chronic Liver Disease Symposium 2019

SESSION III – UPDATE ON PORTAL HTN: ASSESSMENT AND MANAGEMENT

What do I do now? The management of portal hypertensive complications: Varices, ascites, and encephalopathy Rene Romero, MD, Children’s Hospital of Atlanta

When there is good function, but the flow is all wrong: Approach to non-cirrhotic portal hypertension Evelyn Hsu, MD, Seattle Children’s Hospital

The role of the interventional radiologist in the treatment of portal HTN: How can I help you?  Jared R. Green, MD, Ann and Robert H. Lurie Children’s Hospital (SLIDES NOT AVAILABLE)

When to consider surgery in the treatment of portal HTN?  Riccardo Superina, MD, FRCS(C), FACS, Northwestern University  (SLIDES NOT AVAILABLE)

#NASPGHAN19 Liver Symposium (Part 2)

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Although I was unable to attend this year’s liver symposium at NASPGHAN19, I reviewed the lecture notes.  There is some terrific content.  Here are some of the slides (borrowed with permission from NASPGHAN).

Link to complete NASPGHAN Chronic Liver Disease Symposium 2019

SESSION II – FRONTIERS IN LIVER THERAPEUTICS

Keynote Speaker: Outcomes for the future: How do we improve on the status quo? Ronald J. Sokol, MD, FAASLD, Children’s Hospital Colorado  (SLIDES NOT AVAILABLE in onliine handout)

Recognition and stabilization of the pediatric patient with acute liver failure Robert Squires MD Children’s Hospital of Pittsburgh at UPMC

Should I offer treatment for my patients with Hepatitis B or Hepatitis C? Regino P. Gonzalez-Peralta MD, AdventHealth for Children

Are there any medical therapies for NASH?   Marialena Mouzaki, MD, Cincinnati Children’s Hospital Medical Center

This lecture describes a lot of the emerging pharmacologic treatments; none of these are currently recommended.

#NASPGHAN19 Liver Symposium Notes (Part 1)

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Although I was unable to attend this year’s liver symposium at NASPGHAN19, I reviewed the lecture notes.  There is some terrific content.  Here are some of the slides (borrowed with permission from NASPGHAN).

Link to complete NASPGHAN Chronic Liver Disease Symposium 2019

Session I

How do I best evaluate a cholestatic infant? Sanjiv Harpavat MD Texas Children’s Hospital 

Related blog post: What is the evidence that biliary atresia starts in utero?

As for this algorithm, in my opinion, the 1st step needs to be to exclude emergencies associated with infantile cholestasis: coagulopathy, hypoglycemia, sepsis, and checking urine for reducing substances (cow’s milk formula can worsen liver disease if galactosemia is present). Subsequently, evaluation needs to proceed quickly to determine the etiology.

How do I interpret genetic results?  Saul J. Karpen MD, PhD, Emory University School of Medicine/Children’s Healthcare of Atlanta

What do abnormal liver enzyme levels mean in a tween?  William F. Balistreri MD, Cincinnati Children’s Hospital Medical Center

What do I do with this abnormal radiology finding? Jean Molleston MD, Riley Children’s Hospital

I have not selected slides from Dr. Molleston’s handout –the images are terrific.  For most of the problems that are presented, the lecture notes do not provide specific recommendations for management.

Disclaimer: NASPGHAN/gutsandgrowth assumes no responsibility for any use or operation of any method, product, instruction, concept or idea contained in the material herein or for any injury or damage to persons or property (whether products liability, negligence or otherwise) resulting from such use or operation. The discussion, views, and recommendations as to medical procedures, choice of drugs and drug dosages herein are the sole responsibility of the authors. Because of rapid advances in the medical sciences, the Society cautions that independent verification should be made of diagnosis and drug dosages. The reader is solely responsible for the conduct of any suggested test or procedure. Some of the slides reproduced in this syllabus contain animation in the power point version. This cannot be seen in the printed version.

 

 

30 -Year Outcomes with Biliary Atresia

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M Fanna et al. JPGN 2019; 69: 416-24. In this retrospective 30-year study (1986-2015) from France, patients were examined in 4 time cohorts: 1986-96, 1997-2002, 2003-9, and 2010-5.

  • Age at Kasai operation remained stable throughout the study period -median 59 days.
  • Early Kasai was associated with a reduced need for liver transplantation. 25-year survival with native liver was 38%, 27%, 22%, and 19% for patients operated in first, second, third months or later respectively.
  • Clearance of jaundice (total bilirubin ≤20 micromol/L) after Kasai did not change appreciably in the time cohorts and was 38.8%.
  • 753 (of 1428 in cohort) patients underwent liver transplantation.
  • Overall survival of entire cohort was 87% (including all levels of followup).
  • Survival after LT was 79% at 28 years.
  • Five-year patient survival after LT was 76%, 91%, 88%, and 92% in the cohorts, indicating better survival more recently.
  • 22% of patients reached age 30 years without transplantation.

The authors note that better outcomes were noted in a long-term study from Japan where there are lower rates of LT needed for biliary atresia. IN Japan 20-year survival with native liver and overall patient survival was 48% and 89%, compared to 26% and 76% in France.

My take: This study indicates that the majority of patients with BA will require liver transplantation and that earlier Kasai operation is associated with a better chance of survival with native liver.  It is likely that data in the U.S. would be more similar to France than Japan based on prior publications.

Related blog posts:

Ecola State Park, OR

Liver Briefs -October 2019

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Briefly noted:

M Mouzaki et al. JPGN 2019; 69: 339-43. In a cohort of 228 patients with 17 (8%) who were receiving psychotropic medications, the use of psychotropic medications was associated with increased nonalcoholic fatty liver disease (NAFLD) severity.  These patients were more likely to be receiving metformin (53% vs 18%) and antihypertensive medications (29% vs 8%).

S Honigbaum et al. JPGN 2019; 69: 344-50. Among 20 infants with biliary atresia, tissue had abundantly expressed lysly oxidase-like 2 (LOXL2) compared to controls.  LOXL2 is an extracellular matrix enzyme that catalyzed cross-linking of collagen and elastin; LOXL2 likely contributes to fibrosis.

What Is The Evidence That Biliary Atresia Starts in Utero?

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A good read: KR Mysore, BL Shneider, S Harpavat. JPGN 2019; 69: 396-403.

This review dissects the evidence that biliary atresia (BA) most often begins in utero.

Key points:

  • Infants with BA have elevated conjugated/direct bilirbuin at birth.
  • Infants with BA have biliary abnormalities on fetal ultrasound (eg. gallbladder abnormalities, biliary cysts).
  • Infants with BA have abnormal gamma-glutamyl transferase levels in their amniotic fluid with low levels noted at gestational age 18-19 weeks.  This finding is not specific for BA as other conditions that affect biliary tree (eg. cystic fibrosis, trisomy 21) can have low levels as well.
  • BA is more common in premature infants.
  • Early recognition is important. In U.S (from 1976-89), transplant-free survival rates were 63% for Kasai when done in 1st 30 days, 44% for 30-60 days, 40% for 60-90 days, and 29% if >90 days.
  • A diagnostic approach is given in Figure 2 but is already out of date due to the availability of MMP-7 testing (article received by JPGN in January 2019).

This review also lists numerous current investigative therapies which include probiotic, steroids, desflurane/sevoflurane (anesthetics), pentoifyline, IVIG, vancomycin, meloxicam, GCSF, Bone marrow stem cells, N-acetylcysteine, and obeticholic acid.

My take: This article shows that the clock on liver injury begins in utero in most cases of BA and this will have implications on pathogenesis and management.

Related blog posts:

Cannon Beach, OR from Ecola State Park