NASPGHAN Postgraduate Course 2017 (Part 3): Biliary Atresia, NAFLD, SMOFlipid, Pancreatic Pain

This blog entry has abbreviated/summarized these presentations. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

Here is a link to postgraduate course syllabus: NASPGHAN PG Syllabus – 2017

Biliary Atresia: Update on diagnostic and prognostic biomarkers and therapeutic interventions

Cara Mack    Children’s Hospital of Colorado

Key points:

  • 84% of biliary atresia is isolated; 16% are syndromic with other defects
  • Direct bilirubin is (mildly) elevated at birth in patients with biliary atresia
  • Total bilirubin 3 months after Kasai predicts outcome. If <2 mg/dL, then unlikely to need a transplant in the first 2 years of life.
  • Reviewed biomarkers including Th1, Autotaxin, IL-8

Therapeutic interventions:

  • Nutritional support. Better nutrition improves outcomes after liver transplantation.
  • Fat soluble vitamin supplementation
  • Cholangitis prevention. Some studies have shown that prophylactic antibiotics may reduce incidence of cholangitis.
  • No therapeutic interventions that delay progression of this disease



CHILDREN Cohort Mgt of Vitamin Supplementation

Steroids are not helpful after Kasai procedure

Diagnosis and Management of Pediatric NAFLD 2017

Stavra Xanthokos   Cincinnati Children’s Hospital Medical Center

Key points:

  • NAFLD is #2 cause of liver transplantation in adults and on its way to becoming #1
  • ALT is still the best screening tool; NASPGHAN guidelines recommends screening overweight/obese children 9-11 years of age
  • Ultrasound has poor sensitivity and specificity for NAFLD; it is helpful for detecting gallbladder disease
  • Bariatric surgery has been effective for NAFLD


SMOFlipid and the Pediatric Patient

Peter Wales  Hospital for Sick Children (Toronto)

Slides are not available in syllabus

Key points:

  • Improving outcomes noted in the intestinal failure population
  • Dr. Wales reviewed proposed improvements with Omega-3 lipids -less cholestasis, less hepatitis, and less fibrosis
  • Compared improvements with lipid minimization (1 g/kg/day) compared to newer agents: omegaven and SMOFlipid. Additional studies are needed due to limitations of previous studies
  • Discussed SMOFlipid vs. Intralipid trial at 5 centers in Canada. N=24.
  • At SickKids: SMOFlipid for all preterms at admission & for term infants after 2 weeks of PN. Dosing 2-2.5 g/kg & now accounts for 85% of lipid usage at institution
  • None of the lipid products were designed for preterm infants. Intralipid has a pediatric indication and other products are used off label
  • Lipid restriction probably affects brain size/development; thus, a lipid agent that allows for higher doses likely will be beneficial for developmental outcomes.  The retina can be used as a biomarker of the brain affects of lipids.

Painful Chronic Pancreatitis: Management/therapeutic interventions

Vikesh Singh  Johns Hopkins University School of Medicine

Slides are not available in syllabus



N2U Part 5 -Biliary Atresia and Kwashiokor

2015 N2U Syllabus & Presentations

Growth Failure, Macro- and Micronutrients, and Biliary Atresia  James Heubi   (Syllabus pg 62 –68)

Case in Point:  AK is a 5-month-old Hispanic male with biliary atresia s/p hepatoportoenterostomy at age 6 weeks who was seen in clinic with a 2 month history of poor nutritional intake (full details on syllabus pg 62).

Initial Focus/Management:

  • Increasing caloric density 24 cal with MCT-containing formula, then up to 30 cal (avoid monotherapy with portagen due to EFA deficiency); if not effective, then nocturnal tube feedings (NG/NJ) are likely to be needed. Parenteral supplementation –not being used at Cincinnati in pretransplant patients.
  • Ascites, when present, limits fluid volume intakes. Aldactone (often at about 3 mg/kg/day) can be helpful; check to make sure urine sodium indicates some natriuresis.
  • Fat soluble vitamin supplementation/micronutrient supplementation (when needed). Check levels at about 3 months of age. If Vitamin D (25-OH) is greater than 20 (in pediatrics), this is probably reasonable in this population. With Alagille/hyperlipidemic patients, need to correct vitamin E for cholesterol (or total lipid) (Sokol RJ, Heubi JE, et al. NEJM 1984; 310: 1209-12).
  • For biliary atresia, direct bilirubin >2 indicates need for fat soluble monitoring; in other cholestatic conditions (eg. Alagille, PFIC), don’t rely on direct bilirubin as fat soluble deficiency can develop with lower direct bilirubins.
  • Vitamin D supplementation: 1000 units/kg/day Drisdol D3 (expensive). D3 preferred but D2 usually OK. Monitor levels and increase dosing if needed. Check monthly until adequate level. Alternatives: Bio-D-Mulsion Forte (D3), Nature’s Blend Ultra Strength (D3).
  • Vitamin E supplementation: Liqui-E (w TPGS) or Nutr-E-sol 15-25 IU/kg/day. Alternative: Aqua-E
  • Vitamin A supplementation/monitoring: AquaADEKs is reasonable supplement. Harder to monitor vitamin A levels.
  • If failed Kasai, likely headed to transplantation fairly quickly.

Kwashiokor –Rob Shulman (Syllabus pgs 21-33)

Case in point: 15 mo –Fed a diet of coconut and rice milks managed by pediatrician and chiropractor.  (This can occur with BRAT diets as well.)

Key points:

  • Terminology: from language spoken in a region of Ghana. Term developed to describe the sickness a baby gets when the new baby comes. This is a result of child who gets displaced from breastfeeding as the result of a sibling being born. Willaims CD. Lancet 1935; 226: 1151-52. Original description
  • Etiology: protein deficiency, protein quality, infection (‘pushes them over the edge’)
  • Microbiome in Kwashiokor References: Tilg et al. Nature Rev. 2013;10:261-262.  Smith et al. Science. 2013;339:548-554. Initial study showed discrepant microbiome in identical twins with and without Kwashiokor. Followup study by placing stool (from Kwashiokor and from healthy children) in mice. Stool from twin with Kwashiokor resulted in mice malnutrition (Garrett W. NEJM 2013; 368: 1746-47). (Related blogs:Gut microbiomes of Malawian twin pairs discordant for … and Microbiome and the risk of Kwashiokor | gutsandgrowth )

Feeding plan/ Prevention of Refeeding Syndrome:

  • Check labs –including protime, zinc, phosphorus, potassium, magnesium
  • Oral feedings with standard formulas/diet (usually). Limit feeding volumes initially for 1st week Kwashiokor (page 37 in syllabus) –about ½ full caloric intake (consider ½ strength formula).
  • ‘Advancing diet slowly is not needed with other forms of malnutrition.’
  • Give multivitamin. In 3rd world, it is recommended to add additional vitamin A (200,000 units once).
  • Hold off on iron (even in multivitamin) until improved for a few weeks.
  • Albumin infusions are not recommended àassociated with worse outcomes
  • Refeeding syndrome is an iatrogenic disease! This is associated with Kwashiokor and not with other malnutrition diseases.
  • Add Kphos to feedings (eg Neutraphos, NeutraphosK). Usually drop in phosphorus drop most likely in first 48 hours –monitor carefully in first few days and again during increments in feeding.
  • In 3rd World countries, addition of antibiotics (amoxicillin or cefdinir for 7 d) to therapeutic regimens for uncomplicated severe acute malnutrition associated with a significant improvement in recovery and mortality rates. In U.S. this translates to low threshold for using antibiotics but not required in every case.

Disclaimer: This blog entry has abbreviated/summarized this presentation. Though not intentional, some important material is likely to have been omitted; in addition, transcription errors are possible as well.

These blog posts are for educational purposes only. Specific dosing of medications/diets (along with potential adverse effects) should be confirmed by prescribing physician/nutritionist.  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.

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