#NASPGHAN18 Highlights (Part 2)

I did not make it to this year’s meeting but did get a chance to catch up on a lot information via the PG 2018 Syllabus and based on information posted online.

Here are a couple of highlights for me:

My favorite slide from postgraduate course -Dr. Robert Kramer

Slides regarding the topic of Treat-toTarget Dr. Eric Benchimol:

Slides regarding GI symptoms and autism from Dr. Kara Margolis:

Slide regarding the frequency of bariatric surgery: Dr. Rohit Kohli:

Slides regarding intestinal failure population from Dr. Conrad Cole:

From Dr. Miranda van Tilburg regarding psychological therapies for functional GI disorders:

Disclaimer: 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.

Don’t Forget the Kidneys in Children with Intestinal Failure

Increasingly, kidney problems are recognized in children with intestinal failure/short bowel syndrome who receive long-term parenteral nutrition.  A recent study (H Billing et al JPGN 2018; 66: 751-54) highlights the experience with this issue at a pediatric intestinal rehabilitation center in Germany.

Key findings:

  • Among 50 patients with a median age of 4.2 years, 76% had proteinuria
  • 30% had chronic kidney disease –indicated by reduced creatinine clearance of <90 min (1.73 squared)/min
  • Hypercalciuria was identified in 30 patients (60%)
  • Nephrocalcinosis was identified in 9 patients (18%)

The authors note that end-stage renal failure has not been reported in association with intestinal failure, though proteinuria is associated as a risk factor.

My take: This observational study shows a high frequency of kidney issues in children with intestinal failure. With improvements in survival, chronic kidney disease could become a more significant clinical issue.


Tweet below indicates need for careful nutrition input when children are placed on unusual diets, including the ketogenic diet.

#NASPGHAN17 Presentation: Reducing Hospitalization in Intestinal Failure Patients

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.

Risk Factors for Hospitalization Among Pediatric Intestinal Failure Patients

Tatyana Hofmekler, Janet Figueroa, Hilina Kassa, Rene Romero, Andi Shane.

Dr Hofmekler is now part of GI Care for Kids (my group) and provided a terrific presentation.

NASPGHAN Annual Mtg 2017

Key points:

  • In this study, there were no social or demographic factors which were identified which were associated with increased hospitalization
  • Having a colon and an ileocecal valve lowered the risk of hospitalization
  • The use of SBBO treatment was associated with increased hospitalization though this may have been a marker of more severe disease
  • Vascular catheter infections were reduced compared to study at same institution previously but remained an important risk factor for hospitalization

My take: this study illustrates the challenges in reducing hospitalization.  While the authors did not identify social/demographic factors, my experience is that there are some families who are much more capable than others in taking care of children with complex problems.  If all children had the best parents, that would truly allow the hospitalization rate to be reduced much lower.


#NASPGHAN17 Presentations at Annual Meeting: GGT in PSC, Nutrition for Intestinal Failure

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.

Improvement in GGT Predicts Event-free Survival in Primary Sclerosing Cholangitis Regardless of Ursodeoxycholic Acic Treatment. 

Mark Deneau et al. (Grand Watkins Prize).

Key points:

  • PSC is difficult to study due to its rarity and due to its slow progression; thus surrogate biomarkers are needed.
  • Alkaline phosphatase is not a good biomarker in children
  • GGT level at one year after diagnosis was predictive of prognosis
  • Ursodeoxycholic acid does not appear to be effective

Optimizing Nutrition in Intestinal Failure

Justine Turner, University of Alberta

Key points:

  • Human milk is an ideal “formula” for infants, including those with intestinal failure
  • Oral feedings are important
  • Combination of bolus feeds and continuous feeds is reasonable
  • SMOFlipid allows higher lipid dose administration without hepatoxicity; this may improve cognitive outcomes
  • Amino acid based formulas have higher osmolality which can contribute to diarrhea

Patients with >50% of small bowel and >50% of colon were most likely to achieve enteral autonomy (GIFT registry)



Intestinal Failure -Concise Review

A recent review (CP Duggan, T Jaksic. NEJM 2017; 377: 666-75) concisely reviews recent advances in pediatric intestinal failure.  Most of the review has been covered elsewhere in this blog.  A couple of key points:

Outcomes of intestinal failure:

  • The authors note that a 2012 study identified a 25% mortality rate of infants enrolled between 2000-2004.  “More recent advances have resulted in substantially improved survival rates (>90%).”

Epidemiology of intestinal failure:

  • Using a definition of needing parenteral nutrition for more than 42 days after bowel resection or a residual small-bowel length of less than 25% of normal (for gestational age), intestinal failure was identified in 24.5 cases per 100,000 live births
  • Among infants with birth weight <1500 g, the incidence is 7 per 1000 live births.
  • Frequent causes: necrotizing enterocolitis, gastroschisis

Adaptation of Intestine:

  • Improved chances of attaining enteral autonomy if longer residual small bowel, younger age at time of intestinal resection, preservation of ileocecal valve, absence of severe liver disease, diagnosis of necrotizing enterocolitis, and normal motility.

Parenteral nutrition:

  • Lower rates of liver disease noted with routine restriction of soy-based fat emulsions to 1 g per kilogram
  • Fish oil preparations (with n-3 fatty acids): switching to fish oil preparation “reduces biochemical measures of cholestasis.”
  • Newer preparations of fat emulsions: Smoflipid, Clinolipid are FDA-approved for adults.  Smoflipid, in small studies, is associated with lower conjugated bilirubin compared with soy-based lipids.

Enteral nutrition:

  • “Prompt initiation of enteral feeding after bowel resection has been reported to improve the rate of enteral autonomy….little justification for prolonged ‘gut rest'”
  • The authors note that human milk is often chosen for enteral nutrition and when unavailable, amino acid based formulas are typically chosen due to “more favorable outcomes than protein hydrolysates.”
  • Chronic diarrhea is improved with drip feedings, though bolus feeds may have trophic effects.  “In our experience, a combined approach (e.g. continuous feeding at night and bolus feeding during the day) is feasible.”
  • Oral motor stimulation is important.  Thus, try to give oral human milk feeds when feasible.

Medical Therapies:

  • Acid blockers: used for hyperacidity after massive resection
  • Loperamide
  • Bile acid sequestrants (eg. cholestyramine)
  • NOT evaluated in intestinal failure: octreotide, racecadotril, crofelemer
  • Motility agents
  • Antibiotics for bacterial overgrowth.  “Cyclical use (1 week per month) of broad-spectrum antibiotics…is the mainstay of therapy…at many centers.”
  • Probiotics: “No evidence of benefit in small studies; risk of sepsis”
  • Pancreatic enzymes: rarely used. Indicated if pancreatic atrophy or exocrine insufficiency
  • Growth factors: Teduglutide -licensed for adults, studies in children are ongoing

Surgical Therapies:

  • Central lines
  • Gastrostomy Tubes
  • STEP procedure or possibly lengthening procedure (Bianchi).  STEP procedure is less technically difficult.

Previous related blog entries:

Bone Health and Intestinal Failure

Link: Bone Health of Children with Intestinal Failure (Thanks to Kipp Ellsworth for this reference) E Neelis et al. DOI: http://dx.doi.org/10.1016/j.clnu.2017.02.014

From Abstract:


A retrospective study was performed including all children with IF between 2000 and 2015 who underwent a DXA measurement and/or a hand radiograph. Z-scores of BMD total body (BMD TB) and lumbar spine (BMD LS), bone mineral apparent density (BMAD) and bone health index (BHI) were collected. A low BMD and low BHI were defined as a Z-score ≤ -2. DXA and DXR results were compared for cases in which a DXA and hand radiograph were performed within a 6 months’ interval.


Forty-six children were included. Overall, 24.3% of the children had a low BMD at the first DXA at a median age of 6 years; correction for growth failure (n=6)) reduced this to 16.2%. Fifty percent had a low BHI at the first hand radiograph. Median DXA and BHI Z-scores were significantly lower than reference scores. Age, duration of PN and surgical IF were related to lower Z-scores at the first DXA. Paired DXA and DXR results (n=18) were compared, resulting in a Cohen’s kappa of 0.746 (‘substantial’) for BMD TB. Spearman’s correlation coefficient for BHI and BMD TB Z-scores was 0.856 (p<0.001). Hand radiography had a sensitivity of 90% and specificity of 86% (BMD TB).


Up to 50% of the children had a low BMD. Children with IF have a significantly poorer bone health than the reference population, also after weaning off PN. Bone health assessment by DXA and DXR showed good agreement, especially for Z-scores ≤ -2. DXR assessment using BoneXpert software seems to be feasible for monitoring of bone health in children with IF.


PICC versus Broviac for TPN in Intestinal Failure

LinkA comparison of Broviac® and peripherally inserted central catheters in children with intestinal failure


Central venous catheters (CVCs) are a source of morbidity for children with intestinal failure (IF). Complications include infection, breakage, occlusion, and venous thrombosis. Broviacs® have traditionally been preferred, but peripherally inserted central catheters (PICCs) are gaining popularity. This study compares complications between Broviacs® and PICCs in children with IF.


After IRB approval, children with IF receiving parenteral nutrition (2012–2016) were reviewed. Complications were compared between Broviacs® and PICCs using the generalized estimation equation population-averaged Poisson regression model. P values <0.05 were considered significant.


36 children (0.1–16 years) with IF were reviewed, accounting for 27,331 catheter days, 108 Broviacs® (3F–9F), and 54 PICCs (2-11F). Broviacs® had a significantly higher infection rate (4.2 vs. 2.6/1000 catheter days, p = 0.011), but PICCs were more likely to break (1.56 vs. 0.26/1000 catheter days, p = 0.002). When comparing same size catheters (3F), there were no significant differences in infection, breakage, or occlusion. Twelve children (33%) had central venous thrombosis, all after Broviac® placement. Three children (8%) had basilic vein thrombosis after PICC placement.


Although Broviacs® and PICCs had similar complication rates, there were fewer central venous thromboses associated with PICCs. This should be considered when choosing catheters for children with IF.


My take: Despite some possible advantages of PICC in this study, a prospective randomized study is needed if one is to make a compelling argument regarding better outcomes.  Given the retrospective nature of this study, it could be that the sicker patients may have been more likely to receive a Broviac® and this could have skewed the results.