Neurodevelopment Impairment in the Majority of Extremely Preterm Infants with Short Bowel Syndrome

Link to article (paywall)/abstract: Neurodevelopmental and Growth Outcomes of Extremely Preterm Infants with Short Bowel Syndrome

Key finding from study:

  • Moderate-severe neurodevelopmental impairment was present in 77% of children with extreme prematurity and with short bowel syndrome compared to 44% with extreme prematurity without necrotizing enterocolitis, spontaneous intestinal perforation or short bowel syndrome. 

One of the authors, Ira Adams-Chapman, recently passed away (link to obituary: Ira Adams-Chapman, 1965-2020). She and I were residents together in Cincinnati. She was a terrific person.

Is GLP2 Worth $300K per Year?

E Ramos Boluda et al. JPGN 2020; 71: 734-739. Experience With Teduglutide in Pediatric Short Bowel Syndrome: First Real-life Data

S Hill. JPGN 2020; 71: 697-698 (editorial) Use of GLP-2 May Herald a New Era of Improved Outcome of Short Bowel Syndrome-associated Intestinal Failure

The study and associated editorial highlight the effectiveness of GLP-2 in a prospective cohort of 17 patients with short bowel syndrome. It is noted that Dr. Hill has received funding from the pharmaceutical manufacturer of the product.

Key findings:

  •  A total of 12 of 17 patients achieved parenteral independence: 3 patients after 3 months of treatment, 4 patients at 6 months, and 5 after 12 months.
  • The percentage able to wean off parenteral nutrition was 17%, 44%, and 60% at 3, 6, and 12 months respectively. Only 1 patient did not exhibit improvement
  • Plasma citrulline levels, a marker for enteral autonomy, increased from a baseline average of 20 micromol/l to 37.5, 46.75, and37.9at 3, 6, and 12 months respectively.
  • Adverse reactions included abdominal pain 30%, nauseas 18%, injection-site reactions 22%, and headache 16%.

Both the editorial and the study comment briefly on the cost of the therapy. The editorial also notes the current recommendation for surveillance endoscopy in view of a hypothetical risk of malignancy.

My take: Is GLP2 Worth the Cost? It probably depends on who is paying and long-term safety data. Perhaps, we will develop tools to improve prediction of which patients will achieve enteral autonomy with GLP2 who would otherwise require ongoing parenteral nutrition.

Related blog posts:

Nutrition Pearls -Fiber in Short Bowel and Good Growth with Cystic Fibrosis

One useful resource for NASPGHAN members (NASPGHAN Nutrition Pearls) has been the short monthly nutrition pearl videos (about 10 of them so far). Here are some pointers from the most recent of these.

In October: Fiber for Short Bowel Syndrome –Beneficial for those with a colon in continuity:

Commercial products with limited data supporting use in short bowel syndrome
All of the fiber products are fermented in colon and may be beneficial. Highlighted products are more likely to help with stool consistency (thickening).

In September: Growth in Cystic Fibrosis

Related blog posts for Short Bowel Syndrome:

Related blog posts for Cystic Fibrosis:

Short Gut Diet -CHOA Approach

Recently Kipp Ellsworth, with input from members of the nutritional team, developed our first institutional Short Gut Diet.

Per Kipp, this diet is “designed to facilitate digestion while minimizing abdominal pain and ostomy/stool output in our inpatients with truncated intestinal anatomy.  Previously, clinicians ordered a regular diet for our short gut patients, with parents and nurses providing oversight of the ordering process based on their knowledge of short gut diet precepts.  Obviously this non-standardized approach resulted in significant noncompliance, another onerous daily task for nursing, and a failure of inpatient short gut diet principles reinforcement.  I anticipate the new diet serving as an omnipresent education tool, reinforcing short gut diet precepts for patients and parents during their inpatient stays.”

Related blog posts:

Using Spot Urine Sodiums

A recent study (AKN Pedersen et al. JPEN https://doi.org/10.1002/jpen.1593) shows the utility of obtaining urine spot sodiums in patients with an ileostomy. Thanks to Kipp Ellsworth for sharing this reference.

Full link: A Single Urine Sodium Measurement May Validly Estimate 24‐hour Urine Sodium Excretion in Patients With an Ileostomy

Background: Sodium deficiency in patients with an ileostomy is associated with chronic dehydration and may be difficult to detect. We aimed to investigate if the sodium concentration in a single spot urine sample may be used as a proxy for 24‐hour urine sodium excretion.

Design: In this prospective, observational study, we included 16 adult individuals: 8 stable patients with an ileostomy and 8 healthy volunteers with intact intestines

Key finding:

  • There was a high and statistically significant correlation between 24‐hour natriuresis and urine sodium concentrations in both morning spot samples (n = 8, Spearman’s rho [ρ] = 0.78, P = 0.03) and midday spot samples (n = 8, ρ = 0.82, P = 0.02) in the patients with an ileostomy.

My take: In patients with ileostomy (and also short bowel syndrome), periodic urine sodium values (from morning or mid-day) will help detect subclinical sodium depletion.

Related blog posts:

 

Atlanta Botanical Gardens

Prevalence of Bloodstream Infections in Children with SBS and Fever

Abstract Link: Prevalence of Bloodstream Infections in Children With Short‐Bowel Syndrome With a Central Line Presenting to Emergency Department With Fever

AC Fifi et al JPEN; https://doi.org/10.1002/jpen.1701

This retrospective study with 246 encounters identified the rate of bloodstream infections (BSI) in children with short bowel syndrome (SBS).

Key findings:

  • The adjusted calculated prevalence rate for BSI in children with SBS and fever was 55% (95% CI, 42.3%–65.4%)
  • There were 114 gram‐negative infections (72.6%), 46 gram‐positive infections (29.3%), and 17 fungal infections (10.8%)
  • Each additional 10 units above 20 mg/L CRP increased the odds of BSI by 26%. There was no association between WBC count and the presence of BSI

My take: This study supports the practice of using broad‐spectrum antibiotics in children with SBS and fever.

Related blog posts:

Atlanta Botanical Garden

#NASPGHAN19 Intestinal Failure Session Part 2

Our Spooky Pumpkin

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

Beth Carter  Trophic Growth Factors: A Practical View

Key Points:

  • GLP-2 has been approved as agent for intestinal failure for children (May 2019)
  • Studies thus far have shown good safety but concerns remain (?increased risk of polyposis, increased growth of neoplasm) and as such increased surveillance needed for patients receiving GLP-2
  • Cost in adults ~$295,000 per year
  • Most patients need to continue GLP-2 to maintain effect

Arthur Kasti  Abstract 218  Microbial Metabolites as Markers of Intestinal Dysbiosis in Pediatric Short Bowel Syndrome

This was a terrific presentation. Key points:

  • Microbiome in SBS patients is less diverse
  • Current diagnosis of bacterial overgrowth is difficult and definitive diagnosis is often impractical
  • Several metabolites may be helpful in diagnosis of bacterial overgrowth

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 Intestinal Failure Session Part 1

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.

Bone Health, Especially for IBD and Short Gut

Several colleagues with birthdays this week and next–Happy Birthday!

At our ICN population management meeting (as well as at a recent nutrition colloquium), Dr. Karen Loechner provided a timely update on bone health for our group.  Some of her slides are pictured below and a link to full slides follows.

Some of the points that I found interesting:

  • New hologic scans are much quicker (as little as 15 secs for some images) than typical DXA scans
  • While sodas have been associated with weaker bones, the main mechanism is likely displacement of milk from diet rather than direct effects
  • Adjust DXA results for height age
  • Think about vertebral compression fractures in children with mobility problems and painful symptoms

 

 

Full Link: Sticks and Stones Pediatric Osteoporosis