The authors have devised a SQUIRT score for short bowel syndrome. The higher score indicates better enteral tolerance with a highest score of 13 and a lowest score is 3. It is a composite score based on composition, frequency and volume/blowouts. The authors define a blow-out to be a stool loose enough and large enough for the child to require a change of clothes.
How to Score:
Consistency (choose best stool of the day) Playdough, Formed 5 Peanut Butter, Pasty 4 Pudding, Seedy 3 Pea Soup, Loose 2 Water 1
Occurrences (over 24 hours) 1 to 3 4 4 to 6 3 7 to 9 2 Greater Than 10 1 Ostomy Present 0
Volume With Ostomy Without Ostomy (mL/kg) (# of blowouts)
< 10 mL/kg 8 0 =4
10 -14 mL/kg 7 15-19 mL/kg 6 1 =3
20-24 mL/kg 5 2 =2 25-29 mL/kg 4
30-34 mL/kg 3
35 mL/kg 2
>35 mL/kg 1 >3 =1
The authors state that this scoring system has been used in their institution. “This tool can provide objective information to guide clinical decision making. Even patients with ostomies, for whom we can measure stool volume, benefit from SQUIRT scores because including the variable of stool consistency provides a more nuanced assessment than relying on volume.”
My take:This type of scoring system would be useful, primarily in patients in which the volume of stool is difficult to measure. It would benefit from trials to validate its utility.
Methods: Retrospective study. Among 180 patients, 35 required long-term parenteral nutrition (SBS-IF group) and 145 acquired full oral feeding within 6 months (oral feeding group) over mean f/u of 7.9 years.
Both bowel matting (OR, 14.2, P = .039) and secondarily diagnosed atresia or stenosis (OR, 17.78, P = .001) were independent postnatal predictors of SBS-IF.
An initial residual small bowel length of more than 50 cm was the best predictive cut-off for nutritional autonomy, with a sensitivity of 67% and a specificity of 100%
My take: This study identifies bowel matting and atresia/stenosis as additional factors in predicting nutritional autonomy.
Lately, there have been a lot of articles on neurocognitive function. The latest (A Gold et al. JPGN 2020; 70: 225-31) describes the myriad of problems facing children with intestinal failure (IF). The authors literally used 12 different measures of neurocognitive and academic measures –though not all 28 subjects had each of these measures (Table 2).
The authors specifically excluded 5 children with severe neurodevelopmental problems that precluded participation in standardized assessment and 10 children who were transplant recipients.
Also, when judging the results, it is important to keep in mind that their cohort had a good maternal education level; 68% were college graduates.
13 of 28 (46%) received a diagnosis of cognitive/learning DSM diagnosis
29% met diagnostic criteria for a learning disability, 7% for ADHD, and 11% for intellectual disability; comparison Canadian prevalence rates are 4%, 5%, and 1% respectively
The number of first-year septic episodes was associated with poorer outcomes; ≥2 or more episodes increased the likelihood.
Sustained cholestasis was associated with poor outcomes
The average level of intellectual functioning in their sample of 28 children was within 1 standard deviation of the population mean
There are a lot of risk factors for neurodevelopment impairment in these children with IF: prematurity, nutritional status/specific nutrient deficiencies, cholestasis, need for anesthesia/surgeries
My take: More than half of children with IF had neurodevelopemental impairment. In this cohort, recurrent sepsis in the first year of life and sustained cholestasis were associated risk factors.
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
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.
A recent study (C Belza et al. JPEN 2019; https://doi.org/10.1002/jpen.1692) showed that SMOFlipid reduced the frequency of cholestasis in intestinal failure patients. Thanks to Kipp Ellsworth for reference.
This was a retrospective cohort study of infants with IF with a minimum follow‐up of 12 months in 2008–2016. Patients were stratified into 2 groups: group 1 received SMOFlipid; group 2 was a historical cohort who received Intralipid. The primary outcome was liver function evaluated using conjugated bilirubin (CB) levels…
Thirty‐seven patients were evaluated (17 = SMOFlipid, 20 = Intralipid). SMOFlipid patients were less likely to reach CB of 34 (24% vs 55%, P = 0.05), 50 µmol/L (11.8% vs 45%; P = 0.028), and did not require Omegaven (0% vs 30%; P = 0.014). CB level at 3 months after initiation of parenteral nutrition (PN) was lower in patients receiving SMOFlipid (0 vs 36 µmol/L; P = 0.01). Weight z‐scores were improved for patients receiving SMOFlipid at 3 months (−0.932 vs −2.092; P = 0.028) and 6 months (−0.633 vs −1.614; P = 0.018).
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.
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.
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.
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.
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.
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.
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.
“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.
Acid blockers: used for hyperacidity after massive resection
Bile acid sequestrants (eg. cholestyramine)
NOT evaluated in intestinal failure: octreotide, racecadotril, crofelemer
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
STEP procedure or possibly lengthening procedure (Bianchi). STEP procedure is less technically difficult.