Postoperative Enteral Nutrition Guidelines Reduce the Risk of Intestinal Failure-Associated Liver Disease in Surgical Infants.

OBJECTIVE: To assess the effectiveness of postoperative feeding guidelines in reducing the incidence and severity of intestinal failure-associated liver disease (IFALD) among infants. STUDY DESIGN: Two cohorts of infants <6 months old undergoing intestinal surgery were compared: preguideline (retrospective data from 2007 to 2013; n = 83) and postguideline (prospective data from 2013 to 2016; n = 81). The guidelines included greater initial enteral nutrition volumes of 20 mL/kg/d and daily feeding advancement if tolerated. The primary outcomes were incidence of IFALD (peak direct bilirubin [DB] >2 mg/dL) and severity (DB >5 mg/dL for moderate-severe). Multiple logistic regression was used to determine the odds of developing IFALD. Other outcomes were time to reach 50% and 100% goal calories from enteral nutrition and the incidence of necrotizing enterocolitis after feeding. RESULTS: The incidence of IFALD decreased from 71% to 51% (P = .031), and median peak DB decreased from 5.7 to 2.4 mg/dL (P = .001). After adjusting for diagnosis and prematurity, the odds of developing IFALD of any severity were reduced by 60% (OR 0.40, 95% CI 0.20-0.85), and the odds of developing moderate-to-severe IFALD were reduced by 72% (OR 0.28, 95% CI 0.13-0.58) with guideline use. Time to reach 50% enteral nutrition decreased from a median of 10 to 6 days (P = .020) and time to reach 100% enteral nutrition decreased from 35 to 21 days (P = .035) with guideline use. The incidence of necrotizing enterocolitis after initiating enteral nutrition did not change (5% vs 9%, P = .346). CONCLUSIONS: Implementation of feeding guidelines reduced time to reach feeding goals, significantly reducing IFALD incidence and severity.

A decade of the modified Atkins diet (2003­2013): Results, insights, and future directions.

The modified Atkins diet has been used since 2003 for the treatment of children and adults with refractory epilepsy.This "alternative" ketogenic diet is started in clinic, without fasting, hospitalization, and restriction of protein,calories, or fluid intake. Now after 10 years of continued use, approximately 400 patients have been reported in over 30 studies of the modified Atkins diet as treatment for intractable seizures, with results demonstrating similar efficacy to the ketogenic diet and improved tolerability. The modified Atkins diet is being increasingly used in the adult population. Clinical trials have provided insight into the mechanisms of action of dietary therapies overall. This review will discuss the past decade of experience with the modified Atkins diet as well as predictions for its role in the treatment of epilepsy a decade from now.

A Proof-of-Principle, Case-Control Study to Compensate for Potential Carbohydrates in Liquid Antiseizure Drugs in Children on the Ketogenic Diet.

INTRODUCTION: Since its creation, patients on ketogenic diet are told to avoid liquid medications due to theoretical concerns of "hidden" carbohydrates. However, switching from liquid to tablet formulations can be problematic, especially for infants and young children. We theorized that increasing the daily ketogenic ratio might compensate for liquid antiseizure drug carbohydrates. METHODS: Two tables were created (for 3:1 and 4:1 ketogenic ratios), with variables including daily volume of antiseizure drugs and calories. Cases were those who had their ratio increased and liquid medications continued. Children already on tablet formulations, emergency situations, were primarily those included as controls. RESULTS: From May 2016 through August 2018, 59 children (33 cases and 26 controls) ages 0.3-14 years were started on the classic ketogenic diet. Compensated antiseizure drugs most commonly included levetiracetam, clobazam, and valproate (mean volume 16 mL/d (range: 3-62 mL/d)). Adjusted ratios for younger children and infants on a 3:1 diet ranged from 3.1 to 3.5:1 and older children on a 4:1 diet from 4.2 to 4.7:1. There was no difference between cases and controls in achieving large ketosis (76% vs 77%), weight gain (1.4 vs 1.2 kg), 1 month >50% seizure reduction (52% vs 50%), or >90% seizure reduction (30% vs 35%). Four (12%) cases had zero or small urinary ketosis, which improved when antiseizure drugs were switched to tablets or discontinued. CONCLUSIONS: This proof-of-principle study demonstrates feasibility of compensating for carbohydrates in liquid medications by increases in the daily ketogenic ratio. This ratio adjustment protocol may help ease an already complex adjustment to dietary therapy.

Evaluation of the Utility of a Dietary Therapy Second Opinion Clinic.

Fine-tuning ketogenic diets to achieve better seizure control may influence families to seek second opinions. Since 2009, Johns Hopkins Hospital has provided second opinions for children followed at other ketogenic diet centers. We retrospectively reviewed 65 consecutive children seen in this clinic; parents were also sent a 2-page survey. The mean age was 6.6 years and dietary therapy had been used a median 9 months. Seizure reduction >50% was achieved in 65%, including 35% with >90% reduction. Parent questions included how to improve seizure control (65%), ideal diet duration (18%), and confirmation of the plan (11%). The most common recommendations were anticonvulsant reduction (43%), adding oral citrates/calcium/vitamins (38%), and carnitine supplementation (31%). Diet discontinuation was more frequently suggested in those children with <50% seizure reduction (60% vs 20%, P = .001). Recommendations were successful in 78%, and the visit was reported as useful by 88%.

Malnutrition increases the risk of 30-day complications after surgery in pediatric patients with Crohn disease.

BACKGROUND: Pediatric patients with Crohn disease (CD) are frequently malnourished, yet how this affects surgical outcomes has not been evaluated. This study aims to determine the effects of malnourishment in children with CD on 30-day outcomes after surgery. STUDY DESIGN: The ACS NSQIP-Pediatric database from 2012 to 2015 was used to select children aged 5-18 with CD who underwent bowel surgery. BMI-for-age Z-scores were calculated based on CDC growth charts and 2015 guidelines of pediatric malnutrition were applied to categorize severity of malnutrition into none, mild, moderate, or severe. Malnutrition's effects on 30-day complications. Propensity weighted multivariable regression was used to determine the effect of malnutrition on complications were evaluated. RESULTS: 516 patients were included: 349 (67.6%) without malnutrition, 97 (18.8%) with mild, 49 (9.5%) with moderate, and 21 (4.1%) with severe malnutrition. There were no differences in demographics, ASA class, or elective/urgent case type. Overall complication rate was 13.6% with malnutrition correlating to higher rates: none 9.7%, mild 18.6%, moderate 20.4%, and severe 28.6% (p < 0.01). In propensity-matched, multivariable analysis, malnutrition corresponded with increased odds of complications in mild and severely malnourished patients (mild OR = 2.1 [p = 0.04], severe OR 3.26 [p = 0.03]). CONCLUSION: Worsening degrees of malnutrition directly correlate with increasing risk of 30-day complications in children with CD undergoing major bowel surgery. These findings support BMI for-age z scores as an important screening tool for preoperatively identifying pediatric CD patients at increased risk for postoperative complications. Moreover, these scores can guide nutritional optimization efforts prior to elective surgery. LEVEL OF EVIDENCE: IV.

Impact of Child Life Services on Children and Families Admitted to Start the Ketogenic Diet.

Traditionally the ketogenic diet is started as an inpatient admission to the hospital. Starting in January 2015, child life services were made formally available during ketogenic diet admissions to help families cope. One-page surveys were then provided to 15 parents on the day of discharge and again after 3 months. Every family believed that the child life services were helpful. Children who were developmentally appropriate/mildly delayed had higher parent-reported anxiety scores than those who were moderate to severely delayed (4.4 vs 1.0, P = .02). At 3 months, child life services were deemed very helpful for the parents (mean score: 8.9, range: 5-10), and were more helpful for the parent than the child (mean 6.2, range 1-10, P = .047). One of the most helpful services was a prior phone call to parents 1 week prior. In this small pilot study, child life involvement during the start of the ketogenic diet was highly useful.

Mucous fistula refeeding decreases parenteral nutrition exposure in postsurgical premature neonates.

BACKGROUND/PURPOSE: Premature neonates can develop intraabdominal conditions requiring emergent bowel resection and enterostomy. Parenteral nutrition (PN) is often required, but results in cholestasis. Mucous fistula refeeding allows for functional restoration of continuity. We sought to determine the effect of refeeding on nutrition intake, PN dependence, and PN associated hepatotoxicity while evaluating the safety of this practice. METHODS: A retrospective review of neonates who underwent bowel resection and small bowel enterostomy with or without mucous fistula over 2years was undertaken. Patients who underwent mucous fistula refeeding (RF) were compared to those who did not (OST). Primary outcomes included days from surgery to discontinuation of PN and goal enteral feeds, and total days on PN. Secondary outcomes were related to PN hepatotoxicity. RESULTS: Thirteen RF and eleven OST were identified. There were no significant differences among markers of critical illness (p>0.20). In the interoperative period, RF patients reached goal enteral feeds earlier than OST patients (median 28 versus 43days; p=0.03) and were able to have PN discontinued earlier (median 25 versus 41days; p=0.04). Following anastomosis, the magnitude of effect was more pronounced, with RF patients reaching goal enteral feeds earlier than OST patients (median 7.5 versus 20days; p≤0.001) and having PN discontinued sooner (30.5 versus 48days; p=0.001). CONCLUSIONS: RF neonates reached goal feeds and were able to be weaned from PN sooner than OST patients. A prospective multicenter trial of refeeding is needed to define the benefits and potential side effects of refeeding in a larger patient population in varied care environments.