Children With Intestinal Failure Undergoing Intestinal Rehabilitation Are at Risk for Essential Fatty Acid Deficiency

Gunnar, Riikka; Lumia, Mirka; Pakarinen, Mikko P.; Merras‐Salmio, Laura

doi:10.1002/jpen.1152

Cited by 11 publications

(8 citation statements)

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“…Systemic inflammation and poor nutrition during the neonatal period affect growth and hence have a negative effect on neurological development (7). IF children with more severe disease forms require longer PN, are at risk for repeat infections, and are more likely to develop nutritional deficiencies during enteral rehabilitation (10). The adverse effect of prolonged PN is also shown in our study where children who continued on PN during the study period had lower IQ scores than children who had rehabilitated to EN.…”

Section: Discussionmentioning

confidence: 99%

Neonatal Intestinal Failure Is Independently Associated With Impaired Cognitive Development Later in Childhood

Gunnar

Kanerva²,

Salmi³

et al. 2020

J. pediatr. gastroenterol. nutr.

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Objective: The impact of pediatric intestinal failure (IF) on neurodevelopment beyond infancy has not been systematically studied. Our aim was to evaluate cognitive and motor impairment and to identify risk factors for adverse outcomes among children with IF. Methods: We conducted a cross-sectional single-center study at the Helsinki University Children's Hospital. Patients with IF with >60 days of parental nutrition (PN) dependency aged between 3 and 16 years (n = 40) were invited to participate. The cognitive and motor skills were evaluated using validated tests: Wechsler Preschool and Primary Scale of Intelligence, 3rd edition, Wechsler Intelligence Scale for Children, 4th edition, and Movement Assessment Battery for Children, 2nd edition. Results: All the patients attending the study tests (n = 30, males = 24) were included. Their median age, gestational age, and birth weight was 7.5 (range 3–16) years, 35 (interquartile range [IQR] 28–38) weeks and 2238 (IQR 1040–3288) grams, respectively. Median duration of PN was 13 (IQR 5–37) months and 9 patients were currently on PN. Median intelligence quotient was 78 (IQR 65–91) and 10 (35%) patients had an intelligence quotient under 70 (−2 standard deviation). Significant motor impairment was detected in 10 patients (36%) and milder difficulties in 8 (28%). Adverse cognitive outcome was associated with neonatal short bowel syndrome, number of interventions under general anesthesia, and length of inpatient status, whereas adverse motor outcome was associated with prematurity. Conclusion: Clinically significant cognitive and motor impairments are alarmingly common among neonatal patients with IF. We recommend early neurodevelopmental follow-up for all children with IF.

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Section: Discussionmentioning

confidence: 99%

Neonatal Intestinal Failure Is Independently Associated With Impaired Cognitive Development Later in Childhood

Gunnar

Kanerva²,

Salmi³

et al. 2020

J. pediatr. gastroenterol. nutr.

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“…Multidisciplinary intestinal rehabilitation teams in expert centers have a significant advantage in patient care in terms of optimizing PN, promoting enteral nutrition, and reducing complications such as infections and liver diseases while providing correctly timed listing for intestinal transplant when indicated ( 10 ). Even after weaning off PN, children with SBS are at risk for further complications and malnutrition necessitating long-term expert follow-up ( 11 , 12 ). Important complications of pediatric SBS include infections, associated with both central venous catheter (CVC) and the diseased gut.…”

Section: Therapy and Outcomes Of Pediatric Short Bowel Syndromementioning

confidence: 99%

Infection Prevention and Management in Pediatric Short Bowel Syndrome

Merras‐Salmio

Pakarinen

2022

Front. Pediatr.

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Short bowel syndrome (SBS) is a rare disease with potentially life-threatening consequences. In addition to intestinal failure-associated liver disease, infections and other complications related to central venous catheters (CVCs) cause a significant burden to patients with SBS and may even necessitate an intestinal transplant eventually. The need for long-term central venous access and the intestinal dysfunction associated with SBS drive the need for intestinal failure-specific approach to prevent and treat infections in patients with SBS. In bacterial infections, the line can often be salvaged with proficient antibiotic therapy. Repeated catheter replacements are predisposed to recurrent infections and thrombotic complications, which may limit the long-term survival of patients with SBS. Protocol-based CVC access procedures and daily care including taurolidine and ethanol catheter locks have been shown to reduce infection rates substantially. Compromised intestinal function in SBS predisposes to small bowel bacterial overgrowth, mucosal injury, and increased permeability. These pathophysiological changes are concentrated in a subset of patients with excessive bowel dilatation and frequent bowel-derived infections. In such patients, reconstructive intestinal surgery may be indicated. Probiotics have not been effective in infection prevention in SBS and carry a significant risk of complications. While more studies focusing on the prevention of infections and their complications are needed, protocol-based approach and multidisciplinary teams in the care of patients with SBS have been shown to reduce complications and improve outcomes.

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“…Recent reports of EFAD in patients with IF who are receiving PN have raised renewed concerns regarding the safety of MO ILE lipids. [44][45][46][47] These reports, however, are predominantly casebased, and in some cases were managed by lipid restriction. EFAD can lead to intellectual disability and poor growth in children.…”

Section: Introductionmentioning

confidence: 99%

Composite lipid emulsion use and essential fatty acid deficiency in pediatric patients with intestinal failure with high parenteral nutrition dependence: A retrospective cohort study

Belza

Courtney‐Martin

Wong-Sterling

et al. 2023

J Parenter Enteral Nutr

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BackgroundReports of essential fatty acid deficiency (EFAD) in patients receiving parenteral nutrition (PN) and a composite lipid (mixed oil intravenous lipid emulsion [MO ILE]) are predominantly when managed by lipid restriction. The objective of this study was to determine the prevalence of EFAD in patients with intestinal failure (IF) who are PN dependent without lipid restriction.MethodsWe retrospectively evaluated patients, ages 0–17 years, followed by our intestinal rehabilitation program between November 2020 and June 2021 with PN dependency index (PNDI) of >80% on a MO ILE. Demographic data, PN composition, PN days, growth, and plasma fatty acid profile were collected. A plasma triene‐tetraene (T:T) ratio >0.2 indicated EFAD. Summary statistics and Wilcoxon rank sum test evaluated to compare between PNDI category and ILE administration (grams/kilograms/day). P < 0.05 was considered significant.ResultsTwenty‐six patients (median age, 4.1 years [interquartile range (IQR) = 2.4–9.6]) were included. The median duration of PN was 1367 days (IQR = 824–3195). Sixteen patients had a PNDI of 80%–120% (61.5%). Fat intake for the group was 1.7 g/kg/day (IQR = 1.3–2.0). The median T:T ratio was 0.1 (IQR = 0.1–0.2) with no values >0.2. Linoleic and arachidonic acid were low in 85% and 19% of patients, respectively; however, Mead acid was normal in all patients.ConclusionThis report is the largest to date on the EFA status of patients with IF on PN. These results suggest that, in the absence of lipid restriction, EFAD is not a concern when using MO ILEs in children receiving PN for IF.

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Children With Intestinal Failure Undergoing Intestinal Rehabilitation Are at Risk for Essential Fatty Acid Deficiency

Abstract: IF children with supplemental PN carry the highest overall risk for EFAD. Young age, current PN, and short remaining small bowel also increase the risk for EFAD.

Cited by 11 publications

References 44 publications

Neonatal Intestinal Failure Is Independently Associated With Impaired Cognitive Development Later in Childhood

Neonatal Intestinal Failure Is Independently Associated With Impaired Cognitive Development Later in Childhood

Infection Prevention and Management in Pediatric Short Bowel Syndrome

Composite lipid emulsion use and essential fatty acid deficiency in pediatric patients with intestinal failure with high parenteral nutrition dependence: A retrospective cohort study

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