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Nutritional therapy complications in children with ultra-short bowel syndrome include growth deficiency but not
Katarzyna Olszewska1, Janusz Ksiazyk1, Dariusz Kozlowski2
1Department of Paediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, Warsaw, Poland.
Insights
Children with ultra-short bowel syndrome (USBS) experience growth deficiencies and bone issues. Those needing daily parenteral nutrition show low citrulline levels, but cholestasis was not observed in this rare condition.
Area of Science:
- Pediatric Gastroenterology
- Nutritional Science
- Endocrinology
Background:
- Ultra-short bowel syndrome (USBS) is a rare condition affecting children.
- Limited research exists on the long-term nutritional status of children with USBS.
- Home parenteral nutrition (PN) is a critical intervention for these patients.
Purpose of the Study:
- To evaluate the nutritional status of children with USBS on home PN.
- To assess serum citrulline concentrations as a marker of intestinal function.
- To investigate the prevalence of cholestasis in this pediatric population.
Main Methods:
- A cohort of 17 children with USBS was studied.
- Data collected included anthropometric measurements, bone mineral density, and 25-hydroxyvitamin D levels.
- Serum citrulline concentrations and bilirubin levels were analyzed.
Main Results:
- Children with USBS exhibited growth deficiency (median SDS for BMI: -1.2).
- 87% had decreased bone mineral density and 53% had vitamin D deficiency.
- Patients requiring daily PN had serum citrulline < 10 μmol/L; no cholestasis was detected.
Conclusions:
- Children with USBS often present with growth deficits, poor bone mineralization, and vitamin D deficiency.
- Low serum citrulline (< 10 μmol/L) is associated with the need for daily PN in USBS.
- Home PN appears to prevent cholestasis in children with USBS.
Aim:
Children with ultra-short bowel syndrome (USBS) have not been extensively studied to date because the condition is rare. The aim of the study was to assess the nutritional status of children with USBS receiving home parenteral nutrition, using citrulline serum concentration and cholestasis.
Methods:
We studied 17 patients with USBS, with a median age of 6.6 years and median duration of parenteral nutrition of 6.6 years. The study was carried out at The Children's Memorial Health Institute, Warsaw, from January 2014 to January 2015.
Results:
The median standard deviation score (SDS) was -1.2 for body mass according to chronological age, -1.72 according to height and -0.59 according to height for age. Patients requiring seven days per week parenteral nutrition had a citrulline concentration below 10 μmol/L. Decreased bone-mineral density was observed in 87% of the patients. Low values of 25-hydroxyvitamin D were found in 53% of the children. None of the patients had elevated conjugated bilirubin levels above 34.2 μmol/L.
Conclusion:
Children with USBS were growth deficient according to their chronological age, with frequent abnormal bone mineralisation and vitamin D deficiency. Children requiring parenteral nutrition seven days a week had citrulline concentrations below 10 μmol/L. Cholestasis was not seen.
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