Chronic Intestinal Failure in Children: An International Multicenter Cross-Sectional Survey

Abstract

Background: The European Society for Clinical Nutrition and Metabolism database for chronic intestinal failure (CIF) was analyzed to investigate factors associated with nutritional status and the intravenous supplementation (IVS) dependency in children. Methods: Data collected: demographics, CIF mechanism, home parenteral nutrition program, z-scores of weight-for-age (WFA), length or height-for-age (LFA/HFA), and body mass index-for-age (BMI-FA). IVS dependency was calculated as the ratio of daily total IVS energy over estimated resting energy expenditure (%IVSE/REE). Results: Five hundred and fifty-eight patients were included, 57.2% of whom were male. CIF mechanisms at age 1–4 and 14–18 years, respectively: SBS 63.3%, 37.9%; dysmotility or mucosal disease: 36.7%, 62.1%. One-third had WFA and/or LFA/HFA z-scores < −2. One-third had %IVSE/REE > 125%. Multivariate analysis showed that mechanism of CIF was associated with WFA and/or LFA/HFA z-scores (negatively with mucosal disease) and %IVSE/REE (higher for dysmotility and lower in SBS with colon in continuity), while z-scores were negatively associated with %IVSE/REE. Conclusions: The main mechanism of CIF at young age was short bowel syndrome (SBS), whereas most patients facing adulthood had intestinal dysmotility or mucosal disease. One-third were underweight or stunted and had high IVS dependency. Considering that IVS dependency was associated with both CIF mechanisms and nutritional status, IVS dependency is suggested as a potential marker for CIF severity in children.

Definition, classification, and causes of short bowel syndrome

“…These results supported the use of the clinical classification of CIF also as a “severity classification of CIF in adults” to be used in both the clinical setting and research 13 . Similarly, the data from another recent ESPEN international, multicenter, cross‐sectional survey in children suggested that IVS dependency, calculated as the ratio of daily total IVS energy requirement to estimated resting energy expenditure, may be a potential marker for CIF severity in this age category, but this requires confirmation 2 …”

“…13 In children, SBS is mainly due to intestinal volvulus, intestinal malformations, and necrotizing enterocolitis. 2 The ESPEN multicenter survey, including 1880 adult patients, showed that the case mix of patients with SBS-CIF differs between countries. 13 In the entire group, 60.7% of patients were women; SBS type 1 constituted 60.0% of cases; SBS type 2 constituted…”

“…Short bowel syndrome (SBS) is the main pathophysiologic cause of chronic intestinal failure (CIF), accounting for nearly two-thirds of cases in adults 1 and one-half in children. 2 Intravenous supplementation (IVS) of nutrients, provided at the patient's home by home parenteral nutrition (HPN) programs, is the primary and life-saving treatment for CIF. 3 With time and proper management, SBS-associated CIF (SBS-CIF) can be reversible in some patients, such that they can be weaned off IVS.…”

“…10 Both CIF due to benign disease and SBS-CIF have been included in the Orphanet list, the European list of rare diseases, as code 294422 and 104008, respectively. 11 The definition, classification, and causes of CIF and SBS have been recently devised and investigated by ESPEN [1][2][3][4][5]12,13 to facilitate communication and cooperation among professionals in clinical practice, organization, management, and research.…”

Novel Therapeutic Approaches for Mitigating Complications in Short Bowel Syndrome

“…This is typically seen in conditions such as necrotizing enterocolitis, volvulus, and intestinal atresia or strictures [ 8 ]. However, most relevant to this review is the generalized gut atrophy secondary to the state induced by TPN, resulting from a lack of luminal contents [ 1 , 9 , 10 ]. Although the benefits of TPN are widely known and TPN is used as a life-saving treatment worldwide, longer durations of TPN can cause severe liver injury, and lack of luminal contents can induce serious and deadly complications [ 10 , 11 ].…”