Chronic Intestinal Failure in Children: An International Multicenter Cross-Sectional Survey
Antonella Lezo, Antonella Diamanti, Evelyne Marinier, Merit M. Tabbers, Anat Guz‐Mark, Paolo Gandullia, Maria Immacolata Spagnuolo, Sue Protheroe, Noël Peretti, Laura Merras‐Salmio, Jessie M. Hulst, Sanja Kolaček, Looi C. Ee, Joanna Lawrence, Jonathan Hind, Lorenzo D’Antiga, Giovanna Verlato, Ieva Puķīte, Grazia Di Leo, Tim Vanuytsel, Maryana Doitchinova-Simeonova, Lars Ellegård, Luisa Masconale, María Maíz-Jiménez, Sheldon C. Cooper, Giorgia Brillanti, Elena Nardi, Anna Simona Sasdelli, Simon Lal, Loris Pironi
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.