Cognitive deficits in school-age children with severe short bowel syndrome.

BACKGROUND/PURPOSE: Improved therapies for the management of short bowel syndrome (SBS) have resulted in the prolonged survival of many children. By early childhood, the physiological sequelae of severe SBS include delayed physical development and metabolic imbalances. However, little is known about how SBS affects brain development. Although many parents report school problems, no controlled study has evaluated the integrity of the central nervous system in SBS children. The purpose of this study was to investigate the neuropsychological status of school-aged SBS children to determine if there were characteristic cognitive impairments that might be amenable to early therapeutic intervention. METHODS: SBS children (n = 8; mean age, 116.9 +/- 21 months) were compared with an age-matched cystic fibrosis (CF) control group (n = 8; mean age, 118.1 +/- 14 months). Groups did not differ in age, grade, or absences. Neuropsychological tests with established sensitivity to CNS integrity compared performance over 6 cognitive domains. Emotional status also was measured. Analyses were completed with 2-tailed t tests. RESULTS: Groups did not differ on tests of intellectual ability and emotional function. Language, memory and learning, and problem-solving testing results indicated no significant group differences. However, the SBS group performed more poorly on measures assessing visual-spatial ability, with P values ranging from .002 to .045. In a subset of subjects, we noted significantly slower left-handed, but not right-handed, performance on measures of finger dexterity and psychomotor speed. CONCLUSIONS: Although emotional status did not differ from that of children with CF, SBS patients showed visual-spatial deficits in the company of preserved language, attention and memory, and executive skills. The specificity and consistency of these findings suggests that right hemisphere CNS changes may occur in children with SBS. This unexpected finding, coupled with the indication of left-sided psychomotor slowing in right-handed subjects, raises the possibility that actual brain impairment, rather than developmental delay accompanying slowed physical growth, accounts for these findings. Longitudinal studies are needed to further clarify this issue. The educational significance of the results is discussed.

Coordinated interdisciplinary management of pediatric intestinal failure: a 2-year review.

BACKGROUND/PURPOSE: Intestinal failure is a complex metabolic process that results from malabsorption and malnutrition and provides challenges for a variety of pediatric subspecialists. The purpose of this study was to evaluate the effect of coordinated interdisciplinary team management of children with intestinal failure on nutritional outcome measures. METHODS: The Intestinal Care Center (ICC) is staffed with an interdisciplinary team of pediatric specialists including a gastroenterologist, pediatric surgeon, transplant surgeon, clinical dietitians, and a nutrition support nurse. Using an established registry, the authors conducted a comprehensive evaluation of patient data including anthropometric measures, organ system function, and mode of nutrition support. Disease-associated complications including micronutrient deficiencies, growth delay, and death also were monitored. Nutritional outcome was assessed by transition from enteral to oral feeding, cessation of total parenteral nutrition (TPN), and maintenance of linear growth. RESULTS: Since the inception of the ICC in 1996, 103 patients (69 boys, 34 girls) with intestinal failure have been evaluated with a median age of 2.6 years (range, 0.2 to 21.3 years). Mode of nutritional therapy on initial consultation included TPN (n = 76, 74%), enteral feedings (n = 6, 6%) and oral intake (n = 21, 20%). After intensive management of the 76 patients who were TPN dependent, 22 (29%) subsequently have been weaned from TPN (duration, 0.2 to 17.5 years) to oral (n = 14), oral-enteral (n = 4) or enteral feedings (n = 4). Of the 6 patients who were receiving enteral feedings, 4 (67%) were transitioned to oral feedings. Sixty-eight patients (66%) had evidence of hepatic disease. Of these, 10 underwent transplant, and 23 died (2 posttransplant). Linear growth velocity of neither pre- nor postpubescent patients significantly improved during the 2-year study period. CONCLUSION: Data registry establishment and concurrent interdisciplinary team management of children with intestinal failure provides for innovative treatment approaches and a foundation for retrospective or prospective assessment of children with disease.

Insulinlike growth factor 1 and insulinlike growth factor 3: indices of intestinal failure in children.

BACKGROUND/PURPOSE: A number of pediatric patients with short bowel syndrome (SBS) manifest growth failure despite aggressive nutritional support. Exogenous growth hormone (GH) therapy in children with SBS has proved disappointing. The purpose of this study was to determine if there were characteristic patterns of GH, IGF-1, or IGFBP-3 levels in pediatric SBS patients with profound growth failure in an effort to elucidate an early strategic approach to management of SBS in the subpopulation. METHODS: Forty patients (29 boys, 11 girls; mean age, 5.3 years; range, 0.5 to 18.6 years) with SBS (<30% total bowel length) who received intensive nutrition support and follow-up underwent serological tests for GH, IGF-1, IGFBP-3, and thyroid function. Height (HT), weight (WT), and bone age were assessed relative to age-appropriate percentiles. Growth failure was defined as a HT and WT at less than the fifth percentile and bone age > or = 2 standard deviations below actual age. Residual small bowel length was determined by review of pathological and operative reports. Comparisons between the growth factors, bowel length, and anthropometric data were analyzed by chi2. RESULTS: Two distinct subgroups of patients emerged from our study. Thirty-eight percent of patients (n = 11) had growth failure by anthropometry that was associated significantly with low IGF-1 independently and with both IGF-1 and IGFBP-3 levels (P< 0.05). There were no significant associations with GH level, thyroid function, small bowel length, or the amount of parenteral versus enteral intake in either subgroup of these patients. Low IGF-1 and IGFBP-3 but not GH levels may be indices of intestinal failure in pediatric SBS. Growth in this subpopulation is refractory to aggressive standard approaches to nutritional support and may require early interventions. CONCLUSION: Exogenous IGF-1 and IGFBP-3, not GH, may be beneficial to treat this subpopulation.