Pediatric intestinal failure: Predictors of metabolic bone disease.

PURPOSE: The purpose of this study was to identify risk factors for the development of metabolic bone disease (MBD) in pediatric intestinal failure (IF). METHODS: A retrospective single-center study of 36 pediatric IF patients who were screened for MBD was performed. Bone mineral density (BMD) was measured using dual-energy X-ray absorptiometry (DXA). Simple regression analysis was initially performed to screen predictors, followed by multivariate step-wise linear regression analysis to identify risk factors of MBD. RESULTS: Mean lumbar spine BMD Z-score was -1.16 ± 1.32, and 50.0% of patients had a BMD Z-score less than -1.0. Deficiency of 25-hydroxyvitamin-D (25-OHD <30 ng/ml) was present in the 63.8% of patients, while 25.0% had hyperparathyroidism (intact parathyroid hormone (PTH)>55 pg/ml). Seven patients (19.4%) had bone pain, of which 4 (11.1%) suffered a pathologic fracture. Using multivariate analysis, parenteral nutrition (PN) duration predicted decreased BMD (B=-0.132, p=0.006). Serum 25-OHD nonsignificantly correlated with BMD Z-score (B=0.024, p=0.092). Interestingly, repeat DXA after increasing vitamin D supplementation showed no improvement in BMD Z-score (-1.18 ± 1.49 vs -1.36 ± 1.47, p=0.199). CONCLUSIONS: Pediatric IF is associated with a significant risk of MBD, which is predicted by the duration of PN-dependence. These findings underscore the importance of BMD monitoring. Better therapies for treating IF-associated MBD are needed.

Enteral autonomy in pediatric short bowel syndrome: predictive factors one year after diagnosis.

PURPOSE: This study examined predictors of achieving enteral autonomy among pediatric short bowel syndrome (SBS) patients remaining on parenteral nutrition (PN) beyond one year. METHODS: A retrospective single-institution study of 171 pediatric SBS patients (defined as ≥50% small bowel (SB) loss or ≥60 days of PN with onset before 6 weeks of age) was performed. Multivariate Cox proportional hazards analysis was conducted, with subgroup analysis of patients on PN for ≥1 year (n=59). Primary outcome was successful wean from PN. RESULTS: Over a follow-up of 4.1±4.8 years, 64.3% of children weaned from PN. Mortality was 15.2%. Presence of ≥10% expected SB length (hazard ratio [HR] 6.48, p=0.002) or an ileocecal valve (ICV; HR, 2.86, p<0.001) predicted PN weaning. Of those on PN ≥1 year, the wean rate was 50.8%, and ICV no longer predicted weaning (p=0.153). Predictors among those on PN ≥1 year were: ≥10% expected SB length (HR, 8.27, p=0.010), intestinal atresia (HR, 4.26, p=0.011), and necrotizing enterocolitis (NEC, HR, 2.84, p=0.025). CONCLUSIONS: SBS children on PN ≥1 year continue to wean from PN, and those with ≥10% of predicted SB length, NEC, or atresia are more likely to do so. These findings may help direct management and advice for these challenging patients.

Survival outcomes of pediatric intestinal failure patients: analysis of factors contributing to improved survival over the past two decades.

BACKGROUND: Intestinal failure (IF) is associated with significant and life-threatening complications. Recent studies suggest that treatments for IF in the pediatric population are improving over time. Based on this, we examined whether pediatric IF survival rates have improved in our patient population over the past two decades, and secondarily examined which aspects of patient care contributed to changes in survival. METHODS: We conducted a retrospective chart review of all pediatric patients with IF at our children's hospital from 1990 through 2009. Cox regression analyses were used to determine change in survival rates over time (5-y cohorts), and we examined multiple covariates to determine their potential influence on survival rates over time. RESULTS: A significant improvement in survival of 171 children with IF was noted over the past two decades. Children with an onset of IF in the 1990-1994 cohort had significantly decreased survival compared with children in all subsequent cohorts (P = 0.011). The only intervention that was identified between this time period and future periods was the establishment of a comprehensive intestinal failure clinical care team. While the latter three cohorts were not significantly different, progressively increased survival was noted. Ability to wean off parenteral nutrition (PN) and small bowel length greater than 10% of the expected length were significantly associated with improved survival over this time period (P < 0.01). Other tested covariates, including the more recent use of ethanol lock therapy (to prevent catheter sepsis) and lipid reduction strategies (to treat PN-associated cholestasis) failed to show a significant impact on improved survival. CONCLUSIONS: Despite a striking improvement in survival of children with IF over the past two decades, the only identified intervention that significantly impacted survival was establishment of a comprehensive care team. These findings emphasize the need for multi-disciplinary efforts to care for such complex and challenging children.

Redilation of bowel after intestinal lengthening procedures--an indicator for poor outcome.

BACKGROUND/PURPOSE: Despite a good understanding of short-term outcomes of the longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) procedures, limited data exist on long-term complications. METHODS: This is a 15-year single-institution retrospective chart review of patients who underwent an intestinal lengthening procedure (ILP). Long-term ILP-related complications, their interval to development, patients' ability to wean off parenteral nutrition (PN), and the need for further procedures were analyzed. RESULTS: Of 119 patients with short bowel syndrome, 14 had undergone an ILP. Seven patients had an LILT, and 9 patients had a STEP, including repeat ILPs on the same patient. Overall, 93% of patients had complications. Four patients in the LILT group and 3 patients in the STEP group weaned off PN. Eight patients (57%) experienced bowel redilation after their ILP. The 2 deaths in the study came from this group. Seven required another abdominal operation and only one weaned off PN. There were no significant differences in mean bowel length between the redilated group and the non-re-dilated group. CONCLUSIONS: Complications are common after ILPs, and patients who redilated their bowel after ILP did clinically worse than those who did not.

The adolescent child with short bowel syndrome: new onset of failure to thrive and need for increased nutritional supplementation.

BACKGROUND: Proper nutrition for children with short bowel syndrome (SBS) is challenging. An underappreciated SBS cohort is the group of adolescents in which nutritional demand is markedly increased. We identified several children that previously had weaned off parenteral nutrition but needed re-initiation of parenteral nutrition or increased enteral nutritional supplementation (NS) during adolescence. This study aims to identify characteristics of adolescent SBS patients to better understand their nutritional status. METHODS: A single institution, retrospective review of SBS patients over 10 years was done. Patients were analyzed for NS need, remaining bowel length, height, weight, and essential nutrient levels. RESULTS: Sixteen adolescent SBS patients were identified for analysis. Of these, ten did not require increased NS upon reaching adolescence (group A), and six required increased NS during adolescence (group B). Group B patients required a mean increase in NS of 29.6 kcal/kg per day. Group B patients had significantly shorter remaining bowel length. NS resulted in minimal height increase, but more substantial gains in weight and pubertal development. CONCLUSIONS: Adolescents with SBS may require NS beyond what can be provided enterally. These patients are at risk for nutritional deficiencies, and an aggressive approach to monitoring and supplementing these deficiencies is warranted, especially during this critical period of growth.

The clinical significance of focal active colitis in pediatric patients.

The clinical significance of focal neutrophilic infiltrates in crypt epithelium in colorectal biopsies or focal active colitis has been studied in adult populations, but little is known about this entity in children. The incidence of Crohn's disease in adult patients presenting with focal active colitis has varied between 0% and 13% in previous studies, whereas the incidence of infectious-type colitis has been reported to be nearly 50%. We reviewed 31 cases of focal active colitis diagnosed in pediatric patients without a history of inflammatory bowel disease between 1989 and 2000. Pathologic variables studied included number and location of inflamed crypts and distribution and character of lamina propria inflammation. Clinical follow-up was obtained from patient charts. Two patients were lost to follow-up. Follow-up on the remaining 29 patients ranged from 4 months to 7 years with a mean of 4.2 years. Eight patients (27.6%) developed Crohn's disease. Nine patients (31%) appeared to have acute infectious-type colitis, one with C. difficile. Eight patients (27.6%) had focal active colitis, which did not correlate with their symptoms or ultimate clinical diagnosis. These were termed idiopathic focal active colitis. Two patients were found to have allergic colitis, one had ulcerative colitis, and one had Hirschsprung's disease. Pediatric patients with focal active colitis have a much higher incidence of Crohn's disease than adults with same entity. Hence, it is important to document the presence of focal active colitis in pediatric patients.

Refractory iron deficiency anemia as the primary clinical manifestation of celiac disease.

In the absence of dietary insufficiency, iron deficiency is usually caused by chronic blood loss or intestinal malabsorption. Celiac disease is one of the most common causes of intestinal malabsorption during childhood, and its association with insulin-dependent diabetes mellitus has been previously reported. Here the authors describe an otherwise asymptomatic diabetic adolescent boy with iron deficiency anemia that was not responsive to oral iron therapy. A diagnosis of celiac disease was made based on both anti-endomysial antibody titers and small intestinal biopsy. Institution of a gluten-free diet resulted in correction of the anemia. These observations emphasize the importance of considering a diagnosis of celiac disease in patients with nonresponsive iron deficiency anemia, particularly in the setting of insulin-dependent diabetes mellitus.