Generic and Disease-specific Health-related Quality of Life in Pediatric Intestinal Failure.

OBJECTIVE: The aim of the study was to assess overall and disease-specific health-related quality of life (hrQOL) in patients with pediatric intestinal failure (PIF) and caregivers and elucidate differences from healthy and chronic gastrointestinal (GI) illness cohorts. METHODS: Cross-sectional study of patients with PIF and their caregivers managed at a multidisciplinary intestinal rehabilitation program using the PedsQL Generic Core and the Gastrointestinal Symptoms Module to assess generic and disease-specific hrQOL, respectively. These data were compared to established healthy and chronic GI disease controls. RESULTS: A total of 53 patients (mean age 6.2 ±â€Š3.9 years) and their caregivers were studied. Patients reported lower generic hrQOL than healthy children (73.0 vs 83.84, P < 0.001), but no difference from patients with chronic GI disease (73.0 vs 77.79). In contrast, PIF caregivers perceived similar generic hrQOL compared to a healthy cohort (78.9 vs 82.70), but higher when compared to the GI disease cohort (78.9 vs 72.74, P < 0.01). Patients with PIF and caregivers reported lower psychosocial health scores than healthy controls. Patients and caregivers reported similar disease-specific hrQOL to a cohort with chronic GI disease but significantly lower disease-specific hrQOL than a healthy cohort (P < 0.001 both groups). CONCLUSIONS: Patients with PIF and their caregivers have disparate perceptions of generic hrQOL when compared to healthy and chronic GI disease controls. Both patients and caregivers, however, had significantly lower scores in psychosocial health than healthy controls. In addition, disease-specific hrQOL was substantially lower than healthy controls for PIF patients and caregivers. Further investigation to expand on these findings and identify modifiable variables to improve the psychosocial health score and disease-specific factors would be of high value.

Is Serum Methylmalonic Acid a Reliable Biomarker of Vitamin B12 Status in Children with Short Bowel Syndrome: A Case Series.

We describe 3 patients with short bowel syndrome who had persistently elevated serum methylmalonic acid (MMA) levels while being treated for vitamin B12 deficiency. Following treatment for presumed small bowel bacterial overgrowth, MMA levels normalized. Among patients with short bowel syndrome, MMA levels may have limited specificity for vitamin B12 deficiency.

High Prevalence of Eosinophilic Gastrointestinal Disease in Children With Intestinal Failure.

OBJECTIVES: The aim of the present study was to describe the prevalence and clinical features of gastrointestinal (GI) eosinophilic inflammation among pediatric patients with intestinal failure (IF). METHODS: Medical records of all patients studied in our institution's IF program who underwent GI endoscopy over a 15-year period were reviewed, and clinical, pathologic, nutrition, and laboratory data collected. RESULTS: One hundred five patients underwent 208 GI endoscopic procedures with biopsy. The overall prevalence of eosinophilic inflammation, defined as increased eosinophils in at least 1 tissue type on at least 1 endoscopy, was 39 of 105 (37%). The tissue-specific prevalence of eosinophilic inflammation ranged widely, with the colon/rectosigmoid being the most common (18/68, 26%), followed by the esophagus (17/83, 20%), ileum (9/54, 17%), duodenum (4/83, 5%), and stomach (3/83, 4%). Higher peripheral eosinophil count and hematochezia were associated with eosinophilic inflammation in the colon (P = 0.002 and 0.0004, respectively). The use of a strict elemental diet for 3 months before endoscopy was not associated with a decreased frequency of eosinophilic inflammation in any tissue. CONCLUSIONS: Eosinophilic inflammation is a common histopathological finding in patients with IF. Colonic eosinophilic inflammation is associated with clinical symptoms of GI blood loss, and peripheral eosinophilia, and was not abrogated by a strict elemental diet.

Gastrointestinal bleeding as a complication of serial transverse enteroplasty.

PURPOSE: Serial transverse enteroplasty (STEP) lengthens and tapers bowel in patients with intestinal failure. Evaluation and treatment of serious late gastrointestinal bleeding (GIB) in three STEP patients are described. METHODS: Patients participating in an interdisciplinary intestinal rehabilitation program were reviewed to identify those who underwent STEP and had GIB requiring transfusion. RESULTS: Of 296 patients, 23 underwent STEP, and 3 (13%) had subsequent GIB requiring transfusion. Diagnoses were vanishing gastroschisis/atresia, malrotation/atresia, and gastroschisis.. STEP was performed at ages 3-5 months, using 5-15 stapler-firings with an increase in mean bowel length from 39 to 62 cm. GIB was diagnosed 5-30 months post-op and resulted in 1-7 transfusions per patient. Endoscopy demonstrated staple-line ulceration in two patients and eosinophilic enterocolitis in the third. All were treated with enteral antibiotics, sulfasalazine, and luminal steroids. Those with ulcers responded to bowel rest, and the patient with eosinophilic enterocolitis stabilized with luminal steroids. In all three, hemoglobin levels improved despite persistent occult bleeding. CONCLUSIONS: Significant GIB is a potential late complication of STEP. Endoscopy identified the underlying source of GIB in all three patients. A combination of enteral antibiotics, anti-inflammatory medications, and bowel rest was effective in treating post-STEP GIB, without the need for additional bowel resection.