Resolution of parenteral nutrition-associated jaundice on changing from a soybean oil emulsion to a complex mixed-lipid emulsion.

BACKGROUND AND OBJECTIVE: Resolution of parenteral nutrition (PN)-associated jaundice has been reported in children given a reduced dose of intravenous fat using a fish oil-derived lipid emulsion. The aim of the present study was to examine the effect on PN-associated jaundice of changing from a soybean oil-derived lipid to a mixed lipid emulsion derived from soybean, coconut, olive, and fish oils without reducing the total amount of lipid given. METHODS: Retrospective cohort comparison examining serum bilirubin during 6 months in children with PN-associated jaundice who changed to SMOFlipid (n=8) or remained on Intralipid (n=9). RESULTS: At entry, both groups received most of their energy as PN (SMOFlipid 81.5%, range 65.5-100 vs Intralipid 92.2%, range 60.3-100; P=0.37). After 6 months, both tolerated increased enteral feeding but still received large proportions of their energy as PN (SMOFlipid 68.4%, range 36.6-100 vs Intralipid 50%, range 37.6-76; P=0.15). The median bilirubin at the outset was 143 µmol/L (range 71-275) in the SMOFlipid group and 91 µmol/L (range 78-176) in the Intralipid group. After 6 months, 5 of 8 children in the SMOFlipid and 2 of 9 children in the Intralipid group had total resolution of jaundice. The median bilirubin fell by 99 µmol/L in the SMOFlipid group but increased by 79 µmol/L in the Intralipid group (P=0.02). CONCLUSIONS: SMOFlipid may have important protective properties for the liver and may constitute a significant advance in PN formulation. Randomised trials are needed to study the efficacy of SMOFlipid in preventing PN liver disease.

Atypical disease phenotypes in pediatric ulcerative colitis: 5-year analyses of the EUROKIDS Registry.

BACKGROUND: Definitive diagnosis of pediatric ulcerative colitis (UC) may be particularly challenging since isolated colitis with overlapping features is common in pediatric Crohn's disease (CD), while atypical phenotypes of UC are not uncommon. The Paris classification allows more accurate phenotyping of atypical inflammatory bowel disease (IBD) patients. Our aim was to identify the prevalence of atypical disease patterns in new-onset pediatric UC using the Paris classification. METHODS: Information was collected from the EUROKIDS Registry, an inception cohort of untreated pediatric IBD patients undergoing evaluation at diagnosis. Patients with IBD-unclassified were excluded. Patients with isolated Crohn's colitis served as a control group. RESULTS: Data from 898 pediatric patients (643 UC, 255 CD colitis) were included. Extensive or pancolitis was present in 77% of UC patients and macroscopic rectal sparing in 5%. Rectal sparing was inversely associated with age (mean age with rectal sparing 9.9 years vs. 11.8 without; P = 0.02). Upper gastrointestinal (UGI) involvement occurred in 4% of patients. Erosions in the stomach were present in 3.1% of children, but frank ulcerations in 0.4%; 0.8% of children had erosions or ulcerations limited to the esophagus or duodenum. The corresponding UGI involvement in Crohn's colitis was 22%. A cecal patch occurred in 2% of patients. CONCLUSIONS: Extensive disease and rectal sparing are age-dependent phenotypes in pediatric UC. Rectal sparing, cecal patch, backwash ileitis, and gastric erosions are not uncommon at diagnosis, while gastric ulcerations and erosions in the duodenum or esophagus are. Recognition of atypical phenotypes in pediatric-onset UC is crucial to prevent misclassification of IBD.

Contemporary outcomes for ulcerative colitis inpatients admitted to pediatric hospitals in the United Kingdom.

BACKGROUND: Pediatric ulcerative colitis (UC) care is variable with a lack of appropriate guidelines to guide practice until recently. METHODS: UC inpatients <17 years old admitted to 23 U.K. pediatric hospitals had clinical details collected between September 2010 and 2011. Comparative data for 248 patients were available from a previous audit in 2008. RESULTS: One hundred and seventy-six patients (98 males) of median age 13 years (interquartile range, 10-13) were analyzed; 23 were elective surgical admissions, 47 new diagnoses, and 106 needed acute medical care for established UC. Median length of stay was 6 days (interquartile range, 3-10) with no deaths. Eighty-eight of 126 patients (70%) with active disease had standard stool cultures performed (3 [2%] were positive), and 57 (45%) had Clostridium difficile toxin tested (none positive). Twenty-five of 66 (38%) emergency admissions had an abdominal x-ray on admission, and 13 of 66 patients (20%) had a Pediatric Ulcerative Colitis Activity Index score. There were 3 cases of toxic megacolon and 2 thromboses. Eighty-one of 116 patients (71%) responded to steroids. Nineteen patients who did not respond adequately to steroids received rescue therapy (7 infliximab, 11 ciclosporin, and 1 both) with overall response rate of 90%; 7 patients needed surgery acutely, 5 without previous rescue therapy. Compared with the 2008 data, stool culture rates improved significantly (86 of 121 [71%] versus 76 of 147 [52%], P = 0.001) as did heparinization rates (15 of 150 [10%] versus 5 of 215 [2%], P = 0.002) and rescue therapy usage (17 of 33 [52%] versus 10 of 38 [26%], P = 0.03). CONCLUSIONS: There were signs of improving UC care with significantly increased rates of stool culture and rescue therapy. The majority of sites, however, did not use Pediatric Ulcerative Colitis Activity Index scores.

Improved outcome of referrals for intestinal transplantation in the UK.

AIM: To describe the outcome of children with intestinal failure referred to Birmingham Children's Hospital (BCH) for consideration of intestinal transplantation (ITx), to determine factors for an adverse outcome and to analyse the impact of post-1998 strategies on survival. SUBJECTS AND METHODS: A retrospective analysis was performed of children referred for ITx assessment from January 1989 to December 2003. Children were assessed by a multidisciplinary team and categorised into: (a) stable on parenteral nutrition; (b) unsuitable for transplantation (Tx); and (c) recommended for Tx. To analyse the impact of the post-1998 strategies on survival, a comparison was made between the two eras (pre-1998 and post-1998). RESULTS: 152 children with chronic intestinal failure were identified (63M:89F, median age 10 months (range 1-170)). After assessment, 69 children were considered stable on parenteral nutrition (5-year survival 95%); 28 children were unsuitable for Tx (5-year survival 4%); and 55 children were recommended for Tx (5-year survival 35%, which includes 14 children who died waiting for size-matched organs). Twenty three ITx and nine isolated liver transplants (iLTx) were performed. In a multivariate analysis, the following factors in combination had an adverse effect on survival: the presence of a primary mucosal disorder (p = 0.007, OR ratio 3.16, 95% CI 1.37 to 7.31); absence of involvement of a nutritional care team at the referring hospital (p = 0.001, OR ratio 2.55, 95% CI 1.44 to 4.52); and a serum bilirubin>100 micromol/l (p = 0.001, OR ratio 3.70, 95% CI 1.84 to 7.47). Earlier referral (median serum bilirubin 78 micromol/l in the post-1998 era compared with 237 micromol/l in the pre-1998 era, p = 0.001) may be a contributory factor to improved survival. The strategies of combined en bloc reduced liver/small bowel transplantation and iLTx resulted in fewer deaths on the waiting list in the post-1998 era (2 deaths in post-1998 era v 12 deaths in pre-1998 era). The overall 3-year survival in the post-1998 era (69%) has improved compared with the pre-1998 era (31%; p<0.001) CONCLUSION: The changing characteristics at the time of referral, including earlier referral and innovative surgical strategies have resulted in improved long-term survival of children referred for ITx.