A European Survey on Digestive Perianastomotic Ulcerations, a Rare Crohn-like Disorder Occurring in Children and Young Adults.

OBJECTIVES: Digestive perianastomotic ulcerations (DPAU) resembling Crohn disease lesions are long-term complications of intestinal resections, occurring in children and young adults. They are known to be uncommon, severe and difficult to treat. METHODS: In the absence of recommendations, we performed a large European survey among the members of the ESPGHAN working group on inflammatory bowel disease (IBD) in order to collect the experience of expert pediatric gastroenterologists on DPAU. RESULTS: Fifty-one patients (29 boys and 22 girls) were identified from 19 centers in 8 countries. Most patients were followed after necrotizing enterocolitis (n = 20) or Hirschsprung disease (n = 11). The anastomosis was performed at a median age (interquartile range) of 6 [1-23] months, and first symptoms occurred 39 [22-106] months after surgery. Anemia was the most prevalent symptom followed by diarrhea, abdominal pain, bloating, and failure to thrive. Hypoalbuminemia, elevated CRP, and fecal calprotectin were common. Deep ulcerations were found in 59% of patients usually proximally to the anastomosis (68%). During a median follow-up of 40 [19-67] months, treatments reported to be the most effective included exclusive enteral nutrition (31/35, 88%), redo anastomosis (18/22, 82%), and alternate antibiotic treatment (37/64, 58%). CONCLUSIONS: Unfortunately, persistence of symptoms, failure to thrive, and abnormal laboratory tests at last follow-up in most of patients show the burden of DPAU lacking optimal therapy and incomplete understanding of the pathophysiology.

Enhanced recovery after surgical correction of adolescent idiopathic scoliosis.

BACKGROUND: Few publications in the literature examine enhanced recovery after scoliosis surgery (ERAS) in children, despite significant scientific interest in adults. The objective of the current study was to describe an ERAS protocol for surgical correction of adolescent idiopathic scoliosis (AIS) and its results. METHODS: ERAS outcomes were measured in two patient cohorts. Historical controls and ERAS groups were selected from patients managed for scoliosis surgery in 2015 and 2018, respectively. The ERAS protocol included fasting minimization, carbohydrate loading, the avoidance of background morphine infusions, perioperative opioid-sparing protocols, the use of a cooling brace, early physiotherapy, feeding and oral medications, and the early removal of urinary catheters and surgical drains. The main outcome of the study was hospital length of stay. RESULTS: Overall, 82 controls and 81 ERAS patients were recruited. ERAS protocols were observed in over 80% of patients for almost items. Median length of hospital stay was significantly lower in the ERAS group (- 3 [95% confidence interval: -2; -4] days). Median morphine consumption was reduced by 25% and 35% on days 2 and 3, respectively. The incidence of PONV did not differ between the two groups, and the incidence of constipation decreased slightly but significantly in the ERAS group on day 2. Pain intensity at rest and movement were lower in the ERAS group at day 2 and 3. CONCLUSIONS: The current study suggests an ERAS protocol after adolescent idiopathic scoliosis surgery is associated with reduced hospital length of stay and improved postoperative care.

Nod2 Protects the Gut From Experimental Colitis Spreading to Small Intestine.

BACKGROUND AND AIMS: Nucleotide oligomerization domain 2 [NOD2] mutations are key risk factors for Crohn's disease [CD]. NOD2 contributes to intestinal homeostasis by regulating innate and adaptive immunity together with intestinal epithelial function. However, the exact roles of NOD2 in CD and other NOD2-associated disorders remain poorly known. METHODS: We initially observed that NOD2 expression was increased in epithelial cells away from inflamed areas in CD patients. To explore this finding, Nod2 mRNA expression, inflammation, and cytokines expression were examined in the small bowel of wild-type [WT], Nod2 knockout and Nod2 mutant mice after rectal instillation of 2,4,6-trinitrobenzene sulphonic acid [TNBS]. RESULTS: In WT mice, Nod2 upregulation upstream to rectal injury was associated with pro-inflammatory cytokine expression but no overt histological inflammatory lesions. Conversely, in Nod2-deficient mice the inflammation spread from colitis to ileum and duodenum. CONCLUSIONS: Nod2 protects the gut from colitis spreading to small intestine.

Infant deaths in slings.

Although the incidence of sudden unexpected death in infancy (SUDI) decreased markedly after campaigns to promote supine positioning during sleeping, it has remained unchanged over the last decade. Epidemiological data suggest a role for new causes such as suffocation, asphyxia, and entrapment. Health authorities in several countries have issued warnings about slings used to carry infants. However, few reports of infant deaths in slings have been published in medical journals. Our paediatric intensive care unit has admitted two infants who experienced cardiorespiratory arrest while carried in a sling. Diagnostic investigations including a post-mortem examination established asphyxia as the mechanism of death. In conclusion, baby slings may carry a risk of SUDI, either by compression of the baby into a forward-flexed position or by direct suffocation. European recommendations for the cautious use of baby slings should be disseminated to families and professionals involved in caring for infants, as done recently in Australia, Canada, and the USA.

Prolonged enteral feeding is often required to avoid long-term nutritional and metabolic complications after esophagogastric dissociation.

BACKGROUND: Total esophagogastric dissociation (TED) was first described in 1997 by Bianchi as a new surgical procedure to treat severe gastroesophageal reflux disease (GERD) in children with neurological impairment. Recently, TED has been proposed in other conditions, such as esophageal atresia, esotracheal cleft, or caustic esophageal lesions. Although the long-term results in terms of GERD control have been previously reported, those regarding the nutritional and metabolic status have never been documented. PATIENTS AND METHODS: All patients without neurological impairment with TED between 1999 and 2004 at Robert Debre Hospital and Jeanne de Flandre Hospital, France, were prospectively investigated, paying particular attention to their metabolic and nutritional status (blood concentration of iron and vitamins A, D, E, and B12; lipid malabsorption; and hyperglycemia test) and growth. RESULTS: Seventeen children underwent TED. Six received primary procedures, whereas 11 were operated on because of severe respiratory diseases or failure to thrive. The mean follow-up was 6 years (range 3-8 years). Two children died (12%). Seven children were weaned from enteral nutrition support, but 5 of them had failure to thrive, steatorrhea, and/or malabsorption of vitamin B12 and/or fat-soluble vitamins. Eight patients had dumping syndrome, which was symptomatic in 6 cases. CONCLUSIONS: TED is an effective procedure for treatment of GERD. However, nutritional and metabolic complications including dumping syndrome and chronic digestive malabsorption are frequent after TED, especially after enteral nutrition weaning. A long-term follow-up of these patients is thus necessary and prolonged enteral nutrition support is recommended.

Nod2 regulates the host response towards microflora by modulating T cell function and epithelial permeability in mouse Peyer's patches.

Nucleotide oligomerisation domain 2 (NOD2) mutations are associated with susceptibility to Crohn's disease and graft-versus-host disease, two human disorders related with dysfunctions of Peyer's patches (PPs). In Nod2(-/-) mice transcellular permeability and bacterial translocation are increased in PPs. In this study, we show that both anti-CD4(+) and anti-interferon gamma (anti-IFNgamma) monoclonal antibodies abrogate this phenotype and reduce the expression of tumour necrosis factor (TNF) receptor 2 and the long isoform of myosin light chain kinase, thus demonstrating that immune T cells influence the epithelial functions. In turn, intraperitoneal injection of ML-7 (a myosin light chain kinase inhibitor) normalises the values of CD4(+) T cells, IFNgamma and TNFalpha. This reciprocal cross-talk is under the control of the gut microflora as shown by the normalisation of all parameters after antibiotic treatment. Toll-like receptor 2 (TLR2) and TLR4 expression were increased in Nod2(-/-) mice under basal conditions and TLR2 and TLR4 agonists induced an increased transcellular permeability in Nod2(+/+) mice. Muramyldipeptide (a Nod2 agonist) or ML-7 was able to reverse this phenomenon. It thus appears that Nod2 modulates the cross-talk between CD4(+) T cells and the epithelium recovering PP and that it downregulates the pro-inflammatory effect driven by the ileal microflora, likely by inhibiting the TLR pathways.

Hypertension in childhood cancer: a frequent complication of certain tumor sites.

UNLABELLED: The clinical features and management of severe hypertension (HT) (blood pressure > 99th percentile + 5 mm Hg) have been rarely described in pediatric oncology. OBJECTIVES: Retrospective descriptive study of the case files of 31 patients followed in the Institut Curie Department of Pediatric Oncology between 1999 and 2004 and presenting severe HT at the time of diagnosis of their tumor. RESULTS: The median age was 2 years 1 month (range: 3 mo to 6 y 8 mo). Median blood pressure was 99th percentile + 30 mm Hg (range: 99th percentile + 7 mm Hg to 99th percentile + 62 mm Hg). The tumors presented by these children were: Wilms tumor (n=17, ie, 20% of all Wilms tumors treated during this period), neuroblastoma (n=12, ie, 10% of all neuroblastomas treated during this period) or other tumors (n=2). HT was asymptomatic in all children. Initial management consisted of etiologic treatment by primary chemotherapy and/or surgical resection of the tumor, associated with antihypertensive therapy, initially administered by intravenous injection for 12 children (nicardipine, labetalol) and then orally in all children (calcium channel blockers, n=23; angiotensin-converting enzyme inhibitor, n=16; beta-blockers, n=4; alpha/beta-blockers, n=2; diuretics, n=1). Dual therapy was necessary in 7 cases and triple therapy was necessary in 1 case. The median duration of antihypertensive therapy was 40 days (range: 9 to 195). No child developed a serious complication of HT. CONCLUSIONS: Initial HT is a frequent complication of Wilms tumor and neuroblastoma and affects young children (< 2.5 y). It is often severe, asymptomatic, but needs specific treatment and resolves after treatment of the tumor.