Schooling experiences in children with long-gap esophageal atresia compared with children with esophageal atresia and primary anastomosis: a Swedish study.

BACKGROUND: Children with long-gap esophageal atresia (LGEA) risk living with aerodigestive morbidity and mental health difficulties. No previous study has investigated their experiences of schooling, despite the importance of schools in children's development, learning and social relationships. We aimed to describe experiences of schooling in children with LGEA in Sweden in comparison with children with EA who had primary anastomosis. METHOD: Children with LGEA aged 3-17 were recruited nationwide in Sweden. One parent completed a survey on their child's school-based supports (according to definitions from the Swedish National Agency for Education), school absence, school satisfaction, school functioning (PedsQL 4.0), mental health (Strength and Difficulties Questionnaire) and current symptomatology. School data were compared between 26 children with LGEA to that from 95 children with EA who had PA, a hypothesized milder affected group. Mental health level was determined using validated norms; abnormal ≥ 90 percentile. Data were analyzed using descriptives, correlation and Mann-Whitney-U test. Significance level was p < 0.05. RESULTS: Formal school-based support was reported in 17 (65.4%) children with LGEA and concerned support with nutritional intake (60%), education (50%) and medical/special health needs (35%). The prevalence of school-based support was significantly higher compared to children with PA overall (36.8%, p = 0.013) and regarding nutritional intake support (20%, p < 0.001). In children with LGEA, school-based support was related to low birth weight (p = 0.036), young child age (p = 0.014), height ≤ -2SD for age/sex (p = 0.024) and an increased number of aerodigestive symptoms (p < 0.05). All children with LGEA who had abnormal mental health scores had school-based support, except for one child. Nine children with LGEA (36%) had school absence ≥ 1times/month the past year, more frequently because of colds/airway infections (p = 0.045) and GI-specific problems compared to PA (p = 0.003). School functioning scores were not significantly different from children with PA (p = 0.34) but correlated negatively with school-based support (< 0.001) and school absence (p = 0.002). One parent out of 26 reported their child's school satisfaction as "not good". CONCLUSIONS: Children with LGEA commonly receive school-based support, reflecting multifaceted daily needs and disease severity. School absence is frequent and related to poorer school functioning. Future research focusing on academic achievement in children with EA is needed.

Regulation of bile acid metabolism in biliary atresia: reduction of FGF19 by Kasai portoenterostomy and possible relation to early outcome.

BACKGROUND: Fibroblast growth factor 19 (FGF19) is produced in the small intestine and is involved in suppression of hepatic bile acid (BA) synthesis. FGF19 is also expressed in the liver and serum levels are elevated in adults with cholestatic liver disease. This may reflect a rescue mechanism to dampen liver injury caused by increased intrahepatic BAs. OBJECTIVES: To examine circulating FGF19 at early stages of biliary atresia and at short-term follow-up post-Kasai portoenterostomy (KPE) in relation to noncholestatic infants. The relationship between FGF19, BAs and markers for BA synthesis and hepatic gene expression of factors involved in BA metabolism were also evaluated. METHODS: Liver tissue, portal and peripheral blood samples were obtained from fifteen patients at KPE; additional blood was collected 4-6 months after surgery. Two control groups were included; to examine possible changes related to surgery and to compare FGF19 in biliary atresia to noncholestatic infants. RESULTS: Circulating FGF19 levels correlated to its hepatic gene expression at time of KPE in biliary atresia and levels were elevated compared to noncholestatic infants. At follow-up, FGF19 levels were markedly reduced, and the decline coincided with reductions in bilirubin and conjugated chenodeoxycholic acid and with increased levels of the BA synthesis marker C4. CONCLUSION: Elevated circulating FGF19 in biliary atresia is of hepatic origin and reduced following KPE. Changes in serum FGF19 may reflect the level of restoration of the enterohepatic circulation, and this warrants further long-term studies on the role of FGF19 in the cholestatic liver.

Population-based cohort study on the epidemiology of acute appendicitis in children in Sweden in 1987-2013.

BACKGROUND: The long-term incidence of acute appendicitis has been reported to be declining in Europe and North America. Recent reports, however, indicate stabilized or even increased rates. The aim of this study was to investigate the present epidemiology of acute appendicitis and appendicectomy in a population-based cohort of Swedish children. METHODS: The Swedish National Patient Register was queried for all children with acute appendicitis and/or appendicectomy in 1987-2013. Population-based incidence rates were calculated. Rates were age- and sex-adjusted, and analysed for temporal and regional trends. RESULTS: Some 56 774 children with acute appendicitis were identified, of whom 53 478 (94·2 per cent) underwent appendicectomy. The incidence rate of acute appendicitis declined by 43·7 per cent over 26 years, from 177·7 to 100·1 per 100 000 person-years between 1987 and 2013. The most significant reduction was for non-perforated appendicitis, from 138·5 to 68·4 per 100 000 person-years between 1987 and 2009. The incidence rate of perforated appendicitis decreased from 28·0 to 19·9 per 100 000 person-years and negative appendicectomies reduced from 48·5 to 3·6 per 100 000 person-years during the study interval. CONCLUSION: The incidence rates of acute appendicitis and negative appendicectomy have reduced markedly in Swedish children over time, with significantly different trends amongst non-perforated appendicitis and perforated appendicitis. The full explanation for the observed findings is unclear.

A review of conservative treatment of acute appendicitis.

Appendicitis is a common condition in the pediatric population and appendectomy has been the traditional treatment. Both the urgency of the operation and the need for the appendectomy have recently been challenged. In children, this controversy focuses on operative management of perforated appendicitis and appendix abscesses. In adults, the debate has extended to management of nonperforated appendicitis. This review describes the evidence behind these challenges and updates a per-protocol meta-analysis of randomized controlled trials in adults. In the per-protocol meta-analysis, there was no difference between operative versus nonoperative management in failure of treatment. The complication rate was significantly lower in patients treated nonoperatively. However, in the nonoperative group, 10% of patients needed immediate surgery and 17% developed a recurrence during the 1-year follow-up. Overall, 73% of adults with suspected acute appendicitis may not need operative treatment. There are no data in the literature to support nonoperative treatment of acute appendicitis in children.