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BACKGROUND: In patients with Alagille syndrome, cholestasis-associated clinical features can include high serum bile acids and severe pruritus that can necessitate liver transplantation. We aimed to evaluate the efficacy and safety of the ileal bile acid transporter inhibitor odevixibat versus placebo in patients with Alagille syndrome. METHODS: The ASSERT study was a phase 3, double-blind, randomised, placebo-controlled trial that enrolled patients at 21 medical centres or hospitals in ten countries (Belgium, France, Germany, Italy, Malaysia, the Netherlands, Poland, Türkiye, the UK, and the USA). Eligible patients had a genetically confirmed diagnosis of Alagille syndrome, a history of significant pruritus, and elevated serum bile acids. Patients were randomly assigned (2:1) to receive oral odevixibat 120 µg/kg per day or placebo for 24 weeks (in a block size of six and stratified by age: <10 years and ≥10 years to <18 years) via a web-based system. Patients, clinicians, study staff, and people analysing the data were masked to treatment allocation. The primary efficacy endpoint was change in caregiver-reported scratching score (on the PRUCISION instrument; range 0-4) from baseline to weeks 21-24. The prespecified key secondary efficacy endpoint was change in serum bile acid concentration from baseline to the average of weeks 20 and 24. Outcomes were analysed in patients who received at least one dose of study drug (the full analysis set for efficacy outcomes and the safety analysis set for safety outcomes). This trial is registered on ClinicalTrials.gov (NCT04674761) and EudraCT (2020-004011-28), and is completed. FINDINGS: Between Feb 26, 2021, and Sept 9, 2022, 52 patients were randomly assigned to receive odevixibat (n=35) or placebo (n=17), all of whom were included in the analysis sets. The median age was 5·5 years (IQR 3·2 to 8·9). 27 (52%) of 52 patients were male and 25 (48%) were female. The mean scratching score was elevated at baseline in both groups (2·8 [SD 0·5] for odevixibat vs 3·0 [0·6] for placebo). Mean scratching scores at weeks 21-24 were 1·1 (0·9) for odevixibat and 2·2 (1·0) for placebo, representing a least-squares (LS) mean change of -1·7 (95% CI -2·0 to -1·3) for odevixibat and -0·8 (-1·3 to -0·3) for placebo, which was significantly greater for odevixibat than for placebo (difference in LS mean change from baseline -0·9 [95% CI -1·4 to -0·3]; p=0·0024). Odevixibat also resulted in significantly greater reductions in mean serum bile acids from baseline versus placebo (237 µmol/L [SD 115] with odevixibat vs 246 µmol/L [121] with placebo) to the average of weeks 20 and 24 (149 µmol/L [102] vs 271 µmol/L [167]; LS mean change -90 µmol/L [95% CI -133 to -48] with odevixibat vs 22 µmol/L [-35 to 80] with placebo; difference in LS mean change -113 µmol/L [95% CI -179 to -47]; p=0·0012). The most common treatment-emergent adverse events were diarrhoea (ten [29%] of 35 patients in the odevixibat group vs one [6%] of 17 in the placebo group) and pyrexia (eight [23%] vs four [24%]). Seven patients had serious treatment-emergent adverse events during the treatment period: five (14%) in the odevixibat group and two (12%) in the placebo group. No patients discontinued treatment and there were no deaths. INTERPRETATION: Odevixibat could be an efficacious non-surgical intervention to improve pruritus, reduce serum bile acids, and enhance the standard of care in patients with Alagille syndrome. Longer-term safety and efficacy data of odevixibat in this population are awaited from the ongoing, open-label ASSERT-EXT study. FUNDING: Albireo Pharma, an Ipsen company.
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Síndrome de Alagille , Prurido , Humanos , Método Duplo-Cego , Síndrome de Alagille/tratamento farmacológico , Síndrome de Alagille/complicações , Masculino , Feminino , Criança , Adolescente , Prurido/tratamento farmacológico , Prurido/etiologia , Resultado do Tratamento , Ácidos e Sais Biliares/sangue , Adulto , Pré-Escolar , Adulto Jovem , Proteínas de Transporte , Glicoproteínas de Membrana , Metilaminas , TiazepinasRESUMO
BACKGROUND: Data on oral vancomycin for primary sclerosing cholangitis (PSC)-associated inflammatory bowel disease (IBD) are limited. AIMS: Using data from the Paediatric PSC Consortium, to examine the effect of vancomycin on IBD activity. METHODS: In this retrospective multi-centre cohort study, we matched vancomycin-treated and untreated patients (1:3) based on IBD duration at the time of primary outcome assessment. The primary outcome was Physician Global Assessment (PGA) of IBD clinical activity after 1 year (±6 months) of vancomycin. We used generalised estimating equations (GEE) to examine the association between vancomycin and PGA remission, adjusting for IBD type, severity and medication exposures. Secondary outcomes included serum labs and endoscopic remission (global rating of no activity) among those with available data and also analysed with GEE. RESULTS: 113 PSC-IBD patients received vancomycin (median age 12.7 years, 63% male). The matched cohort included 70 vancomycin-treated and 210 untreated patients. Vancomycin was associated with greater odds of IBD clinical remission (odds ratio [OR] 3.52, 95% CI 1.97-6.31; adjusted OR [aOR] 5.24, 95% CI 2.68-10.22). Benefit was maintained in sensitivity analyses restricted to non-transplanted patients and those with baseline moderate-severe PGA. Vancomycin was associated with increased odds of endoscopic remission (aOR 2.76, 95% CI 1.002-7.62; N = 101 with data), and with lower CRP (p = 0.03) and higher haemoglobin and albumin (both p < 0.01). CONCLUSION: Vancomycin was associated with greater odds of IBD clinical and endoscopic remission. Additional, preferably randomised, controlled studies are needed to characterise efficacy using objective markers of mucosal inflammation, and to examine safety and define optimal dosing.
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Antibacterianos , Colangite Esclerosante , Doenças Inflamatórias Intestinais , Vancomicina , Humanos , Vancomicina/administração & dosagem , Vancomicina/efeitos adversos , Colangite Esclerosante/tratamento farmacológico , Colangite Esclerosante/complicações , Feminino , Masculino , Estudos Retrospectivos , Criança , Adolescente , Antibacterianos/administração & dosagem , Antibacterianos/uso terapêutico , Antibacterianos/efeitos adversos , Doenças Inflamatórias Intestinais/tratamento farmacológico , Doenças Inflamatórias Intestinais/complicações , Administração Oral , Resultado do Tratamento , Índice de Gravidade de Doença , Indução de Remissão , Estudos de CoortesRESUMO
Gastroesophageal reflux disease (GERD) is frequent and prolonged in esophageal atresia (EA) pediatric patients requiring routine use of proton pump inhibitors (PPIs). However, there are still controversies on the prophylactic use of PPIs and the efficacy of PPIs on GERD and EA complications in this special condition. The aim of the study is to assess the prophylactic use of PPIs in pediatric patients with EA and its complications. We, therefore, performed a systematic review including all reports on the subject from 1980 to 2022. We conducted meta-analysis of the pooled proportion of PPI-and no PPI groups using random effect model, meta-regression, and estimate heterogeneity by heterogeneity index I2 . Thirty-eight reports on the topic met the criteria selection, representing a cumulative 6044 patients with EA. Prophylactic PPI prescription during the first year of life does not appear to prevent GERD persistence at follow-up and is not associated with a significantly reduced rate of antireflux surgical procedures (ARP). PPIs improve peptic esophagitis and induce remission of eosinophilic esophagitis at a rate of 50%. Their effect on other GERD outcomes is uncertain. Evidence suggests that PPIs do not prevent anastomotic stricture, Barrett's esophagus, or respiratory complications. PPI use in EA can improve peptic and eosinophilic esophagitis but is ineffective on the other EA complications. Side effects of PPIs in EA are almost unknown.
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Atresia Esofágica , Refluxo Gastroesofágico , Inibidores da Bomba de Prótons , Atresia Esofágica/cirurgia , Atresia Esofágica/complicações , Inibidores da Bomba de Prótons/uso terapêutico , Humanos , Refluxo Gastroesofágico/tratamento farmacológico , CriançaRESUMO
This paper presented a national register for esophageal atresia (EA) started in January 2008. We report our experience about the conception of this database and its coordination. Data management and data quality are also detailed. In 2023, more than 2,500 patients with EA are included. Prevalence of EA in France was calculated at 1.8/10,000 live birth. Main clinical results are listed with scientific publications issued directly from the register.
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Atresia Esofágica , Fístula Traqueoesofágica , Humanos , Atresia Esofágica/epidemiologia , Atresia Esofágica/cirurgia , Gerenciamento de Dados , Sistema de Registros , França/epidemiologia , Prevalência , Fístula Traqueoesofágica/epidemiologia , Fístula Traqueoesofágica/cirurgiaRESUMO
To assess the complications of one-step button percutaneous endoscopic gastrostomy (B-PEG) and determine risk factors for developing stomal infections or gastropexy complications. A retrospective study of 679 children who underwent a B-PEG procedure in a single tertiary care center over a 10-year period to December 2020 was conducted. Patient characteristics, early complications (occurring ≤ 7 days after the procedure), late complications (> 7 days after the procedure), and outcomes were collected from medical records. A list of potential risk factors, including age at procedure, prematurity, underlying neurological disease, and undernutrition, was determined a priori. At least 1 year of follow-up was available for 513 patients. Median follow-up duration was 2.8 years (interquartile range 1.0-4.9 years). Major complications were rare (< 2%), and no death was related to B-PEG. Early complications affected 15.9% of the study population, and 78.0% of children presented late complications. Development of granulation tissue was the most common complication followed in frequency by tube dislodgment and T-fastener complications. Only 24 patients (3.5%) presented stomal infections. Young age at the time of PEG placement (odds ratio (OR) 2.34 [1.03-5.30], p = .042) was a risk factor for developing peristomal infection. T-fastener migration occurred in 17.3% of children, and we found underlying neurological disease was a protective factor (OR 0.59 [0.37-0.92], p = .019). Conclusion: B-PEG is a safe method and associated with a low rate of local infection. However, T-fasteners are associated with significant morbidity and require particular attention in young and premature infants. What is Known: ⢠Percutaneous endoscopic gastrostomy (PEG) is the preferred method to provide long-term enteral nutrition in children to prevent malnutrition. The Pull-PEG method is still the most commonly used with complications , such as stomal infection. Since its description, only a few studies have reported postoperative complications of one-step button PEG (B-PEG). What is New: ⢠T-fastener complications were not rare, and underlying neurologic disease was a protective factor. A very low rate of stomal infection was described, and young age at the time of PEG placement was a risk factor. The B-PEG is a safe method with fewer major complications than P-PEG in children.
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Gastrostomia , Desnutrição , Lactente , Humanos , Criança , Gastrostomia/efeitos adversos , Estudos Retrospectivos , Nutrição Enteral/efeitos adversos , Nutrição Enteral/métodos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Fatores de Risco , Desnutrição/etiologiaRESUMO
Objectives: Malnutrition is common in children with biliary atresia (BA) awaiting liver transplantation (LT). Few studies have evaluated the effectiveness of enteral nutrition (EN) in these patients. The objective of this work was to assess the efficacy and tolerance of EN in children with BA awaiting LT. Methods: A total of 31 patients with BA followed between 1995 and 2018 were retrospectively included. Anthropometric indicators (weight, length, and head circumference) and adverse effects of EN were noted at the start (T0) and the end (T1) of EN. The z-scores for anthropometric indicators were compared between T0 and T1. Results: The median age at T0 was 7 months (interquartile range [IQR] 5-9), and the median duration of EN was 9 months (IQR 3-17). The z-scores for anthropometric variables improved from T0 to T1: -1.6 (IQR -2.5 to -1.0) to -0.5 (IQR -1.8 to 0.3) for median weight for age; -1.3 (IQR -2.4 to 0) to -0.4 (IQR -2.0 to 0.7) for length for age; -0.9 (IQR -2.3 to -0.3) to -0.3 (IQR -1.2 to 0.1) for weight for length; and -1.2 (IQR -2.1 to -0.6) to -0.2 (IQR -1.6 to 0.4) for body mass index (p < 0.05 for all comparisons). Nearly all (94%) of the patients had a weight-for-length z-score > -2 at the end of EN; 23% had adverse effects and 10% had complications leading to the cessation of EN. Conclusion: EN is effective and well tolerated in infants with BA awaiting LT.
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Tube feeding is a therapeutic intervention that is aimed at providing nutritional support and is important in the nutritional and gastrointestinal management of children with neurological disability (ND) worldwide. Since the publication of the first European Society of Gastroenterology, Hepatology, and Nutrition (ESPGHAN) consensus paper in 2017, some aspects of tube-feeding modalities have attracted the interest of the scientific community more than others, including the type of enteral formulas, enteral access, and the challenging practice of tube weaning. The purpose of this review was to report on the most recent hot topics and new directions in tube-feeding strategies for children with ND.
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Crianças com Deficiência , Gastroenterologia , Criança , Nutrição Enteral , Gastrostomia , Humanos , Intubação Gastrointestinal , Estado Nutricional , Apoio NutricionalRESUMO
Objective: Despite recent progress in caring for patients born with esophageal atresia (EA), undernutrition and stunting remain common. Our study objective was to assess nutritional status in the first year after birth with EA and to identify factors associated with growth failure. Study design: We conducted a population-based study of all infants born in France with EA between 2010 and 2016. Through the national EA register, we collected prenatal to 1 year follow-up data. We used body mass index and length-for-age ratio Z scores to define patients who were undernourished and stunted, respectively. Factors with P < 0.20 in univariate analyses were retained in a logistic regression model. Results: Among 1,154 patients born with EA, body mass index and length-for-age ratio Z scores at 1 year were available for about 61%. Among these, 15.2% were undernourished and 19% were stunted at the age of 1 year. There was no significant catch-up between ages 6 months and 1 year. Patients born preterm (41%), small for gestational age (17%), or with associated abnormalities (55%) were at higher risk of undernutrition and stunting at age 1 year (P < 0.05). Neither EA type nor surgical treatment was associated with growth failure. Conclusion: Undernutrition and stunting are common during the first year after birth in patients born with EA. These outcomes are significantly influenced by early factors, regardless of EA type or surgical management. Identifying high-risk patient groups with EA (i.e., those born preterm, small for gestational age, and/or with associated abnormalities) may guide early nutritional support strategies.
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OBJECTIVE: To compare the efficacy of, and complications from, the 2 main treatments for achalasia: endoscopic dilatation and surgical cardiomyotomy (Heller's myotomy). STUDY DESIGN: We retrospectively collected data on children treated for achalasia over an 11-year period from 8 tertiary pediatric centers. A line of treatment was defined as performing either Heller's myotomy or 1-3 sessions of endoscopy dilatation over 3 months. Treatment success was a priori defined as clinical improvement and no need for new treatment. RESULTS: Ninety-seven children (median age, 12 years; 57% boys) were included. The median time to diagnosis was 10.5 months, and the median follow-up period was 27 months. Thirty-seven children were treated by Heller's myotomy and 60 by endoscopy dilatation as the first-line treatment. After adjustment for potentially confounding factors, Heller's myotomy was significantly more successful than endoscopy dilatation (hazard ratio, 3.93 [1.74; 8.88]; P = .001), with a median survival without failure of 49 and 7 months, respectively, and with no significant difference in the occurrence of complications (35.2% for Heller's myotomy, 29.7% for endoscopy dilatation, P = .56). Hydrostatic dilatation was as successful as pneumatic dilatation (hazard ratio, 1.35 [0.56; 3.23]; P = .50). CONCLUSIONS: Heller's myotomy is more successful than endoscopy dilatation, with no significant difference in the occurrence of serious complications. This raises the potential role of peroral endoscopic myotomy as an alternative treatment to Heller's myotomy.
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Acalasia Esofágica , Miotomia de Heller , Criança , Masculino , Humanos , Feminino , Acalasia Esofágica/cirurgia , Dilatação , Estudos Retrospectivos , EndoscopiaRESUMO
Objectives: Although transient elastography (TE) is the primary noninvasive method for assessing liver fibrosis, its use remains to be validated in children. This study aims to evaluate the agreement between two-dimensional ultrasound shear wave elastography (2D-SWE) and TE to assess pediatric liver stiffness method. Methods: During the 18-month study, we prospectively included 101 consecutive children (median age: 8.5 years, range: 1 month to 17 years) who required TE for medical reasons, and in whom 2D-SWE measurement was performed within a 3-month follow-up during a routine ultrasound. Liver elasticity values were classified according to the Metavir score using published pediatric norms for TE and according to the manufacturer's reference values for 2D-SWE. The Spearman's correlation coefficient was used to assess the relationship between the elasticity measured by the two techniques. Concordance was described by the Bland-Altman method. Results: A strong correlation (rho = 0.70, p < 0.001) was found between 2D-SWE and TE for the elasticity measures. The strength of correlation was higher among patients older than 6 years (rho = 0.79, p < 0.001). Concordance between liver fibrosis stages assessed by these techniques was moderate [weighted kappa = 0.46, (95% CI: 0.35-0.57)]. When considering stages over F2, 2D-SWE diagnostic performances showed a sensitivity of 85% (95% CI: 74-92) and a specificity of 57% (95% CI: 42-70) compared with TE. Conclusion: Measurements of the liver stiffness using 2D-SWE and TE are strongly correlated. The moderate concordance between these techniques for assessing the liver fibrosis stage provides evidence against alternating between these methods during follow-up of patients with the chronic liver diseases.
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OBJECTIVES: To identify the risk factors for anastomotic, refractory and recurrent strictures and to establish whether anastomotic stricture is associated with antireflux surgery. DESIGN: This prospective national multicentre study included all infants born with oesophageal atresia (OA) over an 8-year period. Data on OA and complications were collected at birth and at 1 year old. Univariate and multivariate analyses were conducted. RESULTS: 1082 patients from 37 centres were included in the study. The prevalence of anastomotic stricture at 1 year old was 23.2%. Anastomosis under tension (defined by the surgeon at the time of repair) and delayed anastomosis (defined as anastomosis performed more than 15 days after birth, excluding delays due to prematurity or severe cardiac diseases) were found to be independent risk factors for anastomotic stricture (2.3 (1.42-3.74) and 4.02 (2.12-7.63), respectively). Patients with anastomotic stricture had a 2.3-fold higher rate of fundoplication compared with others (p=0.001). Anastomosis under tension and delayed anastomosis were found to be independent risk factors for recurrent stricture (1.92 (1.10-3.34) and 5.73 (2.71-12.14), respectively), while delayed anastomosis was the only risk factor for refractory stricture (8.30 (3.34-20.64)). There was a 2.39-fold (1.42-4.04) higher rate of fundoplication in the anastomotic stricture group than in the group without anastomotic stricture (p=0.001). CONCLUSIONS: Patient-related anatomical factors leading to anastomosis under tension and delayed anastomosis increase the risk of anastomotic stricture.
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Atresia Esofágica , Estenose Esofágica , Anastomose Cirúrgica/efeitos adversos , Constrição Patológica/complicações , Constrição Patológica/cirurgia , Atresia Esofágica/complicações , Estenose Esofágica/epidemiologia , Estenose Esofágica/etiologia , Humanos , Lactente , Recém-Nascido , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Prospectivos , Estudos RetrospectivosRESUMO
OBJECTIVE: To evaluate the feasibility of endoscopic gastrojejunal tube (GJT) placement in infants and children. STUDY DESIGN: All children undergoing endoscopic GJT placement between January 2010 and December 2019 were included in this single-center retrospective study. Difficulties with and failure of GJT placement, complication rates, and device longevity, efficacy, and duration were assessed. RESULTS: A total of 107 children, median age 10 months (IQR, 5.0-23.0 months) and median weight 6.6 kg (IQR, 5.3-9.5 kg), underwent endoscopic GJT placement using the gastric stoma to introduce the endoscope (one step: n = 36 of 107; 33.6%). Endoscopic placement was successful in 99%. Eight periprocedure complications occurred, including 1 pneumoperitoneum requiring exsufflation, 2 acute pulmonary hypertension episodes leading to death in 1 case, and 5 episodes of bronchospasm. Minor complications were frequent and mostly mechanical (79%), whereas major complications were rare (5.6%): intussusception (n = 4), intestinal perforation (n = 1), and pneumoperitoneum (n = 1). Ten patients died. Of the 97 patients who lived, 85 (87%) were weaned from jejunal feeding at a median of 179 days (IQR, 69-295 days) after initiation. Among them, 30 (35.2%) required fundoplication. Weight for age z-score was significantly higher at weaning. CONCLUSIONS: GJT placement is feasible in children, even low-weight infants. Complications are frequent but are mostly minor.
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Derivação Gástrica , Pneumoperitônio , Criança , Nutrição Enteral/métodos , Derivação Gástrica/métodos , Gastrostomia , Humanos , Lactente , Intubação Gastrointestinal/métodos , Pneumoperitônio/etiologia , Pneumoperitônio/cirurgia , Estudos RetrospectivosRESUMO
BACKGROUND: Given the high prevalence and complication risks of acid gastroesophageal reflux (GERD) in the first months of life in infants with esophageal atresia, the ESPGHAN/NASPGHAN consensus statement recommends systematic treatment with proton pump inhibitors (PPIs) until the age of 1 year and checking for acid GERD thereafter. However, these recommendations have not been evaluated. METHODS: This prospective study was conducted from 2007 to 2016. We evaluated the prevalence of acid GERD in 100 consecutive infants presenting with esophageal atresia/tracheoesophageal fistula after the age of 18 months when PPI treatment was stopped. The diagnosis of acid GERD was based on positive pH-metry and/or evidence of complications (e.g., peptic esophagitis, need for jejunal nutrition, or antireflux surgery). Those with acid GERD at a median age of 18 months received a control examination every year or adapted to their clinical situation. RESULTS: The prevalence rates of acid GERD were 64.3% at 18 months and 22.8% at the last follow-up (median age 65 months).There is no risk factor for acid GERD identified. CONCLUSIONS: This study shows a high prevalence of acid GERD in late infancy and supports the recommendation of systematic checking for acid GERD when treatment with PPI is stopped. IMPACT: Acid gastroesophageal reflux disease (GERD) is a frequent complication of esophageal atresia in infants. The ESPGHAN/NASPGHAN consensus, which is based on expert opinion, recommends systematic treatment of children with PPI until the age of 1 year. The prevalence rates of acid GERD were 64.3% at 18 months and 22.8% at the last follow-up. This study shows a high prevalence of acid GERD in late infancy and supports the recommendation of systematic checking for acid GERD when treatment with PPI is stopped.
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Atresia Esofágica , Refluxo Gastroesofágico , Fístula Traqueoesofágica , Criança , Pré-Escolar , Atresia Esofágica/complicações , Atresia Esofágica/epidemiologia , Refluxo Gastroesofágico/complicações , Refluxo Gastroesofágico/diagnóstico , Refluxo Gastroesofágico/tratamento farmacológico , Humanos , Lactente , Prevalência , Estudos Prospectivos , Inibidores da Bomba de Prótons/uso terapêutico , Fístula Traqueoesofágica/complicações , Fístula Traqueoesofágica/tratamento farmacológico , Fístula Traqueoesofágica/epidemiologiaRESUMO
BACKGROUND: No consensus currently exists on tube-weaning practice worldwide. The aim of this study was to describe tube-weaning practice (indications and modalities) of a panel of pediatric gastroenterologists involved in the management of prolonged enteral nutrition (EN). METHODS: A cross-sectional survey of pediatric gastroenterologists involved in the management of children receiving prolonged (>3 months) EN who were likely to be weaned was conducted. A questionnaire containing 24 questions was disseminated to the French Speaking Group of Paediatric Gastroenterology, Hepatology, and Nutrition members. The association between weaning practice and the experience of respondents was investigated. RESULTS: In total, 311 professionals were approached, and 64 questionnaires were completed (response rate 21%). Nearly all respondents (n = 61, 95%) reported using individualized weaning programs. Weaning attempts were performed more frequently at home, and follow-up was performed in the outpatient clinic (97%) by progressive EN volume reduction (83%). Inpatient weaning was performed by only 28% of participants. The choice of the setting was predominantly (59%) based on parental request. Management and follow-up involved mainly the referring pediatric gastroenterologist and the dietitian. Fifty-one (80%) of the respondents experienced ≥1 weaning failure. CONCLUSIONS: This is one of only a few studies examining real-life pediatric tube-weaning practices. Considerable practice variation between centers was observed; some of the common practices included the setting of weaning (outpatient vs inpatient) and the eligibility criteria. Increasing knowledge about weaning modalities is the starting point for the design of consensual standardized programs.
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Gastroenterologia , Criança , Estudos Transversais , Nutrição Enteral , Humanos , Inquéritos e Questionários , DesmameRESUMO
Introduction: Scoliosis is a well-described complication of esophageal atresia (EA) caused by the associated spine malformations and/or thoracotomy. However, the sagittal posture abnormalities in patients with EA have not been described. The aim of this study was to evaluate the prevalence of and risk factors for sagittal posture abnormalities at the age of 6 years in patients operated on for EA. Methods: A prospective cohort of 123 patients with EA was examined by the same rehabilitation doctor at the time of a multidisciplinary visit scheduled at the age of 6 years. Children presenting with scoliosis (n = 4) or who missed the consultation (n = 33) were excluded. Univariate and multivariate logistic regression models with Firth's penalized-likelihood approach were used to identify risk factors associated with sagittal posture anomalies. Candidate risk factors included neonatal characteristics, associated malformations, atresia type, postoperative complications, psychomotor development retardation, orthopedic abnormalities, and neurological hypotonia. Results: The prevalence rates of sagittal posture abnormalities were 25.6% (n = 22; 95% CI, 16.7-36.1%). Multivariate analysis showed that minor orthopedic abnormalities (OR: 4.02, 95% CI: 1.29-13.43, P = 0.021), and VACTERL (OR: 3.35, 95% CI: 1.09-10.71, P = 0.042) were significant risk factors for sagittal posture abnormalities. Conclusion: This study shows that sagittal posture anomalies occur frequently in children operated on at birth for EA and are not directly linked to the surgical repair. These children should be screened and treated using postural physiotherapy, especially those with VACTERL and minor orthopedic abnormalities.
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Objectives: Experience of hypnosis in gastrointestinal (GI) endoscopy is scarce in children. Our aims were to assess the rate of successful GI endoscopy performed using hypnosis alone or in combination with midazolam, with or without additional equimolar mixture of oxygen and nitrous oxide (EMONO), and to identify predictive factors of successful endoscopy in children. Methods: This prospective single-centre study included children older than 6 years requiring a diagnostic esophagogastroduodenoscopy (EGD) or rectosigmoidoscopy. Ericksonian hypnosis was performed alone or in combination with midazolam, with or without additional EMONO. Successful endoscopy was defined by a complete and well-tolerated procedure. Levels of satisfaction of the endoscopist, nurse, and patient were assessed. Results: One hundred forty children [70 boys, median age: 12 years (Q1-Q3: 9-14)] were included over a 14-month period. They underwent EGD in 51.4% (n = 72) and rectosigmoidoscopy in 48.6% (n = 68) of cases. EMONO and midazolam were combined with hypnosis in 136 cases (97.1%). Successful endoscopy rate reached 82.9%. The procedure was interrupted due to poor tolerance and was rescheduled under general anaesthesia in 11 patients (7.9%). Predictive factors for successful endoscopy were older age (13 vs. 8 years, OR: 1.34, CI 95% [1.10-1.62], p = 0.003) and type of endoscopy (EGD vs. rectosigmoidoscopy, OR: 16.34 [2.14-124.68], p = 0.007). A good cooperation of the patient was reported by the endoscopist and the nurse in 88.4 and 86.9% of cases, respectively. Ninety-two per cent of patients mentioned that the procedure went well. Conclusions: Our study suggests that hypnosis combined with EMONO and/or midazolam is of additional value to perform diagnostic EGD or rectosigmoidoscopy in children older than 6 years without systematic need for general anaesthesia.
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BACKGROUND AND OBJECTIVES: Respiratory diseases are common in children with esophageal atresia (EA), leading to increased morbidity and mortality in the first year. The primary study objective was to identify the factors associated with readmissions for respiratory causes in the first year in EA children. METHODS: A population-based study. We included all children born between 2008 and 2016 with available data and analyzed factors at birth and 1 year follow-up. Factors with a P value <.10 in univariate analyses were retained in logistic regression models. RESULTS: Among 1460 patients born with EA, 97 (7%) were deceased before the age of 1 year, and follow-up data were available for 1287 patients, who constituted our study population. EAs were Ladd classification type III or IV in 89%, preterm birth was observed in 38%, and associated malformations were observed in 52%. Collectively, 61% were readmitted after initial discharge in the first year, 31% for a respiratory cause. Among these, respiratory infections occurred in 64%, and 35% received a respiratory treatment. In logistic regression models, factors associated with readmission for a respiratory cause were recurrence of tracheoesophageal fistula, aortopexy, antireflux surgery, and tube feeding; factors associated with respiratory treatment were male sex and laryngeal cleft. CONCLUSIONS: Respiratory morbidity in the first year after EA repair is frequent, accounting for >50% of readmissions. Identifying high risk groups of EA patients (ie, those with chronic aspiration, anomalies of the respiratory tract, and need for tube feeding) may guide follow-up strategies.
Assuntos
Atresia Esofágica/epidemiologia , Readmissão do Paciente/estatística & dados numéricos , Transtornos Respiratórios/epidemiologia , Estudos de Coortes , Anormalidades Congênitas/epidemiologia , Nutrição Enteral , Feminino , Seguimentos , França/epidemiologia , Refluxo Gastroesofágico/epidemiologia , Humanos , Lactente , Recém-Nascido Pequeno para a Idade Gestacional , Masculino , Nascimento Prematuro , Sistema de Registros , Fístula Traqueoesofágica/epidemiologiaRESUMO
OBJECTIVES: The objective of this study was to assess the tolerance and efficacy of endoscopic intrapyloric botulinum toxin injection compared with pyloric dilatation in children with gastroparesis. METHODS: This was a retrospective descriptive multicentre study that included pediatric patients treated between 2010 and 2018 at 4 tertiary hospitals. RESULTS: Data were collected for 24 patients. The median age at diagnosis was 2.5âyears (range 0.5-4.7). A total of 46 endoscopic procedures were performed. The endoscopic procedure was multiple in 63% of patients. Among the interventions, 76% were successful and 15% were unsuccessful. The recurrence rate was 57% and the median time to recurrence was 3.7âmonths (0.1-73). The efficacy did not differ significantly between the 2 methods at the first intervention and as a second-line treatment. The recurrence rate also did not differ significantly between the 2 methods. No complications were reported. The median follow-up was 19.8âmonths (1.7-61.7). CONCLUSIONS: In this retrospective multicentre study, endoscopic management of gastroparesis by balloon dilatation or botulinum toxin was safe in children and seemed to be partially efficient within the first months. Symptoms recurred frequently and required repetition of the interventions.