Nutritional status at age 1 year in patients born with esophageal atresia: A population-based, prospective cohort study.

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.

Different Fecal Microbiota in Hirschsprung's Patients With and Without Associated Enterocolitis.

Background And Objectives: Patients with Hirschsprung's disease are at risk of developing Hirschsprung-associated enterocolitis, especially in the first 2 years of life. The pathophysiology of this inflammatory disease remains unclear, and intestinal dysbiosis has been proposed in the last decade. The primary objective of this study was to evaluate in a large cohort if Hirschsprung-associated enterocolitis was associated with alterations of fecal bacterial composition compared with HD without enterocolitis in different age groups.

Esophageal Atresia and Respiratory Morbidity.

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.

Risk Factors of Early Mortality and Morbidity in Esophageal Atresia with Distal Tracheoesophageal Fistula: A Population-Based Cohort Study.

Objective: To identify the risk factors for early mortality and morbidity in a population with distal esophageal atresia (EA)-tracheoesophageal fistula.

Study Design: Cohort study from a national register. Main outcomes and measures included early mortality, hospital length of stay (LoS), need for nutritional support at 1 year of age as a proxy measure of morbidity, and complications during the first year of life.

Analysis of enteric nervous system and intestinal epithelial barrier to predict complications in Hirschsprung's disease.

In Hirschsprung's disease (HSCR), postoperative course remains unpredictable. Our aim was to define predictive factors of the main postoperative complications: obstructive symptoms (OS) and Hirschsprung-associated enterocolitis (HAEC). In this prospective multicentre cohort study, samples of resected bowel were collected at time of surgery in 18 neonates with short-segment HSCR in tertiary care hospitals.

Management of lower urinary tract fibroepithelial polyps in children.

Introduction: Fibroepithelial polyps (FEP) of the lower urinary tract are relatively common in adults but rare in children, with fewer than 250 cases reported in the literature to date.

Objective: The aim of this study was to address the experience of FEP management in children.

Study Design: A retrospective multicenter review was undertaken in children with defined FEP of the lower urinary tract managed between 2008 and 2018.

One-Year Outcome for Congenital Diaphragmatic Hernia: Results From the French National Register.

Objective: To evaluate the status of congenital diaphragmatic hernia (CDH) management in France and to assess predictors of adverse outcomes.

Study Design: We reviewed the first-year outcome of all cases of CDH reported to the French National Register in 2011.

Results: A total of 158 cases were included.

What prenatal ultrasound features are predictable of complex or vanishing gastroschisis? A retrospective study.

Objective: To evaluate prenatal ultrasound parameters as prognostic factors for complex and vanishing gastroschisis.

Methods: Retrospective multicentre study of 200 gastroschisis over 13 years (2000-2013). Collection of prenatal ultrasound evaluation on maternal and fetal growth parameters, intra- and extra-abdominal bowel and stomach dilation, abdominal wall defect diameter and changes in bowel appearance.

DICER1 pleuropulmonary blastoma familial tumour predisposition syndrome: What the paediatric urologist needs to know.

Introduction: Germline-inactivating DICER1 mutations are responsible of a familial tumour susceptibility syndrome with an increased risk of tumours, mainly pleuropulmonary blastoma (PPB). DICER1 mutations also cause a range of other tumours, some of them in urogenital organs (cystic nephroma [CN], ovarian sex cord-stromal tumours, bladder and cervix embryonal rhabdomyosarcoma [ERMS]).

Objective: The aim was to clarify the range of urogenital phenotypes associated with DICER1 mutations and to give practical course of action to paediatric urologist that are exposed to DICER1-related conditions.

[Performance of prenatal diagnosis and postnatal development of congenital lung malformations].

Objectives: For many diseases, the comparison of prenatal diagnosis with a histopathological reality is not always possible. Fetal lung pathology, with its high rate of surgery in postnatal, allows this assessment. This study proposes an approach to the reliability of prenatal diagnosis and analysis of the postnatal development of all children in care for congenital pulmonary malformation (CPM).

Small left colon syndrome in 3 sisters.

Neonatal small left colon syndrome is a rare cause of bowel obstruction. Its etiology remains unknown, but a significant association has been noted between maternal diabetes and small left colon. No reported cases within the same family could be found in the literature, excepting 2 sets of twins.

Epidermal healing in burns: autologous keratinocyte transplantation as a standard procedure: update and perspective.

Background: Treatment of burned patients is a tricky clinical problem not only because of the extent of the physiologic abnormalities but also because of the limited area of normal skin available.

Methods: Literature indexed in the National Center (PubMed) has been reviewed using combinations of key words (burns, children, skin graft, tissue engineering, and keratinocyte grafts). Articles investigating the association between burns and graft therapeutic modalities have been considered.

Study of proliferation and 3D epidermal reconstruction from foreskin, auricular and trunk keratinocytes in children.

Objective: Severe burns in children are conventionally treated with split-thickness skin autografts or epidermal sheets. An alternative approach is to graft isolated keratinocytes. We evaluated foreskin and other anatomic sites as donor sources for autologous keratinocyte graft in children.

Unilateral pulmonary agenesis: a report of four cases, two diagnosed antenatally and literature review.

Pulmonary agenesis is a rare congenital malformation of lung development defined as complete absence of lung tissues, bronchi, and pulmonary vessels; it may be uni- or bilateral. The right-sided form carries the poorest prognosis due to severity of co-existent anomalies. Its diagnostic circumstances are variables: first reported cases were diagnosed at autopsy, but early postnatal as well as fortuitous discovery have been reported.

Esophageal atresia: data from a national cohort.

Purpose: A prospective national register was established in 2008 to record all new cases of live-birth newborns with esophageal atresia (EA). This epidemiological survey was recommended as part of a national rare diseases plan.

Methods: All 38 national centers treating EA participated by completing for each patient at first discharge a questionnaire validated by a national committee of experts.

Foreskin-isolated keratinocytes provide successful extemporaneous autologous paediatric skin grafts.

Severe burns in children are conventionally treated with split-thickness skin autografts or epidermal sheets. However, neither early complete healing nor quality of epithelialization is satisfactory. An alternative approach is to graft isolated keratinocytes.

Leydig cell tumour revealed by bilateral gynecomastia in a 15-year-old adolescent: a patient report.

Pubertal gynecomastia is common, and may affect up to 70% adolescents boys. Its course is spontaneously benign with resolution occurring by age 18 years. Although the exact physio-pathological mechanism remains unknown, it is thought to result from hormonal imbalance between estrogens and testosterone at puberty.

Infantile hypertrophic pyloric stenosis: are viruses involved?

Infantile hypertrophic pyloric stenosis (IHPS) is characterized by abnormal thickening of the internal circular muscle layer. IHPS is known to be due to a combination of genetic and environmental factors, but its precise causes and pathophysiology are poorly understood. The objective of the study is to determine the prevalence of the principal viruses targeting the respiratory and digestive tracts in children with IHPS.

[Pylephlebitis in the child: a challenging diagnosis].

Pylephlebitis or septic thrombophlebitis of the portomesenteric veins is a complication of intra-abdominal infections. The disease is rare in children and the diagnosis is often delayed. The morbidity of pylephlebitis is relatively low, although there is a risk of residual thrombosis.

Quantitative and qualitative study in keratinocytes from foreskin in children: Perspective application in paediatric burns.

Background: We performed a quantitative and qualitative evaluation of keratinocytes from foreskin in children.

Materials And Methods: We harvested 18 foreskins after circumcision. The mean average age of the operated children was 4 years.

Growth in infants in the first two years of life after neonatal repair for unilateral cleft lip and palate.

Objective: To evaluate the growth during the first two years of life in infants after unilateral cleft lip and palate neonatal repair.

Method: All mature infants with nonsyndromic unilateral cleft lip and palate (NSUCLP) born between 2004 and 2007 were included. Information concerning growth was collected.

[Cultured keratinocyte cells from foreskin and future application for burns in children].

Purpose Of The Study: We tested in vitro the keratinocytes capacity for division and differentiation. The donor site was the human foreskin.

Patients And Methods: For 12 months, we harvested 18 foreskins after circumcision.

[Cleft lip and palate: indications for radiological postnatal explorations].

Aim: We evaluated the utility of systematic neonatal radiological explorations in 20 instances of isolated cleft lip and palate in 20 cases.

Methods: This study included 13 infants with prenatal ultrasound diagnosis. The type of cleft and possible associated anomalies were noted.