Novel Pressure-Impedance Parameters for Evaluating Esophageal Function in Pediatric Achalasia.

Objective: In achalasia, absent peristalsis and reduced esophagogastric junction (EGJ) relaxation and compliance underlie dysphagia symptoms. Novel high-resolution impedance manometry variables, that is, bolus presence time (BPT) and trans-EGJ-bolus flow time (BFT) have been developed to estimate the duration of EGJ opening and trans-EGJ bolus flow. The aim of this study was to evaluate esophageal motor function and bolus flow in children diagnosed with achalasia using these variables.

Intra- and interrater reliability of the Chicago Classification of achalasia subtypes in pediatric high-resolution esophageal manometry (HRM) recordings.

Background: Subtyping achalasia by high-resolution manometry (HRM) is clinically relevant as response to therapy and prognosis have shown to vary accordingly. The aim of this study was to assess inter- and intrarater reliability of diagnosing achalasia and achalasia subtyping in children using the Chicago Classification (CC) V3.0.

Probiotics for the Prevention of Nosocomial Diarrhea in Children.

This document provides recommendations developed by the ESPGHAN Working Group on Probiotics and Prebiotics on the role of probiotics in the prevention of nosocomial diarrhea in children based on a systematic review of previously completed systematic reviews and of subsequently published randomized controlled trials. The quality of evidence was assessed using the Grading of Recommendations Assessment, Development and Evaluation guidelines. Recommendations were given only if at least 2 randomized controlled trials examined the same probiotic strain.

Survival in children requiring chronic renal replacement therapy.

Survival in the pediatric end-stage renal disease (ESRD) population has improved substantially over recent decades. Nonetheless, mortality remains at least 30 times higher than that of healthy peers. Patient survival is multifactorial and dependent on various patient and treatment characteristics and degree of economic welfare of the country in which a patient is treated.

Systematic review of nonoperative versus operative treatment of uncomplicated appendicitis.

Background: To compare the risk of complications between initial nonoperative treatment and appendectomy of uncomplicated (simple) appendicitis in children.

Methods: Systematic literature search. Eligible for inclusion were both and randomized controlled trials and cohort studies including children in which the outcome of nonoperative treatment of uncomplicated appendicitis was reported with a minimum follow-up period of one year.

Serological markers for human intestinal ischemia: A systematic review.

Early and accurate diagnosis of intestinal ischemia is important in order to provide rapid and correct treatment and reduce morbidity and mortality rates. Clinical signs and symptoms are often unspecific. This systemic review sums up literature regarding human plasma biomarkers for acute mesenteric ischemia reported during the last ten years.

Development of the Brussels Infant and Toddler Stool Scale ('BITSS'): protocol of the study.

Introduction: The Bristol Stool Form Scale (BSS) which consists of 7 photographs of different stool forms allows assessment of stool consistency (scale 1 for hard lumps to scale 7 for watery stools), in an objective manner in adults. The BSS is also sometimes used to characterise the stools of infants and young children. Despite its use, there is general agreement among paediatric gastroenterologists that the BSS is not adequate to be used in infants and young children who wear diapers; thus, a new scale specifically designed for this population is needed.

Plasma levels of plasminogen activator inhibitor-1 and bleeding phenotype in patients with von Willebrand disease.

Introduction: von Willebrand disease (VWD) is the most common inherited bleeding disorder. In VWD patients, large variations in bleeding tendency are observed, which cannot be completely explained by the variation in von Willebrand factor levels or activities. Thus, there must be additional factors, for instance, changes in fibrinolysis that have an effect on the variation in bleeding tendency in VWD patients.

Unexpected findings after surgery for suspected appendicitis rarely change treatment in pediatric patients; Results from a cohort study.

Background: To determine if non-operative treatment is safe in children with acute appendicitis, we evaluated the incidence of unexpected findings after an appendectomy in children, and the influence they have on subsequent treatment.

Methods: A historical cohort study (January 2004-December 2014) was performed including children, aged 0-17 years, who underwent an appendectomy for the suspicion of acute appendicitis. Patients were divided based upon histopathological examination.

Anterior rectus sheath blocks in children with abdominal wall pain due to anterior cutaneous nerve entrapment syndrome: a prospective case series of 85 children.

Background: Chronic abdominal pain in children may be caused by the anterior cutaneous nerve entrapment syndrome. Local nerve blocks are recommended as an initial treatment in adults. Evidence on effectiveness and safety of such a treatment in children is lacking.

The Dynamic Compression Brace for Pectus Carinatum: Intermediate Results in 286 Patients.

Background: Dynamic brace compression is a novel treatment for patients with pectus carinatum. The dynamic compression system contains a device to measure the flexibility of the thoracic wall and regulate the pressure of the brace.

Methods: Patients referred to our pediatric surgical center were screened for treatment with the dynamic compression brace.

Health-related quality of life of children on treatment for acute lymphoblastic leukemia: A systematic review.

Children with acute lymphoblastic leukemia (ALL) undergo intense anticancer treatment. We systematically reviewed 22 studies evaluating 2,073 ALL patients' health-related quality of life (HRQL) and its clinical/demographic correlates during treatment. Overall HRQL was significantly reduced on treatment.

A comparison of three treatment strategies in recent onset non-systemic Juvenile Idiopathic Arthritis: initial 3-months results of the BeSt for Kids-study.

Background: Combination therapy with prednisone or etanercept may induce earlier and/or more improvement in disease activity in Disease Modifying Anti Rheumatic Drug (DMARD) naïve non-systemic Juvenile Idiopathic Arthritis (JIA) patients. Here we present three months clinical outcome of initial treatments of the BeSt-for-Kids study.

Methods: Included patients were randomized to either: 1.

Systemic corticosteroid regimens for prevention of bronchopulmonary dysplasia in preterm infants.

Background: Cochrane systematic reviews show that systemic postnatal corticosteroids reduce the risk of bronchopulmonary dysplasia (BPD) in preterm infants. However, corticosteroids have also been associated with an increased risk of neurodevelopmental impairment. It is unknown whether these beneficial and adverse effects are modulated by differences in corticosteroid treatment regimens.

Real-Life Outcomes for Patients with Asthma Prescribed Spacers for Use with Either Extrafine- or Fine-Particle Inhaled Corticosteroids.

Background: Spacers are often used with pressurized metered-dose inhalers (pMDIs) to eliminate the need for coordinating inhalation with actuation.

Objective: To investigate the real-life effectiveness of spacers prescribed for use with either extrafine- or fine-particle inhaled corticosteroids (ICSs).

Methods: This historical matched cohort study examined anonymous medical record data over 2 years (1-year baseline, 1-year outcome) for patients with asthma aged 12 to 80 years initiating ICSs by pMDI with or without prescribed spacer.

Disease-associated mutations identify a novel region in human STING necessary for the control of type I interferon signaling.

Background: Gain-of-function mutations in transmembrane protein 173 (TMEM173) encoding stimulator of interferon genes (STING) underlie a recently described type I interferonopathy called STING-associated vasculopathy with onset in infancy (SAVI).

Objectives: We sought to define the molecular and cellular pathology relating to 3 individuals variably exhibiting the core features of the SAVI phenotype including systemic inflammation, destructive skin lesions, and interstitial lung disease.

Methods: Genetic analysis, conformational studies, in vitro assays and ex vivo flow-cytometry were performed.

Objectively diagnosing rumination syndrome in children using esophageal pH-impedance and manometry.

Background: Rumination syndrome is characterized by recurrent regurgitation of recently ingested food into the mouth. Differentiation with other diagnoses and gastroesophageal reflux disease (GERD) in particular, is difficult. Recently, objective pH-impedance (pH-MII) and manometry criteria were proposed for adults.

Pneumovirus-Induced Lung Disease in Mice Is Independent of Neutrophil-Driven Inflammation.

The human pneumovirus respiratory syncytial virus (RSV) is the most common pathogen causing lower respiratory tract disease in young children worldwide. A hallmark of severe human RSV infection is the strong neutrophil recruitment to the airways and lungs. Massive neutrophil activation has been proven detrimental in numerous diseases, yet in RSV the contribution of neutrophils to disease severity, and thereby, the relevance of targeting them, is largely unknown.

Infants Requiring Maintenance Dialysis: Outcomes of Hemodialysis and Peritoneal Dialysis.

Background: The impact of different dialysis modalities on clinical outcomes has not been explored in young infants with chronic kidney failure.

Study Design: Cohort study.

Setting & Participants: Data were extracted from the ESPN/ERA-EDTA Registry.

Changes in Biochemical Parameters of the Calcium-Phosphorus Homeostasis in Relation to Nutritional Intake in Very-Low-Birth-Weight Infants.

Preterm infants are at significant risk to develop reduced bone mineralization based on inadequate supply of calcium and phosphorus (Ca-P). Biochemical parameters can be used to evaluate the nutritional intake. The direct effect of nutritional intake on changes in biochemical parameters has not been studied.

The early postnatal nutritional intake of preterm infants affected neurodevelopmental outcomes differently in boys and girls at 24 months.

Aim: This study assessed whether increased amino acid and energy intake in preterm infants during the first week of life was associated with improved neurodevelopment at the corrected age (CA) of 24 months.

Methods: We evaluated preterm infants from two consecutive cohorts in 2004 (Cohort 1) and 2005 (Cohort 2) with different nutritional intakes in the Netherlands. Nutritional intake and growth were recorded until week 5 and after discharge.

Timing of renal replacement therapy does not influence survival and growth in children with congenital nephrotic syndrome caused by mutations in NPHS1: data from the ESPN/ERA-EDTA Registry.

Background: Congenital nephrotic syndrome (CNS) of the Finnish type, NPHS1, is the most severe form of CNS. Outcomes of renal replacement therapy (RRT) in NPHS1 patients in Europe were analysed using data from the ESPN/ERA-EDTA Registry. As NPHS1 is most prevalent in Finland and the therapeutic approach differs from that in many other countries, we compared outcomes in Finnish and other European patients.

Add-on LABA in a separate inhaler as asthma step-up therapy increased dose of ICS or ICS/LABA combination inhaler.

Asthma management guidelines recommend adding a long-acting β-agonist (LABA) or increasing the dose of inhaled corticosteroid (ICS) as step-up therapy for patients with uncontrolled asthma on ICS monotherapy. However, it is uncertain which option works best, which ICS particle size is most effective, and whether LABA should be administered by separate or combination inhalers. This historical, matched cohort study compared asthma-related outcomes for patients (aged 12-80 years) prescribed step-up therapy as a ≥50% extrafine ICS dose increase or add-on LABA, either a separate inhaler or a fine-particle ICS/LABA fixed-dose combination (FDC) inhaler.

Pediatric low-grade gliomas: implications of the biologic era.

For the past decade, it has been recognized that pediatric low-grade gliomas (LGGs) and glial-neuronal tumors carry distinct molecular alterations with resultant aberrant intracellular signaling in the Ras-mitogen-activated protein kinase pathway. The conclusions and recommendations of a consensus conference of how best to integrate the growing body of molecular genetic information into tumor classifications and, more importantly, for future treatment of pediatric LGGs are summarized here. There is uniform agreement that molecular characterization must be incorporated into classification and is increasingly critical for appropriate management.

Reflux monitoring in children.

Recently, multichannel intraluminal impedance (MII) monitoring was added to the repertoire of tests to evaluate the (patho)physiology of gastroesophageal reflux (GER) in children. Its advantage above the sole monitoring of the esophageal pH lies in the ability of the detection of both acid and nonacid GER and to discern between liquid and gas GER. Currently, combined 24 h pH-MII monitoring is recommended for evaluation of gastro-esophageal reflux disease (GERD) and its relation to symptoms in infants and children, despite the lack of reference values in these age groups.