Stress hyperglycaemia as a result of a catecholamine producing tumour in an infant.

Hyperglycaemia commonly occurs in children presenting at the emergency department. In the absence of diabetic symptoms, this stress-related hyperglycaemia is considered a benign condition. We present a malignant cause of hyperglycaemia in an 11-month-old girl with concomitant symptoms of a neuroendocrine malignancy.

Determining the genome-wide kinship coefficient seems unhelpful in distinguishing consanguineous couples with a high versus low risk for adverse reproductive outcome.

Background: Offspring of consanguineous couples are at increased risk of congenital disorders. The risk increases as parents are more closely related. Individuals that have the same degree of relatedness according to their pedigree, show variable genomic kinship coefficients.

Diabetes IN develOpment (DINO): the bio-psychosocial, family functioning and parental well-being of youth with type 1 diabetes: a longitudinal cohort study design.

Background: Strict glycemic control during adolescence decreases the risk of developing complications later in life, even if this level of control is not maintained afterwards. However, the majority of adolescents with type 1 diabetes (T1D) are in poor control and so far medical or psychological interventions have shown limited success. Adolescence is characterized by major biological, psychosocial, cognitive and parent-child relationship changes and the complex interaction between these developmental trajectories, and its impact on health outcomes is still poorly understood.

Pediatric IBD-unclassified Is Less Common than Previously Reported; Results of an 8-Year Audit of the EUROKIDS Registry.

Background: Inflammatory bowel disease-unclassified (IBD-U) is diagnosed in ∼10% of pediatric and adolescent onset IBD patients. The EUROKIDS registry (2004) initiated by the Porto IBD working group of ESPGHAN prospectively monitors diagnostic workup of newly diagnosed pediatric and adolescent onset IBD patients. We aimed to describe diagnostic workup, phenotype, and change of diagnosis over time in pediatric IBD-U patients.

Long-term quality of life and functional outcome of patients with juvenile idiopathic arthritis in the biologic era: a longitudinal follow-up study in the Dutch Arthritis and Biologicals in Children Register.

Objective: To carry out a longitudinal investigation of functional outcome, health-related quality of life (HRQoL) and treatment strategies in JIA patients who started etanercept >5 years ago.

Methods: We approached patients whose HRQoL changes were described previously in a subanalysis of the Dutch Arthritis and Biologicals in Children register. Recent disease status, co-morbidities and structural damage were retrieved.

Complete Remodeling After Conservative Treatment of a Severely Angulated Odontoid Fracture in a Patient With Osteogenesis Imperfecta: A Case Report.

Study Design: Case report.

Objective: This is the first case report describing successful healing and remodeling of a traumatic odontoid fracture that was dislocated and severely angulated in a patient with osteogenesis imperfecta who was treated conservatively.

Summary Of Background Data: Osteogenesis imperfecta (OI) is a rare genetic disorder resulting in a low bone mass and bone fragility, predisposing these patients to fractures that often occur at a young age.

A Girl with Disseminated Thoracic Echinococcosis.

We present an unusual case of extensively disseminated pulmonary and thoracic echinococcosis in a 10-year-old girl. Thoracic echinococcosis should be considered in any child from an endemic country with cystic lesions on chest imaging. Confirmation of the diagnosis can be difficult and is important before surgery to reduce the risk of further dissemination and anaphylaxis.

RYR1-related myopathies: a wide spectrum of phenotypes throughout life.

Background And Purpose: Although several recent studies have implicated RYR1 mutations as a common cause of various myopathies and the malignant hyperthermia susceptibility (MHS) trait, many of these studies have been limited to certain age groups, confined geographical regions or specific conditions. The aim of the present study was to investigate the full spectrum of RYR1-related disorders throughout life and to use this knowledge to increase vigilance concerning malignant hyperthermia.

Methods: A retrospective cohort study was performed on the clinical, genetic and histopathological features of all paediatric and adult patients in whom an RYR1 mutation was detected in a national referral centre for both malignant hyperthermia and inherited myopathies (2008-2012).

Bone mineral density in children and adolescents with Prader-Willi syndrome: a longitudinal study during puberty and 9 years of growth hormone treatment.

Context: Longitudinal data on bone mineral density (BMD) in children and adolescents with Prader-Willi Syndrome (PWS) during long-term GH treatment are not available.

Objective: This study aimed to determine effects of long-term GH treatment and puberty on BMD of total body (BMDTB), lumbar spine (BMDLS), and bone mineral apparent density of the lumbar spine (BMADLS) in children with PWS.

Design And Setting: This was a prospective longitudinal study of a Dutch PWS cohort.

Methotrexate for maintaining remission in paediatric Crohn's patients with prior failure or intolerance to thiopurines: a multicenter cohort study.

Background And Aims: Methotrexate [MTX] is an immunomodulating drug that can be used to maintain remission in patients with Crohn's disease [CD], but data on efficacy and tolerability in children and teenagers are scarce. We evaluated the long-term efficacy and tolerability of MTX monotherapy after thiopurine therapy in paediatric CD patients.

Methods: A multicenter cohort of paediatric MTX users who stopped thiopurines due to ineffectiveness or intolerance between 2002 and 2012 were included and followed for at least 12 months.

The incidence of type 1 diabetes is still increasing in the Netherlands, but has stabilised in children under five (Young DUDEs-1).

Aim: This study described the incidence and prevalence of type 1 diabetes in children in the Netherlands in 2010-2011 and to compare these results with earlier studies.

Methods: This was a retrospective nationwide cohort study of Dutch children aged 14 years or younger. Patients were identified using health insurance reimbursement registries for hospital care and invoices for insulin.

New insights into factors influencing adult height in short SGA children: Results of a large multicentre growth hormone trial.

Background: Growth hormone (GH) treatment is effective in improving adult height (AH) in short children born SGA. However, there is a wide variation in height gain, even after adjustment for predictive variables. It is therefore important to investigate new factors which can influence the response to GH.

Iron deficiency in the first 6 months of age in infants born between 32 and 37 weeks of gestational age.

Background/objectives: Preterm infants are at risk of iron deficiency (ID). In the Netherlands, preterm infants born after 32 weeks of gestational age (GA) do not receive iron supplementation on a routine basis. We hypothesized that dietary iron intake in these infants might not be sufficient to meet the high iron requirements during the first 6 months of life.

Serum hepcidin measured by immunochemical and mass-spectrometric methods and their correlation with iron status indicators in healthy children aged 0.5-3 y.

Background: The diagnostic use of hepcidin is limited by the absence of standardization and lack of age-specific reference ranges in children in particular. The aim of this study was to determine reference ranges of serum hepcidin in healthy children aged 0.5-3 y using mass spectometry (MS) and a commercial immunochemical (IC) assay, and to investigate its association with other indicators of iron status and inflammation.

Validation of the Rome III criteria and alarm symptoms for recurrent abdominal pain in children.

Objectives: Rome criteria were formulated to define functional gastrointestinal disorders (Rome III criteria, 2006) excluding organic diagnoses when alarm symptoms were absent. The aims of the study were to validate the Rome III criteria as to their capacity to differentiate between organic and functional abdominal pain and to assess the role of alarm symptoms in this differentiation.

Methods: During 2 years all of the patients (ages 4-16 years) presenting with recurrent abdominal pain (Apley criteria) and referred to secondary care were included.

A 'picturesque' case of transition from subcutaneous to oral treatment in neonatal diabetes.

We report a case of a 6-week-old infant with diabetes mellitus based on a genetic defect in the sulfonylurea receptor 1 (SUR1), an ATP-sensitive potassium (KATP) channel protein. A spectacular improvement in glucose regulation was shown by real-time continuous glucose monitoring when switching her from insulin to oral glibenclamide. Children with neonatal onset of diabetes deserve genetic testing in order to replace insulin with oral medication.

The value of Ret-Hb and sTfR in the diagnosis of iron depletion in healthy, young children.

Objectives: Reticulocyte hemoglobin (Ret-Hb) content and soluble transferrin receptor (sTfR) are described as promising biomarkers in the analysis of iron status. However, the value of Ret-Hb and sTfR in the early detection of iron depletion, as frequently observed in children in high-income countries, is unclear. We hypothesized that young children to iron depletion, using the WHO cutoff of ferritin <12 μg/l, would have lower Ret-Hb and higher sTfR concentrations compared to children with a ferritin ⩾level 12 μg/l.

The value of soluble transferrin receptor and hepcidin in the assessment of iron status in children with cystic fibrosis.

Background: The value of ferritin in the diagnosis of iron deficiency is limited in patients with CF since it increases in the presence of inflammation. We hypothesized that the soluble transferrin receptor (sTfR) and hepcidin may provide more information than ferritin in assessing iron status in children with CF.

Methods: We analyzed sTfR and hepcidin in relation to conventional iron status indicators in 49 children with CF.

Trends in prescription of biological agents and outcomes of juvenile idiopathic arthritis: results of the Dutch national Arthritis and Biologics in Children Register.

Background: Treatment of juvenile idiopathic arthritis (JIA) has changed dramatically since the introduction of biological agents in 1999.

Objective: To evaluate trends in prescription patterns of biological agents and the subsequent outcome of JIA.

Methods: The Arthritis and Biologics in Children register (multicentre prospective observational study) aimed to include all consecutive patients with JIA in the Netherlands who had started biological agents since 1999.

Improving the Manchester Triage System for pediatric emergency care: an international multicenter study.

Objectives: This multicenter study examines the performance of the Manchester Triage System (MTS) after changing discriminators, and with the addition use of abnormal vital sign in patients presenting to pediatric emergency departments (EDs).

Design: International multicenter study.

Settings: EDs of two hospitals in The Netherlands (2006-2009), one in Portugal (November-December 2010), and one in UK (June-November 2010).

Occult constipation: faecal retention as a cause of recurrent abdominal pain in children.

Unlabelled: Recurrent abdominal pain (RAP) in children is generally believed to be functional. In practice, many children with RAP become pain-free with laxative therapy. The aims of the study were to establish the role of (occult) constipation in RAP and to investigate whether patients diagnosed with (occult) constipation could be identified by history and physical examination.

The influences of factors associated with decreased iron supply to the fetus during pregnancy on iron status in healthy children aged 0.5 to 3 years.

Objective: To investigate whether maternal anemia, pregnancy-induced diabetes, hypertension and smoking contributed to the recently found high prevalence of iron deficiency in a population of otherwise healthy children.

Study Design: Iron status was assessed in 400 children aged 0.5 to 3 years.

Mechanical ventilation drives inflammation in severe viral bronchiolitis.

Introduction: Respiratory insufficiency due to severe respiratory syncytial virus (RSV) infection is the most frequent cause of paediatric intensive care unit admission in infants during the winter season. Previous studies have shown increased levels of inflammatory mediators in airways of mechanically ventilated children compared to spontaneous breathing children with viral bronchiolitis. In this prospective observational multi-center study we aimed to investigate whether this increase was related to disease severity or caused by mechanical ventilation.