Interpretation of the Juvenile Arthritis Disease Activity Score: responsiveness, clinically important differences and levels of disease activity in prospective cohorts of patients with juvenile idiopathic arthritis.

Objectives: The objectives of this study were to assess 27-joint Juvenile Arthritis Disease Activity Score (JADAS-27) responsiveness, JADAS-27 changes corresponding to clinically important differences and cut-off scores for low and high disease activity in a large prospective JIA cohort.

Methods: JADAS-27 responsiveness, using effect size and standardized response mean (SRM), and changes in the JADAS-27 corresponding to clinically important differences were determined for clinical improvement (ACRpedi30) and worsening (flare). To assess whether various degrees of change in the JADAS-27 could be used to demonstrate improvement or worsening in individual patients, diagnostic parameters were computed for cut-off score changes.

Changes in weight and BMI following the diagnosis of type 1 diabetes in children and adolescents.

The aim of this study was to study weight and body mass index (BMI) before, at, and after diagnosis of type 1 diabetes (T1D) and to identify factors associated with weight gain. Studied retrospectively were 209 children <18 years with T1D followed for 6 years. Data collected included clinical and laboratory data before diagnosis, at diagnosis, and during 6 years of follow-up.

Clinical outcome of ablation for long-standing persistent atrial fibrillation with or without defragmentation.

Objective: To assess the outcome and associated risks of atrial defragmentation for the treatment of long-standing persistent atrial fibrillation (LSP-AF).

Methods: Thirty-seven consecutive patients (60.4 ± 7.

Neurological injury after neonatal cardiac surgery: a randomized, controlled trial of 2 perfusion techniques.

Background: Complex neonatal cardiac surgery is associated with cerebral injury. In particular, aortic arch repair, requiring either deep hypothermic circulatory arrest (DHCA) or antegrade cerebral perfusion (ACP), entails a high risk of perioperative injury. It is unknown whether ACP results in less cerebral injury than DHCA.

On development of functional brain connectivity in the young brain.

Our brain is a complex network of structurally and functionally interconnected regions, shaped to efficiently process and integrate information. The development from a brain equipped with basic functionalities to an efficient network facilitating complex behavior starts during gestation and continues into adulthood. Resting-state functional MRI (rs-fMRI) enables the examination of developmental aspects of functional connectivity (FC) and functional brain networks.

[Pregnancy after isotretinoin use].

Background: Acne is a common problem that is increasingly being effectively treated with isotretinoin, a highly teratogenic agent. According to the pregnancy prevention program, contraception should be used until at least one month after discontinuing the medication.

Case Description: A 29-year-old woman became pregnant shortly after discontinuing isotretinoin.

Treated and untreated women with idiopathic precocious puberty: long-term follow-up and reproductive outcome between the third and fifth decades.

Context: Central precocious puberty (CPP), treated or untreated, may have implications in adulthood.

Objective: To assess the reproductive outcome and social adjustment of former CPP women between the 3rd and 5th decades of life.

Design: Cross-sectional study of an historical cohort.

Using the Internet-based upload blood glucose monitoring and therapy management system in patients with type 1 diabetes.

The aim of this study is to assess the impact of the internet-based upload blood glucose monitoring and therapy management system (Carelink(®)) in patients with type 1 diabetes. Diabetic patients treated with pump infusion for ≥3 months were prospectively randomized to use the CareLink(®) with (4 months) and without (4 months) diabetes-team initiated contact (n = 36, intervention group) or to continue standard care for 4 months and then transfer to the CareLink(®) without diabetes-team initiated contact (n = 34, control group). In the first 4 months, treatment was adjusted monthly by the same team in both groups.

Treatment of neonatal progressive ventricular dilatation: a single-centre experience.

Objective: To describe our experience with a cohort of 295 infants with progressive ventricular dilatation occurring in the antenatal or neonatal period.

Methods: A search was performed in our cranial ultrasound database. All records and images of infants in whom an imaging diagnosis of progressive ventricular dilatation had been made were retrieved.

Placental pathology in full-term infants with hypoxic-ischemic neonatal encephalopathy and association with magnetic resonance imaging pattern of brain injury.

Objective: To investigate the relationship between placental pathology and pattern of brain injury in full-term infants with neonatal encephalopathy after a presumed hypoxic-ischemic insult.

Study Design: The study group comprised full-term infants with neonatal encephalopathy subsequent to presumed hypoxia-ischemia with available placenta for analysis who underwent cerebral magnetic resonance imaging (MRI) within the first 15 days after birth. Macroscopic and microscopic characteristics of the placenta were assessed.

Reduced occipital fractional anisotropy on cerebral diffusion tensor imaging in preterm infants with postnatally acquired cytomegalovirus infection.

Background: Detection of white matter (WM) abnormalities on MRI is important regarding the neurodevelopmental outcome in preterm infants. The long-term neurodevelopmental outcome of preterm infants with postnatal cytomegalovirus (CMV) infection has not been studied extensively.

Objectives: We aimed to assess WM microstructure in preterm infants with postnatal CMV infection using diffusion tensor imaging.

Imaging the premature brain: ultrasound or MRI?

Neuroimaging of preterm infants has become part of routine clinical care, but the question is often raised on how often cranial ultrasound should be done and whether every high risk preterm infant should at least have one MRI during the neonatal period. An increasing number of centres perform an MRI either at discharge or around term equivalent age, and a few centres have access to a magnet in or adjacent to the neonatal intensive care unit and are doing sequential MRIs. In this review, we try to discuss when best to perform these two neuroimaging techniques and the additional information each technique may provide.

Agonists of MAS oncogene and angiotensin II type 2 receptors attenuate cardiopulmonary disease in rats with neonatal hyperoxia-induced lung injury.

Stimulation of MAS oncogene receptor (MAS) or angiotensin (Ang) receptor type 2 (AT2) may be novel therapeutic options for neonatal chronic lung disease (CLD) by counterbalancing the adverse effects of the potent vasoconstrictor angiotensin II, consisting of arterial hypertension (PAH)-induced right ventricular hypertrophy (RVH) and pulmonary inflammation. We determined the cardiopulmonary effects in neonatal rats with CLD of daily treatment during continuous exposure to 100% oxygen for 10 days with specific ligands for MAS [cyclic Ang-(1-7); 10-50 μg·kg(-1)·day(-1)] and AT2 [dKcAng-(1-7); 5-20 μg·kg(-1)·day(-1)]. Parameters investigated included lung and heart histopathology, fibrin deposition, vascular leakage, and differential mRNA expression in the lungs of key genes involved in the renin-angiotensin system, inflammation, coagulation, and alveolar development.

Evaluation of perinatal arterial ischemic stroke using noninvasive arterial spin labeling perfusion MRI.

Background: Arterial spin labeling (ASL) magnetic resonance imaging (MRI) can evaluate brain perfusion in neonates noninvasively. The aim of this study was to investigate whether ASL MRI can demonstrate perfusion abnormalities in neonates diagnosed with perinatal arterial ischemic stroke (PAIS).

Methods: Pulsed ASL perfusion MR images were acquired in the subacute stage (5-6 d after birth) and at follow-up (13 d to 16 wk after birth) in four PAIS patients.

Rapid optical imaging of human breast tumour xenografts using anti-HER2 VHHs site-directly conjugated to IRDye 800CW for image-guided surgery.

Purpose: Molecular optical imaging using monoclonal antibodies is slow with low tumour to background ratio. We used anti-HER2 VHHs conjugated to IRDye 800CW to investigate their potential as probes for rapid optical molecular imaging of HER2-positive tumours by the determination of tumour accumulation and tumour to background levels.

Methods: Three anti-HER2 VHHs (11A4, 18C3, 22G12) were selected with phage display and produced in Escherichia coli.

Cognitive outcome in childhood after unilateral perinatal brain injury.

Aim: The aim of the study was to assess cognitive outcome in children with periventricular haemorrhagic infarction (PVHI) or perinatal arterial ischaemic stroke (PAIS) and relate these findings to early developmental outcome and neonatal magnetic resonance imaging findings.

Method: A neuropsychological assessment was performed in 50 children (26 males, 24 females) with unilateral PVHI (n=21) or PAIS (n=29) at a median age of 11 years 9 months (range 6-20y). This included tests for intelligence, verbal memory, visual-motor integration, word comprehension, attention, reaction times, and executive function.

Factors associated with diabetic ketoacidosis at onset of Type 1 diabetes in children and adolescents.

Aims: To identify risk factors for diabetic ketoacidosis at diagnosis of Type 1 diabetes in children and adolescents.

Methods: In three time periods (1986-1987, 1996-1997 and 2006-2007) 75, 86 and 245 patients, respectively, aged < 20 years were newly diagnosed with Type 1 diabetes in one tertiary care centre. In this retrospective comparative study, data of clinical characteristics, laboratory evaluation at diagnosis, as well as demographic data were retrieved from the patients' files.

Cerebral oxygenation and brain activity after perinatal asphyxia: does hypothermia change their prognostic value?

Background: Hypothermia is an established therapy in term neonates to reduce death and disability after perinatal asphyxia. Near-infrared spectroscopy-monitored regional cerebral oxygen saturation (rScO2) and amplitude-integrated electroencephalogram (aEEG)-monitored background pattern have been shown to be early predictors of long-term neurodevelopmental outcome. The aim of this study was to investigate the prognostic value of rScO2 and aEEG for neurodevelopmental outcome in neonates with hypoxic-ischemic encephalopathy (HIE) treated with hypothermia.

Prediction of gross motor development and independent walking in infants born very preterm using the Test of Infant Motor Performance and the Alberta Infant Motor Scale.

Background: One objective of a neonatal follow-up program is to examine and predict gross motor outcome of infants born preterm.

Aims: To assess the concurrent validity of the Test of Infant Motor Performance (TIMP) and the Alberta Infant Motor Scale (AIMS), the ability to predict gross motor outcome around 15 months corrected age (CA), and to explore factors associated with the age of independent walking.

Methods: 95 infants, born at a gestational age <30 weeks, were assessed around 3, 6 and 15 months CA.

Hydrocortisone treatment for bronchopulmonary dysplasia and brain volumes in preterm infants.

Objective: To assess whether there was an adverse effect on brain growth after hydrocortisone (HC) treatment for bronchopulmonary dysplasia (BPD) in a large cohort of infants without dexamethasone exposure.

Study Design: Infants who received HC for BPD between 2005 and 2011 and underwent magnetic resonance imaging at term-equivalent age were included. Control infants born in Geneva (2005-2006) and Utrecht (2007-2011) were matched to the infants treated with HC according to segmentation method, sex, and gestational age.

Clinical course of asymmetric motor performance and deformational plagiocephaly in very preterm infants.

Objectives: To describe the clinical courses of positional preference and deformational plagiocephaly up to 6 months corrected age (CA) in infants born at gestational age <30 weeks or birth weight <1000 g, and to explore predictive factors for the persistence of these phenomena.

Study Design: A total of 120 infants were examined 3 times each. The presence of deformational plagiocephaly and a score of 0-6 on an asymmetry performance scale served as outcome measures at 6 months CA.

Neonatal neuroimaging predicts recruitment of contralesional corticospinal tracts following perinatal brain injury.

Aim: Unilateral perinatal brain injury may result in recruitment of ipsilateral projections originating in the unaffected hemisphere and development of unilateral spastic cerebral palsy (USCP). The aim of this study was to assess the predictive value of neonatal neuroimaging following perinatal brain injury for recruitment of ipsilateral corticospinal tracts.

Method: Neonatal magnetic resonance imaging (MRI) and cranial ultrasound scans of 37 children (20 males, 17 females; median [range] gestational age 36 wks(+4) [26(+6) -42wks(+5) ] and birthweight 2312 g ([770-5230g]) with unilateral perinatal arterial ischaemic stroke (n=23) or periventricular haemorrhagic infarction (n=14) were reviewed and scored for involvement of the corticospinal trajectory.