Fused high b-value diffusion weighted and T2-weighted MR images in staging of pediatric Hodgkin's lymphoma: A pilot study.

Purpose: To evaluate the value of fused high b-value diffusion weighted and T2-weighted MRI compared to T1-weighted imaging, T2-weighted imaging and DWI for staging pediatric Hodgkin's lymphoma.

Method: 21 consecutive pediatric patients who underwent whole-body MRI at Hodgkin's lymphoma staging were included. Fused, colorized DWI/T2-weighted images were created.

Pharmacokinetics and safety of tobramycin nebulization with the I-neb and PARI-LC Plus in children with cystic fibrosis: A randomized, crossover study.

Aims: We aimed to compare the pharmacokinetics (PK) and safety profile of tobramycin inhalation solution (TIS) using the I-neb device to the standard PARI-LC Plus nebulizer in children with cystic fibrosis.

Methods: A randomized, open-label, crossover study was performed. In 2 separate study visits, blood samples from 22 children were collected following TIS nebulization with I-neb (75 mg) and PARI-LC Plus (300 mg).

Validation of aortic valve 4D flow analysis and myocardial deformation by cardiovascular magnetic resonance in patients after the arterial switch operation.

Background: Aortic regurgitation (AR) and subclinical left ventricular (LV) dysfunction expressed by myocardial deformation imaging are common in patients with transposition of the great arteries after the arterial switch operation (ASO). Echocardiographic evaluation is often hampered by reduced acoustic window settings. Cardiovascular magnetic resonance (CMR) imaging provides a robust alternative as it allows for comprehensive assessment of degree of AR and LV function.

Imaging features of extranodal involvement in paediatric Hodgkin lymphoma.

Detecting extranodal disease in paediatric Hodgkin lymphoma is of great importance for both treatment and prognosis. Different imaging techniques can be used to identify these extranodal sites. This pictorial essay provides an overview of imaging features of extranodal disease manifestation in paediatric Hodgkin lymphoma.

Use of Biosimilars in Pediatric Inflammatory Bowel Disease: An Updated Position Statement of the Pediatric IBD Porto Group of ESPGHAN.

Biologic therapies have changed the outcome of both adult and pediatric patients with Inflammatory Bowel Disease (IBD). In September 2013, the first biosimilar of infliximab was introduced into the pharmaceutical market. In 2015, a first position paper on the use of biosimilars in pediatric IBD was published by the ESPGHAN IBD Porto group.

Clinical signs and symptoms in a joint model of four disease activity parameters in juvenile dermatomyositis: a prospective, longitudinal, multicenter cohort study.

Background: It is currently impossible to predict the prognosis of patients with juvenile dermatomyositis (JDM). The aim of this study was to find clinical features most strongly associated with outcome variables in JDM as a first step towards tailor-made treatment.

Methods: In a large, prospectively followed, multicenter cohort study of 340 patients with JDM, each contributing multiple visits, a Bayesian model of disease activity was developed, using the four continuous outcome variables creatine kinase (CK), childhood myositis assessment score (CMAS), manual muscle testing of 8 muscle groups (MMT8) and the physician's global assessment of disease activity (PGA).

An increase in myeloid cells after severe injury is associated with normal fracture healing: a retrospective study of 62 patients with a femoral fracture.

Background and purpose-Nonunion is common in femoral fractures. Previous studies suggested that the systemic immune response after trauma can interfere with fracture healing. Therefore, we investigated whether there is a relation between peripheral blood cell counts and healing of femur fractures.

Differences in infection prophylaxis measures between paediatric acute myeloid leukaemia study groups within the international Berlin-Frankfürt-Münster (I-BFM) study group.

Prevention of infections is of obvious relevance in paediatric patients with acute myeloid leukaemia (AML). However, recommendations are often non-specific and supported by low-quality evidence, resulting in divergent infection preventive regimens. Using a web-based survey, we investigated the infection prophylaxis guidelines of 22 paediatric AML study groups affiliated to the international Berlin-Frankfürt-Münster study group.

Management and consequences of postoperative fluctuations in plasma sodium concentration after pediatric brain tumor surgery in the sellar region: a national cohort analysis.

Purpose: Severe fluctuations in plasma sodium concentration and plasma osmolarity, including central diabetes insipidus (CDI), may have significant influence on postoperative morbidity and mortality after pediatric brain tumor surgery.The aim of this study was to describe the frequency, severity and neurological consequences of these fluctuations in pediatric brain tumor survivors.

Methods: A retrospective, multi-institutional chart review was conducted among all children who underwent brain tumor surgery in the sellar or suprasellar region in seven university hospitals in the Netherlands between January 2004 and December 2013.

A Multicenter Experience with Long-Acting Somatostatin Analogues in Patients with Congenital Hyperinsulinism.

Background/aims: Congenital hyperinsulinism (CHI) is a rare disease characterized by recurrent severe hypoglycemia. In the diffuse form of CHI, pharmacotherapy is the preferred choice of treatment. Long-acting somatostatin analogues have been used in children as off-label medication.

Predictors and Outcome of Pericardial Effusion After Hematopoietic Stem Cell Transplantation in Children.

Pericardial Effusion (PE) is a potentially life-threatening complication of Hematopoietic Cell Transplantation (HCT). Our study aim was to identify incidence, risk factors, response to treatment, and outcome of PE after pediatric HCT. All patients after HCT at our tertiary center between 2005 and 2010 were included.

Role of Microbial Modulation in Management of Atopic Dermatitis in Children.

The pathophysiology of atopic dermatitis (AD) is multifactorial and is a complex interrelationship between skin barrier, genetic predisposition, immunologic development, skin microbiome, environmental, nutritional, pharmacological, and psychological factors. Several microbial modulations of the intestinal microbiome with pre- and/or probiotics have been used in AD management, with different clinical out-come (both positive, as well as null findings). This review provides an overview of the clinical evidence from trials in children from 2008 to 2017, aiming to evaluate the effect of dietary interventions with pre- and/or pro-biotics for the treatment of AD.

Human neonatal thymectomy induces altered B-cell responses and autoreactivity.

An association between T-cell lymphopenia and autoimmunity has long been proposed, but it remains to be elucidated whether T-cell lymphopenia affects B-cell responses to autoantigens. Human neonatal thymectomy (Tx) results in a decrease in T-cell numbers and we used this model to study the development of autoreactivity. Two cohorts of neonatally thymectomized individuals were examined, a cohort of young (1-5 years post-Tx, n = 10-27) and older children (>10 years, n = 26), and compared to healthy age-matched controls.

Eating behavior during dexamethasone treatment in children with acute lymphoblastic leukemia.

Background And Aim: Large prospective studies on dexamethasone-induced changes in eating behavior, energy, and nutrient intake are lacking in pediatric acute lymphoblastic leukemia (ALL). We prospectively studied eating behavior, energy, nutrient intake, and the effect on leptin and adiponectin levels during dexamethasone administration in children with ALL.

Patients: Parents of patients with ALL (3-16 years) completed a dietary diary for their child during 4 days of dexamethasone (6 mg/m ) administration.

Hearing loss after platinum treatment is irreversible in noncranial irradiated childhood cancer survivors.

Cisplatin and carboplatin are effective antineoplastic agents. They are also considered to be potentially highly ototoxic. To date, no long-term follow-up data from well-documented cohorts with substantial numbers of childhood cancer survivors (CCS) with platinum-related hearing loss are available.

Hearing loss and speech perception in noise difficulties in Fanconi anemia.

Objectives/hypothesis: Fanconi anemia is a hereditary chromosomal instability disorder. Hearing loss and ear abnormalities are among the many manifestations reported in this disorder. In addition, Fanconi anemia patients often complain about hearing difficulties in situations with background noise (speech perception in noise difficulties).

Population pharmacokinetics of intravenous Erwinia asparaginase in pediatric acute lymphoblastic leukemia patients.

Erwinia asparaginase is an important component in the treatment of pediatric acute lymphoblastic leukemia. A large variability in serum concentrations has been observed after intravenous Erwinia asparaginase. Currently, Dutch Childhood Oncology Group protocols dose alterations are based on trough concentrations to ensure adequate asparaginase activity (≥100 IU/L).

Improved SNAPPE-II and CRIB II scores over a 15-year period.

Objective: During the last decades mortality and morbidity of preterm infants have declined in the Western world. We hypothesized that the decrease in mortality in preterm infants was associated with a decrease in illness severity scores (SNAPPE-II and CRIB II scores).

Study Design: Subjects were inborn infants born between January 1997 and December 1999 (period 1) and between January 2006 and December 2011 (period 2) with a gestational age of 26+0 through 28+6 weeks and without congenital malformations (n=394).

Developing a paediatric drug formulary for the Netherlands.

As many drugs in paediatrics are used off-label, prescribers face a lack of evidence-based dosing guidelines. A Dutch framework was developed to provide dosing guidelines based on best available evidence from registration data, investigator-initiated research, professional guidelines, clinical experience and consensus. This has clarified the scientific grounds of drug use for children and encouraged uniformity in prescribing habits in the Netherlands.

Whole-tumor apparent diffusion coefficient measurements in nephroblastoma: Can it identify blastemal predominance?

Purpose: To explore the potential relation between whole-tumor apparent diffusion coefficient (ADC) parameters in viable parts of tumor and histopathological findings in nephroblastoma.

Materials And Methods: Children (n = 52) with histopathologically proven nephroblastoma underwent diffusion-weighted magnetic resonance imaging (MRI) (1.5T) before preoperative chemotherapy.

Predicting the neurobehavioral side effects of dexamethasone in pediatric acute lymphoblastic leukemia.

Although dexamethasone is an effective treatment for acute lymphoblastic leukemia (ALL), it can induce a variety of serious neurobehavioral side effects. We hypothesized that these side effects are influenced by glucocorticoid sensitivity at the tissue level. We therefore prospectively studied whether we could predict the occurrence of these side effects using the very low-dose dexamethasone suppression test (DST) or by measuring trough levels of dexamethasone.

Acute Activation of Metabolic Syndrome Components in Pediatric Acute Lymphoblastic Leukemia Patients Treated with Dexamethasone.

Although dexamethasone is highly effective in the treatment of pediatric acute lymphoblastic leukemia (ALL), it can cause serious metabolic side effects. Because studies regarding the effects of dexamethasone are limited by their small scale, we prospectively studied the direct effects of treating pediatric ALL with dexamethasone administration with respect to activation of components of metabolic syndrome (MetS); in addition, we investigated whether these side effects were correlated with the level of dexamethasone. Fifty pediatric patients (3-16 years of age) with ALL were studied during a 5-day dexamethasone course during the maintenance phase of the Dutch Childhood Oncology Group ALL-10 and ALL-11 protocols.

Pneumococcal conjugate vaccine herd effects on non-invasive pneumococcal pneumonia in elderly.

Background: Herd protection from infant pneumococcal conjugate vaccination is well established for invasive pneumococcal disease (IPD) but not for non-IPD pneumococcal community-acquired pneumonia (PCAP). We assessed the contribution of vaccine-serotypes in non-IPD PCAP in adults 65 years and older in the period 2008-2013.

Methods: This is a post hoc analysis of two prospective studies from the Netherlands.

Hydrocortisone as an Intervention for Dexamethasone-Induced Adverse Effects in Pediatric Patients With Acute Lymphoblastic Leukemia: Results of a Double-Blind, Randomized Controlled Trial.

Purpose: Dexamethasone is a key component in the treatment of pediatric acute lymphoblastic leukemia (ALL), but can induce serious adverse effects. Recent studies have led to the hypothesis that neuropsychological adverse effects may be a result of cortisol depletion of the cerebral mineralocorticoid receptors. We examined whether including a physiologic dose of hydrocortisone in dexamethasone treatment can reduce neuropsychologic and metabolic adverse effects in children with ALL.

Mosaic CREBBP mutation causes overlapping clinical features of Rubinstein-Taybi and Filippi syndromes.

Rubinstein-Taybi syndrome (RTS, OMIM 180849) and Filippi syndrome (FLPIS, OMIM 272440) are both rare syndromes, with multiple congenital anomalies and intellectual deficit (MCA/ID). We present a patient with intellectual deficit, short stature, bilateral syndactyly of hands and feet, broad thumbs, ocular abnormalities, and dysmorphic facial features. These clinical features suggest both RTS and FLPIS.