Inborn errors in RNA polymerase III underlie severe varicella zoster virus infections.

Varicella zoster virus (VZV) typically causes chickenpox upon primary infection. In rare cases, VZV can give rise to life-threatening disease in otherwise healthy people, but the immunological basis for this remains unexplained. We report 4 cases of acute severe VZV infection affecting the central nervous system or the lungs in unrelated, otherwise healthy children who are heterozygous for rare missense mutations in POLR3A (one patient), POLR3C (one patient), or both (two patients).

Childhood obesity-related endothelial dysfunction: an update on pathophysiological mechanisms and diagnostic advancements.

Childhood obesity jeopardizes a healthy future for our society's children as it is associated with increased cardiovascular morbidity and mortality later on in life. Endothelial dysfunction, the first step in the development of atherosclerosis, is already present in obese children and may well represent a targetable risk factor. Technological advancements in recent years have facilitated noninvasive measurements of endothelial homeostasis in children.

Ferritin and body mass index predict cardiac dysfunction in female adolescents with anorexia of the restrictive type.

Background And Aim: Decreased left ventricular mass index in anorexia nervosa is amply reported. The aim of this study is to identify non-burdensome predictors of reduced left yentricular mass/height (cLVM) in a cohort of adolescent restrictive anorexic girls.

Methods: This is a retrospective study of all anorexic girls of the restrictive type referred to our tertiary eating disorder unit between September 2002 and December 2012, for somatic assessment of weig ht loss.

The role of Th17 and Treg responses in the pathogenesis of RSV infection.

The respiratory syncytial virus (RSV) represents the leading cause of viral bronchiolitis and pneumonia in children worldwide and is associated with high morbidity, hospitalization rate, and significant mortality rates. The immune response elicited by RSV is one of the main factors contributing to the pathogenesis of the disease. Two subsets of the cellular immune response, the T helper 17 cell (Th17) and the regulatory T-cell (Treg), and more particularly the balance between these two subsets, might play a significant role in the pathogenesis of the RSV infection.

Diet, exercise, and endothelial function in obese adolescents.

Background And Objectives: Endothelial dysfunction is the first, although reversible, sign of atherosclerosis and is present in obese adolescents. The primary end point of this study was to investigate the influence of a multicomponent treatment on microvascular function. Additional objectives and end points were a reduced BMI SD score, improvements in body composition, exercise capacity, and cardiovascular risk factors, an increase in endothelial progenitor cells (EPCs), and a decrease in endothelial microparticles (EMPs).

Endothelial progenitor cells and endothelial microparticles are independent predictors of endothelial function.

Objective: To examine the degree of microvascular endothelial dysfunction in relation to classical cardiovascular risk factors, arterial stiffness, and numbers of circulating endothelial progenitor cells (EPCs) and endothelial microparticles (EMPs), in obese and normal-weight children.

Study Design: Cross-sectional study with 57 obese (15.2±1.

A spinal arteriovenous fistula in a 3-year old boy.

We present a case of a 3-year-old boy with neurodegeneration. Family history reveals Rendu-Osler-Weber disease. Magnetic resonance imaging (MRI) of the spinal cord and spinal angiography showed a spinal arteriovenous fistula with venous aneurysm, causing compression of the lumbar spinal cord.

Influence of frequent infectious exposures on general and varicella-zoster virus-specific immune responses in pediatricians.

Reexposure to viruses is assumed to strengthen humoral and cellular immunity via the secondary immune response. We studied the effects of frequent exposure to viral infectious challenges on immunity. Furthermore, we assessed whether repetitive exposures to varicella-zoster virus (VZV) elicited persistently high immune responses.

Sleep-disordered breathing and C-reactive protein in obese children and adolescents.

Purpose: Sleep-disordered breathing (SDB) is common among overweight and obese children. It is a risk factor for several health complications, including cardiovascular disease. Inflammatory processes leading to endothelial dysfunction are a possible mechanism linking SDB and cardiovascular disease.

Methodological considerations and practical recommendations for the application of peripheral arterial tonometry in children and adolescents.

Endothelial dysfunction is recognized as the primum movens in the development of atherosclerosis. Its crucial role in both cardiovascular morbidity and mortality has been confirmed. In the past, research was hampered by the invasive character of endothelial function assessment.

Assessment of endothelial dysfunction in childhood obesity and clinical use.

The association of obesity with noncommunicable diseases, such as cardiovascular complications and diabetes, is considered a major threat to the management of health care worldwide. Epidemiological findings show that childhood obesity is rapidly rising in Western society, as well as in developing countries. This pandemic is not without consequences and can affect the risk of future cardiovascular disease in these children.

Functional respiratory imaging as a tool to assess upper airway patency in children with obstructive sleep apnea.

Objective: We aim to investigate if anatomical and functional properties of the upper airway using computerized 3D models derived from computed tomography (CT) scans better predict obstructive sleep apnea (OSA) severity than standard clinical markers.

Methods: Consecutive children with suspected OSA underwent polysomnography, clinical assessment of upper airway patency, and a CT scan while awake. A three-dimensional (3D) reconstruction of the pharyngeal airway was built from these images, and computational fluid dynamics modeling of low inspiratory flow was performed using open-source software.

Metabolic disregulation in obese adolescents with sleep-disordered breathing before and after weight loss.

Objective: Sleep-disordered breathing (SDB) is prevalent in obesity. Weight loss is one of the most effective treatment options. The aim was to assess the association of SDB and metabolic disruption before and after weight loss.

Erythema ab igne in an adolescent with anorexia nervosa.

Objective: A rare skin lesion, erythema ab igne (EAI) is presented, in an adolescent female with anorexia nervosa.

Method: Clinical records of this patient were compared with a pubmed search about EAI in patients with an eating disorder.

Results: The patient presented with localized, spider-like, erythematous and hyperpigmented skin lesions on the lower abdomen and on both thighs.

Legislation and refusal of blood transfusion by a minor Jehovah-Witness in Belgium.

The refusal of blood transfusion by Jehovah's Witnesses in critical situations constitutes an ethical and juridical dilemma. The refusal to receive blood products by Jehovah's Witnesses is based on biblical verses. Recurring arguments to sustain this refusal regard the right to self-determination and the right to freedom of faith.

The health and economic burden of chickenpox and herpes zoster in Belgium.

Varicella-zoster virus causes chickenpox (CP) and after reactivation herpes zoster (HZ). Vaccines are available against both diseases warranting an assessment of the pre-vaccination burden of disease. We collected data from relevant Belgian databases and performed five surveys of CP and HZ patients.

Weight loss and sleep-disordered breathing in childhood obesity: effects on inflammation and uric acid.

Sleep-disordered breathing (SDB) is prevalent in childhood obesity. It may be an independent risk factor for the metabolic syndrome. Possible mechanisms are inflammation and oxidative stress.

Visceral leishmaniasis in a child infected with the human immunodeficiency virus in a non-endemic region.

We reported the case of a boy who fled from Chechnya to Belgium. He was diagnosed with a human immune deficiency virus (HIV)/Visceral leishmaniasis (VL) coinfection. In both countries, the prevalence of HIV-infected children is low and VL is not endemic.

A solid pseudopapillary tumour of the pancreas presenting after an abdominal trauma.

We present the case of 12-year-old girl who was referred with persistent abdominal pain and a palpable mass after blunt trauma and whose final diagnosis was a solid pseudopapillary tumour of the pancreas. This is the second case in our hospital of a solid pseudopapillary tumour of the pancreas presenting after a blunt abdominal trauma. Solid pseudopapillary tumour of the pancreas is a rare neoplasm.