[Severe hypoglycemia in children and adolescents with type 1 diabetes: risks factors and management].

Hypoglycemia is one of the most common acute complications in the treatment of type 1 diabetes. It is the result of a mismatch between insulin dose, food consumed, and recent exercise. Hypoglycemia occurs more frequently in younger children and with lower HbA1c levels.

[Biological variation of glycation and mean blood glucose have greater influence on Hba1c levels in type 1 young diabetic patients than glucose instability].

The aim of the study was to assess the relative influence of mean blood glucose (MBG), glucose instability (GI) and biological variation of glycohemoglobin (BVG) on HbA1c. The study included 378 unselected young type 1 diabetic patients with a diabetes duration > 1 year. There were 1,409 visits with simultaneous HbA1c determinations and self-monitoring of BG meter downloads.

[Management of type 1 diabetes (insulin, diet, sport): "Dorchy's recipes"].

The principal aims of therapeutic management of the child, adolescent and adult with type 1 diabetes are to allow good quality of life and to avoid long-term complications by maintaining blood glucose concentrations close to the normal range and an HbA1c level under 7%. The number of daily insulin injections, 2 or > or = 4, by itself does not necessarily give better results, but the 4-injection regimen allows greater freedom, taking into account that the proper insulin adjustment is difficult before adolescence. Successful glycemic control in young patients depends mainly on the quality and intensity of diabetes education.

[(R)evolution in pediatric diabetology].

Before the discovery of insulin 87 years ago, all diabetic children died within a few weeks or months following diagnosis. Since then, improvements in the treatment and live of young diabetics have sometimes occurred in (r)evolutions that have caused debate among physicians. They are briefly reviewed in this paper.

[Pediatric diabetology at the University Children's Hospital Queen Fabiola in Brussels is 40 years old].

By the end of medical school at the Free University of Brussels (ULB) in 1969, I began my specialization in pediatrics. Immediately, my mentor, Professor Helmut Loeb led me into pediatric diabetes which was non-existent in Belgium. Forty years later, the diabetes clinic for children and adolescents at the University Children's Hospital Queen Fabiola in Brussels has the largest number of young patients in Belgium, social medical activities and clinical research, with the best protective glycated hemoglobin levels (proven in international comparisons from Hvidøre Study Group on Childhood Diabetes) in relation to potentially invalidating complications in the short and long term.

Effect of ankle-foot orthoses on gait in typically developing children: Developmental trend in segmental coordination.

Objective: As orthoses, and particularly ankle-foot orthoses, are widely used in the management of children with motor disorders, including cerebral palsy, we aimed to study their effect in normal children in order to add to normative gait data, which are essential for diagnosing, understanding and treating abnormal gait patterns.

Design: We analyzed the effect of ankle-foot orthoses on classical gait parameters and lower limb segments coordination patterns in typically developing children in two age groups reflecting different neuromaturational/developmental situations. We recorded 3D kinematic gait patterns in 9 children (4-5 years) and 11 children (9-10 years) walking barefoot or wearing bilateral solid ankle-foot orthoses maintaining the ankle joint angle at a neutral position.

Angelman syndrome: current understanding and research prospects.

Angelman syndrome is a neurogenetic disorder characterized by developmental delay, severe intellectual disability, absent speech, exuberant behavior with happy demeanor, motor impairment, and epilepsy, due to deficient UBE3A gene expression that may be caused by various abnormalities of chromosome 15. Recent findings in animal models demonstrated altered dendritic spine formation as well as both synaptic [including gamma-aminobutyric acid (GABA)(A) and N-methyl-D-aspartate (NMDA) transmission] and nonsynaptic (including gap junction) influences in various brain regions, including hippocampus and cerebellar cortex. Reversal of selected abnormalities in rescue genetically engineered animal models is encouraging, although it should not be misinterpreted as promising "cure" for affected patients.

Ventricular septal defect closure in Taussig-Bing heart: the "pulmonic rule".

Accurate ventricular septal defect patch sizing and tailoring remain challenging in many surgical procedures. Surgical exposure frequently limits complete visualization of the ventricular septal defect. Moreover, examination of the heart cavity under cardioplegic arrest may lead to skewed appreciation of the ventricular septal defect caliber and shape.

Gender differences in inflammatory markers in children.

No clear explanation exists to understand how sex hormones and/or chromosomes affect the immune system. In vitro studies of human lymphoid cells also show sex differences in immune function. To evaluate these differences in frequent pediatric emergencies, we analyze the expression of inflammatory markers (C-reactive protein, erythrocyte sedimentation rate, and neutrophil count) underlying inflammatory processes in children: 482 children (241 girls and 241 boys) hospitalized for pneumonia (n = 384), pyelonephritis (n = 39), or bronchiolitis (n = 59) matched for age and sex.

Consensus on the appropriate use of intrathecal baclofen (ITB) therapy in paediatric spasticity.

Among features of motor disorders in children, spasticity is associated with considerable morbidity and problems in care, particularly in severely affected patients. Intrathecal baclofen (ITB) has been increasingly used as a relatively specific treatment modality for spasticity. To date, most of the evidence for its use in paediatric patients has come from retrospective and uncontrolled studies, although randomised, controlled trials of screening ITB and ITB therapy itself have recently been published.

Association of parathyroid adenoma and familial hypocalciuric hypercalcaemia in a teenager.

Objective: Familial hypocalciuric hypercalcaemia (FHH) is clinically characterized by mild to moderate parathyroid hormone (PTH)-dependent hypercalcaemia, autosomal dominant pattern of inheritance, and normal to frankly reduced urinary calcium excretion in spite of a high serum calcium (clearance (Ca)/clearance (Cr)<0.01). FHH has a benign course and should be differentiated from primary hyperparathyroidism.

Microbiology of otitis media: a moving target.

The microbiology of acute otitis media (AOM) is linked to the nasopharyngeal commensal flora. This respiratory ecosystem undergoes various selective pressures, such as antibiotic consumption and vaccine use. Socio-economic conditions also influence the bacterial composition of the nasopharynx.

Gianotti-Crosti syndrome following hepatitis A vaccination.

We report a case of Gianotti-Crosti syndrome in a child following hepatitis A vaccination in order to insist that currently available hepatitis A vaccines are highly immunogenic and that Gianotti-Crosti syndrome is a possible minor adverse reaction.

Kytococcus schroeteri infection of a ventriculoperitoneal shunt in a child.

Kytococcus schroeteri is a newly described micrococcal species and, to date, has been associated mostly with endocarditis. Six infections attributable to this opportunistic pathogen have been described since 2002, when the first case was identified. We describe here the first pediatric case of a K.

Behavior and neuropsychiatric manifestations in Angelman syndrome.

Angelman syndrome has been suggested as a disease model of neurogenetic developmental condition with a specific behavioral phenotype. It is due to lack of expression of the UBE3A gene, an imprinted gene located on chromosome 15q. Here we review the main features of this phenotype, characterized by happy demeanor with prominent smiling, poorly specific laughing and general exuberance, associated with hypermotor behavior, stereotypies, and reduced behavioral adaptive skills despite proactive social contact.

Genetic forms of nephrotic syndrome: a single-center experience in Brussels.

The aim of the study was to present our experience in treating children with genetic forms of nephrotic syndrome and diagnosing these diseases. We retrospectively reviewed the clinical data, mutational analyses, histopathological features, treatment modalities, and outcome of 26 consecutive children (20 families) suffering from congenital and/or steroid-resistant nephrotic syndrome who were assessed by genetic analysis. Ten out of 26 children (38%) had congenital nephrotic syndrome, 4/26 (15%) had infantile nephrotic syndrome, 10/26 (38%) had late-onset nephrotic syndrome, and 2/26 (9%) had asymptomatic proteinuria.

[Pediatric nephrology from the fetal kidney to the kidney graft].

The department of pediatric uro-nephrology was created in 1977 in Brugmann hospital. Since then, various sectors have been developed including: hemodialysis and peritoneal dialysis, kidney transplantation, urological and genital surgery, antenatal screening and rapid management of uronephropathies, treatment of voiding dysfunction and neurogenic bladder, management of tubular and glomerular diseases. The progress in genetics, medical imaging, obstetrics, neonatology and surgery has allowed us to take care of our young patients within a multidisciplinary framework.

Corneal opacities in the Hallermann-Streiff syndrome.

We present six patients with typical Hallermann-Streiff syndrome. All have microphthalmia and were operated for congenital cataract. Three of the patients developed a severe glaucoma and one patient presented repeated uveal effusions.

A de novo SOX10 mutation causing severe type 4 Waardenburg syndrome without Hirschsprung disease.

Type 4 Waardenburg syndrome represents a well define entity caused by neural crest derivatives anomalies (melanocytes, intrinsic ganglion cells, central, autonomous and peripheral nervous systems) leading, with variable expressivity, to pigmentary anomalies, deafness, mental retardation, peripheral neuropathy, and Hirschsprung disease. Autosomal dominant mode of inheritance is prevalent when Sox10 gene mutation is identified. We report the natural history of a child who presented with synophrys, vivid blue eye, deafness, bilateral complete semicircular canals agenesis with mental retardation, subtle signs for peripheral neuropathy and lack of Hirschsprung disease.