Congenital hyperinsulinism.

Hyperinsulinism is the most common cause of hypoglycemia in infants. In many cases conservative treatment is not effective and surgical intervention is required. Differentiation between diffuse and focal forms and localization of focal lesions are the most important issues in preoperative management.

Ergocalciferol supplementation in children with CKD delays the onset of secondary hyperparathyroidism: a randomized trial.

Background And Objectives: Vitamin D deficiency is an important contributor to the development of hyperparathyroidism and is independently associated with cardiovascular and bone disease. The hypothesis was that nutritional vitamin D (ergocalciferol) supplementation in children with CKD stages 2-4 delays the onset of secondary hyperparathyroidism.

Design, Setting, Participants, & Measurements: A randomized, double-blinded, placebo-controlled study in children with CKD2-4 who had 25-hydroxyvitamin D [25(OH)D] deficiency was conducted.

Vitamin D deficiency is associated with short stature and may influence blood pressure control in paediatric renal transplant recipients.

Vitamin D deficiency is common in adult renal transplant recipients, but data in children are scarce. Vitamin D is shown to have multiple effects on the cardiovascular system, renal function, and maintenance of bone health. We hypothesized that 25(OH)D deficiency is common in pediatric renal transplant recipients, and may be associated with hyperparathyroidism, short stature, renal function, and blood pressure control.

Development of the paediatric pain profile: role of video analysis and saliva cortisol in validating a tool to assess pain in children with severe neurological disability.

The Paediatric Pain Profile (PPP) is a 20-item behavior-rating scale designed to assess pain in children with severe to profound neurological impairment. Three raters independently used the PPP to rate behavior of 29 children (mean age 9.6, SD 5.

Establishing content validity of the oral assessment guide in children and young people.

There is a need for accurate and consistent oral assessment to measure mucosal changes and oral complications associated with cancer therapies. Mucositis is an important and common side effect of cancer therapies that merits the identification of improved health-care interventions. Developing appropriate and reliable oral assessment instruments for use with children is relevant to the evaluation of these interventions.

Gene therapy for children with cystic fibrosis--who has the right to choose?

It may be unethical to deny children with cystic fibrosis access to ethically approved clinical trials from which they might benefit

Infantile hyperinsulinism associated with enteropathy, deafness and renal tubulopathy: clinical manifestations of a syndrome caused by a contiguous gene deletion located on chromosome 11p.

We describe the clinical features of a new syndrome causing hyperinsulinism in infancy (HI), severe enteropathy, profound sensorineural deafness, and renal tubulopathy in three children born to two pairs of consanguineous parents. This combination of clinical features is explained by a 122-kb contiguous gene deletion on the short arm of chromosome 11. It deletes 22 of the 39 exons of the gene coding for the SUR1 component of the KATP channel on the pancreatic beta-cell thereby causing severe HI.

Exhaled nitric oxide increases following admission for intravenous antibiotics in children with cystic fibrosis.

Background: Lack of standardisation for the measurement of exhaled nitric oxide (NO) (FENO) has resulted in conflicting data in cystic fibrosis (CF). The aim of this study was to assess whether FENO is a useful non-invasive marker of lung disease in CF by assessing the effect of intravenous (IV) antibiotics on FENO.

Methods: FENO was measured on line, according to recently published ERS/ATS guidelines, using a chemiluminescence analyser together with pulmonary function in 14 CF children prior to and following a course of IV antibiotics.

Arterial distensibility in children and teenagers: normal evolution and the effect of childhood vasculitis.

Background: Polyarteritis nodosa is a necrotising vasculitis of the medium sized and small muscular arteries. The inflammatory and subsequent reparative processes may alter the arterial mechanical properties. The effect of vasculitic damage on arterial distensibility has never been explored however.

Does oral folic acid lower total homocysteine levels and improve endothelial function in children with chronic renal failure?

Background: Accelerated vascular disease is common in chronic renal failure (CRF) and accounts for significant mortality and morbidity. Elevated homocysteine levels may contribute by an effect on endothelial function.

Methods And Results: We performed a double-blind placebo-controlled randomized crossover trial of folic acid at 5 mg/m2 in 25 normotensive children 12+/-3 (7 to 17) years of age with CRF (glomerular filtration rate 26.

Medical versus surgical treatment in children with severe bilateral vesicoureteric reflux and bilateral nephropathy: a randomised trial.

Background: Nephropathy associated with vesicoureteric reflux (VUR) and urinary tract infection can result in end-stage renal failure, hypertension, or both. Whether long-term VUR contributes to these outcomes is unknown. We compared, in a randomised trial, medical with surgical management of children with bilateral severe VUR and bilateral nephropathy.

Serial assessment of left ventricular diastolic function after Fontan procedure.

Objective: To assess longitudinal changes in systemic ventricular diastolic function late after the Fontan procedure.

Design And Patients: Prospective study of 13 patients at 2.8 (2.

The spectral main sequence of human saccades.

Despite the many models of saccadic eye movements, little attention has been paid to the shape of saccade trajectories. Some investigators have argued that saccades are driven by a rectangular "bang-bang" neural control signal, whereas others have emphasized the similarity to fast arm movement trajectories, such as the "minimum jerk" profile. However, models have not been tested rigorously against empirical trajectories.

Ictal hemiparesis.

Two subjects with ictal hemiparesis are described. Both children presented with evolving paresis associated with seizure activity. Structural neuroimaging remained consistently normal, although EEG demonstrated slow-wave activity, and SPECT scanning in one child showed perfusion asymmetry.

Development of a radiopharmaceutical activity schedule for technetium-99m dimercaptosuccinic acid in children based on image quality criteria.

The aim of this study was to determine an activity schedule (amount of administered activity in relation to body weight) for technetium-99m dimercaptosuccinic acid examinations in children, from information present in renal scintigraphic images. Scans from 48 children (5 weeks to 14.8 years old) were graded for image quality according to the clarity of both kidney outline and internal structure.