Dexamethasone therapy and endogenous cortisol production in severe pediatric head injury.

A prospective randomised study was performed on 25 children aged 1.4 to 15.8 years with severe head injury (Glasgow Coma Scale less than or equal to 7) to determine the clinical effectiveness and the impact on endogenous cortisol production of high-dose steroid therapy. Thirteen patients (group 1) received dexamethasone 1 mg/kg/day during the first 3 days and 12 (group 2) not. All patients were treated with a standardized regimen. Urinary free cortisol was measured by radioimmunoassay, and the clinical data were recorded at hourly intervals. Outcome was assessed 6 months later using the Glasgow Outcome Scale. We found a higher frequency of bacterial pneumonias in the dexamethasone-treated patients (7/13 versus 2/12). Group 1 showed a suppression of endogenous cortisol production from day 1 to day 6. In group 2, mean free cortisol was up to 5-fold higher than under basal conditions. The results in group 2 showed that the endogenous steroid production reacts adequately to the stress of severe head injury. It probably is sufficient to elicit maximum glucocorticoid effects. There was no other statistically significant difference in the clinical and laboratory data between the two groups. We conclude that dexamethasone in high doses suppresses endogenous cortisol production up to 6 days and may increase the risk of bacterial infection without affecting the outcome or the clinical and laboratory data.

Neurologie [News in neurology 2013].

In 2013, perampanel is approved as an add-on treatment for generalised and focal seizures in pharmaco-resistant epilepsy. New anticoagulants are superior to antivitamin K in stroke secondary prevention in case of atrial fibrillation. DBS remains a valid therapeutic option for advanced Parkinson's disease. Intranasal ketamine seems to reduce the intensity of severe migraine aura. High concentrations of topic capsaicin improve post-herpetic neuralgia. In Alzheimer's disease, statins might deteriorate cognitive functions. Oral immuno-modifing treatments for relapsing remitting multiple sclerosis have shown to slow cerebral atrophy progression at two years.

Effectiveness of a pediatric palliative home care team as experienced by parents and health care professionals.

OBJECTIVES: Little data are available on palliative home care for children. The objective of this study was to evaluate the effectiveness of a specialized pediatric palliative home care team (PPHCT) as experienced by parents and health care professionals (HCPs). METHODS: Parents and HCPs involved in the care of terminally ill children who died and whom the PPHCT was in charge of were surveyed with questionnaires focusing on satisfaction with the PPHCT, satisfaction with the course of the dying phase, and the development of anxiety, depression, and prolonged grief disorder. RESULTS: Forty-three parent dyads participated (return rate, 88%). Satisfaction with the PPHCT scored a median of 10 (numeric rating scale, 0-10). The child's death was predominantly experienced as very peaceful (median, 9); 71% died at home. According to parents, involvement of the PPHCT led to highly significant (p<0.001) improvements in the children's symptoms and quality of life, as well as in aspects of communication and administrative barrier reduction. Anxiety was detected in 25% of parents, depression in 19%, and prolonged grief disorder in 13%. HCPs (return rate, 83%) evaluated all investigated care domains (particularly cooperation/communication/family support) as being significantly improved (p<0.001). Thirty-five percent of HCPs felt uncertain concerning pediatric palliative care; 79% would welcome specific training opportunities. CONCLUSIONS: Involvement of a PPHCT is experienced as a substantial improvement of care by parents and HCPs. Coordination of palliative care during the last phase of life appears to be an important quality factor for the home care of dying children and their families.

Pédiatrie 1. Formule Quadratique: une nouveauté pédiatrique et pratique pour estimer le taux de filtration glomérulaire [Quadratic formula: a new pediatric advance for glomerular filtration rate estimation].

A new formula for glomerular filtration rate estimation in pediatric population from 2 to 18 years has been developed by the University Unit of Pediatric Nephrology. This Quadratic formula, accessible online, allows pediatricians to adjust drug dosage and/or follow-up renal function more precisely and in an easy manner.

A novel pediatric biventricular assist device: in vitro test results.

Most ventricular assist devices (VADs) currently used in infants are extracorporeal. These VADs require long-term anticoagulation therapy and extensive surgery, and two devices are needed for biventricular support. We designed a biventricular assist device based on shape memory alloy that reproduces the hemodynamic effects of cardiomyoplasty, supporting the heart with a compressing movement, and evaluated its performance in a dedicated mockup system. Nitinol fibers are the device's key component. Ejection fraction (EF), cardiac output (CO), and generated systolic pressure were measured on a test bench. Our test bench settings were a preload range of 0-15 mm Hg, an afterload range of 0-160 mm Hg, and a heart rate (HR) of 20, 30, 40, and 60 beats/min. A power supply of 15 volts and 3.5 amperes was necessary. The EF range went from 34.4% to 1.2% as the afterload and HR increased, along with a CO from 180 to 6 ml/min. The device generated a maximal systolic pressure of 25 mm Hg. Cardiac compression for biventricular assistance in child-sized heart using shape memory alloy is technically feasible. Further testing remains necessary to assess this VAD's in vivo performance range and its reliability.

Peripapillary neovascular membrane: A rare cause of acute vision loss in pediatric idiopathic intracranial hypertension.

We report a 14-year-old boy who presented with vision loss secondary to peripapillary neovascular membrane (PPNVM) as the initial and only symptom of papilledema secondary to idiopathic intracranial hypertension. After one lumbar puncture, visual acuity progressively recovered during the course of 1 week and further improved with the administration of oral acetazolamide. One year after the onset of vision loss, the patient's visual acuity had recovered to baseline measurements. The previously active PPNVM had involuted into a residual peripapillary fibrotic scar. To our knowledge, this is the first report of PPNVM complicating idiopathic intracranial hypertension in a child.

Nouvelles thérapies en rhumatologie pédiatrique [New therapies in pediatric rheumatology].

Biotherapies are recent treatments, which target molecules implicated in the pathogenesis of inflammatory diseases. In pediatric rheumatology, we use anti-TNF-alpha and abatacept in JIA patients with polyarticular involvement, whereas anti-IL-6 and anti-IL-1 blockers are efficacious in the systemic form of JIA and other auto-inflammatory conditions. These new treatments have significantly improved the control of articular and systemic inflammation and the prognosis of rheumatic diseases. Their effect and their safety on the long-term need to be assessed on large cohorts of patients. Due to the impact of these chronic illnesses on the young patient and its family, and the required specific knowledge, the care of these children should be provided by a multidisciplinary team linked to a centre of competence.

Hémato-oncologie pédiatrique: de la biologie cellulaire aux traitements ciblés et individualisés [Pediatric hemato-oncology: from cellular biology to individualized targeted therapies].

Progresses in pediatric oncology over the last decades have been dramatic and allow current cure rates above 80%. There are mainly due to multicentre clinical trials aiming at optimizing chemotherapy protocols as well as local therapies in a stepwise approach. Most of the new anticancer drugs currently in development are based on targeted therapies, directed to specific targets present only in or on tumor cells, like growth factor receptors, mechanisms involved in proliferation, DNA repair, apoptosis, tumor invasion or angiogenesis. Concerning bone marrow transplantation also, new strategic approaches are in advanced development. They aim at reducing treatment induced toxicity and enhancing efficacy at the same time. This short paper would like to point out these new technologies, which should be known by the general practitioner.

Nouvelles stratégies thérapeutiques en oncologie ophtalmo-pédiatrique [New strategies in pediatric ophthalmic oncology]

Le rétinoblastome représente 11% de tous les cancers apparaissant pendant la première année de vie. Pour éviter les effets secondaires de la chimiothérapie systémique ou de la radiothérapie externe, de nouveaux médicaments et de nouvelles techniques de traitement focalisé ont été développés. Des voies d'administration telles la voie périoculaire (topotécan) ou la voie artérielle ophtalmique directe (carboplatine) sont utilisées aujourd'hui dans certains cas résistants. La radiothérapie actuelle, appliquée exclusivement en deuxième intention, fait appel également à des techniques ciblées, permettant d'éviter les tissus sains et de réduire le risque de cancers radio-induits non oculaires. La radiothérapie stéréotaxique conformationnelle ou conformale et la proton-thérapie font ainsi partie du nouvel arsenal thérapeutique du rétinoblastome. Retinoblastoma represents 11% of all cancers during the first year of life. New drugs and focal treatments have been developed in order to avoid the side effects of systemic chemotherapy and external radiotherapy. New targeted and local administration strategies such as periocular chemotherapy (topotecan) or direct ophthalmic artery delivery (carboplatin), are already used today in selected resistant cases. Radiotherapy, presently indicated only as a second-line treatment, is also subject to new techniques, targeting tumors more closely to avoid involving healthy tissue and reduce the risk of radio-induced nonocular tumors. Stereotactic conformal radiotherapy and proton therapy may thus be included in the new range of treatment methods in retinoblastoma

Prise en charge des jeunes patients avec endocrinopathies pediatriques chroniques: les défis d'une transition souvent difficile [Caring for patients with pediatric endocrinopathies and diabetes into adulthood: challenges of an often difficult transition].

The success of therapies for a number of pediatric disorders has posed new challenges for the long-term follow-up of adolescents with chronic endocrinopathies. Unfortunately, too many patients are lost during the transfer from pediatric to adult clinics. The transition process should be well-organized and include the young person and family. Recognizing the special needs of these adolescents is an important step in developing patient-centered approaches to care that enable patients to develop autonomy and self care skills. Key elements in this process include structured policies and guidelines, communication and close collaboration between pediatric and adult clinics, and integrating nurse clinicians in the transition process to help close the gaps in care.

Deep sclerectomy combined with trabeculectomy in pediatric glaucoma

Résumé : Ce travail porte sur l'étude rétrospective d'une série de jeunes patients opérés de glaucomes pédiatriques. Le but est d'évaluer le résultat au long cours d'une intervention chirurgicale combinant une sclérectomie profonde et une trabéculectomie (sclérectomie profonde pénétrante). Durant la période de mars 1997 à octobre 2006, 28 patients on été suivis pour évaluer le résultat de cette chirurgie effectuées sur 35 yeux. Un examen ophtalmologique complet a été pratiqué avant la chirurgie, 1 et 7 jours, puis 1, 2, 3, 4, 6, 9, 12 mois, enfin tous les 6 mois après l'opération. Les critères d'évaluation du résultat postopératoire sont : les changements de pression intraoculaire, le traitement antiglaucomateux adjuvant, le taux de complication, le nombre de reprises chirurgicales,- l'erreur de réfraction, la meilleure acuité visuelle corrigée, l'état et le diamètre de la cornée. L'âge moyen est de 3.6 ± 4.5 ans et le suivi moyen de 3.6 ± 2.9 ans. La pression intraoculaire préopératoire de 31.9 ± 11.5 mmHg baisse de 58.3% (p<0.005) à la fin du suivi. Sur les 14 patients dont l'acuité visuelle a pu être mesurée, 8 (57.1 %) ont une acuité égale ou supérieure à 5/10e, 3 (21.4%) une acuité de 2/10e après intervention. Le taux de succès cumulatif complet à 9 ans est de 52.3%, le succès relatif 70.6%. Les complications menaçant la vision (8.6%) ont été plus fréquentes dans les cas de glaucome réfractaire. Pour conclure la sclérectomie profonde combinée à une trabéculectomie est une technique chirurgicale développée afin de contrôler la pression intraoculaire dans les cas de glaucomes congénitaux, juvéniles et secondaires. Les résultats intermédiaires sont encourageants et prometteurs. Les cas préalablement opérés avant cette nouvelle technique ont cependant un pronostic moins favorable. Le nombre de complications menaçant la vision est essentiellement lié à la sévérité du glaucome et au nombre d'interventions préalables. Abstract : Purpose : To evaluate the outcomes of combined deep sclerectomy and trabeculectomy (penetrating deep sclerectomy) in pediatric glaucoma. Design : Retrospective, non-consecutive, non-comparative, interventional case series. Participants : Children suffering from pediatric glaucoma who underwent surgery between March 1997 and October 2006 were included in this study. Methods : A primary combined deep sclerectomy and trabeculectomy was performed in 35 eyes of 28 patients. Complete examinations were performed before surgery, postoperatively at 1 and 7 days, at 1, 2, 3, 4, 6, 9, 12 months and then every 6 months after surgery. Main Outcome Measures : Surgical outcome was assessed in terms of intraocular pressure (IOP) change, additional glaucoma medication, complication rate, need for surgical revision, as well as refractive errors, best corrected visual acuity (BCVA), and corneal clarity and diameters. Results : The mean age before surgery was 3.6 ± 4.5 years, and the mean follow-up was 3.5 ± 2.9 years. The mean preoperative IOP was 31.9 ± 11.5 mmHg. At the end of follow-up, the mean IOP decreased by 58.3% (p<0.005), and from 14 patients with available BCVA 8 patients (57.1 %) achieved. 0.5 (20/40) or better, 3 (21.4%) 0.2 (20/100), and 2 (14.3%) 0.1 (20/200) in their better eye. The mean refractive error (spherical equivalent) at final follow-up visits was +0.83 ± 5.4. Six patients (43%) were affected by myopia. The complete and qualified success rates, based on a cumulative survival curve, after- 9 years were 52.3% and 70.6%, respectively (p<0.05). Sight threatening complications were more common (8.6%) in refractory glaucomas. Conclusions : Combined deep sclerectomy and trabeculectomy is a surgical technique developed to control IOP in congenital, secondary and juvenile glaucomas. The intermediate results are satisfactory and promising. Previous classic glaucoma surgeries performed before this new technique had less favourable results. The number of sight threatening complications is related to the severity of glaucoma and number of previous surgeries.

Optimisation des doses en tomographie computérisée pédiatrique [Patient dose optimization in pediatric computerized tomography]

The development of CT applications might become a public health problem if no effort is made on the justification and the optimisation of the examinations. This paper presents some hints to assure that the risk-benefit compromise remains in favour of the patient, especially when one deals with the examinations of young patients. In this context a particular attention has to be made on the justification of the examination. When performing the acquisition one needs to optimise the extension of the volume investigated together with the number of acquisition sequences used. Finally, the use of automatic exposure systems, now available on all the units, and the use of the Diagnostic Reference Levels (DRL) should allow help radiologists to control the exposure of their patients.

The AO comprehensive classification of pediatric long-bone fractures: a web-based multicenter agreement study.

The first AO comprehensive pediatric long-bone fracture classification system has been proposed following a structured path of development and validation with experienced pediatric surgeons. A Web-based multicenter agreement study involving 70 surgeons in 15 clinics and 5 countries was conducted to assess the reliability and accuracy of this classification when used by a wide range of surgeons with various levels of experience. Training was provided at each clinic before the session. Using the Internet, participants could log in at any time and classify 275 supracondylar, radius, and tibia fractures at their own pace. The fracture diagnosis was made following the hierarchy of the classification system using both clinical terminology and codes. kappa coefficients for the single-surgeon diagnosis of epiphyseal, metaphyseal, or diaphyseal fracture type were 0.66, 0.80, and 0.91, respectively. Median accuracy estimates for each bone and type were all greater than 80%. Depending on their experience and specialization, surgeons greatly varied in their ability to classify fractures. Pediatric training and at least 2 years of experience were associated with significant improvement in reliability and accuracy. Kappa coefficients for diagnosis of specific child patterns were 0.51, 0.63, and 0.48 for epiphyseal, metaphyseal, and diaphyseal fractures, respectively. Identified reasons for coding discrepancies were related to different understandings of terminology and definitions, as well as poor quality radiographic images. Results supported some minor adjustments in the coding of fracture type and child patterns. This classification system received wide acceptance and support among the surgeons involved. As long as appropriate training could be performed, the system classification was reliable, especially among surgeons with a minimum of 2 years of clinical experience. We encourage broad-based consultation between surgeons' international societies and the use of this classification system in the context of clinical practice as well as prospectively for clinical studies.

Role of surgery in pediatric epilepsy.

Twenty five percent of patients with intractable epilepsy have surgically remediable epilepsy syndromes. This article reviews the treatment paradigm for pediatric epilepsy and also the indications, methods, and surgical options for the subgroup of patients with surgically remediable epileptic disorders based on our experience in the management of these children. The article also discusses the rationale for offering surgery and the timing of surgery in these patients. The study of surgically remediable epilepsy can best be divided into focal, sub hemispheric, hemispheric and multifocal epileptic syndromes. These syndromes have both acquired and congenital etiologies and can be treated by resective or disconnective surgery. The surgical management of these conditions (with the exception of multifocal epilepsy) provides Engel's Class 1 outcome(complete seizure freedom) in approximately 80% of children. The consequences of seizure freedom leads to a marked improvement in the quality of life of these children. The benefits to society, of allowing a child to grow to adulthood with normal cognition to earn a livelihood and contribute actively to society, cannot be understated.