[Cannabidiol: its use in refractory epilepsies].

Introduction: Some epileptic syndromes are characterised by seizures that are difficult to control and are associated to delayed neuropsychomotor development, which results in a deterioration in the patient's quality of life as well as in that of his or her family.

Aim: To evaluate the use of cannabidiol as adjuvant therapy in patients with refractory epilepsies.

Patients And Methods: An observational study was conducted by means of a survey addressed to the patient's caregiver.

[Possible treatments for infantile spinal atrophy].

The new treatments of spinal muscular atrophy (SMA) due by SMN1 gene deletions are reviewed. There are several ways to increase the protein SMN, its activity and persistence in the tissues. Neuroprotective drugs as olesoxime or riluzole, and drugs acting by epigenetic mechanisms, as histone deacetylase inhibitors, have shown positive effects in preclinical studies but no clear efficacy in clinical trials.

[Clinical guidelines for infantile-onset Pompe disease].

Infantile-onset Pompe disease has a fatal prognosis in the short term unless it is diagnosed at an early stage and enzyme replacement therapy is not started as soon as possible. A group of specialists from different disciplines involved in this disease have reviewed the current scientific evidence and have drawn up an agreed series of recommendations on the diagnosis, treatment and follow-up of patients. We recommend establishing enzyme treatment in any patient with symptomatic Pompe disease with onset within the first year of life, with a clinical and enzymatic diagnosis, and once the CRIM (cross-reactive immunological material) status is known.

Cost-minimization analysis in the treatment of spasticity in children with cerebral palsy with botulinum toxin type A: an observational, longitudinal, retrospective study.

Objective: Cost-minimization analysis of onabotulinumtoxinA and abobotulinumtoxinA, taking into account the real dose administered to children with spasticity associated with dynamic equinus foot deformity due to cerebral palsy.

Method: A single centre, observational, longitudinal, and retrospective study which included spastic paediatric patients aged 2-to-18-years and treated with onabotulinumtoxinA or abobotulinumtoxinA from December 1995 to October 2012, in the Paediatric Neurology Unit of a first-level Spanish hospital. A longitudinal analysis of spasticity severity was made to confirm the similar efficacy of both treatments.

Cardiac preload responsiveness in children with cardiovascular dysfunction or dilated cardiomyopathy: a multicenter observational study.

Objectives: To characterize cardiac preload responsiveness in pediatric patients with cardiovascular dysfunction and dilated cardiomyopathy using global end-diastolic volume index, stroke volume index, cardiac index, and extravascular lung water index.

Design: Prospective multicenter observational study.

Setting: Medical/surgical PICUs of seven Spanish University Medical Centers.

[Erythropoietin as a protective factor in rat CNS cells receiving radiotherapy -an in vitro study].

Aim: To investigate the effect of erythropoietin in cultured rat cerebral cortex cells receiving radiotherapy.

Materials And Methods: Cerebral cortex was taken from 17-day-old Wistar rat embryos and placed in culture. At 72 hours, the cultures were divided into two groups, one receiving 30 pM erythropoietin alpha and the other was the control group.

[Treatment with antisense oligonucleotides in Duchenne's disease].

In this paper I review the results of the treatments directed to modify the mRNA of dystrophin with the goal of converting the severe Duchenne type to the milder Becker muscular dystrophy. Antisense oligomers potential to modify Duchenne muscular dystrophy (DMD) gene expression and therapeutic strategies to induce ribosomal read-through of nonsense mutations (PTC124) are described. They are an important advance in the treatment of DMD, so far unspecific.

Asynchrony, neural drive, ventilatory variability and COMFORT: NAVA versus pressure support in pediatric patients. A non-randomized cross-over trial.

Purpose: To determine if neurally adjusted ventilatory assist (NAVA) improves asynchrony, ventilatory drive, breath-to-breath variability and COMFORT score when compared to pressure support (PS).

Methods: This is a non-randomized short-term cross-over trial in which 12 pediatric patients with asynchrony (auto-triggering, double triggering or non-triggered breaths) were enrolled. Four sequential 10-min periods of data were recorded after 20 min of ventilatory stabilization (wash-out) at each of the following settings: baseline PS with the ventilator settings determined by the attending physician (1-PS(b)); PS after optimization (2-PS(opt)); NAVA level set so that maximum inspiratory pressure (P(max)) equaled P(max) in PS (3-NAVA); same settings as in 2-PS(opt) (4-PS(opt)).

[Consensus document from the Spanish Society of Paediatric Infectious Diseases (SEIP) on the diagnosis and treatment of congenital cytomegalovirus infection].

Cytomegalovirus (CMV) is the leading cause of congenital infection in developed countries, affecting 0.3 to 0.6% of all live births in Europe.

[Effect of intensive treatment with atorvastatin versus standard doses of statins on the risk of stroke. A meta-analysis from five randomized trials including 25,709 patients].

Aim: To evaluate the efficacy of intensive lipid lowering treatment with atorvastatin versus standard doses of statins (simvastatin, atorvastatin, lovastatin or pravastatin) on the risk of stroke, using meta-analytic techniques.

Patients And Methods: The meta-analysis included 5 randomized trials that met the inclusion criteria: comparison between intensive atorvastatin treatment (atorvastatin 80 mg or target LDL < 70 mg/dL) and standard doses of statins, with a clinical follow-up of more than 12 months and stroke as clinical endpoint. TNT (n = 9,917) atorvastatin 80 mg vs.

[Congenital brachial palsy: its epidemiology and sequelae. A retrospective study of our case mix].

Aim: To conduct a retrospective study of cases of congenital brachial palsy, focusing on its incidence, clinical manifestations and long-term course.

Patients And Methods: A systematized study of all the cases of congenital brachial palsy detected in the Hospital La Paz between January 1994 and December 2003 was carried out with the aim of recording data on the pregnancy, gestational age, type of delivery, presentation, sex and weight at birth, Apgar test and the arterial pH of the umbilical cord. It also sought to estimate the type of brachial palsy, the side that was affected, electromyographic findings, associated insults, treatment and progress.

[General anesthesia in mentally disabled patients undergoing dental surgery].

Objective: To describe the anesthetic technique used, the evaluation of airway patency, and the perioperative complications in patients with lower than average intelligence (mentally disabled) who are administered general anesthesia for dental surgery.

Material And Methods: We carried out a prospective, descriptive, comparative study of mentally disabled ASA 2-3 patients. The patients were distributed in 2 groups: mild to moderate mental disability and severe to very severe mental disability.

[Nitrogen balance in adolescent patients with chronic renal insufficiency].

Unlabelled: Protein restriction in patients with chronic renal failure may be potentially beneficial, however the dangers in growing children of such restrictions must be considered. We analyzed the nitrogen balance in 60 chronic renal failure children (47 male y 13 female) Group A: 36 preadolescents. GFR was 54.