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Introduction Pneumocystis jirovecii pneumonia (PJP) is a life-threatening condition in immunocompromised children. Our aim is to analyze the epidemiologic and clinical characteristics of PJP cases in our setting, describing the prognosis and related risk factors. Methods Retrospective study including all pediatric patients (≤18 years) with PJP admitted to our hospital (January 1989–December 2016). Case definition: patient with acute pneumonitis and P. jirovecii detection in bronchoalveolar lavage or tracheal aspirate using methenamine silver or direct antibody fluorescence staining, or Real-Time Polymerase Chain Reaction. Results Twenty-five cases (0.9 cases/year) were identified. Median age was 2.2 years (interquartile range: 0.5–12.3), 64% were male, and 12% were receiving appropriate antimicrobial prophylaxis. Cytomegalovirus coinfection was detected in 26% cases. The most common underlying diseases were primary immunodeficiencies (36%) and 16% were human immunodeficiency virus (HIV)-infected children. Eighteen were admitted to the pediatric intensive care unit (PICU) and overall 30-day mortality was 20% (31.25% in HIV non-infected vs 0% in HIV-infected patients; OR: 0.33, 95% CI: 0.02–7.24, p = 0.55). Clinical outcome was worse in girls and those patients requiring adjuvant steroid therapy. HIV non-infected patients, higher initial LDH, younger age and shorter time elapsed between diagnosis of PJP and the underlying disease were identified as risk factors to be admitted to the PICU (p = 0.05, p = 0.026, p = 0.04 and p = 0.001 respectively). Conclusion Accompanying the widespread use of combined antiretroviral therapy, PJP has been diagnosed almost exclusively in HIV non-infected children at our institution. Moreover, significant higher morbidity rates associated with PJP are seen in this group of patients.

STUDY OBJECTIVES: The majority of studies investigating the association between sleep and Alzheimer's disease (AD) biomarkers have been performed in healthy participants. Our objective was to investigate the association between sleep and several biomarkers that reflect distinct aspects of AD physiopathology. METHODS: The cohort included 104 individuals with mild-moderate AD. The participants were submitted to one-night polysomnography, and cerebrospinal fluid was collected in the following morning to measure the selected biomarkers associated with amyloid deposition, tau pathology, neurodegeneration, axonal damage, synaptic integrity, neuroinflammation, and oxidative damage. RESULTS: There was a positive correlation between neurofilament light (NF-L) and the time spent in stage 1 of non-rapid eyes movement (NREM) (N1) sleep and a negative correlation between this marker and the time spent in stage 3 of NREM (N3) sleep. Accordingly, we observed that deep sleep was associated with lower levels of NF-L, whereas light sleep increased the probability of having higher levels of this marker. Furthermore, chitinase-3-like-1 (YKL-40) was negatively correlated with sleep efficiency, the time spent in stage 2 of NREM (N2) sleep, and the time spent in N3 sleep. Conversely, there was a positive correlation between N3 sleep and the oxidative protein damage markers N-ε-(carboxyethyl)lysine and N-ε-(malondialdehyde)lysine. CONCLUSIONS: There were significant correlations between sleep parameters and AD biomarkers related to axonal damage and neuroinflammation, such as NF-L and YKL-40. A lack of deep sleep was associated with higher levels of NF-L. This highlights a potential role for NF-L as a biomarker of sleep disruption in patients with mild-moderate AD in addition to its role in predicting neurodegeneration and cognitive decline. This study was supported by the Generalitat of Catalonia, Department of Health (PERIS 2019 SLT008/18/00050) and “Fundació La Marató TV3” (464/C/2014) to G.P.R.; by the Spanish Ministry of Economy and Competitiveness, Institute of Health Carlos III (grant number P114/00328), the Spanish Ministry of Science, Innovation and University (RTI 2018–099) of Catalonia, and Agency for Management of University and Research grants (2017 SGR696) to R.P. This study has been co-financed by FEDER funds from the European Union (“A way to build Europe”). IRBLleida is a CERCA Programme/Generalitat of Catalonia. F.D. was supported by the Agency for Management of University and Research grants (FI_B100153).

Rehabilitation for return to training is an interdisciplinary, specific and individualised process that is geared towards the sportsman's optimal availability for competition after a sports injury. This process begins after medical discharge and continues up to the return to play, involving the different professionals in the field of healthcare and led by the sports physician. Planning consists of three phases, defined by muscular action (isometric, concentric and eccentric); kinetic chain (closed or open); range of movement (internal, medial, external and total); and exercise orientation (general, directed and specific). Return to play must be agreed upon on the basis of objective information on the recovery stage of the injury, the state of fitness, and the sportsman's own perception.

Background Pantothenate kinase-associated neurodegeneration is a progressive neurological disorder occurring in both childhood and adulthood. The objective of this study was to design and pilot-test a disease-specific clinical rating scale for the assessment of patients with pantothenate kinase-associated neurodegeneration. Methods In this international cross-sectional study, patients were examined at the referral centers following a standardized protocol. The motor examination was filmed, allowing 3 independent specialists in movement disorders to analyze 28 patients for interrater reliability assessment. The scale included 34 items (maximal score, 135) encompassing 6 subscales for cognition, behavior, disability, parkinsonism, dystonia, and other neurological signs. Results Forty-seven genetically confirmed patients (30 ± 17 years; range, 6-77 years) were examined with the scale (mean score, 62 ± 21; range, 20-106). Dystonia with prominent cranial involvement and atypical parkinsonian features were present in all patients. Other common signs were cognitive impairment, psychiatric features, and slow and hypometric saccades. Dystonia, parkinsonism, and other neurological features had a moderate to strong correlation with disability. The scale showed good internal consistency for the total scale (Cronbach's α = 0.87). On interrater analysis, weighted kappa values (0.30-0.93) showed substantial or excellent agreement in 85% of the items. The scale also discriminated a subgroup of homozygous c.1583C>T patients with lower scores, supporting construct validity for the scale. Conclusions The proposed scale seems to be a reliable and valid instrument for the assessment of pediatric and adult patients with pantothenate kinase-associated neurodegeneration. Additional validation studies with a larger sample size will be required to confirm the present results and to complete the scale validation testing. © 2017 International Parkinson and Movement Disorder Society

Summary Objective To reduce ventilator-associated infections (VARI) and improve outcomes for children. Methods This prospective interventional cohort study was conducted in a paediatric intensive care unit (PICU) over three periods: pre-intervention, early post-intervention, and late post-intervention. These children were on mechanical ventilation (MV) for ≥48h. Results Overall, 312 children (11.9% of whom underwent tracheostomy) and 6187 ventilator-days were assessed. There was a significant reduction in ventilator-associated pneumonia (VAP) among tracheostomized patients (8.16, 3.27, and 0.65 per 1000 tracheostomy ventilation-days before the intervention, after the general bundle implementation, and after the tracheostomy intervention, respectively). The median time from onset of MV to diagnosis of ventilator-associated tracheobronchitis (VAT) increased from 5.5 to 48 days in the late post-intervention period ( p =0.004), and was associated with a significant increase in median 28-day ventilator-free days and PICU-free days. Tracheostomy (odds ratio 7.44) and prolonged MV (odds ratio 2.75) were independent variables significantly associated with VARI. A trend towards a reduction in PICU mortality was observed, from 28.4% to 16.6% (relative risk 0.58). Conclusions The implementation of a care bundle to prevent VARI in children had a different impact on VAP and VAT, diminishing VAP rates and delaying VAT onset, resulting in reduced healthcare resource use. Tracheostomized children were at increased risk of VARI, but preventive measures had a greater impact on them.

Introduction: Neuroinflammation is a potential player in neurodegenerative conditions, particularly the aggressive ones, such as multiple system atrophy (MSA). Previous reports on cytokine levels in MSA using serum or cerebrospinal fluid (CSF) have been inconsistent, including small samples and a limited number of cytokines, often without comparison to Parkinson's disease (PD), a main MSA differential diagnosis. Methods: Cross-sectional study of CSF levels of 38 cytokines using a multiplex assay in 73 participants: 39 MSA patients (19 with parkinsonian type [MSAp], 20 with cerebellar type [MSAc]; 31 probable, 8 possible), 19 PD patients and 15 neurologically unimpaired controls. None of the participants was under non-steroidal anti-inflammatory drugs at the time of the lumbar puncture. Results: There were not significant differences in sex and age among participants. In global non-parametric comparisons FDR-corrected for multiple comparisons, CSF levels of 5 cytokines (FGF-2, IL-10, MCP-3, IL-12p40, MDC) differed among the three groups. In pair-wise FDR-corrected non-parametric comparisons 12 cytokines (FGF-2, eotaxin, fractalkine, IFN-α2, IL-10, MCP-3, IL-12p40, MDC, IL-17, IL-7, MIP-1β, TNF-α) were significantly higher in MSA vs. non-MSA cases (PD + controls pooled together). Of these, MCP-3 and MDC were the most significant ones, also differed in MSA vs. PD, and were significant MSA-predictors in binary logistic regression models and ROC curves adjusted for age. CSF levels of fractalkine and MIP-1α showed a strong and significant positive correlation with UMSARS-2 scores. Conclusion: Increased CSF levels of cytokines such as MCP-3, MDC, fractalkine and MIP-1α deserve consideration as potential diagnostic or severity biomarkers of MSA. info:eu-repo/semantics/publishedVersion

Introduction The finding of mutations of the COQ2 gene and reduced coenzyme Q10 levels in the cerebellum in multiple system atrophy (MSA) suggest that coenzyme Q10 is relevant to MSA pathophysiology. Two recent studies have reported reduced coenzyme Q10 levels in plasma and serum (respectively) of MSA patients compared to Parkinson's disease and/or control subjects, but with largely overlapping values, limited comparison with other parkinsonisms, or dependence on cholesterol levels. We hypothesized that cerebrospinal fluid (CSF) is reliable to assess reductions in coenzyme Q10 as a candidate biomarker of MSA. Methods In this preliminary cross-sectional study we assessed CSF coenzyme Q10 levels in 20 patients with MSA from the multicenter Catalan MSA Registry and of 15 PD patients, 10 patients with progressive supranuclear palsy (PSP), and 15 control subjects from the Movement Disorders Unit Biosample Collection of Hospital Clinic de Barcelona. A specific ELISA kit was used to determine CSF coenzyme Q10 levels. CSF coenzyme Q10 levels were compared in MSA vs. the other groups globally, pair-wise, and by binary logistic regression models adjusted for age, sex, disease severity, disease duration, and dopaminergic treatment. Results CSF coenzyme Q10 levels were significantly lower in MSA than in other groups in global and pair-wise comparisons, as well as in multivariate regression models. Receiver operating characteristic curve analyses yielded significant areas under the curve for MSA vs. PD, PSP and controls. Conclusions These findings support coenzyme Q10 relevance in MSA. Low CSF coenzyme Q10 levels deserve further consideration as a biomarker of MSA.

Objectives To assess the prediction accuracy of the 2008 US Centers for Disease Control and Prevention (CDC) definitions for ventilator-associated pneumonia (VAP)/ventilator-associated tracheobronchitis (VAT), 2013 CDC definitions for ventilator-associated events (VAE) and a new VAE algorithm in the paediatric (Ped) population, the Ped-VAE. Methods We performed a prospective 13-month cohort study at a multidisciplinary paediatric intensive care unit (PICU). Primary endpoints were duration of ventilation episode, PICU or hospitalization length of stay from episode and episode mortality. Episodes without VAE (or VAP/VAT) served as comparison groups. Results One hundred eight episodes of ventilation (99 children) with 2554 ventilator-days were assessed. In episodes not meeting 2008 CDC definitions, a median of 6 ventilator-days (PICU stay 11 days) was documented (with eight deaths), not significantly different from episodes not meeting VAE or Ped-VAE definitions. Using 2008 CDC criteria, 11 (10.2%) respiratory infections (eight tracheobronchitis) were identified, seven VAEs using 2013 CDC criteria (6.4%) and 29 (26.8%) using Ped-VAE criteria (relative risk vs. 2008 CDC criteria 2.58; 95% confidence interval 1.36–4.91). In contrast with their comparison groups, episodes meeting 2008 CDC criteria did not significantly predict outcomes, whereas VAEs (only four possible VAPs) were associated with significantly more ventilation and PICU length of stay (12-day/8-day increase) and sevenfold increase in mortality. Ped-VAE did not increase mortality, but it was associated with 4-day increase in ventilation and PICU length of stay, with ten possible VAPs, and atelectasis (9/12) as the main paediatric ventilator-associated condition. Conclusions The 2008 CDC criteria did not predict outcomes, whereas VAE only identified very severe events. The Ped-VAE algorithm had more accuracy predicting outcomes by characterizing lower oxygenation changes and identifying hypoxaemia severity, a major driver of management.

Objective To examine the presence and characteristics of idiopathic REM sleep behavior disorder (IRBD) in a representative Caucasian sample from the elderly community of Lleida, Spain, attending primary care centers. Methods Participants were individuals aged 60 years or older who underwent routine visits in two primary care centers. They underwent a two-stage study; a validated screening single question for IRBD diagnosis (RBD1Q) followed by, in those who endorsed positive answer, clinical assessment by a neurologist plus video-polysomnography (V-PSG). Results Of 539 individuals (56.4% women, mean age 72.86 ± 8.20 years), 28 (5.2%) endorsed positively the RBD1Q. Four of these 28 refused further assessments. Four of the 24 remaining subjects underwent clinical assessment but refused V-PSG. Of the 20 who underwent clinical assessment plus V-PSG, REM sleep was not recorded in four (20%, all four taking antidepressants). V-PSG ruled out RBD in 12 subjects who had obstructive sleep apnea (n = 9), periodic limb movement disorder in sleep (n = 2) and normal sleep (n = 1). IRBD was diagnosed in four individuals giving an estimated prevalence of 0.74% (95% CI = 0.29–1.89). They were three men and one woman between 74 and 82 years of age who never reported dream-enacting behaviors to their doctors because they thought they represented a normal phenomenon despite suffering sleep-related injuries. These patients had history of violent sleep behaviors with an interval between estimated RBD onset and V-PSG of 4.5 ± 4.2 years. Conclusions IRBD is not uncommon in the elderly community and its demographic and clinical profile is similar to those diagnosed in sleep centers.

Study objectives To describe a group of patients referred because of abnormal sleep behaviors that were suggestive of rapid eye movement (REM) sleep behavior disorder (RBD) in whom video-polysomnography ruled out RBD and showed the reported behaviors associated with vigorous periodic limb movements during sleep (PLMS). Aims and methods Clinical history and video-polysomnography review of patients identified during routine visits in a sleep center. Results Patients were 15 men and 2 women with a median age of 66 (range: 48-77) years. Reported sleep behaviors were kicking (n = 17), punching (n = 16), gesticulating (n = 8), falling out of bed (n = 5), assaulting the bed partner (n = 2), talking (n = 15), and shouting (n = 10). Behaviors resulted in injuries in 3 bed partners and 1 patient. Twelve (70.6%) patients were not aware of displaying abnormal sleep behaviors that were only noticed by their bed partners. Ten (58.8%) patients recalled unpleasant dreams such as being attacked or chased. Video-polysomnography showed (1) frequent and vigorous stereotyped PLMS involving the lower limbs, upper limbs, and trunk (median PLMS index 61.2; median PLMS index in NREM sleep 61.9; during REM sleep only 8 patients had PLMS and their median PLMS index in REM sleep was 39.5); (2) abnormal behaviors (e.g., punching, groaning) during some of the arousals that immediately followed PLMS in NREM sleep; and (3) ruled out RBD and other sleep disorders such as obstructive sleep apnea. Dopaminergic agents were prescribed in 14 out of the 17 patients and resulted in improvement of abnormal sleep behaviors and unpleasant dreams in all of them. After dopaminergic treatment, follow-up video-polysomnography in 7 patients showed a decrease in the median PLMS index from baseline (108.9 vs. 19.2, p = .002) and absence of abnormal behaviors during the arousals. Conclusions Abnormal sleep behaviors and unpleasant dreams simulating RBD symptomatology may occur in patients with severe PLMS. In these cases, video-polysomnography ruled out RBD and identified prominent PLMS followed by arousals containing abnormal behaviors. Our cases represent an objectively documented subtype of periodic limb movement disorder causing abnormal sleep behaviors.

Sleep-related movement and behaviour disorders may have an impact on sleep quality and lead to daytime symptoms. These groups of conditions include diseases such as restless legs syndrome, periodic leg movements, and REM and NREM parasomnias. The knowledge of their clinical features and management is of utmost importance for the neurologist and sleep specialist. Frequently, these patients are referred to such specialists and it is relevant to know that certain sleep disorders may be associated with other neurological conditions.

Rehabilitation for return to training is an interdisciplinary, specific and individualised process to achieve optimal availability for competition after an athlete’s sports injury. This process requires a transversal, specific, progressive and previously planned design. The model will be individualized according to the injury, the athlete, the sport and its competitive schedule. The design is divided into 3 different phases and the duration of each one will be determined by premises previously set out for the achievement of specific clinical and conditional goals. Decision making process is assessed by objective information, but performance on professional context is a key factor to consider.

Background: Twenty-four-hour ambulatory blood pressure monitoring (ABPM) provides the most accurate efficacy assessment of an antihypertensive agent throughout a 24-hour dosing interval. The objective of this prospective, randomised, double-blind, parallel-group, multicentre study was to compare the antihypertensive efficacy of imidapril versus candesartan cilexetil using ABPM. Methods: After screening and a single-blind, placebo run-in phase, ambulatory adult patients with mild to moderate hypertension (defined as a mean office sitting diastolic BP [DBP] and systolic BP [SBP], respectively, of 90–109mm Hg and 140–179mm Hg, and a mean ABPM DBP and SBP, respectively, of ≥80mm Hg and ≥125mm Hg) were randomised to once-daily treatment with imidapril or candesartan cilexetil for 12 weeks. ABPM was performed at baseline and at the end of the 12-week treatment period in 112 patients (imidapril group, n = 55; candesartan cilexetil group, n = 57). To achieve the target BP of ≤140/90mm Hg, imidapril was titrated from 5 mg/day to 20 mg/day and candesartan cilexetil was titrated from 4 mg/day to 16 mg/day. Results: Significant (p < 0.001) and similar decreases from baseline in clinic mean DBP and SBP, in mean 24-hour ABPM, DBP and SBP awake and asleep, and in mean BP (MBP) were observed in both treatment groups. In addition, significant and similar reductions in DBP and SBP were observed during the early morning acceleration period in both treatments. The reduction in BP load was higher with imidapril than with candesartan cilexetil: 44.6% versus 34.5% reduction in DBP load and 38.0% versus 32.9% reduction in SBP load, respectively. With respect to the average deviation index expressing a load index, the reduction with imidapril was 41.0% versus 33.6% with candesartan cilexetil. The percentage of DBP dipper patients remained identical before and after treatment in both groups. With regard to SBP, the percentage of dippers increased from 38.2% to 45.5% in the imidapril group and decreased from 54.4% to 42.1% in the candesartan cilexetil group. The incidence of adverse events was similar between the treatment groups and no cases of dry cough were reported. Conclusion: Imidapril in once-daily doses of up to 20mg was shown to be at least as effective as candesartan cilexetil given in once-daily doses of up to 16mg in reducing BP throughout the entire 24-hour dosing interval. Both drugs were well tolerated.

STUDY OBJECTIVES To measure the sleep spindle characteristics in patients with unilateral thalamic stroke. DESIGN A prospective study of patients with thalamic stroke and age-matched healthy controls. SETTING Department of Neurology of a University Hospital. PARTICIPANTS Thirteen patients (mean age: 67 years, SD: 13,44) with an isolated, unilateral acute thalamic stroke and 18 healthy age-matched volunteers. INTERVENTIONS A polysomnogram recording from 14 scalp EEG electrodes performed during 2 consecutive nights, the second or third week after the stroke. Only the sleep of the second night was analyzed. MEASUREMENTS AND RESULTS Sleep spindles were counted during two separate 10-minute epochs of stage II. Spindles appearing synchronously in both sides with similar amplitude were called "bilateral." Spindles with twice the amplitude in one side than the other were "right" or "left-side predominant". There were 8 patients with posterolateral, 3 with global and 2 with anterior lesions. Eight were right and 5 left-sided. The number of spindles was similar in patients (39.8 +/- 23.4 in 20 minutes) than controls (26.07 +/- 29.07; p=0.173). Spindles with a centroparietal (34%) and centroparieto-occipital localization (22%) were the most frequent. In controls approximately 66% of the spindles had a bilateral and symmetric distribution over the scalp, 23% of the spindles were predominantly left-sided and 5% were predominantly right-sided. In patients, bilateral spindles decreased (p

Aims and topics The international symposium on luminescence spectrometry (ISLS) series started in 1984 when Willy Bayens organized the first six editions at Ghent University. From 1996, the ISLS has been conducted periodically every 2 years in different countries. The main purpose of the conference is to provide a forum to present new developments and innovations in the field of luminescence spectroscopy and their main applications, from photoluminescence and electrogenerated luminescence to bioluminescence and chemiluminescence, regarding both basic science and applications in biosciences and pharmaceutical, environmental, and food analysis. The 2012 meeting was mainly focused on “Luminescence spectroscopy: bioanalytical and biophysical aspects.” Symposium sessions took place in the Historic Building of the University of Barcelona (Fig. 1), which was raised in 1871 by Elies Rogent (it is possible to have a virtual look at http://www.ub.edu/museuvirtual/visitavirtualEH/ index_es.html).

Esmolol is a cardioselective beta-blocker with very rapid onset of action and short half-life due to its metabolism by blood-borne esterases. This unique profile among currently available beta-blockers renders esmolol highly useful in critical care situations. However, published experience with the use of esmolol in critically ill children is scant. The case of a 4-year-old boy with secondary long QT syndrome and ventricular tachycardia successfully treated with esmolol is presented.

Fragile X syndrome is the most common form of hereditary mental retardation. The molecular basis of this syndrome is mainly a CGG expansion in the 5' untranslated region of the FMR1 gene. Expansions with more than 200 CGG repeats abolish gene expression causing the classical fragile X phenotype. Premutation carriers (55-200 CGG) have normal cognitive function with increased risk of developing premature ovarian failure and fragile X-associated tremor-ataxia syndrome (FXTAS). Some clinical features associated with FXTAS, such as tremor, gait ataxia, cognitive decline, and generalized brain atrophy, are also seen in other movement disorders. Ninety-five patients referred for HD, who tested negative for the expansion in the IT15 gene, were screened for FMR1 CGG-repeat expansion. One FMR1 premutation male carrier was detected, giving an FXTAS frequency of 1.6%. Our results highlight that FXTAS is still not well diagnosed; therefore, we recommend FMR1 premutation screenings in all patients with late-onset tremor, ataxia, and cognitive dysfunction.

The incidence and prevalence of immunocompromised patients has increased (higher number of transplantations and improved management of primary immunodeficiencies). Nevertheless, the management strategies of these patients in the pediatric ICU (PICU) remain a challenge because of their important death rate. The study objective was to analyse the morbimortality and prognosis of immunosuppressed patients requiring critical care due to a medical cause.

Acute respiratory failure (ARF) in immunocompromised patients is associated with a high mortality rate. The aim of this investigation is to study these patients' behavior patterns to collect data that can allow us to improve their survival rate.