6 research outputs found

    Selective decontamination of the digestive tract: all questions answered?

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    Although many studies have shown beneficial effects of SDD on the incidence of respiratory tract infections, SDD did not become routine practice because mortality reduction was not demonstrated in individual trials, beneficial effects on duration of ventilation, ICU stay or hospital stay were not demonstrated, cost-efficacy had not been demonstrated, and selection of antibiotic resistance was considered a serious side-effect. A recent study has now shown improved patient survival and lower prevalence of antibiotic resistance in patients receiving SDD. Why could this study show mortality reduction, where all others studies had failed before? And do the microbiological data unequivocally prove protective effects of SDD on emergence of antibiotic resistance? Interestingly, the reported mortality reductions exceeds even the most optimistic predictions from previous meta-analyses, but a clear explanation is not yet evident. The data on antibiotic resistance, however, are rather superficial and do not allow to interpret the underlying epidemiological dynamics. Therefore, the recent findings are provocative and shed new light on the SDD issue, warranting studies confirming its beneficial effects but also addressing several important aspects related to study design

    Effects of Postural Management on Hip Migration in Children with Cerebral Palsy : A Systematic Review

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    Purpose: To review the existing literature on the effects of postural management on hip migration in children with cerebral palsy. Methods: A systematic literature search was performed using 5 databases. Quality of articles was assessed and study designs were appraised according to the American Academy of Cerebral Palsy and Developmental Medicine Systematic Review Method. Results: Eight of 655 identified studies were included, reporting postural management in children with cerebral palsy. Seven of 8 studies reported positive effects on hip migration after postural management interventions. However, level of evidence and quality of the articles were low. Conclusion: The evidence for postural management to prevent or reduce hip migration in children with cerebral palsy is limited by the lack of high-quality studies. Strong recommendations for clinical practice are not possible. Future high-quality research is crucial to improve our understanding of the effects of postural management to prevent hip migration in children with cerebral palsy

    Beneficial effect of BH4 treatment in a 15-year-old boy with biallelic mutations in DNAJC12

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    Background: Biallelic mutations in DNAJC12 were recently identified as a BH4-responsive cause of hyperphenylalaninemia (HPA). Outcome was only favorable when treatment was initiated early in life. We report on a 15-year-old boy with HPA due to a homozygous deletion in DNAJC12 in whom – despite his advanced age – treatment was initiated. Case: A boy with developmental delay, an extrapyramidal movement disorder, and persistently elevated plasma phenylalanine levels was diagnosed with DNAJC12 deficiency at the age of 15 years. Diagnosis was made upon exome reanalysis revealing a homozygous 6.9 kb deletion in DNAJC12 which had not been detected by the standard exome analysis pipeline. Treatment with the BH4 analog sapropterin dihydrochloride (10 mg/kg/day) was initiated and evoked a 50% reduction of the plasma phenylalanine levels. More strikingly, a marked improvement in daily functioning and improved exercise tolerance was noted. Additionally, gait analysis before and after treatment initiation revealed a partial normalization of his movement disorder. Conclusion: Patients with hyperphenylalaninemia due to DNAJC12 deficiency may benefit from treatment with a BH4 analog – even when introduced at a later age

    Impacts of multiple stressors on freshwater biota across spatial scales and ecosystems

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    Climate and land-use change drive a suite of stressors that shape ecosystems and interact to yield complex ecological responses (that is, additive, antagonistic and synergistic effects). We know little about the spatial scales relevant for the outcomes of such interactions and little about effect sizes. These knowledge gaps need to be filled to underpin future land management decisions or climate mitigation interventions for protecting and restoring freshwater ecosystems. This study combines data across scales from 33 mesocosm experiments with those from 14 river basins and 22 cross-basin studies in Europe, producing 174 combinations of paired-stressor effects on a biological response variable. Generalized linear models showed that only one of the two stressors had a significant effect in 39% of the analysed cases, 28% of the paired-stressor combinations resulted in additive effects and 33% resulted in interactive (antagonistic, synergistic, opposing or reversal) effects. For lakes, the frequencies of additive and interactive effects were similar for all spatial scales addressed, while for rivers these frequencies increased with scale. Nutrient enrichment was the overriding stressor for lakes, with effects generally exceeding those of secondary stressors. For rivers, the effects of nutrient enrichment were dependent on the specific stressor combination and biological response variable. These results vindicate the traditional focus of lake restoration and management on nutrient stress, while highlighting that river management requires more bespoke management solutions

    Causes of permanent childhood hearing impairment

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    Item does not contain fulltextINTRODUCTION: The causes of Permanent Childhood Hearing Impairment (PCHI) are often quoted as being hereditary in 50%, acquired in 25%, and unknown in 25% of cases. Interest in the causes of PCHI has grown recently due to increasing diagnostic possibilities. We investigated the evidence for the reported distribution of these causes. METHODS: Population-based study and a systematic review. Inclusion criteria for population-based study: children born between 2003 and 2005, resident in The Netherlands at birth, known at an Audiology Center with PCHI at the age of 3-5 years. The causes of PCHI were determined prospectively by detection of congenital cytomegalovirus on dried blood spots and/or genetic diagnostic investigations in addition to reviewing data from medical records. A systematic review was carried out using three terms (hearing loss, infant, and etiology) and limited to articles published between January 1997 and July 2009. Main outcome measures were: the (weighted) proportions of the various causes of PCHI following diagnostic investigations. RESULTS: In the study-population (n = 185) a hereditary cause was found in 38.9%, acquired cause in 29.7%, miscellaneous cause in 7.1%, and the cause remained unknown in 24.3%. The systematic review of the literature (n = 9 articles) resulted in a weighted mean of 30.4% hereditary, 19.2% acquired, and 48.3% unknown causes of PCHI. DISCUSSION: The systematic review and the results of the population-based study provided little support for the generally accepted distribution of causes of PCHI
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