Hypertension as the trigger for posterior reversible encephalopathy syndrome in paediatric renal patients: An important diagnosis that should not be missed

Background. Posterior reversible encephalopathy syndrome (PRES) is a reversible neurological condition presenting with seizures and visual disturbances and diagnosed on magnetic resonance imaging (MRI). Little is understood about its pathogenesis, particularly in children, but it is thought to be related to hypertension.Objectives. To review the presentation, diagnosis and outcome of PRES in paediatric renal patients at Chris Hani Baragwanath Academic Hospital, Johannesburg, South Africa, between 1 January 2000 and 31 January 2017 and compare these with published case reports to date.Methods. This was a retrospective analysis of five new cases and a review of the existing literature.Results. The five reported patients were all hypertensive at the time of diagnosis and presented with seizures. Most (91%) of the 64 reviewed patients were also hypertensive at initial presentation. All five of the reported and 91% of the reviewed patients presented with seizures. The most common pattern of change on MRI occurred in the parietal and occipital regions. Complete neurological recovery occurred in four of the five reported and 87.5% of the reviewed patients.Conclusion. All patients presented clinically with hypertensive crises and radiological evidence of PRES. Seizures were the most common presenting symptom. The prognosis for paediatric patients with PRES is favourable, so it is important to confirm the diagnosis in low-resource settings where intensive care is limited.

Posterior urethral valves in South African boys: Outcomes and challenges

Background. Posterior urethral valves (PUV) are a common cause of congenital obstructive nephropathy. The outcome of patients with PUV at Chris Hani Baragwanath Academic Hospital in Johannesburg, South Africa, has not been documented previously.Objectives. To describe the outcome of patients diagnosed with PUV over a 29-year period from January 1985 to December 2013, and to analyse risk factors for chronic kidney disease.Methods. This was a retrospective record review of boys aged <14 years diagnosed with PUV at Chris Hani Baragwanath Academic Hospital. PUV was diagnosed by a voiding cystourethrogram (VCUG) and/or at cystoscopy. Valves were resected primarily or after vesicostomy. The glomerular filtration rate was calculated using the Schwartz formula, and stratified as normal or decreased for age at presentation and at the final visit.Results. Records of 181 patients were analysed. The diagnosis was made during the first year of life in 139 patients (76.8%). Clinical presentation included urinary tract infection (UTI) in 109 patients (60.2%), palpable bladder in 98 (54.1%), palpable kidney in 85 (46.9%), and poor urinary stream in 78 (43.1%). An ultrasound scan was reported normal in 10.9%. Vesicostomy was performed in 80 patients (44.2%) and primary valve ablation in 101 (55.8%), with vesicostomy being more prevalent in the pre-2000 era. The median duration of follow-up was 21 months (interquartile range 5 - 79) and renal outcome at last visit was normal in 117 patients (64.6%). The presence of bladder diverticula was associated with a favourable renal outcome. Thirteen patients (7.2%) died, and 102 (56.3%) defaulted from follow-up.Conclusions. PUV frequently presents with UTI and palpable bladder and/or kidneys. Findings on ultrasound were normal in 10.9% of our patients with PUV. A VCUG is indicated in the presence of palpable kidneys or bladder even if the ultrasound scan is normal. Bladder diverticula as a pressure-release mechanism are renoprotective. Vesicostomy or primary valve ablation did not affect final renal outcome. Chronic kidney disease occurred in 34.8% of patients after surgical correction. Adherence to scheduled appointments is problematic in this population. Long-term follow-up is mandatory.

Gallium scintigraphy in the diagnosis and total lymphoid irradiation of Takayasu's arteritis

Takayasu's arteritis (TA) in children causes appreciable morbidity and mortality, predominantly as a result of the complication of renovascular hypertension (RVH). Ten children with TA, complicated by RVH, were treated at our centre over the past decade. An initial raised erythrocyte sedimentation rate (ESR) and a purified protein derivative greater than 15 mm were present in every case. More recently, gallium scintigraphy has been used to demonstrate sites of active inflammation in affected vessels (3/4 patients) which became negative after total lymphoid irradiation (TU). The latter was used in the last 6 children, and appeared to be effective in controlling disease activity as evinced in the normalisation of their ESRs and negative findings on gallium scintigraphy (in all 3 patients with prior active inflammation). Because of vascular damage caused by the vasculitic process, surgical intervention is often required to improve organ perfusion, particularly of the kidney/so Renal autografting (or allografting) seems preferable (6/11 kidneys functional) to renal bypass grafting (5/5 kidneys clotted). Patient survival improved when TU was used in addition to standard surgical and medical therapy; this included steroids and antituberculous therapy with TU, and steroids and cyclophosphamide in the two relapses. Five of 6 patients treated with TU were alive after 32 - 54 months' follow-up, while 4 patients who received standard medical and surgical therapy but not TU all died within 18 months of diagnosis. Gallium scintigraphy is a helpful diagnostic tool in assessing vasculitic activity in TA; TU is an important mode of immunosuppression, but still needs to be compared with cyclophosphamide as the major immunosuppressive

Aerosolized amikacin for treatment of pulmonary Mycobacterium avium infections: an observational case series

BACKGROUND: Current systemic therapy for nontuberculous mycobacterial pulmonary infection is limited by poor clinical response rates, drug toxicities and side effects. The addition of aerosolized amikacin to standard oral therapy for nontuberculous mycobacterial pulmonary infection may improve treatment efficacy without producing systemic toxicity. This study was undertaken to assess the safety, tolerability and preliminary clinical benefits of the addition of aerosolized amikacin to a standard macrolide-based oral treatment regimen. CASE PRESENTATIONS: Six HIV-negative patients with Mycobacterium avium intracellulare pulmonary infections who had failed standard therapy were administered aerosolized amikacin at 15 mg/kg daily in addition to standard multi-drug macrolide-based oral therapy. Patients were monitored clinically and serial sputum cultures were obtained to assess response to therapy. Symptomatic improvement with radiographic stabilization and eradication of mycobacterium from sputum were considered markers of success. Of the six patients treated with daily aerosolized amikacin, five responded to therapy. All of the responders achieved symptomatic improvement and four were sputum culture negative after 6 months of therapy. Two patients became re-infected with Mycobacterium avium intracellulare after 7 and 21 months of treatment. One of the responders who was initially diagnosed with Mycobacterium avium intracellulare became sputum culture positive for Mycobacterium chelonae resistant to amikacin after being on intermittent therapy for 4 years. One patient had progressive respiratory failure and died despite additional therapy. There was no evidence of nephrotoxicity or ototoxicity associated with therapy. CONCLUSION: Aerosolized delivery of amikacin is a promising adjunct to standard therapy for pulmonary nontuberculous mycobacterial infections. Larger prospective trials are needed to define its optimal role in therapy of this disease

Reperfusion therapy for ST elevation acute myocardial infarction in Europe: description of the current situation in 30 countries

Aims Patient access to reperfusion therapy and the use of primary percutaneous coronary intervention (p-PCI) or thrombolysis (TL) varies considerably between European countries. The aim of this study was to obtain a realistic contemporary picture of how patients with ST elevation myocardial infarction (STEMI) are treated in different European countries. Methods and results The chairpersons of the national working groups/societies of interventional cardiology in European countries and selected experts known to be involved in the national registries joined the writing group upon invitation. Data were collected about the country and any existing national STEMI or PCI registries, about STEMI epidemiology, and treatment in each given country and about PCI and p-PCI centres and procedures in each country. Results from the national and/or regional registries in 30 countries were included in this analysis. The annual incidence of hospital admission for any acute myocardial infarction (AMI) varied between 90–312/100 thousand/year, the incidence of STEMI alone ranging from 44 to 142. Primary PCI was the dominant reperfusion strategy in 16 countries and TL in 8 countries. The use of a p-PCI strategy varied between 5 and 92% (of all STEMI patients) and the use of TL between 0 and 55%. Any reperfusion treatment (p-PCI or TL) was used in 37–93% of STEMI patients. Significantly less reperfusion therapy was used in those countries where TL was the dominant strategy. The number of p-PCI procedures per million per year varied among countries between 20 and 970. The mean population served by a single p-PCI centre varied between 0.3 and 7.4 million inhabitants. In those countries offering p-PCI services to the majority of their STEMI patients, this population varied between 0.3 and 1.1 million per centre. In-hospital mortality of all consecutive STEMI patients varied between 4.2 and 13.5%, for patients treated by TL between 3.5 and 14% and for patients treated by p-PCI between 2.7 and 8%. The time reported from symptom onset to the first medical contact (FMC) varied between 60 and 210 min, FMC-needle time for TL between 30 and 110 min, and FMC-balloon time for p-PCI between 60 and 177 min. Conclusion Most North, West, and Central European countries used p-PCI for the majority of their STEMI patients. The lack of organized p-PCI networks was associated with fewer patients overall receiving some form of reperfusion therapy

Ethnobotany in the Nepal Himalaya

<p>Abstract</p> <p>Background</p> <p>Indigenous knowledge has become recognized worldwide not only because of its intrinsic value but also because it has a potential instrumental value to science and conservation. In Nepal, the indigenous knowledge of useful and medicinal plants has roots in the remote past.</p> <p>Methods</p> <p>The present study reviews the indigenous knowledge and use of plant resources of the Nepal Himalayas along the altitudinal and longitudinal gradient. A total of 264 studies focusing on ethnobotany, ethnomedicine and diversity of medicinal and aromatic plants, carried out between 1979 and 2006 were consulted for the present analysis. In order to cross check and verify the data, seven districts of west Nepal were visited in four field campaigns.</p> <p>Results</p> <p>In contrast to an average of 21–28% ethnobotanically/ethnomedicinally important plants reported for Nepal, the present study found that up to about 55% of the flora of the study region had medicinal value. This indicates a vast amount of undocumented knowledge about important plant species that needs to be explored and documented. The richness of medicinal plants decreased with increasing altitude but the percentage of plants used as medicine steadily increased with increasing altitude. This was due to preferences given to herbal remedies in high altitude areas and a combination of having no alternative choices, poverty and trust in the effectiveness of folklore herbal remedies.</p> <p>Conclusion</p> <p>Indigenous knowledge systems are culturally valued and scientifically important. Strengthening the wise use and conservation of indigenous knowledge of useful plants may benefit and improve the living standard of poor people.</p

Enhanced Syllable Discrimination Thresholds in Musicians

Speech processing inherently relies on the perception of specific, rapidly changing spectral and temporal acoustic features. Advanced acoustic perception is also integral to musical expertise, and accordingly several studies have demonstrated a significant relationship between musical training and superior processing of various aspects of speech. Speech and music appear to overlap in spectral and temporal features; however, it remains unclear which of these acoustic features, crucial for speech processing, are most closely associated with musical training. The present study examined the perceptual acuity of musicians to the acoustic components of speech necessary for intra-phonemic discrimination of synthetic syllables. We compared musicians and non-musicians on discrimination thresholds of three synthetic speech syllable continua that varied in their spectral and temporal discrimination demands, specifically voice onset time (VOT) and amplitude envelope cues in the temporal domain. Musicians demonstrated superior discrimination only for syllables that required resolution of temporal cues. Furthermore, performance on the temporal syllable continua positively correlated with the length and intensity of musical training. These findings support one potential mechanism by which musical training may selectively enhance speech perception, namely by reinforcing temporal acuity and/or perception of amplitude rise time, and implications for the translation of musical training to long-term linguistic abilities.Grammy FoundationWilliam F. Milton Fun

The exposure of the hybrid detector of the Pierre Auger Observatory

The Pierre Auger Observatory is a detector for ultra-high energy cosmic rays. It consists of a surface array to measure secondary particles at ground level and a fluorescence detector to measure the development of air showers in the atmosphere above the array. The "hybrid" detection mode combines the information from the two subsystems. We describe the determination of the hybrid exposure for events observed by the fluorescence telescopes in coincidence with at least one water-Cherenkov detector of the surface array. A detailed knowledge of the time dependence of the detection operations is crucial for an accurate evaluation of the exposure. We discuss the relevance of monitoring data collected during operations, such as the status of the fluorescence detector, background light and atmospheric conditions, that are used in both simulation and reconstruction.Comment: Paper accepted by Astroparticle Physic

The Fluorescence Detector of the Pierre Auger Observatory

The Pierre Auger Observatory is a hybrid detector for ultra-high energy cosmic rays. It combines a surface array to measure secondary particles at ground level together with a fluorescence detector to measure the development of air showers in the atmosphere above the array. The fluorescence detector comprises 24 large telescopes specialized for measuring the nitrogen fluorescence caused by charged particles of cosmic ray air showers. In this paper we describe the components of the fluorescence detector including its optical system, the design of the camera, the electronics, and the systems for relative and absolute calibration. We also discuss the operation and the monitoring of the detector. Finally, we evaluate the detector performance and precision of shower reconstructions.Comment: 53 pages. Submitted to Nuclear Instruments and Methods in Physics Research Section