Reninoma: an uncommon cause of renin-mediated hypertension

Reninoma (juxtaglomerular cell tumor) is a rare cause of renin-mediated hypertension. Reninomas are usually diagnosed in adolescents and young adults with occasional reports in younger children. Patients typically present with a long history of headaches leading to a diagnosis of severe hypertension that responds well to antihypertensive treatment targeting the renin-angiotensin-aldosterone system. The clue to clinical diagnosis is the presence of hypokalemia and metabolic alkalosis on the first blood sample drawn before any treatment is instituted. Elevated blood levels of renin and aldosterone confirm the clinical suspicion of renin-mediated hypertension. Diagnostic imaging is employed to identify the source of excessive renin production. While renal ultrasound can miss reninoma, contrast CT or magnetic resonance imaging of the kidneys are diagnostic modalities of choice leading to the correct diagnosis. Renal vein renin sampling with lateralization might help to identify the site of excessive renin production. Nephron-sparing surgery is curative with maintenance of normal blood pressure after discontinuation of antihypertensive medications in the majority of patients. In this paper, we present the case of reninoma in an adolescent girl emphasizing clinical presentation, diagnostic evaluation, and medical and surgical treatment of this rare tumor. We also discuss important points in the management of children presenting with renin-mediated hypertension

Early Miocene tectono-sedimentary shift in the eastern North Alpine Foreland Basin and its relation to changes in tectonic style in the Eastern Alps

A striking difference along the Alpine Orogen is the style of collisional tectonics during the Oligo-Miocene, with the onset of escape tectonics in the Eastern Alps (Fig. 1A). The indentation of the Adriatic Plate into the Eastern Alpine Orogen resulted in the formation of conjugate dextral and sinistral strike-slip faults in the vicinity of the Tauern Window. Moreover, major changes occurred in the foreland of the Eastern and Southern Alps in the Early Miocene, with the cessation of the northern Alpine front propagation and the onset of thrusting along the Southern Alpine Front. In this study, we present new results from structural, stratigraphic and subsidence analyses of the eastern North Alpine Foreland Basin (NAFB; Fig. 1B) as part of the “Mountain Building in 4 Dimensions” project, German branch of the European AlpArray initiative, which aims at better understanding the deep crustal-mantle structures of the Alpine Orogen and their relation to surface processes. Our results show a first phase of onset of foreland sedimentation in the eastern NAFB between c. 33-28 Ma, followed by a strong tectonic-driven subsidence between c. 28-25 Ma ending by a phase of erosion and the formation of a basin-wide Northern Slope Unconformity (NSU; Fig. 1C & 1D). During this time period, the rift-related Mesozoic normal faults of the European platform were reactivated and are capped by the NSU (Fig. 1D). We interpret this phase as an increase in the flexure of the subducting European Plate under the growing Alpine Orogen. Between 25-19 Ma, the eastern NAFB remained in a deep-marine, underfilled state with a gently increase in subsidence. A major shift took place around 19-17 Ma with strong tectonic-driven uplift, ranging from 200 m (absolute minimum) to 1200 m depending on uncertainties on paleo-water depths, and rapid sedimentary infill of the basin (Fig. 1C & 1D). We discuss the possible causes for this major tectono-sedimentary shift in the eastern NAFB in relation to contemporaneous changes in collisional tectonics within the Eastern and Southern Alps, and with a potential Early Miocene slab break-off event beneath the Eastern Alps

Estimating global injuries morbidity and mortality : methods and data used in the Global Burden of Disease 2017 study

Background: While there is a long history of measuring death and disability from injuries, modern research methods must account for the wide spectrum of disability that can occur in an injury, and must provide estimates with sufficient demographic, geographical and temporal detail to be useful for policy makers. The Global Burden of Disease (GBD) 2017 study used methods to provide highly detailed estimates of global injury burden that meet these criteria. Methods: In this study, we report and discuss the methods used in GBD 2017 for injury morbidity and mortality burden estimation. In summary, these methods included estimating cause-specific mortality for every cause of injury, and then estimating incidence for every cause of injury. Non-fatal disability for each cause is then calculated based on the probabilities of suffering from different types of bodily injury experienced. Results: GBD 2017 produced morbidity and mortality estimates for 38 causes of injury. Estimates were produced in terms of incidence, prevalence, years lived with disability, cause-specific mortality, years of life lost and disability-adjusted life-years for a 28-year period for 22 age groups, 195 countries and both sexes. Conclusions: GBD 2017 demonstrated a complex and sophisticated series of analytical steps using the largest known database of morbidity and mortality data on injuries. GBD 2017 results should be used to help inform injury prevention policy making and resource allocation. We also identify important avenues for improving injury burden estimation in the future

Global burden of 369 diseases and injuries in 204 countries and territories, 1990–2019: a systematic analysis for the Global Burden of Disease Study 2019

Background: In an era of shifting global agendas and expanded emphasis on non-communicable diseases and injuries along with communicable diseases, sound evidence on trends by cause at the national level is essential. The Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) provides a systematic scientific assessment of published, publicly available, and contributed data on incidence, prevalence, and mortality for a mutually exclusive and collectively exhaustive list of diseases and injuries. Methods: GBD estimates incidence, prevalence, mortality, years of life lost (YLLs), years lived with disability (YLDs), and disability-adjusted life-years (DALYs) due to 369 diseases and injuries, for two sexes, and for 204 countries and territories. Input data were extracted from censuses, household surveys, civil registration and vital statistics, disease registries, health service use, air pollution monitors, satellite imaging, disease notifications, and other sources. Cause-specific death rates and cause fractions were calculated using the Cause of Death Ensemble model and spatiotemporal Gaussian process regression. Cause-specific deaths were adjusted to match the total all-cause deaths calculated as part of the GBD population, fertility, and mortality estimates. Deaths were multiplied by standard life expectancy at each age to calculate YLLs. A Bayesian meta-regression modelling tool, DisMod-MR 2.1, was used to ensure consistency between incidence, prevalence, remission, excess mortality, and cause-specific mortality for most causes. Prevalence estimates were multiplied by disability weights for mutually exclusive sequelae of diseases and injuries to calculate YLDs. We considered results in the context of the Socio-demographic Index (SDI), a composite indicator of income per capita, years of schooling, and fertility rate in females younger than 25 years. Uncertainty intervals (UIs) were generated for every metric using the 25th and 975th ordered 1000 draw values of the posterior distribution. Findings: Global health has steadily improved over the past 30 years as measured by age-standardised DALY rates. After taking into account population growth and ageing, the absolute number of DALYs has remained stable. Since 2010, the pace of decline in global age-standardised DALY rates has accelerated in age groups younger than 50 years compared with the 1990–2010 time period, with the greatest annualised rate of decline occurring in the 0–9-year age group. Six infectious diseases were among the top ten causes of DALYs in children younger than 10 years in 2019: lower respiratory infections (ranked second), diarrhoeal diseases (third), malaria (fifth), meningitis (sixth), whooping cough (ninth), and sexually transmitted infections (which, in this age group, is fully accounted for by congenital syphilis; ranked tenth). In adolescents aged 10–24 years, three injury causes were among the top causes of DALYs: road injuries (ranked first), self-harm (third), and interpersonal violence (fifth). Five of the causes that were in the top ten for ages 10–24 years were also in the top ten in the 25–49-year age group: road injuries (ranked first), HIV/AIDS (second), low back pain (fourth), headache disorders (fifth), and depressive disorders (sixth). In 2019, ischaemic heart disease and stroke were the top-ranked causes of DALYs in both the 50–74-year and 75-years-and-older age groups. Since 1990, there has been a marked shift towards a greater proportion of burden due to YLDs from non-communicable diseases and injuries. In 2019, there were 11 countries where non-communicable disease and injury YLDs constituted more than half of all disease burden. Decreases in age-standardised DALY rates have accelerated over the past decade in countries at the lower end of the SDI range, while improvements have started to stagnate or even reverse in countries with higher SDI. Interpretation: As disability becomes an increasingly large component of disease burden and a larger component of health expenditure, greater research and developm nt investment is needed to identify new, more effective intervention strategies. With a rapidly ageing global population, the demands on health services to deal with disabling outcomes, which increase with age, will require policy makers to anticipate these changes. The mix of universal and more geographically specific influences on health reinforces the need for regular reporting on population health in detail and by underlying cause to help decision makers to identify success stories of disease control to emulate, as well as opportunities to improve. Funding: Bill & Melinda Gates Foundation. © 2020 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY 4.0 licens

Resection of Symptomatic ureteric paraganglioma with appendiceal interposition

We present a rare tumour in an even rarer position requiring a radical excision. The resultant mid ureteric defect was interposed with the novel use of a pedicled appendiceal interposition conduit

Hidden danger of button batteries

his case highlights a rare but important presentation of aorto-oesophageal fistula caused by a concealed foreign body. Primary prevention strategies are needed to address the danger associated with button batteries

Urethral syringocele: unseen but existing

Background: Bulbourethral syringocele is an uncommon and under-diagnosed condition most commonly seen in the paediatric population, although there is increasing recognition in adults. Due to the difficulty in diagnosis, we report our experience of urethral syringocele in a quaternary paediatric hospital, with differing presentations, diagnosis and treatment. Methods: This is a retrospective review of seven cases of children over a period of 14 years, including their presentations, diagnosis, treatment and follow-up. A review of the current literature is presented. Results: The median age of these seven cases at presentation was 11 years (6 days to 16 years). Clinical features varied with age, with obstructive uropathy in a neonate, urinary tract infection in an infant, scrotal abscess in two children and lower urinary tract obstructive symptoms in three teenagers. Diagnostic voiding cystogram diagnosed the majority of syringoceles and two were seen on magnetic resonance imaging. Five boys underwent endoscopic transurethral deroofing and two children required transperineal marsupialization. Long-term follow-up showed all had complete resolution of symptoms. Conclusion: Urethral syringocele presents from the neonatal period to late adolescence, with the presenting features reflective of age. Surgical management can be performed endoscopically or by open approach. Awareness of this condition and inclusion in the differential diagnosis, particularly in the setting of an atypical or recurrent scrotal abscess, could avoid a prolonged therapeutic course