Imperial College London

Dr Frédéric B. Piel

Faculty of MedicineSchool of Public Health

Senior Lecturer
 
 
 
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Contact

 

f.piel

 
 
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1112Sir Michael Uren HubWhite City Campus

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Summary

 

Publications

Publication Type
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90 results found

Piel FB, Parkes B, Hambly P, Roca-Barceló A, McCallion M, Leonardi G, Strosnider H, Yip F, Elliott P, Hansell ALet al., 2020, Software application profile: the Rapid Inquiry Facility 4.0: an open access tool for environmental public health tracking, International Journal of Epidemiology, Vol: 49, Pages: i38-i48, ISSN: 0300-5771

The Rapid Inquiry Facility 4.0 (RIF) is a new user-friendly and open-access tool, developed by the UK Small Area Health Statistics Unit (SAHSU), to facilitate environment public health tracking (EPHT) or surveillance (EPHS). The RIF is designed to help public health professionals and academics to rapidly perform exploratory investigations of health and environmental data at the small-area level (e.g. postcode or detailed census areas) in order to identify unusual signals, such as disease clusters and potential environmental hazards, whether localized (e.g. industrial site) or widespread (e.g. air and noise pollution). The RIF allows the use of advanced disease mapping methods, including Bayesian small-area smoothing and complex risk analysis functionalities, while accounting for confounders. The RIF could be particularly useful to monitor spatio-temporal trends in mortality and morbidity associated with cardiovascular diseases, cancers, diabetes and chronic lung diseases, or to conduct local or national studies on air pollution, flooding, low-magnetic fields or nuclear power plants.

Journal article

Nnodu O, Isa H, Nwegbu M, Ohiaeri C, Adegoke S, Chianumba R, Ugwu N, Brown B, Olaniyi J, Okocha E, Lawson J, Hassan A-A, Diaku-Akinwumi I, Madu A, Ezenwosu O, Tanko Y, Kangiwa U, Girei A, Israel-Aina Y, Ladu A, Egbuzu P, Abjah U, Okolo A, Akbulut-Jerada N, Fernandez M, Piel F, Adekile Aet al., 2019, HemoTypeSC, a low-cost point-of-care testing device for sickle cell disease: promises and challenges, Blood Cells, Molecules, and Diseases, Vol: 78, Pages: 22-28, ISSN: 1079-9796

BackgroundSickle cell disease (SCD) is a neglected burden of growing importance. >312,000 births are affected annually by sickle cell anaemia (SCA). Early interventions such as newborn screening, penicillin prophylaxis and hydroxyurea can substantially reduce the mortality and morbidity associated with SCD. Nevertheless, their implementation in African countries has been mostly limited to pilot projects. Recent development of low-cost point-of-care testing (POCT) devices for sickle haemoglobin (HbS) could greatly facilitate the diagnosis of those affected.MethodsWe conducted the first multi-centre, real-world assessment of a low-cost POCT device, HemoTypeSC, in a low-income country. Between September and November 2017, we screened 1121 babies using both HemoTypeSC and HPLC and confirmed discordant samples by molecular diagnosis.FindingsWe found that, in optimal field conditions, the sensitivity and specificity of the test for SCA were 93.4% and 99.9%, respectively. All 14 carriers of haemoglobin C were successfully identified. Our study reveals an overall accuracy of 99.1%, but also highlights the importance of rigorous data collection, staff training and accurate confirmatory testing. It suggests that HPLC results might not be as reliable in a resource-poor setting as usually considered.InterpretationThe use of such a POCT device can be scaled up and routinely used across multiple healthcare centres in sub-Saharan Africa, which would offer great potential for the identification and management of vast numbers of individuals affected by SCD who are currently undiagnosed.

Journal article

Hockham C, Ekwattanakit S, Bhatt S, Penman B, Gupta S, Viprakasit V, Piel Fet al., 2019, Estimating the burden of α-thalassaemia in Thailand using a comprehensive prevalence database for Southeast Asia, eLife, Vol: 8, ISSN: 2050-084X

Abstract Severe forms of α-thalassaemia, haemoglobin H disease and haemoglobin Bart’s hydrops fetalis, are an important public health concern in Southeast Asia. Yet information on the prevalence, genetic diversity and health burden of α-thalassaemia in the region remains limited. We compiled a geodatabase of α-thalassaemia prevalence and genetic diversity surveys and, using geostatistical modelling methods, generated the first continuous maps of α-thalassaemia mutations in Thailand and sub-national estimates of the number of newborns with severe forms in 2020. We also summarised the current evidence-base for α-thalassaemia prevalence and diversity for the region. We estimate that 3,595 (95% credible interval 1,717 – 6,199) newborns will be born with severe α-thalassaemia in Thailand in 2020, which is considerably higher than previous estimates. Accurate, fine-scale epidemiological data are necessary to guide sustainable national and regional health policies for α-thalassaemia control. Our maps and newborn estimates are an important first step towards this aim. Funding This work was supported by European Union’s Seventh Framework Programme (FP7//2007-2013)/European Research Council [268904 – DIVERSITY]

Journal article

Hockham C, Gupta S, Penman B, Bhatt S, Piel Fet al., 2019, Estimating the burden of α-thalassaemia in Thailand using a comprehensive prevalence database for Southeast Asia, eLife, Vol: 8, ISSN: 2050-084X

Severe forms of α-thalassaemia, haemoglobin H disease and haemoglobin Bart’s hydrops fetalis, are an important public health concern in Southeast Asia. Yet information on the prevalence, genetic diversity and health burden of α-thalassaemia in the region remains limited. We compiled a geodatabase of α-thalassaemia prevalence and genetic diversity surveys and, using geostatistical modelling methods, generated the first continuous maps of α-thalassaemia mutations in Thailand and sub-national estimates of the number of newborns with severe forms in 2020. We also summarised the current evidence-base for α-thalassaemia prevalence and diversity for the region. We estimate that 3595 (95% credible interval 1,717–6,199) newborns will be born with severe α-thalassaemia in Thailand in 2020, which is considerably higher than previous estimates. Accurate, fine-scale epidemiological data are necessary to guide sustainable national and regional health policies for α-thalassaemia management. Our maps and newborn estimates are an important first step towards this aim.

Journal article

De Franceschi L, Lux C, Piel FB, Gianesin B, Bonetti F, Casale M, Graziadei G, Lisi R, Pinto V, Putti MC, Rigano P, Rosso R, Russo G, Spadola V, Pulvirenti C, Rizzi M, Mazzi F, Ruffo G, Forni GLet al., 2019, Access to emergency department of acute events and identification of sickle cell disease in refugees, Blood, Vol: 133, Pages: 2100-2103, ISSN: 1528-0020

Journal article

Hockham C, Bhatt S, Colah R, Mukherjee M, Penman B, Gupta S, Piel Fet al., 2018, The spatial epidemiology of sickle-cell anaemia in India, Scientific Reports, Vol: 8, ISSN: 2045-2322

Sickle-cell anaemia (SCA) is a neglected chronic disorder of increasing global health importance, with India estimated to have the second highest burden of the disease. In the country, SCA is particularly prevalent in scheduled populations, which comprise the most socioeconomically disadvantaged communities. We compiled a geodatabase of a substantial number of SCA surveys carried out in India over the last decade. Using generalised additive models and bootstrapping methods, we generated the first India-specific model-based map of sickle-cell allele frequency which accounts for the district-level distribution of scheduled and non-scheduled populations. Where possible, we derived state- and district-level estimates of the number of SCA newborns in 2020 in the two groups. Through the inclusion of an additional 158 data points and 1.3 million individuals, we considerably increased the amount of data in our mapping evidence-base compared to previous studies. Highest predicted frequencies of up to 10% spanned central India, whilst a hotspot of ~12% was observed in Jammu and Kashmir. Evidence was heavily biased towards scheduled populations and remained limited for non-scheduled populations, which can lead to considerable uncertainties in newborn estimates at national and state level. This has important implications for health policy and planning. By taking population composition into account, we have generated maps and estimates that better reflect the complex epidemiology of SCA in India and in turn provide more reliable estimates of its burden in the vast country. This work was supported by European Union’s Seventh Framework Programme (FP7//2007–2013)/European Research Council [268904 – DIVERSITY]; and the Newton-Bhabha Fund [227756052 to CH]

Journal article

Piel FBJ, Brandon P, Hima D, Anna L H, Paul Eet al., 2018, The challenge of opt-outs from NHS data: a small-area perspective, Journal of Public Health, Vol: 40, Pages: e594-e600, ISSN: 1741-3842

Journal article

Graziadei G, Sainati L, Bonomo P, Venturelli D, Masera N, Casale M, Vassanelli A, Lodi G, Piel FB, Voi V, De Franceschi L, Rigano P, Quota A, Notarangelo LD, Russo G, Rosso R, Allo M, D'Ascola D, Facchini E, Macchi S, Arcioni F, Piperno A, Bonetti F, Palazzi G, Bisconte MG, Sau A, Lisi R, Giona F, Campisi S, Colarusso G, Giordano P, Boscariol G, Marktel S, Filosa A, Origa R, Murgia M, Maroni P, Gianesin B, Badalamenti L, Forni GLet al., 2018, Transfusion therapy in a multi-ethnic Sickle Cell population real-world practice. a preliminary data analysis of multicentre survey, 60th Annual Meeting of the American-Society-of-Hematology (ASH), Publisher: American Society of Hematology, ISSN: 0006-4971

Conference paper

Roth GA, Abate D, Abate KH, Abay SM, Abbafati C, Abbasi N, Abbastabar H, Abd-Allah F, Abdela J, Abdelalim A, Abdollahpour I, Abdulkader RS, Abebe HT, Abebe M, Abebe Z, Abejie AN, Abera SF, Abil OZ, Abraha HN, Abrham AR, Abu-Raddad LJ, Accrombessi MMK, Acharya D, Adamu AA, Adebayo OM, Adedoyin RA, Adekanmbi V, Adetokunboh OO, Adhena BM, Adib MG, Admasie A, Afshin A, Agarwal G, Agesa KM, Agrawal A, Agrawal S, Ahmadi A, Ahmadi M, Ahmed MB, Ahmed S, Aichour AN, Aichour I, Aichour MTE, Akbari ME, Akinyemi RO, Akseer N, Al-Aly Z, Al-Eyadhy A, Al-Raddadi RM, Alahdab F, Alam K, Alam T, Alebel A, Alene KA, Alijanzadeh M, Alizadeh-Navaei R, Aljunid SM, Alkerwi A, Alla F, Allebeck P, Alonso J, Altirkawi K, Alvis-Guzman N, Amare AT, Aminde LN, Amini E, Ammar W, Amoako YA, Anber NH, Andrei CL, Androudi S, Animut MD, Anjomshoa M, Ansari H, Ansha MG, Antonio CAT, Anwari P, Aremu O, Ärnlöv J, Arora A, Arora M, Artaman A, Aryal KK, Asayesh H, Asfaw ET, Ataro Z, Atique S, Atre SR, Ausloos M, Avokpaho EFGA, Awasthi A, Quintanilla BPA, Ayele Y, Ayer R, Azzopardi PS, Babazadeh A, Bacha U, Badali H, Badawi A, Bali AG, Ballesteros KE, Banach M, Banerjee K, Bannick MS, Banoub JAM, Barboza MA, Barker-Collo SL, Bärnighausen TW, Barquera S, Barrero LH, Bassat Q, Basu S, Baune BT, Baynes HW, Bazargan-Hejazi S, Bedi N, Beghi E, Behzadifar M, Behzadifar M, Béjot Y, Bekele BB, Belachew AB, Belay E, Belay YA, Bell ML, Bello AK, Bennett DA, Bensenor IM, Berman AE, Bernabe E, Bernstein RS, Bertolacci GJ, Beuran M, Beyranvand T, Bhalla A, Bhattarai S, Bhaumik S, Bhutta ZA, Biadgo B, Biehl MH, Bijani A, Bikbov B, Bilano V, Bililign N, Bin Sayeed MS, Bisanzio D, Biswas T, Blacker BF, Basara BB, Borschmann R, Bosetti C, Bozorgmehr K, Brady OJ, Brant LC, Brayne C, Brazinova A, Breitborde NJK, Brenner H, Briant PS, Britton G, Brugha T, Busse R, Butt ZA, Callender CSKH, Campos-Nonato IR, Campuzano Rincon JC, Cano J, Car M, Cárdenas R, Carreras G, Carrero JJ, Carter A, Carvalho F, Castañeda-Orjuela CA, Castet al., 2018, Global, regional, and national age-sex-specific mortality for 282 causes of death in 195 countries and territories, 1980–2017: a systematic analysis for the Global Burden of Disease Study 2017, The Lancet, Vol: 392, Pages: 1736-1788, ISSN: 0140-6736

BackgroundGlobal development goals increasingly rely on country-specific estimates for benchmarking a nation's progress. To meet this need, the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2016 estimated global, regional, national, and, for selected locations, subnational cause-specific mortality beginning in the year 1980. Here we report an update to that study, making use of newly available data and improved methods. GBD 2017 provides a comprehensive assessment of cause-specific mortality for 282 causes in 195 countries and territories from 1980 to 2017.MethodsThe causes of death database is composed of vital registration (VR), verbal autopsy (VA), registry, survey, police, and surveillance data. GBD 2017 added ten VA studies, 127 country-years of VR data, 502 cancer-registry country-years, and an additional surveillance country-year. Expansions of the GBD cause of death hierarchy resulted in 18 additional causes estimated for GBD 2017. Newly available data led to subnational estimates for five additional countries—Ethiopia, Iran, New Zealand, Norway, and Russia. Deaths assigned International Classification of Diseases (ICD) codes for non-specific, implausible, or intermediate causes of death were reassigned to underlying causes by redistribution algorithms that were incorporated into uncertainty estimation. We used statistical modelling tools developed for GBD, including the Cause of Death Ensemble model (CODEm), to generate cause fractions and cause-specific death rates for each location, year, age, and sex. Instead of using UN estimates as in previous versions, GBD 2017 independently estimated population size and fertility rate for all locations. Years of life lost (YLLs) were then calculated as the sum of each death multiplied by the standard life expectancy at each age. All rates reported here are age-standardised.FindingsAt the broadest grouping of causes of death (Level 1), non-communicable diseases (NCDs) comprised the greatest f

Journal article

Lobitz S, Telfer P, Cela E, Allaf B, Angastiniotis M, Backman Johansson C, Badens C, Bento C, Bouva MJ, Canatan D, Charlton M, Coppinger C, Daniel Y, de Montalembert M, Ducoroy P, Dulin E, Fingerhut R, Frömmel C, García-Morin M, Gulbis B, Holtkamp U, Inusa B, James J, Kleanthous M, Klein J, Kunz JB, Langabeer L, Lapouméroulie C, Marcao A, Marín Soria JL, McMahon C, Ohene-Frempong K, Périni J-M, Piel FB, Russo G, Sainati L, Schmugge M, Streetly A, Tshilolo L, Turner C, Venturelli D, Vilarinho L, Yahyaoui R, Elion J, Colombatti R, with the endorsement of EuroBloodNet, the European Reference Network in Rare Haematological Diseaseset al., 2018, Newborn screening for sickle cell disease in Europe: recommendations from a Pan‐European Consensus Conference, British Journal of Haematology, Vol: 183, Pages: 648-660, ISSN: 1365-2141

Sickle Cell Disease (SCD) is an increasing global health problem and presents significant challenges to European health care systems. Newborn screening (NBS) for SCD enables early initiation of preventive measures and has contributed to a reduction in childhood mortality from SCD. Policies and methodologies for NBS vary in different countries, and this might have consequences for the quality of care and clinical outcomes for SCD across Europe. A two-day Pan-European consensus conference was held in Berlin in April 2017 in order to appraise the current status of NBS for SCD and to develop consensus-based statements on indications and methodology for NBS for SCD in Europe. More than 50 SCD experts from 13 European countries participated in the conference. The aim of this paper is to summarise the discussions and present consensus recommendations which can be used to support development of NBS programmes in European countries where they do not yet exist, and to review existing programmes.

Journal article

GBD 2016 Healthcare Access and Quality Collaborators, 2018, Measuring performance on the Healthcare Access and Quality Index for 195 countries and territories and selected subnational locations: a systematic analysis from the Global Burden of Disease Study 2016, Lancet, Vol: 391, Pages: 2236-2271, ISSN: 0140-6736

BACKGROUND: A key component of achieving universal health coverage is ensuring that all populations have access to quality health care. Examining where gains have occurred or progress has faltered across and within countries is crucial to guiding decisions and strategies for future improvement. We used the Global Burden of Diseases, Injuries, and Risk Factors Study 2016 (GBD 2016) to assess personal health-care access and quality with the Healthcare Access and Quality (HAQ) Index for 195 countries and territories, as well as subnational locations in seven countries, from 1990 to 2016. METHODS: Drawing from established methods and updated estimates from GBD 2016, we used 32 causes from which death should not occur in the presence of effective care to approximate personal health-care access and quality by location and over time. To better isolate potential effects of personal health-care access and quality from underlying risk factor patterns, we risk-standardised cause-specific deaths due to non-cancers by location-year, replacing the local joint exposure of environmental and behavioural risks with the global level of exposure. Supported by the expansion of cancer registry data in GBD 2016, we used mortality-to-incidence ratios for cancers instead of risk-standardised death rates to provide a stronger signal of the effects of personal health care and access on cancer survival. We transformed each cause to a scale of 0-100, with 0 as the first percentile (worst) observed between 1990 and 2016, and 100 as the 99th percentile (best); we set these thresholds at the country level, and then applied them to subnational locations. We applied a principal components analysis to construct the HAQ Index using all scaled cause values, providing an overall score of 0-100 of personal health-care access and quality by location over time. We then compared HAQ Index levels and trends by quintiles on the Socio-demographic Index (SDI), a summary measure of overall development. As derive

Journal article

Gregory K, Piel FBJ, Elliott V, 2018, Sickle cell disease, Nature Reviews Disease Primers, Vol: 4, ISSN: 2056-676X

Sickle cell disease (SCD) is a group of inherited disorders caused by mutations in HBB, which encodes haemoglobin subunit β. The incidence is estimated to be between 300,000 and 400,000 neonates globally each year, the majority in sub-Saharan Africa. Haemoglobin molecules that include mutant sickle β-globin subunits can polymerize; erythrocytes that contain mostly haemoglobin polymers assume a sickled form and are prone to haemolysis. Other pathophysiological mechanisms that contribute to the SCD phenotype are vaso-occlusion and activation of the immune system. SCD is characterized by a remarkable phenotypic complexity. Common acute complications are acute pain events, acute chest syndrome and stroke; chronic complications (including chronic kidney disease) can damage all organs. Hydroxycarbamide, blood transfusions and haematopoietic stem cell transplantation can reduce the severity of the disease. Early diagnosis is crucial to improve survival, and universal newborn screening programmes have been implemented in some countries but are challenging in low-income, high-burden settings.

Journal article

Piel FBJ, Williams TN, 2017, Subphenotypes of sickle cell disease in Africa, Blood, Vol: 130, Pages: 2157-2158, ISSN: 1528-0020

Journal article

Piel FBJ, Rigano P, De Francesci L, Sainati L, Piga A, Cappellini MD, Fidone C, Masera N, Palazzi G, Gianesin B, Forni GLet al., 2017, Real-life experience with hydroxyurea in sickle cell disease: A multicenter study in a cohort of patients with heterogeneous descent, Blood Cells, Molecules and Diseases, Vol: 69, Pages: 82-89, ISSN: 1079-9796

We conducted the first nation-wide cohort study of sickle cell disease (SCD) in Italy, a Southern European country exposed to intense recent flux migration from endemic areas for SCD. We evaluate the impact of hydroxyurea on a total of 652 pediatric and adult patients from 33 Reference Centers for SCD (mean age 24.5 ± 15 years, 51.4% males). Hydroxyurea median treatment duration was 7 years (range: < 1 year to 29 years) at a mean therapeutic dose of 18 ± 4.7 mg/kg/day. Hydroxyurea was associated with a significant increase in mean total and fetal hemoglobin and a significant decrease in mean hemoglobin S, white blood and platelet counts, and lactate dehydrogenase levels. Hydroxyurea was associated with a significant reduction in the incidence of acute chest syndrome (− 29.3%, p < 0.001), vaso-occlusive crisis (− 34.1%, p < 0.001), hospitalization (− 53.2%, p < 0.001), and bone necrosis (− 6.9%, p < 0.001). New silent cerebral infarction (SCI) occurred during treatment (+ 42.4%, p < 0.001) but not stroke (+ 0.5%, p = 0.572). These observations were generally consistent upon stratification for age, descent (Caucasian or African), genotype (βS/βS, βS/β0 or βS/β+) and duration of treatment (< or ≥ 10 years). There were no new safety concerns observed compared to those commonly reported in the literature. Our study, conducted on a large population of patients with different descent and compound state supports the benefits of hydroxyurea therapy as a treatment option. Registered at clinical trials.gov (NCT02709681).

Journal article

Wang H, Abajobir A, Abate KH, Rawaf S, Murray CGL, GBD 2016 Mortality Collaboratorset al., 2017, Global, regional, and national under-5 mortality, adultmortality, age-specific mortality, and life expectancy,1970–2016: a systematic analysis for the Global Burden ofDisease Study 2016, The Lancet, Vol: 390, Pages: 1084-1150, ISSN: 0140-6736

BackgroundDetailed assessments of mortality patterns, particularly age-specific mortality, represent a crucial input that enables health systems to target interventions to specific populations. Understanding how all-cause mortality has changed with respect to development status can identify exemplars for best practice. To accomplish this, the Global Burden of Diseases, Injuries, and Risk Factors Study 2016 (GBD 2016) estimated age-specific and sex-specific all-cause mortality between 1970 and 2016 for 195 countries and territories and at the subnational level for the five countries with a population greater than 200 million in 2016.MethodsWe have evaluated how well civil registration systems captured deaths using a set of demographic methods called death distribution methods for adults and from consideration of survey and census data for children younger than 5 years. We generated an overall assessment of completeness of registration of deaths by dividing registered deaths in each location-year by our estimate of all-age deaths generated from our overall estimation process. For 163 locations, including subnational units in countries with a population greater than 200 million with complete vital registration (VR) systems, our estimates were largely driven by the observed data, with corrections for small fluctuations in numbers and estimation for recent years where there were lags in data reporting (lags were variable by location, generally between 1 year and 6 years). For other locations, we took advantage of different data sources available to measure under-5 mortality rates (U5MR) using complete birth histories, summary birth histories, and incomplete VR with adjustments; we measured adult mortality rate (the probability of death in individuals aged 15–60 years) using adjusted incomplete VR, sibling histories, and household death recall. We used the U5MR and adult mortality rate, together with crude death rate due to HIV in the GBD model life table system, to

Journal article

Piel FB, Steinberg MH, Rees DC, 2017, Sickle Cell Disease., N Engl J Med, Vol: 377, Pages: 305-305

Journal article

Piel FB, Steinberg M, Rees D, 2017, Sickle Cell Disease (Reply Letter), New England Journal of Medicine, Vol: 377, Pages: 302-303, ISSN: 0028-4793

Journal article

Piel FBJ, Steinberg MH, Rees DC, 2017, Sickle cell disease, New England Journal of Medicine, Vol: 376, Pages: 1561-1573, ISSN: 0028-4793

Sickle cell disease is an increasing global health problem. Estimates suggest that every year approximately 300,000 infants are born with sickle cell anemia, which is defined as homozygosity for the sickle hemoglobin (HbS) gene (i.e., for a missense mutation [Glu6Val, rs334] in the β-globin gene [HBB]) and that this number could rise to 400,000 by 2050.1 Although early diagnosis, penicillin prophylaxis, blood transfusion, transcranial Doppler imaging, hydroxyurea, and hematopoietic stem-cell transplantation can dramatically improve survival and quality of life for patients with sickle cell disease, our understanding of the role of genetic and nongenetic factors in explaining the remarkable phenotypic diversity of this mendelian disease is still limited. Better prediction of the severity of sickle cell disease could lead to more precise treatment and management. Beyond well-known modifiers of disease severity, such as fetal hemoglobin (HbF) levels and α-thalassemia, other genetic variants might affect specific subphenotypes. Similarly, although the influence of altitude and temperature has long been reflected in advice to patients with sickle cell disease, recent studies of nongenetic factors, including climate and air quality, suggest more complex associations between environmental factors and clinical complications.2 New treatments and management strategies accounting for these genetic and nongenetic factors could substantially and rapidly improve the quality of life and reduce health care costs for patients with sickle cell disease.

Journal article

Piel FB, Tewari S, Brousse V, Analitis A, Font A, Menzel S, Chakravorty S, Thein SL, Inusa B, Telfer P, de Montalembert M, Fuller G, Katsouyanni K, Rees Det al., 2017, Associations between environmental factors and hospital admissions for sickle cell disease, Haematologica, Vol: 102, Pages: 666-675, ISSN: 0390-6078

Sickle cell disease is an increasing global health burden. This inherited disease is characterized by a remarkable phenotypic heterogeneity, which can only partly be explained by genetic factors. Environmental factors are likely to play an important role but studies of their impact on disease severity are limited and their results are often inconsistent. This study investigated associations between a range of environmental factors and hospital admissions of young patients with sickle cell disease in London and in Paris between 2008 and 2012. Specific analyses were conducted for subgroups of patients with different genotypes and for the main reasons for admissions. Generalized additive models and distributed lag non-linear models were used to assess the magnitude of the associations and to calculate relative risks. Some environmental factors significantly influence the numbers of hospital admissions of children with sickle cell disease, although the associations identified are complicated. Our study suggests that meteorological factors are more likely to be associated with hospital admissions for sickle cell disease than air pollutants. It confirms previous reports of risks associated with wind speed (risk ratio: 1.06/stan-dard deviation; 95% confidence interval: 1.00-1.12) and also with rainfall (1.06/standard deviation; 95% confidence interval: 1.01-1.12). Maximum atmospheric pressure was found to be a protective factor (0.93/standard deviation; 95% confidence interval: 0.88-0.99). Weak or no associations were found with temperature. Divergent associations were identified for different genotypes or reasons for admissions, which could partly explain the lack of consistency in earlier studies. Advice to patients with sickle cell disease usually includes avoiding a range of environmental conditions that are believed to trigger acute complications, including extreme temperatures and high altitudes. Scientific evidence to support such advice is limited and sometimes con

Journal article

Premawardhena A, Allen A, Piel FBJ, Fisher C, Perera L, Rodrigo R, Goonathilaka G, Ramees L, Peto T, Olivieri N, Weatherall DJet al., 2016, The evolutionary and clinical implilcations of the uneven distribution of the frequency of the inherited haemoglobin variants over short geographical distances, British Journal of Haematology, Vol: 176, Pages: 475-484, ISSN: 1365-2141

Studies of the frequency of heterozygous carriers for common inheriteddiseases of haemoglobin in over 7500 adolescent children in 25 districts inSri Lanka have disclosed a highly significant variation over very short geo-graphical distances. A further analysis of these findings, including theirrelationship to the past frequency and distribution of malaria, climatic vari-ation, altitude, ethnic origin and consanguinity rates, have provided evi-dence regarding the evolutionary basis for the variable distribution of theseconditions over short distances. It is likely that the complex interplaybetween malaria and the environment, together with related ethnic andsocial issues, exists in many countries across the tropical belt. Hence, theseobservations emphasise the importance of micromapping heterozygote dis-tributions in high-frequency countries in order to define their true burdenand the facilities required for the prevention and management of thehomozygous and compound heterozygous disorders that result from theirinteraction.

Journal article

Wang H, Naghavi M, Allen C, Barber RM, Bhutta ZA, Carter A, Casey DC, Charlson FJ, Chen AZ, Coates MM, Coggeshall M, Dandona L, Dicker DJ, Erskine HE, Ferrari AJ, Fitzmaurice C, Foreman K, Forouzanfar MH, Fraser MS, Pullman N, Gething PW, Goldberg EM, Graetz N, Haagsma JA, Hay SI, Huynh C, Johnson C, Kassebaum NJ, Kinfu Y, Kulikoff XR, Kutz M, Kyu HH, Larson HJ, Leung J, Liang X, Lim SS, Lind M, Lozano R, Marquez N, Mensah GA, Mikesell J, Mokdad AH, Mooney MD, Nguyen G, Nsoesie E, Pigott DM, Pinho C, Roth GA, Salomon JA, Sandar L, Silpakit N, Sligar A, Sorensen RJD, Stanaway J, Steiner C, Teeple S, Thomas BA, Troeger C, VanderZanden A, Vollset SE, Wanga V, Whiteford HA, Wolock T, Zoeckler L, Abate KH, Abbafati C, Abbas KM, Abd-Allah F, Abera SF, Abreu DMX, Abu-Raddad LJ, Abyu GY, Achoki T, Adelekan AL, Ademi Z, Adou AK, Adsuar JC, Afanvi KA, Afshin A, Agardh EE, Agarwal A, Agrawal A, Kiadaliri AA, Ajala ON, Akanda AS, Akinyemi RO, Akinyemiju TF, Akseer N, Al Lami FH, Alabed S, Al-Aly Z, Alam K, Alam NKM, Alasfoor D, Aldhahri SF, Aldridge RW, Alegretti MA, Aleman AV, Alemu ZA, Alexander LT, Alhabib S, Ali R, Alkerwi A, Alla F, Allebeck P, Al-Raddadi R, Alsharif U, Altirkawi KA, Martin EA, Alvis-Guzman N, Amare AT, Amegah AK, Ameh EA, Amini H, Ammar W, Amrock SM, Andersen HH, Anderson B, Anderson GM, Antonio CAT, Aregay AF, Arnlov J, Arsenijevic VSA, Al A, Asayesh H, Asghar RJ, Atique S, Arthur Avokpaho EFG, Awasthi A, Azzopardi P, Bacha U, Badawi A, Bahit MC, Balakrishnan K, Banerjee A, Barac A, Barker-Collo SL, Barnighausen T, Barregard L, Barrero LH, Basu A, Basu S, Bayou YT, Bazargan-Hejazi S, Beardsley J, Bedi N, Beghi E, Belay HA, Bell B, Bell ML, Bello AK, Bennett DA, Bensenor IM, Berhane A, Bernabe E, Betsu BD, Beyene AS, Bhala N, Bhalla A, Biadgilign S, Bikbov B, Bin Abdulhak AA, Biroscak BJ, Biryukov S, Bjertness E, Blore JD, Blosser CD, Bohensky MA, Borschmann R, Bose D, Bourne RRA, Brainin M, Brayne CEG, Brazinova A, Breitborde NJK, Brenner H, Brewer JD Bet al., 2016, Global, regional, and national life expectancy, all-cause mortality, and cause-specific mortality for 249 causes of death, 1980-2015: a systematic analysis for the Global Burden of Disease Study 2015, Lancet, Vol: 388, Pages: 1459-1544, ISSN: 0140-6736

BackgroundImproving survival and extending the longevity of life for all populations requires timely, robust evidence on local mortality levels and trends. The Global Burden of Disease 2015 Study (GBD 2015) provides a comprehensive assessment of all-cause and cause-specific mortality for 249 causes in 195 countries and territories from 1980 to 2015. These results informed an in-depth investigation of observed and expected mortality patterns based on sociodemographic measures.MethodsWe estimated all-cause mortality by age, sex, geography, and year using an improved analytical approach originally developed for GBD 2013 and GBD 2010. Improvements included refinements to the estimation of child and adult mortality and corresponding uncertainty, parameter selection for under-5 mortality synthesis by spatiotemporal Gaussian process regression, and sibling history data processing. We also expanded the database of vital registration, survey, and census data to 14 294 geography–year datapoints. For GBD 2015, eight causes, including Ebola virus disease, were added to the previous GBD cause list for mortality. We used six modelling approaches to assess cause-specific mortality, with the Cause of Death Ensemble Model (CODEm) generating estimates for most causes. We used a series of novel analyses to systematically quantify the drivers of trends in mortality across geographies. First, we assessed observed and expected levels and trends of cause-specific mortality as they relate to the Socio-demographic Index (SDI), a summary indicator derived from measures of income per capita, educational attainment, and fertility. Second, we examined factors affecting total mortality patterns through a series of counterfactual scenarios, testing the magnitude by which population growth, population age structures, and epidemiological changes contributed to shifts in mortality. Finally, we attributed changes in life expectancy to changes in cause of death. We documented each step of the GB

Journal article

Kassebaum NJ, Arora M, Barber RM, Bhutta ZA, Brown J, Carter A, Casey DC, Charlson FJ, Coates MM, Coggeshall M, Cornaby L, Dandona L, Dicker DJ, Erskine HE, Ferrari AJ, Fitzmaurice C, Foreman K, Forouzanfar MH, Fullman N, Gething PW, Goldberg EM, Graetz N, Haagsma JA, Johnson CO, Kemmer L, Khalil IA, Kinfu Y, Kutz MJ, Kyu HH, Leung J, Liang X, Lim SS, Lozano R, Mensah GA, Mikesell J, Mokdad AH, Mooney MD, Naghavi M, Nguyen G, Nsoesie E, Pigott DM, Pinho C, Rankin Z, Reinig N, Salomon JA, Sandar L, Smith A, Sorensen RJD, Stanaway J, Steiner C, Teeple S, Thomas BA, Troeger C, VanderZanden A, Wagner JA, Wanga V, Whiteford HA, Zhou M, Zoeckler L, Abajobir AA, Abate KH, Abbafati C, Abbas KM, Abd-Allah F, Abraham B, Abubakar I, Abu-Raddad LJ, Abu-Rmeileh NME, Achoki T, Ackerman IN, Adebiyi AO, Adedeji IA, Adsuar JC, Afanvi KA, Afshin A, Agardh EE, Agarwal A, Agarwal SK, Ahmed MB, Kiadaliri AA, Ahmadieh H, Akseer N, Al-Aly Z, Alam K, Alam NKM, Aldhahri SF, Alegretti MA, Aleman AV, Alemu ZA, Alexander LT, Ali R, Alkerwi A, Alla F, Allebeck P, Alsharif U, Altirkawi KA, Martin EA, Alvis-Guzman N, Amare AT, Amberbir A, Amegah AK, Amini H, Ammar W, Amrock SM, Anderson GM, Anderson BO, Antonio CAT, Anwari P, Ärnlöv J, Arsenijevic VSA, Artaman A, Asayesh H, Asghar RJ, Avokpaho EFGA, Awasthi A, Quintanilla BPA, Azzopardi P, Bacha U, Badawi A, Balakrishnan K, Banerjee A, Barac A, Barker-Collo SL, Bärnighausen T, Barregard L, Barrero LH, Basu S, Bayou TA, Beardsley J, Bedi N, Beghi E, Bell B, Bell ML, Benjet C, Bennett DA, Bensenor IM, Berhane A, Bernabé E, Betsu BD, Beyene AS, Bhala N, Bhansali A, Bhatt S, Biadgilign S, Bienhoff K, Bikbov B, Abdulhak AAB, Bisanzio D, Bjertness E, Blore JD, Borschmann R, Boufous S, Bourne RRA, Brainin M, Brazinova A, Breitborde NJK, Brugha TS, Buchbinder R, Buckle GC, Butt ZA, Calabria B, Campos-Nonato IR, Campuzano JC, Carabin H, Carapetis JR, Cárdenas R, Carrero JJ, Castañeda-Orjuela CA, Rivas JC, Catalá-López F, Cavalleri F, Chang J, Chiang PP Cet al., 2016, Global, regional, and national disability-adjusted life-years (DALYs) for 315 diseases and injuries and healthy life expectancy (HALE), 1990–2015: a systematic analysis for the Global Burden of Disease Study 2015, The Lancet, Vol: 388, Pages: 1603-1658, ISSN: 0140-6736

SummaryBackground Healthy life expectancy (HALE) and disability-adjusted life-years (DALYs) provide summary measures of health across geographies and time that can inform assessments of epidemiological patterns and health system performance, help to prioritise investments in research and development, and monitor progress toward the Sustainable Development Goals (SDGs). We aimed to provide updated HALE and DALYs for geographies worldwide and evaluate how disease burden changes with development. Methods We used results from the Global Burden of Diseases, Injuries, and Risk Factors Study 2015 (GBD 2015) for all-cause mortality, cause-specific mortality, and non-fatal disease burden to derive HALE and DALYs by sex for 195 countries and territories from 1990 to 2015. We calculated DALYs by summing years of life lost (YLLs) and years of life lived with disability (YLDs) for each geography, age group, sex, and year. We estimated HALE using the Sullivan method, which draws from age-specific death rates and YLDs per capita. We then assessed how observed levels of DALYs and HALE differed from expected trends calculated with the Socio-demographic Index (SDI), a composite indicator constructed from measures of income per capita, average years of schooling, and total fertility rate. Findings Total global DALYs remained largely unchanged from 1990 to 2015, with decreases in communicable, neonatal, maternal, and nutritional (Group 1) disease DALYs offset by increased DALYs due to non-communicable diseases (NCDs). Much of this epidemiological transition was caused by changes in population growth and ageing, but it was accelerated by widespread improvements in SDI that also correlated strongly with the increasing importance of NCDs. Both total DALYs and age-standardised DALY rates due to most Group 1 causes significantly decreased by 2015, and although total burden climbed for the majority of NCDs, age-standardised DALY rates due to NCDs declined. Nonetheless, age-standardised DALY

Journal article

Forouzanfar MH, Afshin A, Alexander LT, Anderson HR, Bhutta ZA, Biryukov S, Brauer M, Burnett R, Cercy K, Charlson FJ, Cohen AJ, Dandona L, Estep K, Ferrari AJ, Frostad JJ, Fullman N, Gething PW, Godwin WW, Griswold M, Kinfu Y, Kyu HH, Larson HJ, Liang X, Lim SS, Liu PY, Lopez AD, Lozano R, Marczak L, Mensah GA, Mokdad AH, Moradi-Lakeh M, Naghavi M, Neal B, Reitsma MB, Roth GA, Salomon JA, Sur PJ, Vos T, Wagner JA, Wang H, Zhao Y, Zhou M, Aasvang GM, Abajobir AA, Abate KH, Abbafati C, Abbas KM, Abd-Allah F, Abdulle AM, Abera SF, Abraham B, Abu-Raddad LJ, Abyu GY, Adebiyi AO, Adedeji IA, Ademi Z, Adou AK, Adsuar JC, Agardh EE, Agarwal A, Agrawal A, Kiadaliri AA, Ajala ON, Akinyemiju TF, Al-Aly Z, Alam K, Alam NKM, Aldhahri SF, Aldridge RW, Alemu ZA, Ali R, Alkerwi A, Alla F, Allebeck P, Alsharif U, Altirkawi KA, Martin EA, Alvis-Guzman N, Amare AT, Amberbir A, Amegah AK, Amini H, Ammar W, Amrock SM, Andersen HH, Anderson BO, Antonio CAT, Anwari P, Ärnlöv J, Artaman A, Asayesh H, Asghar RJ, Assadi R, Atique S, Avokpaho EFGA, Awasthi A, Quintanilla BPA, Azzopardi P, Bacha U, Badawi A, Bahit MC, Balakrishnan K, Barac A, Barber RM, Barker-Collo SL, Bärnighausen T, Barquera S, Barregard L, Barrero LH, Basu S, Batis C, Bazargan-Hejazi S, Beardsley J, Bedi N, Beghi E, Bell ML, Bello AK, Bennett DA, Bensenor IM, Berhane A, Bernabé E, Betsu BD, Beyene AS, Bhala N, Bhansali A, Bhatt S, Biadgilign S, Bikbov B, Bisanzio D, Bjertness E, Blore JD, Borschmann R, Boufous S, Bourne RRA, Brainin M, Brazinova A, Breitborde NJK, Brenner H, Broday DM, Brugha TS, Brunekreef B, Butt ZA, Cahill LE, Calabria B, Campos-Nonato IR, Cárdenas R, Carpenter DO, Casey DC, Castañeda-Orjuela CA, Rivas JC, Castro RE, Catalá-López F, Chang J, Chiang PP, Chibalabala M, Chimed-Ochir O, Chisumpa VH, Chitheer AA, Choi JJ, Christensen H, Christopher DJ, Ciobanu LG, Coates MM, Colquhoun SM, Cooper LT, Cooperrider K, Cornaby L, Cortinovis M, Crump JA, Cuevas-Nasu L, Damasceno A, Dandona R, Darby SC, Dargan PIet al., 2016, Global, regional, and national comparative risk assessment of 79 behavioural, environmental and occupational, and metabolic risks or clusters of risks, 1990-2015: a systematic analysis for the Global Burden of Disease Study 2015, The Lancet, Vol: 388, Pages: 1659-1724, ISSN: 0140-6736

SummaryBackground The Global Burden of Diseases, Injuries, and Risk Factors Study 2015 provides an up-to-date synthesis of the evidence for risk factor exposure and the attributable burden of disease. By providing national and subnational assessments spanning the past 25 years, this study can inform debates on the importance of addressing risks in context. Methods We used the comparative risk assessment framework developed for previous iterations of the Global Burden of Disease Study to estimate attributable deaths, disability-adjusted life-years (DALYs), and trends in exposure by age group, sex, year, and geography for 79 behavioural, environmental and occupational, and metabolic risks or clusters of risks from 1990 to 2015. This study included 388 risk-outcome pairs that met World Cancer Research Fund-defined criteria for convincing or probable evidence. We extracted relative risk and exposure estimates from randomised controlled trials, cohorts, pooled cohorts, household surveys, census data, satellite data, and other sources. We used statistical models to pool data, adjust for bias, and incorporate covariates. We developed a metric that allows comparisons of exposure across risk factors—the summary exposure value. Using the counterfactual scenario of theoretical minimum risk level, we estimated the portion of deaths and DALYs that could be attributed to a given risk. We decomposed trends in attributable burden into contributions from population growth, population age structure, risk exposure, and risk-deleted cause-specific DALY rates. We characterised risk exposure in relation to a Socio-demographic Index (SDI). Findings Between 1990 and 2015, global exposure to unsafe sanitation, household air pollution, childhood underweight, childhood stunting, and smoking each decreased by more than 25%. Global exposure for several occupational risks, high body-mass index (BMI), and drug use increased by more than 25% over the same period. All risks jointly evaluated

Journal article

Vos T, Allen C, Arora M, Barber RM, Bhutta ZA, Brown A, Carter A, Casey DC, Charlson FJ, Chen AZ, Coggeshall M, Cornaby L, Dandona L, Dicker DJ, Dilegge T, Erskine HE, Ferrari AJ, Fitzmaurice C, Fleming T, Forouzanfar MH, Fullman N, Gething PW, Goldberg EM, Graetz N, Haagsma JA, Johnson CO, Kassebaum NJ, Kawashima T, Kemmer L, Khalil IA, Kinfu Y, Kyu HH, Leung J, Liang X, Lim SS, Lopez AD, Lozano R, Marczak L, Mensah GA, Mokdad AH, Naghavi M, Nguyen G, Nsoesie E, Olsen H, Pigott DM, Pinho C, Rankin Z, Reinig N, Salomon JA, Sandar L, Smith A, Stanaway J, Steiner C, Teeple S, Thomas BA, Troeger C, Wagner JA, Wang H, Wanga V, Whiteford HA, Zoeckler L, Abajobir AA, Abate KH, Abbafati C, Abbas KM, Abd-Allah F, Abraham B, Abubakar I, Abu-Raddad LJ, Abu-Rmeileh NME, Ackerman IN, Adebiyi AO, Ademi Z, Adou AK, Afanvi KA, Agardh EE, Agarwal A, Kiadaliri AA, Ahmadieh H, Ajala ON, Akinyemi RO, Akseer N, Al-Aly Z, Alam K, Alam NKM, Aldhahri SF, Alegretti MA, Alemu ZA, Alexander LT, Alhabib S, Ali R, Alkerwi A, Alla F, Allebeck P, Al-Raddadi R, Alsharif U, Altirkawi KA, Alvis-Guzman N, Amare AT, Amberbir A, Amini H, Ammar W, Amrock SM, Andersen HH, Anderson GM, Anderson BO, Antonio CAT, Aregay AF, Ärnlöv J, Artaman A, Asayesh H, Assadi R, Atique S, Avokpaho EFGA, Awasthi A, Quintanilla BPA, Azzopardi P, Bacha U, Badawi A, Balakrishnan K, Banerjee A, Barac A, Barker-Collo SL, Bärnighausen T, Barregard L, Barrero LH, Basu A, Bazargan-Hejazi S, Bell B, Bell ML, Bennett DA, Bensenor IM, Benzian H, Berhane A, Bernabé E, Betsu BD, Beyene AS, Bhala N, Bhatt S, Biadgilign S, Bienhoff K, Bikbov B, Biryukov S, Bisanzio D, Bjertness E, Blore J, Borschmann R, Boufous S, Brainin M, Brazinova A, Breitborde NJK, Brown J, Buchbinder R, Buckle GC, Butt ZA, Calabria B, Campos-Nonato IR, Campuzano JC, Carabin H, Cárdenas R, Carpenter DO, Carrero JJ, Castañeda-Orjuela CA, Rivas JC, Catalá-López F, Chang J, Chiang PP, Chibueze CE, Chisumpa VH, Choi JJ, Chowdhury R, Christensen H, Christopher DJ, Ciobet al., 2016, Global, regional, and national incidence, prevalence, and years lived with disability for 310 diseases and injuries, 1990–2015: a systematic analysis for the Global Burden of Disease Study 2015, The Lancet, Vol: 388, Pages: 1545-1602, ISSN: 0140-6736

Background Non-fatal outcomes of disease and injury increasingly detract from the ability of the world's population to live in full health, a trend largely attributable to an epidemiological transition in many countries from causes affecting children, to non-communicable diseases (NCDs) more common in adults. For the Global Burden of Diseases, Injuries, and Risk Factors Study 2015 (GBD 2015), we estimated the incidence, prevalence, and years lived with disability for diseases and injuries at the global, regional, and national scale over the period of 1990 to 2015. Methods We estimated incidence and prevalence by age, sex, cause, year, and geography with a wide range of updated and standardised analytical procedures. Improvements from GBD 2013 included the addition of new data sources, updates to literature reviews for 85 causes, and the identification and inclusion of additional studies published up to November, 2015, to expand the database used for estimation of non-fatal outcomes to 60 900 unique data sources. Prevalence and incidence by cause and sequelae were determined with DisMod-MR 2.1, an improved version of the DisMod-MR Bayesian meta-regression tool first developed for GBD 2010 and GBD 2013. For some causes, we used alternative modelling strategies where the complexity of the disease was not suited to DisMod-MR 2.1 or where incidence and prevalence needed to be determined from other data. For GBD 2015 we created a summary indicator that combines measures of income per capita, educational attainment, and fertility (the Socio-demographic Index [SDI]) and used it to compare observed patterns of health loss to the expected pattern for countries or locations with similar SDI scores. Findings We generated 9·3 billion estimates from the various combinations of prevalence, incidence, and YLDs for causes, sequelae, and impairments by age, sex, geography, and year. In 2015, two causes had acute incidences in excess of 1 billion: upper respiratory infections (

Journal article

GBD 2015 Collaborators, Piel FBJ, 2016, Measuring the health-related Sustainable Development Goals in 188 countries: a baseline analysis from the Global Burden of Disease Study 2015, The Lancet, Vol: 388, Pages: 1813-1850, ISSN: 0140-6736

BackgroundIn September, 2015, the UN General Assembly established the Sustainable Development Goals (SDGs). The SDGs specify 17 universal goals, 169 targets, and 230 indicators leading up to 2030. We provide an analysis of 33 health-related SDG indicators based on the Global Burden of Diseases, Injuries, and Risk Factors Study 2015 (GBD 2015).MethodsWe applied statistical methods to systematically compiled data to estimate the performance of 33 health-related SDG indicators for 188 countries from 1990 to 2015. We rescaled each indicator on a scale from 0 (worst observed value between 1990 and 2015) to 100 (best observed). Indices representing all 33 health-related SDG indicators (health-related SDG index), health-related SDG indicators included in the Millennium Development Goals (MDG index), and health-related indicators not included in the MDGs (non-MDG index) were computed as the geometric mean of the rescaled indicators by SDG target. We used spline regressions to examine the relations between the Socio-demographic Index (SDI, a summary measure based on average income per person, educational attainment, and total fertility rate) and each of the health-related SDG indicators and indices.FindingsIn 2015, the median health-related SDG index was 59·3 (95% uncertainty interval 56·8–61·8) and varied widely by country, ranging from 85·5 (84·2–86·5) in Iceland to 20·4 (15·4–24·9) in Central African Republic. SDI was a good predictor of the health-related SDG index (r2=0·88) and the MDG index (r2=0·92), whereas the non-MDG index had a weaker relation with SDI (r2=0·79). Between 2000 and 2015, the health-related SDG index improved by a median of 7·9 (IQR 5·0–10·4), and gains on the MDG index (a median change of 10·0 [6·7–13·1]) exceeded that of the non-MDG index (a median change of 5·5 [2·1–8·

Journal article

Tewari S, Brousse V, Piel FB, Menzel S, Rees DCet al., 2016, Environmental determinants of severity in sickle cell disease, Haematologica, Vol: 100, Pages: 1108-1116, ISSN: 0390-6078

Sickle cell disease causes acute and chronic illness, and median life expectancy is reduced by at least 30 years in all countries, with greater reductions in low-income countries. There is a wide spectrum of severity, with some patients having no symptoms and others suffering frequent, life-changing complications. Much of this variability is unexplained, despite increasingly sophisticated genetic studies. Environmental factors, including climate, air quality, socio-economics, exercise and infection, are likely to be important, as demonstrated by the stark differences in outcomes between patients in Africa and USA/Europe. The effects of weather vary with geography, although most studies show that exposure to cold or wind increases hospital attendance with acute pain. Most of the different air pollutants are closely intercorrelated, and increasing overall levels seem to correlate with increased hospital attendance, although higher concentrations of atmospheric carbon monoxide may offer some benefit for patients with sickle cell disease. Exercise causes some adverse physiological changes, although this may be off-set by improvements in cardiovascular health. Most sickle cell disease patients live in low-income countries and socioeconomic factors are undoubtedly important, but little studied beyond documenting that sickle cell disease is associated with decreases in some measures of social status. Infections cause many of the differences in outcomes seen across the world, but again these effects are relatively poorly understood. All the above factors are likely to account for much of the pathology and variability of sickle cell disease, and large prospective studies are needed to understand these effects better.

Journal article

Kassebaum NJ, Fleming TD, Flaxman A, Phillips DE, Steiner C, Barber RM, Hanson SW, Moradi-Lakeh M, Coffeng LE, Haagsma J, Kyu HH, Graetz N, Lim SS, Vos T, Naghavi M, Murray C, Yonemoto N, Singh J, Jonas JB, Santos IS, Bensenor IM, Lotufo PA, Rana SM, Gessner BD, Ukwaja KN, Piel FB, Khader YS, Bhala N, Antonio CAT, Thrift AG, Mendoza W, Zaki MES, Larsson A, Nand D, Malekzadeh R, Li Y, Jin KY, Levy TSet al., 2016, The Global Burden of Anemia, Hematology/Oncology Clinics of North America, Vol: 30, Pages: 247-308, ISSN: 0889-8588

Journal article

Kyu HH, Pinho C, Wagner JA, Brown JC, Bertozzi-Villa A, Charlson FJ, Coffeng LE, Dandona L, Erskine HE, Ferrari AJ, Fitzmaurice C, Fleming TD, Forouzanfar MH, Graetz N, Guinovart C, Haagsma J, Higashi H, Kassebaum NJ, Larson HJ, Lim SS, Mokdad AH, Moradi-Lakeh M, Odell SV, Roth GA, Serina PT, Stanaway JD, Misganaw A, Whiteford HA, Wolock TM, Hanson SW, Abd-Allah F, Abera SF, Abu-Raddad LJ, AlBuhairan FS, Amare AT, Antonio CAT, Artaman A, Barker-Collo SL, Barrero LH, Benjet C, Bensenor IM, Bhutta ZA, Bikbov B, Brazinova A, Campos-Nonato I, Castaneda-Orjuela CA, Catala-Lopez F, Chowdhury R, Cooper C, Crump JA, Dandona R, Degenhardt L, Dellavalle RP, Dharmaratne SD, Faraon EJA, Feigin VL, Fuerst T, Geleijnse JM, Gessner BD, Gibney KB, Goto A, Gunnell D, Hankey GJ, Hay RJ, Hornberger JC, Hosgood HD, Hu G, Jacobsen KH, Jayaraman SP, Jeemon P, Jonas JB, Karch A, Kim D, Kim S, Kokubo Y, Defo BK, Bicer BK, Kumar GA, Larsson A, Leasher JL, Leung R, Li Y, Lipshultz SE, Lopez AD, Lotufo PA, Lunevicius R, Lyons RA, Majdan M, Malekzadeh R, Mashal T, Mason-Jones AJ, Melaku YA, Memish ZA, Mendoza W, Miller TR, Mock CN, Murray J, Nolte S, Oh I-H, Olusanya BO, Ortblad KF, Park E-K, Paternina Caicedo AJ, Patten SB, Patton GC, Pereira DM, Perico N, Piel FB, Polinder S, Popova S, Pourmalek F, Quistberg DA, Remuzzi G, Rodriguez A, Rojas-Rueda D, Rothenbacher D, Rothstein DH, Sanabria J, Santos IS, Schwebel DC, Sepanlou SG, Shaheen A, Shiri R, Shiue I, Skirbekk V, Sliwa K, Sreeramareddy CT, Stein DJ, Steiner TJ, Stovner LJ, Sykes BL, Tabb KM, Terkawi AS, Thomson AJ, Thorne-Lyman AL, Towbin JA, Ukwaja KN, Vasankari T, Venketasubramanian N, Vlassov VV, Vollset SE, Weiderpass E, Weintraub RG, Werdecker A, Wilkinson JD, Woldeyohannes SM, Wolfe CDA, Yano Y, Yip P, Yonemoto N, Yoon S-J, Younis MZ, Yu C, Zaki MES, Naghavi M, Murray CJL, Vos Tet al., 2016, Global and National Burden of Diseases and Injuries Among Children and Adolescents Between 1990 and 2013 Findings From the Global Burden of Disease 2013 Study, JAMA PEDIATRICS, Vol: 170, Pages: 267-287, ISSN: 2168-6203

Journal article

Piel FB, 2016, The present and future global burden of the inherited disorders of hemoglobin, Hematology/Oncology Clinics of North America, Vol: 30, Pages: 327-341, ISSN: 1558-1977

Journal article

Piel FB, Williams TN, 2016, Sickle cell Anemia: History and Epidemiology, Sickle Cell Anemia From Basic Science to Clinical Practice, Editors: Conran, Costa, Publisher: Springer, ISBN: 9783319067124

Book chapter

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