DSpace Repository

Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000–2021: a systematic analysis from the Global Burden of Disease Study 2021

Show simple item record

dc.creator Wickramasinghe, Nuwan Darshana
dc.creator Yigit, Arzu
dc.creator Yiğit, Vahit
dc.creator Kassebaum, Nicholas J
dc.creator Murray, Christopher J L
dc.creator Naghavi, Mohsen
dc.creator Hay, Simon I
dc.creator Zheng, Peng
dc.creator Zangiabadian, Moein
dc.creator Zaman, Burhan Abdullah
dc.creator Yunusa, Ismaeel
dc.creator Wang, Cong
dc.creator Valadan Tahbaz, Sahel
dc.creator Umair, Muhammad
dc.creator Temsah, Mohamad-Hani
dc.creator Taye, Birhan Tsegaw
dc.creator Tat, Nathan Y
dc.creator Tamuzi, Jacques JL Lukenze
dc.creator Szeto, Mindy D
dc.creator Sunuwar, Dev Ram
dc.creator Suleman, Muhammad
dc.creator Singh, Jasvinder A
dc.creator Sibhat, Migbar Mekonnen
dc.creator Shetty, Adithi
dc.creator Shaker, Elaheh
dc.creator Schumacher, Austin E
dc.creator Samy, Abdallah M
dc.creator Saheb Sharif-Askari, Fatemeh
dc.creator Saeed, Umar
dc.creator Saddik, Basema
dc.creator Rezaei, Nazila
dc.creator Redwan, Elrashdy Moustafa Mohamed
dc.creator Rawaf, Salman
dc.creator Rahman, Mosiur
dc.creator Rahimi, Mehran
dc.creator Qattea, Ibrahim
dc.creator Piel, Frédéric B
dc.creator Petcu, Ionela-Roxana
dc.creator Pawar, Shrikant
dc.creator Pati, Siddhartha
dc.creator Patel, Jay
dc.creator Owolabi, Mayowa O
dc.creator Onikan, Adeyinka Omoniyi
dc.creator Okonji, Osaretin Christabel
dc.creator Nzoputam, Ogochukwu Janet
dc.creator Nzoputam, Chimezie Igwegbe
dc.creator Nwatah, Vincent Ebuka
dc.creator Nri-ezedi, Chisom Adaobi
dc.creator Noyes, Taylor
dc.creator Noubiap, Jean Jacques
dc.creator Nayak, Biswa Prakash
dc.creator Naveed, Muhammad
dc.creator Natto, Zuhair S
dc.creator Nassereldine, Hasan
dc.creator Narasimha Swamy, Sreenivas
dc.creator Nair, Tapas Sadasivan
dc.creator Mustafa, Ghulam
dc.creator Monasta, Lorenzo
dc.creator Mokdad, Ali H
dc.creator Misganaw, Awoke
dc.creator Minhas, Sonica
dc.creator Mestrovic, Tomislav
dc.creator Meresa, Haftu Asmerom
dc.creator Marasini, Bishnu P
dc.creator Majeed, Azeem
dc.creator Mahjoub, Soleiman
dc.creator Mahajan, Preetam Bhalchandra
dc.creator Lim, Stephen S
dc.creator Landires, Iván
dc.creator Krishan, Kewal
dc.creator Kompani, Farzad
dc.creator Kisa, Adnan
dc.creator Khanmohammadi, Shaghayegh
dc.creator Khan, Imteyaz A
dc.creator Kassahun, Woldeteklehaymanot Dagne
dc.creator Karaye, Ibraheem M
dc.creator Kandel, Himal
dc.creator Kalantar, Farnaz
dc.creator Joseph, Nitin
dc.creator Jha, Ravi Prakash
dc.creator Jebai, Rime
dc.creator Jayaram, Shubha
dc.creator James, Peter Bai
dc.creator Iwu, Chidozie C D
dc.creator Ikiroma, Adalia I
dc.creator Ibitoye, Segun Emmanuel
dc.creator Hassen, Mohammed Bheser
dc.creator Hassan, Treska S
dc.creator Hasani, Hamidreza
dc.creator Gupta, Vivek Kumar
dc.creator Goleij, Pouya
dc.creator Golechha, Mahaveer
dc.creator Gill, Paramjit Singh
dc.creator Ghaffari, Kazem
dc.creator Gardner, William M
dc.creator Force, Lisa M
dc.creator Fatehizadeh, Ali
dc.creator Fagbamigbe, Adeniyi Francis
dc.creator Eyawo, Oghenowede
dc.creator Etaee, Farshid
dc.creator Esezobor, Christopher Imokhuede
dc.creator Elmonem, Mohamed A
dc.creator Elhadi, Muhammed
dc.creator Dongarwar, Deepa
dc.creator Djalalinia, Shirin
dc.creator Diaz, Daniel
dc.creator Dhimal, Meghnath
dc.creator Demisse, Fitsum Wolde
dc.creator Dang, Anh Kim
dc.creator Danaei, Bardia
dc.creator Dai, Xiaochen
dc.creator Dadras, Omid
dc.creator Chu, Dinh-Toi
dc.creator Chattu, Vijay Kumar
dc.creator Butt, Muhammad Hammad
dc.creator Boloor, Archith
dc.creator Bitaraf, Saeid
dc.creator Bishai, Jessica Devin
dc.creator Bilalaga, Mariah Malak
dc.creator Bhutta, Zulfiqar A
dc.creator Bhat, Ajay Nagesh
dc.creator Bhardwaj, Prarthna V
dc.creator Bhardwaj, Pankaj
dc.creator Bhagavathula, Akshaya Srikanth
dc.creator Bensenor, Isabela M
dc.creator Bedane, Deriba Abera
dc.creator Barrow, Amadou
dc.creator Bardhan, Mainak
dc.creator Banakar, Morteza
dc.creator Banach, Maciej
dc.creator Bagherieh, Sara
dc.creator Badiye, Ashish D
dc.creator Atout, Maha Moh'd Wahbi
dc.creator Arulappan, Judie
dc.creator Aravkin, Aleksandr Y
dc.creator Amu, Hubert
dc.creator AlRyalat, Saif Aldeen S
dc.creator Ahmad, Aqeel
dc.creator Ahinkorah, Bright Opoku
dc.creator Addo, Isaac Yeboah
dc.creator Abu-Zaid, Ahmed
dc.creator Abu-Gharbieh, Eman
dc.creator Aboye, Melka Biratu
dc.creator Aboagye, Richard Gyan
dc.creator Hagins, Hailey
dc.creator Fuller, Kia
dc.creator Wilner, Lauren B
dc.creator Vilchis Tella, Victor
dc.creator Lonberg, Nikhil
dc.creator Teply, Corey
dc.creator Oron, Assaf P
dc.creator McHugh, Theresa A
dc.creator Thomson, Azalea M
dc.date 2023-08-01T00:00:00Z
dc.date.accessioned 2025-02-25T10:38:48Z
dc.date.available 2025-02-25T10:38:48Z
dc.identifier d10bcde3-8833-4967-a372-05b45cddc9ba
dc.identifier 10.1016/s2352-3026(23)00118-7
dc.identifier https://avesis.sdu.edu.tr/publication/details/d10bcde3-8833-4967-a372-05b45cddc9ba/oai
dc.identifier.uri http://acikerisim.sdu.edu.tr/xmlui/handle/123456789/101447
dc.description Background: Previous global analyses, with known underdiagnosis and single cause per death attribution systems, provide only a small insight into the suspected high population health effect of sickle cell disease. Completed as part of the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2021, this study delivers a comprehensive global assessment of prevalence of sickle cell disease and mortality burden by age and sex for 204 countries and territories from 2000 to 2021. Methods: We estimated cause-specific sickle cell disease mortality using standardised GBD approaches, in which each death is assigned to a single underlying cause, to estimate mortality rates from the International Classification of Diseases (ICD)-coded vital registration, surveillance, and verbal autopsy data. In parallel, our goal was to estimate a more accurate account of sickle cell disease health burden using four types of epidemiological data on sickle cell disease: birth incidence, age-specific prevalence, with-condition mortality (total deaths), and excess mortality (excess deaths). Systematic reviews, supplemented with ICD-coded hospital discharge and insurance claims data, informed this modelling approach. We employed DisMod-MR 2.1 to triangulate between these measures—borrowing strength from predictive covariates and across age, time, and geography—and generated internally consistent estimates of incidence, prevalence, and mortality for three distinct genotypes of sickle cell disease: homozygous sickle cell disease and severe sickle cell β-thalassaemia, sickle-haemoglobin C disease, and mild sickle cell β-thalassaemia. Summing the three models yielded final estimates of incidence at birth, prevalence by age and sex, and total sickle cell disease mortality, the latter of which was compared directly against cause-specific mortality estimates to evaluate differences in mortality burden assessment and implications for the Sustainable Development Goals (SDGs). Findings: Between 2000 and 2021, national incidence rates of sickle cell disease were relatively stable, but total births of babies with sickle cell disease increased globally by 13·7% (95% uncertainty interval 11·1–16·5), to 515 000 (425 000–614 000), primarily due to population growth in the Caribbean and western and central sub-Saharan Africa. The number of people living with sickle cell disease globally increased by 41·4% (38·3–44·9), from 5·46 million (4·62–6·45) in 2000 to 7·74 million (6·51–9·2) in 2021. We estimated 34 400 (25 000–45 200) cause-specific all-age deaths globally in 2021, but total sickle cell disease mortality burden was nearly 11-times higher at 376 000 (303 000–467 000). In children younger than 5 years, there were 81 100 (58 800–108 000) deaths, ranking total sickle cell disease mortality as 12th (compared to 40th for cause-specific sickle cell disease mortality) across all causes estimated by the GBD in 2021. Interpretation: Our findings show a strikingly high contribution of sickle cell disease to all-cause mortality that is not apparent when each death is assigned to only a single cause. Sickle cell disease mortality burden is highest in children, especially in countries with the greatest under-5 mortality rates. Without comprehensive strategies to address morbidity and mortality associated with sickle cell disease, attainment of SDG 3.1, 3.2, and 3.4 is uncertain. Widespread data gaps and correspondingly high uncertainty in the estimates highlight the urgent need for routine and sustained surveillance efforts, further research to assess the contribution of conditions associated with sickle cell disease, and widespread deployment of evidence-based prevention and treatment for those with sickle cell disease. Funding: Bill & Melinda Gates Foundation.
dc.language eng
dc.rights info:eu-repo/semantics/closedAccess
dc.title Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000–2021: a systematic analysis from the Global Burden of Disease Study 2021
dc.type info:eu-repo/semantics/article


Files in this item

Files Size Format View

There are no files associated with this item.

This item appears in the following Collection(s)

Show simple item record

Search DSpace


Advanced Search

Browse

My Account