{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T21:12:13Z","timestamp":1791925933781,"version":"4.3.4"},"reference-count":28,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2025,11,19]],"date-time":"2025-11-19T00:00:00Z","timestamp":1763510400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,11,19]],"date-time":"2025-11-19T00:00:00Z","timestamp":1763510400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Haemophilia"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Glanzmann Thrombasthenia (GT) is a rare autosomal recessive bleeding disorder characterised by defective platelet aggregation due to abnormalities in the glycoprotein IIb\/IIIa complex. With a lack of large real\u2010world database studies, our study aimed to characterise the demographic, clinical and treatment profiles of GT patients using a large, national database.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A retrospective cohort study was conducted using the EPIC Cosmos database from 1 January 2013 to 31 December 2024. Patients with at least one encounter coded with the SNOMED code for GT (32942005) were included. The primary outcome was the prevalence of GT, calculated using the total database population as the denominator. Secondary outcomes included bleeding manifestations, comorbidities, transfusion utilisation, medication use, sex\u2010based differences and mortality, with statistical comparisons performed using Chi\u2010squared tests.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A total of 1059 GT patients were identified, 58.5% female. The estimated prevalence of GT was 1 in 352,435, higher than prior US estimates. The most frequent bleeding manifestations were abnormal uterine bleeding (29.7% of females aged 11\u201345), epistaxis (22.6%) and hematuria (16.1%). Anaemia was the most common comorbidity (35.6%), with iron deficiency anaemia present in 31.0%. Transfusion dependency was frequent, with 16.4% of patients requiring blood or component transfusions. No significant sex\u2010based differences were observed. Mortality increased with age, with an overall annualised rate of 1222 per 100,000 GT patients, exceeding that of the general US population.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>GT is more prevalent in the United States than previously recognised and is associated with substantial clinical burden from recurrent bleeding, anaemia and transfusion dependency.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/hae.70170","type":"journal-article","created":{"date-parts":[[2025,11,19]],"date-time":"2025-11-19T18:20:04Z","timestamp":1763576404000},"page":"155-165","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Characterising Glanzmann Thrombasthenia in the United States: Real\u2010World Data From the EPIC Cosmos Dataset"],"prefix":"10.1111","volume":"32","author":[{"given":"Divyaswathi","family":"Citla\u2010Sridhar","sequence":"first","affiliation":[{"name":"Department of Pediatrics University of Arkansas for Medical Sciences  Little Rock Arkansas USA"},{"name":"Arkansas Children's Hospital  Little Rock Arkansas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Arkansas for Medical Sciences  Little Rock Arkansas USA"},{"name":"Arkansas Children's Hospital  Little Rock Arkansas USA"},{"name":"Department of Biomedical Informatics University of Arkansas for Medical Sciences  Little Rock Arkansas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,11,19]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2018.214239"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.25910"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1055\/s\u20100044\u20101782519"},{"key":"e_1_2_12_5_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2014.121384"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.2147\/jbm.S271744"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2014.121475"},{"key":"e_1_2_12_8_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2022.281973"},{"key":"e_1_2_12_9_1","unstructured":"Research Data Assistance Center.CMS Cell Size Suppression Policy(Research Data Assistance Centre 2025) accessed September 10 2025 https:\/\/resdac.org\/articles\/cms\u2010cell\u2010size\u2010suppression\u2010policy."},{"key":"e_1_2_12_10_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41592\u2010019\u20100686\u20102"},{"key":"e_1_2_12_11_1","doi-asserted-by":"publisher","DOI":"10.2147\/jbm.S71319"},{"key":"e_1_2_12_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tmrv.2016.01.001"},{"key":"e_1_2_12_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/mbc.0000000000001286"},{"key":"e_1_2_12_14_1","doi-asserted-by":"publisher","DOI":"10.4103\/joah.joah_68_18"},{"key":"e_1_2_12_15_1","unstructured":"\u2018United Kingdom Haemophilia Centre Doctors\u2019 Organisation \u201cUKHCDO Annual Report 2023 and Bleeding Disorder Statistics for the Financial Year 2022\/23 \u201d accessed February 11 2025 https:\/\/www.ukhcdo.org\/home\u20102\/annual\u2010reports\/."},{"key":"e_1_2_12_16_1","unstructured":"Office for National Statistics \u201cPopulation estimates for England and Wales: Mid\u20102023 \u201d accessed February 11 2025 https:\/\/www.ons.gov.uk\/peoplepopulationandcommunity\/populationandmigration\/populationestimates\/bulletins\/populationestimatesforenglandandwales\/mid2023#cite\u2010this\u2010statistical\u2010bulletin."},{"key":"e_1_2_12_17_1","unstructured":"\u201cPlatelet Disorders \u201d accessed February 11 2025 https:\/\/www.cdc.gov\/hemophilia\u2010community\u2010counts\/php\/data\u2010research\/2024\u2010march\u2010platelet\u2010disorders.html."},{"key":"e_1_2_12_18_1","unstructured":"\u201cWorld Federation of Hemophilia Report on the Annual Global Survey 2023 \u201d accessed February 11 2025 https:\/\/www1.wfh.org\/publications\/files\/pdf\u20102525.pdf."},{"key":"e_1_2_12_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14973"},{"key":"e_1_2_12_20_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V75.7.1383.1383"},{"key":"e_1_2_12_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/jth.14886"},{"key":"e_1_2_12_22_1","doi-asserted-by":"publisher","DOI":"10.2147\/jbm.S389241"},{"key":"e_1_2_12_23_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.14559"},{"key":"e_1_2_12_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.transci.2018.07.010"},{"key":"e_1_2_12_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/hae.15126"},{"issue":"521","key":"e_1_2_12_26_1","article-title":"Mortality in the United States, 2023","author":"Murphy S. 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