{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,18]],"date-time":"2026-07-18T03:43:28Z","timestamp":1784346208736,"version":"3.55.0"},"reference-count":14,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2022,2,17]],"date-time":"2022-02-17T00:00:00Z","timestamp":1645056000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2022,5]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Owing to the unique pathophysiology of anaemia in haemoglobin Bart's hydrops fetalis (HBHF), a transfusion strategy based on beta\u2010thalassemia guidelines is suboptimal for chronically transfused HBHF patients. A more aggressive transfusion aimed at reducing the proportion of non\u2010functional HbH and improving the \u201cfunctional\u201d haemoglobin (f\u2010Hb) can lead to reduced haemolysis and improved tissue oxygenation. However, the optimal transfusion targets for these parameters are not yet defined. In this retrospective, longitudinal study on four chronically transfused patients with HBHF, we used receiver operating characteristic curves to find a pre\u2010transfusion f\u2010Hb of 106\u00a0g\/l and a HbH of 16.1% to be the optimal thresholds to achieve a normal soluble transferrin receptor and lactate dehydrogenase, respectively.<\/jats:p>","DOI":"10.1111\/bjh.18077","type":"journal-article","created":{"date-parts":[[2022,2,18]],"date-time":"2022-02-18T00:02:51Z","timestamp":1645142571000},"page":"373-376","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Optimizing transfusion therapy for survivors of Haemoglobin Bart's hydrops fetalis syndrome: Defining the targets for <scp>haemoglobin\u2010H<\/scp> fraction and \u201cfunctional\u201d haemoglobin level"],"prefix":"10.1111","volume":"197","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6926-7502","authenticated-orcid":false,"given":"Ali","family":"Amid","sequence":"first","affiliation":[{"name":"Division of Hematology\/Oncology, BC Children's Hospital  Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nick","family":"Barrowman","sequence":"additional","affiliation":[{"name":"Division of Biostatistics, Children's Hospital of Eastern Ontario  Ottawa Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isaac","family":"Odame","sequence":"additional","affiliation":[{"name":"Division of Haematology\/Oncology, Hospital for Sick Children  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Melanie","family":"Kirby\u2010Allen","sequence":"additional","affiliation":[{"name":"Division of Haematology\/Oncology, Hospital for Sick Children  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,2,17]]},"reference":[{"key":"e_1_2_7_2_1","volume-title":"Guidelines for the clinical management of thalassaemia","author":"Cappellini MD","year":"2008"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2012-12-471441"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V91.7.2213"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1404415"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2019-317626"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2021005916"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2015-10-673889"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.3324\/haematol.2017.178368"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-06-2373"},{"issue":"5","key":"e_1_2_7_11_1","first-page":"397","article-title":"Serum erythropoietin and circulating transferrin receptor in thalassemia intermedia patients with heterogeneous genotypes","volume":"81","author":"Camaschella C","year":"1996","journal-title":"Haematologica"},{"issue":"1","key":"e_1_2_7_12_1","first-page":"1","article-title":"The soluble transferrin receptor: biological aspects and clinical usefulness as quantitative measure of erythropoiesis","volume":"77","author":"Beguin Y","year":"1992","journal-title":"Haematologica"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1537-2995.1997.37297203514.x"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1017\/CBO9780511596582.020"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/imj.14823"}],"container-title":["British Journal of Haematology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bjh.18077","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/bjh.18077","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/bjh.18077","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,24]],"date-time":"2023-08-24T01:13:37Z","timestamp":1692839617000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/bjh.18077"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,2,17]]},"references-count":14,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2022,5]]}},"alternative-id":["10.1111\/bjh.18077"],"URL":"https:\/\/doi.org\/10.1111\/bjh.18077","archive":["Portico"],"relation":{},"ISSN":["0007-1048","1365-2141"],"issn-type":[{"value":"0007-1048","type":"print"},{"value":"1365-2141","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,2,17]]},"assertion":[{"value":"2021-12-22","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-01-24","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-02-17","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}