{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T17:16:02Z","timestamp":1771262162976,"version":"3.50.1"},"reference-count":6,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2017,4,28]],"date-time":"2017-04-28T00:00:00Z","timestamp":1493337600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","award":["P30 CA008748"],"award-info":[{"award-number":["P30 CA008748"]}],"id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2017,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Hemoglobin S\/Black (<jats:sup>A<\/jats:sup>\u03b3\u03b4\u03b2)<jats:sup>0<\/jats:sup>\u2010thalassemia is a rare sickle cell disease (SCD) variant. On the basis of limited descriptions in the literature, the disease is reported as a mild microcytic anemia with an uncomplicated course. We report the clinical and laboratory data of nine patients whose diagnoses were confirmed by DNA\u2010based techniques. Despite having mild anemia and high fetal hemoglobin level postinfancy, these patients developed many of the classic complications of SCD, including vaso\u2010occlusive crisis, acute chest syndrome, avascular necrosis, and cholelithiasis. On the basis of these findings, we recommend that patients with this rare disorder receive specialized hematology care according to SCD guidelines.<\/jats:p>","DOI":"10.1002\/pbc.26596","type":"journal-article","created":{"date-parts":[[2017,4,28]],"date-time":"2017-04-28T19:06:46Z","timestamp":1493406406000},"source":"Crossref","is-referenced-by-count":10,"title":["The clinical severity of hemoglobin S\/Black (<sup>A<\/sup>\u03b3\u03b4\u03b2)<sup>0<\/sup>\u2010thalassemia"],"prefix":"10.1002","volume":"64","author":[{"given":"Maria I.","family":"Cancio","sequence":"first","affiliation":[{"name":"Department of Pediatrics Memorial Sloan Kettering Cancer Center  New York New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Banu","family":"Aygun","sequence":"additional","affiliation":[{"name":"Cohen Children's Medical Center of New York  New Hyde Park New York"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David H. K.","family":"Chui","sequence":"additional","affiliation":[{"name":"Hemoglobin Diagnostic Reference Laboratory Departments of Medicine Pathology and Laboratory Medicine Boston Medical Center  Boston Massachusetts"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennifer A.","family":"Rothman","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Duke University Medical Center  Durham North Carolina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. Paul","family":"Scott","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Medical College of Wisconsin Blood Research Institute Children's Research Institute  Milwaukee Wisconsin"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeremie H.","family":"Estepp","sequence":"additional","affiliation":[{"name":"Department of Hematology St. Jude Children's Research Hospital  Memphis Tennessee"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jane S.","family":"Hankins","sequence":"additional","affiliation":[{"name":"Department of Hematology St. Jude Children's Research Hospital  Memphis Tennessee"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,4,28]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/gkh006"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.1985.tb02999.x"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2141.2011.08916.x"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.3109\/03630269.2010.526890"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2013-09-528067"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8652(199701)54:1<40::AID-AJH6>3.0.CO;2-4"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpbc.26596","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.26596","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,5]],"date-time":"2023-10-05T03:37:51Z","timestamp":1696477071000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.26596"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,4,28]]},"references-count":6,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2017,11]]}},"alternative-id":["10.1002\/pbc.26596"],"URL":"https:\/\/doi.org\/10.1002\/pbc.26596","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"value":"1545-5009","type":"print"},{"value":"1545-5017","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,4,28]]},"article-number":"e26596"}}