{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,2]],"date-time":"2026-05-02T19:31:29Z","timestamp":1777750289179,"version":"3.51.4"},"reference-count":0,"publisher":"S. Karger AG","license":[{"start":{"date-parts":[[2009,2,18]],"date-time":"2009-02-18T00:00:00Z","timestamp":1234915200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"},{"start":{"date-parts":[[2009,2,18]],"date-time":"2009-02-18T00:00:00Z","timestamp":1234915200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/karger.com\/pages\/terms-and-conditions"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Haematol"],"abstract":"<jats:p>We have identified 7 patients with Hb H disease as homozygotes for a mutation in the polyadenylation site (AATAAA \u2192 AATAAG) and have compared their hematological data with those of Hb H patients having other types of \u03b1-thalassemia determinants. All 7 patients exhibited moderate anemia with microcytosis and hypochromia being similar to that observed in the other patients. Relatives with a heterozygosity for this mutation are borderline microcytic and hy-pochromic without a significant anemia but with a low in vitro \u03b1\/\u03b2 chain synthesis ratio. Analyses of the hemoglobin components identified low levels of Hb A&lt;sub&gt;2&lt;\/sub&gt; and Hb H that were comparable to those found in other patients with Hb H disease; the level of the \u03b6-chain was low (average 0.14%).<\/jats:p>","DOI":"10.1159\/000204657","type":"journal-article","created":{"date-parts":[[2009,2,20]],"date-time":"2009-02-20T14:45:47Z","timestamp":1235141147000},"page":"82-85","source":"Crossref","is-referenced-by-count":31,"title":["Hb H Disease Caused by a Homozygosity for the AATAAA\u2192 AATAAG Mutation in the Polyadenylation Site of the \u03b12-Globin Gene: Hematological Observations"],"prefix":"10.1159","author":[{"given":"You-Jun","family":"Fei","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Reyhan","family":"\u00d6ner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gulsen","family":"B\u00f6zkurt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Li-Hao","family":"Gu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"\u00c7igdem","family":"Altay","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aytemiz","family":"Gurgey","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Slaheddine","family":"Fattoum","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erol","family":"Baysal","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Titus H.J.","family":"Huisman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"127","published-online":{"date-parts":[[2009,2,18]]},"container-title":["Acta Haematologica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/204657","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/karger.com\/article-pdf\/doi\/10.1159\/000204657","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T11:54:38Z","timestamp":1777463678000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000204657"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,2,18]]},"references-count":0,"URL":"https:\/\/doi.org\/10.1159\/000204657","archive":["Portico"],"relation":{},"ISSN":["0001-5792","1421-9662"],"issn-type":[{"value":"0001-5792","type":"print"},{"value":"1421-9662","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,2,18]]}}}