{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T03:57:08Z","timestamp":1760241428784,"version":"build-2065373602"},"reference-count":34,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2018,2,28]],"date-time":"2018-02-28T00:00:00Z","timestamp":1519776000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>In sickle cell disease (SCD), hydroxyurea (HU) treatment decreases the number of vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) by increasing fetal hemoglobin (HbF). Data are lacking regarding the frequency of HU dose modification or whether sub-therapeutic doses (&lt;15 mg\/kg\/day) are beneficial. We reviewed the medical records of 140 patients from 2010 to 2014. The laboratory parameters and SCD complications were compared between the first and last visits based on HU use. Fifty patients (36%) never took HU or suspended HU (\u201cno HU\u201d group). Among patients taking &lt;15 mg\/kg\/day HU on their first visit, half remained at the same dose, and the other half increased to \u226515 mg\/kg\/day. Among patients taking \u226515 mg\/kg\/day, 17% decreased to &lt;15 mg\/kg\/day, and 83% stayed at \u226515 mg\/kg\/day. The \u201cno HU\u201d group had fewer episodes of VOC and ACS. Both HU treatment groups had a reduction in both complications (p &lt; 0.0001). This improvement was observed in all SCD phenotypes. The white blood cell (WBC) counts were found to be lower, and HbF increased in both HU groups (p = 0.004, 0.001). The maximal HbF response to HU in HbS\/\u03b2+-thalassemia was 20%, similar to those observed for HbSS (19%) and HbS\/\u03b20-thalassemia (22%). HbS\/\u03b2+-thalassemia could have a similar disease severity as HbSS or HbS\/\u03b20-thalassemia. Patients with HbS\/\u03b20-thalassemia or HbS\/\u03b2+-thalassemia phenotypes responded to HU.<\/jats:p>","DOI":"10.3390\/ijms19030681","type":"journal-article","created":{"date-parts":[[2018,2,28]],"date-time":"2018-02-28T12:54:12Z","timestamp":1519822452000},"page":"681","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Chronic Administration of Hydroxyurea (HU) Benefits Caucasian Patients with Sickle-Beta Thalassemia"],"prefix":"10.3390","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9568-9688","authenticated-orcid":false,"given":"Rosario","family":"Di Maggio","sequence":"first","affiliation":[{"name":"Campus of Haematology Franco and Piera Cutino, AOOR Villa Sofia-V. Cervello, 90142 Palermo, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthew","family":"Hsieh","sequence":"additional","affiliation":[{"name":"Molecular and Clinical Hematology Branch, National Institute of Diabetes and Digestive and Kidney Diseases\/National Heart, Lung, and Blood Institute, Bethesda, MD 20814, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiongce","family":"Zhao","sequence":"additional","affiliation":[{"name":"Office of Clinical Director, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD 20814, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giuseppina","family":"Calvaruso","sequence":"additional","affiliation":[{"name":"Campus of Haematology Franco and Piera Cutino, AOOR Villa Sofia-V. Cervello, 90142 Palermo, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paolo","family":"Rigano","sequence":"additional","affiliation":[{"name":"Campus of Haematology Franco and Piera Cutino, AOOR Villa Sofia-V. Cervello, 90142 Palermo, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Disma","family":"Renda","sequence":"additional","affiliation":[{"name":"Campus of Haematology Franco and Piera Cutino, AOOR Villa Sofia-V. Cervello, 90142 Palermo, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John","family":"Tisdale","sequence":"additional","affiliation":[{"name":"Molecular and Clinical Hematology Branch, National Institute of Diabetes and Digestive and Kidney Diseases\/National Heart, Lung, and Blood Institute, Bethesda, MD 20814, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aurelio","family":"Maggio","sequence":"additional","affiliation":[{"name":"Campus of Haematology Franco and Piera Cutino, AOOR Villa Sofia-V. Cervello, 90142 Palermo, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2018,2,28]]},"reference":[{"key":"ref_1","unstructured":"World Health Organization Regional Office for Africa (2010). Sickle-Cell Disease: A Strategy for the WHO African Region, WHO. 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