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This is a retrospective cohort study of Sickle Cell Disease (SCD) patients attending 32 treatment centers across Italy. The aim of this study will be to report the Italian experience with the use of hydroxyurea in a large cohort of SCD patients and to evaluate the benefits and safety of this intervention for the prevention and management of a wide range of clinical morbidities
The indication for hydroxyurea initiation was 2-3 vaso-occlusive crisis and/or hospitalizations in the last year.
The study will analyze demographics (age and gender), origin, genotype, clinical phenotype (vaso-occlusive or hemolytic), transfusion history (including exchange), and folic acid use, average laboratory values up to three years pre-hydroxyurea and for the period post-hydroxyurea therapy including total hemoglobin level, fetal hemoglobin level, hemoglobin S level, white blood count, platelet count, lactate dehydrogenase level, total and direct bilirubin levels, aspartate and alanine aminotransferase levels, and serum creatinine level.
The incidence of complications pre- and post-hydroxyurea therapy will be also analyzed including: stroke, silent cerebral infraction, acute chest syndrome, vaso-occlusive crisis, hospitalization, pulmonary hypertension, leg ulcers, bone necrosis, and kidney injury. Safety data included adverse events as reported by the treating physician and the incidence of malignancy or death as well as pregnancy incidents and their outcomes will be also pointed out.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| SCD patients | Patients followed in 32 Italian Centers. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Physician standard-of-care in SCD patients | Other |
|
| Measure | Description | Time Frame |
|---|---|---|
| Changes in laboratory parameters is being assessed | Increases or decreases in percentage of total hemoglobin, fetal hemoglobin and hemoglobin S level will be assessed. Changing of white blood cells and platelets counts, lactate dehydrogenase, bilirubin, aspartate aminotransferase and serum creatinine level will be also evaluated. | An average of 3 years before and an average of 3 years after initiation of hydroxyurea therapy |
| Changes in complication rates is being assessed | Changing in the incidence of stroke, silent cerebral infraction, acute chest syndrome, vaso-occlusive crisis, pulmonary hypertension, leg ulcers, bone necrosis and kidney injury will be evaluated. | An average of 3 years before and an average of 3 years after initiation of hydroxyurea therapy |
| Rate of hospitalizations | Changing in rate of hospitalizations before and after start hydroxyurea therapy | An average of 3 years before and an average of 3 years after initiation of hydroxyurea therapy |
| Measure | Description | Time Frame |
|---|---|---|
| Changing in the incidence of complications according to specific subgroups | We also stratified the analysis according to age (≥18 years), origin (Italian and African), genotype (βS/β0, βS/β+ and βS/βS) duration of hydroxyurea treatment (≥10 years) and hydroxyurea dose(≥15 mg/kg/day). | An average of 3 years before and an average of 3 years after initiation of hydroxyurea therapy |
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Inclusion Criteria:
Exclusion Criteria
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A population of SCD (Homozigous HbS and Beta Thal /HbS) patients followed in 32 Italian Centers was included in this analysis.
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| Name | Affiliation | Role |
|---|---|---|
| Paolo Rigano, MD | Servico Integrado de Tecnicas Endovasculares | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Ospedali Civili Riuniti di Sciacca | Sciacca | Agrigento | 92019 | Italy | ||
| Ospedale Vittorio Emanuele III |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 29107441 | Derived | Rigano P, De Franceschi L, Sainati L, Piga A, Piel FB, Cappellini MD, Fidone C, Masera N, Palazzi G, Gianesin B, Forni GL; Italian Multicenter Study of Hydroxyurea in Sickle Cell Anemia Investigators. Real-life experience with hydroxyurea in sickle cell disease: A multicenter study in a cohort of patients with heterogeneous descent. Blood Cells Mol Dis. 2018 Mar;69:82-89. doi: 10.1016/j.bcmd.2017.08.017. Epub 2017 Oct 9. |
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| Gela |
| Caltanisetta |
| 93012 |
| Italy |
| Azienda Ospedaliero-Universitaria | Bari | Italy |
| U.O. Oncoematologia Pediatrica Ospedali Civili | Brescia | 25100 | Italy |
| Ospedale "A. Perrino" | Brindisi | 72100 | Italy |
| Azienda Ospedaliera Universitaria Di Cagliari | Cagliari | 09121 | Italy |
| Ospedale Vittorio Emanuele | Catania | 95123 | Italy |
| ARNAS "Garibaldi" | Catania | 95124 | Italy |
| University of Catania | Catania | 95124 | Italy |
| A.O. "Pugliese-Ciaccio" | Catanzaro | 88100 | Italy |
| Osp.San Giovanni Di Dio | Crotone | Italy |
| Azienda Ospedaliero Universitaria Meyer | Florence | 50139 | Italy |
| E.O. Ospedali Galliera | Genova | 16128 | Italy |
| Università degli Studi di Milano | Milan | 20122 | Italy |
| Azienda Ospedaliero-Universitaria di Modena - Policlinico | Modena | 41124 | Italy |
| Clinica pediatrica Monza S. Gerardo | Monza | 20052 | Italy |
| Azienda Ospedaliera San Gerardo di Monza | Monza | 20900 | Italy |
| Aorn A. Cardarelli | Naples | Italy |
| Clinica di Onco-Ematologia Pediatrica, Università di Padova | Padova | 35128 | Italy |
| A.R.N.A.S. "Civico" | Palermo | 90100 | Italy |
| Azienda Ospedaliera Ospedali Riuniti Villa Sofia Cervello | Palermo | 90146 | Italy |
| Azienda Ospedaliero-Universitaria di Parma | Parma | 43126 | Italy |
| Policlinico San Matteo | Pavia | 27100 | Italy |
| Centro Emofilia e Medicina Trasfusionale - Pres. Ospedaliero | Ravenna | 48121 | Italy |
| A. O. Bianchi Melacrino Morelli | Reggio Calabria | 89100 | Italy |
| Ospedale S. Eugenio - FF UOSD DH Talassemici | Roma | 00144 | Italy |
| Università Cattolica del Sacro Cuore - Policlinico A.Gemelli | Roma | 00168 | Italy |
| U.O.S. Talassemia P.O. Umberto I° | Syracuse | 96100 | Italy |
| Università degli Suidi di Torino | Torino | 90146 | Italy |
| Policlinico G.B. Rossi | Verona | 37122 | Italy |
| ID | Term |
|---|---|
| D000755 | Anemia, Sickle Cell |
| ID | Term |
|---|---|
| D000745 | Anemia, Hemolytic, Congenital |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D006453 | Hemoglobinopathies |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
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