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Hydroxyurea in the Treatment of Sickle Cell Disease

Completed
Conditions
Sickle Cell Disease
Interventions
Other: Physician standard-of-care in SCD patients
Registration Number
NCT02709681
Lead Sponsor
Società Italiana Talassemie ed Emoglobinopatie
Brief Summary

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

Detailed Description

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.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
628
Inclusion Criteria
  • Sickle Cell Disease affected patients
  • 2-3 vaso-occlusive crisis and/or hospitalizations in the last year

Exclusion Criteria

  • none
Exclusion Criteria

Not provided

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Arm && Interventions
GroupInterventionDescription
SCD patientsPhysician standard-of-care in SCD patientsPatients followed in 32 Italian Centers.
Primary Outcome Measures
NameTimeMethod
Changes in laboratory parameters is being assessedAn average of 3 years before and an average of 3 years after initiation of hydroxyurea therapy

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.

Changes in complication rates is being assessedAn average of 3 years before and an average of 3 years after initiation of hydroxyurea therapy

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.

Rate of hospitalizationsAn average of 3 years before and an average of 3 years after initiation of hydroxyurea therapy

Changing in rate of hospitalizations before and after start hydroxyurea therapy

Secondary Outcome Measures
NameTimeMethod
Changing in the incidence of complications according to specific subgroupsAn average of 3 years before and an average of 3 years after initiation of hydroxyurea therapy

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).

Trial Locations

Locations (30)

Azienda Ospedaliero Universitaria Meyer

🇮🇹

Firenze, Italy

Policlinico San Matteo

🇮🇹

Pavia, Italy

Centro Emofilia e Medicina Trasfusionale - Pres. Ospedaliero

🇮🇹

Ravenna, Italy

Azienda Ospedaliero-Universitaria

🇮🇹

Bari, Italy

Ospedale "A. Perrino"

🇮🇹

Brindisi, Italy

Ospedale Vittorio Emanuele

🇮🇹

Catania, Italy

A.O. "Pugliese-Ciaccio"

🇮🇹

Catanzaro, Italy

Clinica pediatrica Monza S. Gerardo

🇮🇹

Monza, Italy

Ospedale Vittorio Emanuele III

🇮🇹

Gela, Caltanisetta, Italy

Osp.San Giovanni Di Dio

🇮🇹

Crotone, Italy

Clinica di Onco-Ematologia Pediatrica, Università di Padova

🇮🇹

Padova, Italy

Ospedali Civili Riuniti di Sciacca

🇮🇹

Sciacca, Agrigento, Italy

U.O. Oncoematologia Pediatrica Ospedali Civili

🇮🇹

Brescia, Italy

Azienda Ospedaliera Universitaria Di Cagliari

🇮🇹

Cagliari, Italy

University of Catania

🇮🇹

Catania, Italy

E.O. Ospedali Galliera

🇮🇹

Genova, Italy

Università degli Studi di Milano

🇮🇹

Milano, Italy

Ospedale S. Eugenio - FF UOSD DH Talassemici

🇮🇹

Roma, Italy

ARNAS "Garibaldi"

🇮🇹

Catania, Italy

Azienda Ospedaliera San Gerardo di Monza

🇮🇹

Monza, Italy

Aorn A. Cardarelli

🇮🇹

Napoli, Italy

U.O.S. Talassemia P.O. Umberto I°

🇮🇹

Siracusa, Italy

Policlinico G.B. Rossi

🇮🇹

Verona, Italy

Azienda Ospedaliero-Universitaria di Modena - Policlinico

🇮🇹

Modena, Italy

A.R.N.A.S. "Civico"

🇮🇹

Palermo, Italy

A. O. Bianchi Melacrino Morelli

🇮🇹

Reggio Calabria, Italy

Azienda Ospedaliera Ospedali Riuniti Villa Sofia Cervello

🇮🇹

Palermo, Italy

Università Cattolica del Sacro Cuore - Policlinico A.Gemelli

🇮🇹

Roma, Italy

Università degli Suidi di Torino

🇮🇹

Torino, Italy

Azienda Ospedaliero-Universitaria di Parma

🇮🇹

Parma, Italy

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