Effect of Hydroxyurea on Mortality and Morbidity in Adult Sickle Cell Anemia

Steinberg, Martin H.; Barton, Franca B.; Castro, Oswaldo; Pegelow, Charles H.; Ballas, Samir K.; Kutlar, Abdullah; Orringer, Eugene P.; Bellevue, Rita; Olivieri, Nancy F.; Eckman, James R.; Varma, Mala; Ramírez, Gilberto Parra; Adler, Brian K.; Smith, Wally R.; Carlos, Timothy M.; Ataga, Kenneth I.; DeCastro, Laura; Bigelow, Carolyn; Saunthararajah, Yogen; Telfer, Margaret; Vichinsky, Elliott; Claster, Susan; Shurin, Susan B.; Bridges, Kenneth R.; Waclawiw, Myron A.; Bonds, Duane R.; Terrin, Michael L.

doi:10.1001/jama.289.13.1645

Cited by 771 publications

(515 citation statements)

References 34 publications

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“…Despite the lack of evidence that hydroxyurea therapy prevents organ damage, four large longitudinal studies have consistently demonstrated a survival advantage for children and adults on hydroxyurea therapy, when compared with their counterparts not on therapy. Long-term followup from the MSH study that included only adults with severe SCA demonstrated sustained clinical efficacy and 40% lower mortality after 9 years of follow up in participants electing to take hydroxyurea therapy after completion of the trial when compared with those not electing to start hydroxyurea therapy after completing the trial [57]. This survival benefit was maintained at 17.5 years [58].…”

Section: Hydroxyurea Therapymentioning

confidence: 89%

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

2015

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Over the past 40 years, public health measures such as universal newborn screening, penicillin prophylaxis, vaccinations, and hydroxyurea therapy have led to an impressive decline in sickle cell disease (SCD)-related childhood mortality and SCD-related morbidity in high-income countries. We remain cautiously optimistic that the next 40 years will be focused on meeting current challenges in SCD by addressing chronic complications of SCD to reduce mortality and improve quality of life in a growing population of adults with SCD in high-income countries, while simultaneously decreasing the disparity of medical care between high and low-income countries.

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Section: Hydroxyurea Therapymentioning

confidence: 89%

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

2015

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“…Based on recent data demonstrating improved survival with hydroxyurea use, including patients with milder SCD phenotypes, we recommended hydroxyurea use for all adults without contra-indications or receiving chronic transfusions [17,18]. For those with a high rate of acute care utilization despite hydroxyurea therapy, we recommended a trial of prophylactic transfusions after careful consideration of risks of iron overload and alloimmunization [19].…”

Section: Discussionmentioning

confidence: 99%

Intensive management of high‐utilizing adults with sickle cell disease lowers admissions

et al. 2015

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A minority of super-utilizing adults with sickle cell disease (SCD) account for a disproportionate number of emergency department (ED) and hospital admissions. We performed a retrospective cohort study comparing the rate of admission before and after the opening of a clinic for adults with SCD. Unique to this clinic was an intensive management strategy, focusing on super-utilizing adults with 12 or more admissions per year. ED/hospital and 30 days re-admission rates were compared, 1 year pre-and post-intervention, for those adults who established in the clinic. Prior to the intervention, 17 super-utilizers, comprising 15% of the preintervention cohort (n 5 115), accounted for 58% of the total admissions and had an admission rate of 28 per patient-year. When pre-and post-intervention years were compared, rate of ED/hospital admission per patient-year for super-utilizers decreased from 27.9 to 13.5 (P < 0.001), while there was not a significant reduction for the entire cohort (7.1 vs. 6.1, P 5 0.84). Similarly, the decrease in rate of 30 day re-admission was larger for the super-utilizers (13.5 per patient-year to 1.8, P < 0.001), than the whole cohort (2.6 per patient-year to 0.7, P 5 0.006). Among the super-utilizers, the reduced rate of admission from the pre-to post-clinic intervention year equated to 252 fewer ED/hospital admissions and 227 fewer 30 day readmissions. This management strategy focusing on super-utilizing adults with SCD lowered admission and 30 day re-admission rate.

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“…Among chemotherapy agents, Hydroxyurea (HU) is commonly used as an anti-cancer agent [4]. For instance, HU is an effective reagent which was widely applied for treatment of acute angina, breast cancer, several hematological malignancies including chronic myelogenous leukemia (CML), sickle cell anemia, myeloproliferative disorders, and other known cancers previously [5][6][7][8][9]. In addition to its beneficial properties several disadvantages and side effects such as skin disorders were reported to be associated HU in consumers [10,11].…”

Section: Introductionmentioning

confidence: 99%

In Vitro Evaluation of the Efficacy of Liposomal and Pegylated Liposomal Hydroxyurea

et al. 2013

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Breast cancer is one of the most frequent cancer types within women population. Hydroxyurea (HU) is a chemotherapy compound for treatment of patients with cancer diagnosis, including breast cancer associated with several adverse effects. In this study, we applied nanotechnology to decreased drug side effects along with improvement of therapeutic index. Liposomation is widely used in modern pharmacological developments in order to enhance the effects of the drugs. To achieve this, in this study a mixture of phosphatidylcholine and cholesterol was made up and HU was added to the resultant mixture, was then pegylated using Polyethylene Glycol 2000 to increase resistance, applicability and solubility. The mean diameters of nanoliposomal and pegylated nanoliposomal HU were measured by Zeta sizer device and obtained about 402.5 and 338.2 nm. The efficiency of non-pegylated and pegylated liposomal HU was 70.8 and 64.2, respectively. Releasing HU in both formulations was estimated about 25.8 and 21.7 %. Also, this study investigated the cytotoxicity effect of nanoliposomal and pegylated nanoliposomal HU using MTT assay. Results of this investigation showed that the cytotoxic properties of pegylated HU was 3.6 % more than those non-pegylated form, while was 38.93 % more than ordinary from of HU. This study showed that the stability, releasing pattern and cytotoxicity of the pegylated nanoliposomal HU is better than that of nanoliposomal HU.

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Effect of Hydroxyurea on Mortality and Morbidity in Adult Sickle Cell Anemia

Abstract: CUTE CHEST SYNDROME AND painful episodes are the most common precedents of death in adults with sickle cell anemia (SCA). 1 A randomized, doubleblinded, placebo-controlled trial, the

Cited by 771 publications

References 34 publications

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

Intensive management of high‐utilizing adults with sickle cell disease lowers admissions

In Vitro Evaluation of the Efficacy of Liposomal and Pegylated Liposomal Hydroxyurea

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