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Research ArticleOriginal Article
Open Access

The role of hydroxyurea in decreasing the occurrence of vasso-occulusive crisis in pediatric patients with sickle cell disease at King Saud Medical City in Riyadh, Saudi Arabia

Fauzia R. Azmet, Fawaz Al-Kasim, Walid M. Alashram and Khawar Siddique
Saudi Medical Journal January 2020, 41 (1) 46-52; DOI: https://doi.org/10.15537/smj.2020.1.24698
Fauzia R. Azmet
From the Department of Pediatric Hematology and Oncology, King Saud Medical City, Riyadh, Kingdom of Saudi Arabia
MRCPCH, SFPHO
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  • For correspondence: [email protected]
Fawaz Al-Kasim
From the Department of Pediatric Hematology and Oncology, King Saud Medical City, Riyadh, Kingdom of Saudi Arabia
FRCP, CFPHO
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Walid M. Alashram
From the Department of Pediatric Hematology and Oncology, King Saud Medical City, Riyadh, Kingdom of Saudi Arabia
MBBS, DCH
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Khawar Siddique
From the Department of Pediatric Hematology and Oncology, King Saud Medical City, Riyadh, Kingdom of Saudi Arabia
MSc, MBA
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References

  1. ↵
    1. Piel FB,
    2. Patil AP,
    3. Howes RE,
    4. Nyangiri OA,
    5. Gething PW,
    6. Dewi M,
    7. et al.
    (2013) Global epidemiology of sickle haemoglobin in neonates:a contemporary geostatistical model-based map and population estimates. Lancet 381:142–151.
    OpenUrlCrossRefPubMedWeb of Science
  2. ↵
    1. Piel FB,
    2. Hay SI,
    3. Gupta S,
    4. Weatherall DJ,
    5. Williams TN
    (2013) Global burden of sickle cell anaemia in children under five 2010-2050:modelling based on demographics, excess mortality, and interventions. PLoS Med 10:e1001484.
    OpenUrlCrossRefPubMed
  3. ↵
    1. Pauling L,
    2. Itano HA,
    3. Singer SJ,
    4. Wells IC
    (1949) Sickle cell anemia, a molecular disease. Science 110:543–548.
    OpenUrlFREE Full Text
  4. ↵
    1. Al-Qurashi MM,
    2. El-Mouzan MI,
    3. Al-Herbish AS,
    4. Al-Salloum AA,
    5. Al-Omar AA
    (2008) The prevalence of sickle cell disease in Saudi children and adolescents. A community-based survey. Saudi Med J 29:1480–1483.
    OpenUrlPubMed
  5. ↵
    1. Cannas G,
    2. Poutrel S,
    3. Thomas X
    (2017) Hydroxycarbamine:from an old drug used in malignant hemopathies to a current standard in sickle cell disease. Mediterr J Hematol Infect Dis 9:e2017015.
    OpenUrl
  6. ↵
    1. Eaton WA,
    2. Bunn HF
    (2017) Treating sickle cell disease by targeting HbS polymerization. Blood 129:2719–2726.
    OpenUrlAbstract/FREE Full Text
  7. ↵
    1. Cokic VP,
    2. Smith RD,
    3. Beleslin-Cokic BB,
    4. Njoroge JM,
    5. Miller JL,
    6. Gladwin MT,
    7. et al.
    (2003) Hydroxyurea induces fetal hemoglobin by the nitric oxide-dependent activation of soluble guanylyl cyclase. J Clin Invest 111:231–239.
    OpenUrlCrossRefPubMedWeb of Science
  8. ↵
    1. Ware RE,
    2. Davis BR,
    3. Schultz WH,
    4. Brown RC,
    5. Aygun B,
    6. Sarnaik S,
    7. et al.
    (2016) Hydroxycarbamide versus chronic transfusion for maintenance of transcranial doppler flow velocities in children with sickle cell anaemia-TCD with transfusions changing to hydroxyurea (TWiTCH):a multicentre, open-label, phase 3, non-inferiority trial. Lancet 387:661–670.
    OpenUrlCrossRefPubMed
  9. ↵
    1. Mulaku M,
    2. Opiyo N,
    3. Karumbi J,
    4. Kitonyi G,
    5. Thoithi G,
    6. English M
    (2013) Evidence review of hydroxyurea for the prevention of sickle cell complications in low-income countries. Arch Dis Child 98:908–914.
    OpenUrlAbstract/FREE Full Text
  10. ↵
    1. Wang WC,
    2. Ware RE,
    3. Miller ST,
    4. Iyer RV,
    5. Casella JF,
    6. Minniti CP,
    7. et al.
    (2011) Hydroxycarbamide in very young children with sickle-cell anaemia:a multicentre, randomised, controlled trial (BABY HUG). Lancet 377:1663–1672.
    OpenUrlCrossRefPubMedWeb of Science
    1. Charache S,
    2. Terrin ML,
    3. Moore RD,
    4. Dover GJ,
    5. Barton FB,
    6. Eckert SV,
    7. et al.
    (1995) Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. nvestigators of the multicenter study of hydroxyurea in sickle cell anemia. N Engl J Med 332:1317–1322.
    OpenUrlCrossRefPubMedWeb of Science
    1. Kinney TR,
    2. Helms RW,
    3. O'Branski EE,
    4. Ohene-Frempong K,
    5. Wang W,
    6. Daeschner C,
    7. et al.
    (1999) Safety of hydroxyurea in children with sickle cell anemia:results of the HUG-KIDS study, a phase I/II trial. Pediatric hydroxyurea group. Blood 94:1550–1554.
    OpenUrlAbstract/FREE Full Text
    1. Hankins JS,
    2. Helton KJ,
    3. McCarville MB,
    4. Li CS,
    5. Wang WC,
    6. Ware RE
    (2008) Preservation of spleen and brain function in children with sickle cell anemia treated with hydroxyurea. Pediatr Blood Cancer 50:293–297.
    OpenUrlCrossRefPubMedWeb of Science
    1. Zimmerman SA,
    2. Schultz WH,
    3. Davis JS,
    4. Pickens CV,
    5. Mortier NA,
    6. Howard TA,
    7. et al.
    (2004) Sustained long-term hematologic efficacy of hydroxyurea at maximum tolerated dose in children with sickle cell disease. Blood 103:2039–2045.
    OpenUrlAbstract/FREE Full Text
    1. Estepp JH,
    2. Smeltzer MP,
    3. Kang G,
    4. Aygun B,
    5. Ware RE,
    6. Nottage K
    (2014) Higher fetal hemoglobin following escalation of hydroxyurea to maximum tolerated dose provides clinical benefit to children with sickle cell anemia. Blood 124:85.
    OpenUrl
    1. Hankins JS,
    2. Ware RE,
    3. Rogers ZR,
    4. Wynn LW,
    5. Lane PA,
    6. Scott JP,
    7. et al.
    (2005) Long-term hydroxyurea therapy for infants with sickle cell anemia:the HUSOFT extension study. Blood 106:2269–2275.
    OpenUrlAbstract/FREE Full Text
    1. Thornburg CD,
    2. Files BA,
    3. Luo Z,
    4. et al.
    (2016) Impact of hydroxyurea on clinical events in the BABY HUG trial. Blood 128:2869.
    OpenUrlFREE Full Text
  11. ↵
    1. Puffer E,
    2. Schatz J,
    3. Roberts CW
    (2007) The association of oral hydroxyurea therapy with improved cognitive functioning in sickle cell disease. Child Neuropsychol 13:142–154.
    OpenUrlCrossRefPubMed
  12. ↵
    1. Strunk RC,
    2. Cohen RT,
    3. Cooper BP,
    4. Rodeghier M,
    5. Kirkham FJ,
    6. Warner JO,
    7. et al.
    (2014) Wheezing symptoms and parental asthma are associated with a physician diagnosis of asthma in children with sickle cell anemia. J Pediatr 164:821–826.
    OpenUrl
  13. ↵
    1. Strouse JJ,
    2. Heeney MM
    (2012) Hydroxyurea for the treatment of sickle cell disease:efficacy, barriers, toxicity, and management in children. Pediatr Blood Cancer 59:365–371.
    OpenUrlCrossRefPubMedWeb of Science
  14. ↵
    1. Al-Jam'a AH,
    2. Al-Dabbous IA
    (2002) Hydroxyurea in sickle cell disease patients from Eastern Saudi Arabia. Saudi Med J 23:277–281.
    OpenUrlPubMed
  15. ↵
    1. Al Hawsawi ZM,
    2. Turkistani WA
    (2008) Effect of hydroxyurea in children with sickle cell disease in Saudi Arabia. Journal of Taibah University Medical Sciences 3:129–134.
    OpenUrl
  16. ↵
    1. Alkanhal HN,
    2. Bakrman AK,
    3. Alzahrani AM,
    4. Alotaibi MS,
    5. Bin Salamah AA
    (2014) Adherence to hydroxyurea therapy in patients with sickle cell disease at King Khalid University hospital in Riyadh. J Blood Disord Transfus 5:2.
    OpenUrl
  17. ↵
    1. Lobo CL,
    2. Pinto JF,
    3. Nascimento EM,
    4. Moura PG,
    5. Cardoso GP,
    6. Hankins JS
    (2013) The effect of hydroxcarbamide therapy on survival of children with sickle cell disease. Br J Haematol 161:852–860.
    OpenUrlCrossRefPubMed
  18. ↵
    1. Sharef SW,
    2. Al-Hajri M,
    3. Beshlawi I,
    4. Al-Shahrabally A,
    5. Elshinawy M,
    6. Zachariah M,
    7. et al.
    (2013) Optimizing hydroxyurea use in children with sickle cell disease:low dose regimen is effective. Eur J Haematol 90:519–524.
    OpenUrlCrossRefPubMed
  19. ↵
    1. Phillips K,
    2. Healy L,
    3. Smith L,
    4. Keenan R
    (2018) Hydroxyurea therapy in UK children with sickle cell anaemia:A single-centre experience. Pediatr Blood Cancer, 65.
  20. ↵
    1. Wang WC,
    2. Ware RE,
    3. Miller ST,
    4. Iyer RV,
    5. Casella JF,
    6. Minniti CP,
    7. et al.
    (2011) Hydroxycarbamide in very young children with sickle-cell anaemia:a multicentre, randomised, controlled trial (BABY HUG). Lancet 377:1663–1672.
    OpenUrlCrossRefPubMedWeb of Science
  21. ↵
    1. Quarmyne MO,
    2. Dong W,
    3. Theodore R,
    4. Anand S,
    5. Barry V,
    6. Adisa O,
    7. et al.
    (2017) Hydroxyurea effectiveness in children and adolescents with sickle cell anemia:A large retrospective, population-based cohort. Am J Hematol 92:77–81.
    OpenUrlCrossRefPubMed
  22. ↵
    1. Ware RE
    (2010) How I use hydroxyurea to treat young patients with sickle cell anemia. Blood 115:5300–5311.
    OpenUrlAbstract/FREE Full Text
  23. ↵
    1. Heeney MM,
    2. Ware RE
    (2010) Hydroxyurea for children with sickle cell disease. Hematol Oncol Clin North Am 24:199–214.
    OpenUrlCrossRefPubMed
  24. ↵
    1. McGann PT,
    2. Ware RE
    (2015) Hydroxyurea therapy for sickle cell anemia. Expert Opin Drug Saf 14:1749–1758.
    OpenUrlCrossRefPubMed
  25. ↵
    1. Silva-Pinto AC,
    2. Angulo IL,
    3. Brunetta DM,
    4. Neves FI,
    5. Bassi SC,
    6. Santis GC,
    7. et al.
    (2013) Clinical and hematological effects of hydroxyurea therapy in sickle cell patients:a single-center experience in Brazil. Sao Paulo Med J 131:238–243.
    OpenUrlPubMed
  26. ↵
    1. Miller ST,
    2. Sleeper LA,
    3. Pegelow CH,
    4. Enos LE,
    5. Wang WC,
    6. Weiner SJ,
    7. et al.
    (2000) Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med 342:83–89.
    OpenUrlCrossRefPubMedWeb of Science
  27. ↵
    1. Ferster A,
    2. Vermylen C,
    3. Cornu G,
    4. Buyse M,
    5. Corazza F,
    6. Devalck C,
    7. et al.
    (1996) Hydroxyurea for treatment of severe sickle cell anemia:a pediatric clinical trial. Blood 88:1960–1964.
    OpenUrlAbstract/FREE Full Text
    1. Gladwin MT,
    2. Shelhamer JH,
    3. Ognibene FP,
    4. Pease-Fye ME,
    5. Nichols JS,
    6. Link B,
    7. et al.
    (2002) Nitric oxide donor properties of hydroxyurea in patients with sickle cell disease. Br J Haematol 116:436–444.
    OpenUrlCrossRefPubMedWeb of Science
    1. Hillery CA,
    2. Du MC,
    3. Wang WC,
    4. Scott JP
    (2000) Hydroxyurea therapy decreases the in vitro adhesion of sickle erythrocytes to thrombospondin and laminin. Br J Haematol 109:322–327.
    OpenUrlCrossRefPubMedWeb of Science
    1. Odièvre MH,
    2. Bony V,
    3. Benkerrou M,
    4. Lapouméroulie C,
    5. Alberti C,
    6. Ducrocq R,
    7. et al.
    (2008) Modulation of erythroid adhesion receptor expression by hydroxyurea in children with sickle cell disease. Haematologica 93:502–510.
    OpenUrlAbstract/FREE Full Text
  28. ↵
    1. Jain DL,
    2. Sarathi V,
    3. Desai S,
    4. Bhatnagar M,
    5. Lodha A
    (2012) Low fixed-dose hydroxyurea in severely affected Indian children with sickle cell disease. Hemoglobin 36:323–332.
    OpenUrlCrossRefPubMed
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The role of hydroxyurea in decreasing the occurrence of vasso-occulusive crisis in pediatric patients with sickle cell disease at King Saud Medical City in Riyadh, Saudi Arabia
Fauzia R. Azmet, Fawaz Al-Kasim, Walid M. Alashram, Khawar Siddique
Saudi Medical Journal Jan 2020, 41 (1) 46-52; DOI: 10.15537/smj.2020.1.24698

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The role of hydroxyurea in decreasing the occurrence of vasso-occulusive crisis in pediatric patients with sickle cell disease at King Saud Medical City in Riyadh, Saudi Arabia
Fauzia R. Azmet, Fawaz Al-Kasim, Walid M. Alashram, Khawar Siddique
Saudi Medical Journal Jan 2020, 41 (1) 46-52; DOI: 10.15537/smj.2020.1.24698
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Keywords

  • sickle cell disease
  • hydroxyurea
  • pediatric
  • vaso-occlusive crisis

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