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Study of Prognostic Factors of Death in Children with Sickle Cell Diseases Followed at the Albert Royer National Children's Hospital Center, Dakar, Senegal

Received: 9 December 2019    Accepted: 2 January 2020    Published: 21 January 2020
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Abstract

Objectives. The objectives of our study are: i) estimate the survival time of cases of sickle cell diseases monitored at the Albert Royer National Children's Hospital Center, Dakar, Senegal from 1990 to 2010, ii) identify prognostic factors related to deaths. Methods. Comprehensive and retrospective survival analysis of the prognostic factors of death of the 1650 patients with sickle cell disease followed in the sickle cell management unit of Albert Royer National Children's Hospital Center from January 1st, 1991 to December 31, 2010 (20 years). 17 variables were studied: gender, ethnicity, geographical origin, age of the patient at onset of the disease, age of the patient at onset of follow-up, number of vaso-occlusive crisis in the last year of follow-up duration of which is ≥72h, type of hemoglobinopathy, baseline hemoglobin level, fetal hemoglobin level at time of diagnosis, vaccination with at least one antigen, regular folic acid intake, regular penicillin intake, hydroxyurea therapy, acute complications (severe infections, severe anemia and serious vaso-occlusive accidents) in the last year of follow-up and chronic complications. The Cox model was used. Results. We conducted 1650 observations with 44 deaths, and a lethality of 2.6%. The death incidence rate is 3.51 deaths per 100 person-years. The Cox model highlighted the prognostic factors which significantly explain the model (p < 0.05). Vaccination with at least one antigen and the existence of chronic complications improved patient survival. However, the number of vaso-occlusive crisis in the last year of follow-up (duration is ≥72h), the existence of serious vaso-occlusive accidents in the last year of follow-up, the very low baseline hemoglobin level (≤5mg/100 mn), the early age of late follow-up (≥15 months) reduced patients survival. Conclusions. The prognosis of patients with sickle cell disease followed at the Albert Royer National Children's Hospital Center (ARNCHC) is difficult to establish. In fact, in addition to genetic, clinical and evolutionary factors, there are poorly understood environmental and socio-economic factors that affect survival. A prospective study would shed more light on the prognostic factors of death in children with sickle cell disease.

Published in American Journal of Pediatrics (Volume 6, Issue 1)
DOI 10.11648/j.ajp.20200601.11
Page(s) 1-11
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This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

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Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Sickle Cell Disease, Cox Model, Survival Study, Senegal

References
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    Boubacar Gueye, Cheikh Tacko Diop, Ndéye Marième Diagne, Aminata Paye, Ibrahima Diagne. (2020). Study of Prognostic Factors of Death in Children with Sickle Cell Diseases Followed at the Albert Royer National Children's Hospital Center, Dakar, Senegal. American Journal of Pediatrics, 6(1), 1-11. https://doi.org/10.11648/j.ajp.20200601.11

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    Boubacar Gueye; Cheikh Tacko Diop; Ndéye Marième Diagne; Aminata Paye; Ibrahima Diagne. Study of Prognostic Factors of Death in Children with Sickle Cell Diseases Followed at the Albert Royer National Children's Hospital Center, Dakar, Senegal. Am. J. Pediatr. 2020, 6(1), 1-11. doi: 10.11648/j.ajp.20200601.11

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    AMA Style

    Boubacar Gueye, Cheikh Tacko Diop, Ndéye Marième Diagne, Aminata Paye, Ibrahima Diagne. Study of Prognostic Factors of Death in Children with Sickle Cell Diseases Followed at the Albert Royer National Children's Hospital Center, Dakar, Senegal. Am J Pediatr. 2020;6(1):1-11. doi: 10.11648/j.ajp.20200601.11

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  • @article{10.11648/j.ajp.20200601.11,
      author = {Boubacar Gueye and Cheikh Tacko Diop and Ndéye Marième Diagne and Aminata Paye and Ibrahima Diagne},
      title = {Study of Prognostic Factors of Death in Children with Sickle Cell Diseases Followed at the Albert Royer National Children's Hospital Center, Dakar, Senegal},
      journal = {American Journal of Pediatrics},
      volume = {6},
      number = {1},
      pages = {1-11},
      doi = {10.11648/j.ajp.20200601.11},
      url = {https://doi.org/10.11648/j.ajp.20200601.11},
      eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajp.20200601.11},
      abstract = {Objectives. The objectives of our study are: i) estimate the survival time of cases of sickle cell diseases monitored at the Albert Royer National Children's Hospital Center, Dakar, Senegal from 1990 to 2010, ii) identify prognostic factors related to deaths. Methods. Comprehensive and retrospective survival analysis of the prognostic factors of death of the 1650 patients with sickle cell disease followed in the sickle cell management unit of Albert Royer National Children's Hospital Center from January 1st, 1991 to December 31, 2010 (20 years). 17 variables were studied: gender, ethnicity, geographical origin, age of the patient at onset of the disease, age of the patient at onset of follow-up, number of vaso-occlusive crisis in the last year of follow-up duration of which is ≥72h, type of hemoglobinopathy, baseline hemoglobin level, fetal hemoglobin level at time of diagnosis, vaccination with at least one antigen, regular folic acid intake, regular penicillin intake, hydroxyurea therapy, acute complications (severe infections, severe anemia and serious vaso-occlusive accidents) in the last year of follow-up and chronic complications. The Cox model was used. Results. We conducted 1650 observations with 44 deaths, and a lethality of 2.6%. The death incidence rate is 3.51 deaths per 100 person-years. The Cox model highlighted the prognostic factors which significantly explain the model (p < 0.05). Vaccination with at least one antigen and the existence of chronic complications improved patient survival. However, the number of vaso-occlusive crisis in the last year of follow-up (duration is ≥72h), the existence of serious vaso-occlusive accidents in the last year of follow-up, the very low baseline hemoglobin level (≤5mg/100 mn), the early age of late follow-up (≥15 months) reduced patients survival. Conclusions. The prognosis of patients with sickle cell disease followed at the Albert Royer National Children's Hospital Center (ARNCHC) is difficult to establish. In fact, in addition to genetic, clinical and evolutionary factors, there are poorly understood environmental and socio-economic factors that affect survival. A prospective study would shed more light on the prognostic factors of death in children with sickle cell disease.},
     year = {2020}
    }
    

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  • TY  - JOUR
    T1  - Study of Prognostic Factors of Death in Children with Sickle Cell Diseases Followed at the Albert Royer National Children's Hospital Center, Dakar, Senegal
    AU  - Boubacar Gueye
    AU  - Cheikh Tacko Diop
    AU  - Ndéye Marième Diagne
    AU  - Aminata Paye
    AU  - Ibrahima Diagne
    Y1  - 2020/01/21
    PY  - 2020
    N1  - https://doi.org/10.11648/j.ajp.20200601.11
    DO  - 10.11648/j.ajp.20200601.11
    T2  - American Journal of Pediatrics
    JF  - American Journal of Pediatrics
    JO  - American Journal of Pediatrics
    SP  - 1
    EP  - 11
    PB  - Science Publishing Group
    SN  - 2472-0909
    UR  - https://doi.org/10.11648/j.ajp.20200601.11
    AB  - Objectives. The objectives of our study are: i) estimate the survival time of cases of sickle cell diseases monitored at the Albert Royer National Children's Hospital Center, Dakar, Senegal from 1990 to 2010, ii) identify prognostic factors related to deaths. Methods. Comprehensive and retrospective survival analysis of the prognostic factors of death of the 1650 patients with sickle cell disease followed in the sickle cell management unit of Albert Royer National Children's Hospital Center from January 1st, 1991 to December 31, 2010 (20 years). 17 variables were studied: gender, ethnicity, geographical origin, age of the patient at onset of the disease, age of the patient at onset of follow-up, number of vaso-occlusive crisis in the last year of follow-up duration of which is ≥72h, type of hemoglobinopathy, baseline hemoglobin level, fetal hemoglobin level at time of diagnosis, vaccination with at least one antigen, regular folic acid intake, regular penicillin intake, hydroxyurea therapy, acute complications (severe infections, severe anemia and serious vaso-occlusive accidents) in the last year of follow-up and chronic complications. The Cox model was used. Results. We conducted 1650 observations with 44 deaths, and a lethality of 2.6%. The death incidence rate is 3.51 deaths per 100 person-years. The Cox model highlighted the prognostic factors which significantly explain the model (p < 0.05). Vaccination with at least one antigen and the existence of chronic complications improved patient survival. However, the number of vaso-occlusive crisis in the last year of follow-up (duration is ≥72h), the existence of serious vaso-occlusive accidents in the last year of follow-up, the very low baseline hemoglobin level (≤5mg/100 mn), the early age of late follow-up (≥15 months) reduced patients survival. Conclusions. The prognosis of patients with sickle cell disease followed at the Albert Royer National Children's Hospital Center (ARNCHC) is difficult to establish. In fact, in addition to genetic, clinical and evolutionary factors, there are poorly understood environmental and socio-economic factors that affect survival. A prospective study would shed more light on the prognostic factors of death in children with sickle cell disease.
    VL  - 6
    IS  - 1
    ER  - 

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Author Information
  • Health and Sustainable Development Department, Alouine Diop University, Bambeye, Senegal

  • Health and Sustainable Development Department, Alouine Diop University, Bambeye, Senegal

  • Principal Hospital of Dakar, Dakar, Senegal

  • Albert Royer National Children's Hospital Center, Dakar, Senegal

  • Health Training and Research Unit, Gaston Berger University, Saint-Louis, Senegal

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