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
Volume 6, Issue 1, March 2020, Pages: 1-11
Received: Dec. 9, 2019; Accepted: Jan. 2, 2020; Published: Jan. 21, 2020
Views 114      Downloads 53
Authors
Boubacar Gueye, Health and Sustainable Development Department, Alouine Diop University, Bambeye, Senegal
Cheikh Tacko Diop, Health and Sustainable Development Department, Alouine Diop University, Bambeye, Senegal
Ndéye Marième Diagne, Principal Hospital of Dakar, Dakar, Senegal
Aminata Paye, Albert Royer National Children's Hospital Center, Dakar, Senegal
Ibrahima Diagne, Health Training and Research Unit, Gaston Berger University, Saint-Louis, Senegal
Article Tools
Follow on us
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.
Keywords
Sickle Cell Disease, Cox Model, Survival Study, Senegal
To cite this article
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, American Journal of Pediatrics. Vol. 6, No. 1, 2020, pp. 1-11. doi: 10.11648/j.ajp.20200601.11
Copyright
Copyright © 2020 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
References
[1]
Montalembert GR de. Drépanocytose chez l’enfant. In: EMC (Elsevier SAS, Paris), Pédiatrie, 4-080-A-20, 2006.
[2]
Wajcman H, Galacteros F. Drépanocytose : laboratoire et étude de l’hémoglobine. Bull Soc Pathol Exot, 2001, 94, 80-84.
[3]
Piel FB, Hay SI, Gupta S, Weatherall DJ, Williams TN. Global Burden of Sickle Cell Anaemia in Children under Five, 2010–2050: Modelling Based on Demographics, Excess Mortality, and Interventions. PLoS Med [Internet]. juill 2013 [cité 30 juill 2015]; 10 (7). Disponible sur: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3712914/.
[4]
Piel FB, Patil AP, Howes RE, Nyangiri OA, Gething PW, Dewi M, et al. Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimates. Lancet. janv 2013; 381: 142-51.
[5]
Ware RE. Is Sickle Cell Anemia a Neglected Tropical Disease? PLoS Negl Trop Dis [Internet]. 30 mai 2013 [cité 5 août 2015]; 7 (5). Disponible sur: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3671937/.
[6]
Galacteros F. Drépanocytose. Physiopathologie et diagnostic. Rev Prat. 1995; (45): 351-60.
[7]
Buchanan GR, DeBaun MR, Quinn CT, Steinberg MH. Sickle Cell Disease. J Am Soc Hematol. 2004; 35-47.
[8]
Kete CV. Dépistage néonatal de la drépanocytose par la méthode d’isoelectrofocalisation de l’hémoglobine (cas de 518 nouveau-nés au centre hospitalier Abass Ndao de Dakar) [Thèse Doctorat en Pharmacie]. [Dakar (Sénégal)]: Université Cheikh Anta Diop; 1998.
[9]
Gody JC, Yanza MC, Boka - Yao A. Aspects de la drépanocytose au complexe pédiatrique de Bangui (République de la Centrafrique) : à propos de 123 cas. Médecine Afr Noire. 2007; 54 (11): 596-600.
[10]
Elira-Dokekias A. Étude analytique des facteurs d’aggravation de la maladie drepanocytaire au Congo. Médecine Afr Noire. 1996; 43 (5): 279-85.
[11]
Cameron BF, Christian E, Lobel JS, Gaston MH. Evaluation of Clinical Severity in Sickle Cell Disease. J Natl Med Assoc. mai 1983; 75 (5): 483-7.
[12]
Van-Dunen J, Alves J, Bernardino L, Figueiroa J, Braga C, Do Nascimento Mde L, et al. Factors associated with sickle cell disease mortality among hospitalized Angolan children and adolescents. West Afr J Med. 2007; 26 (4): 269-73.
[13]
Diop S, Cissé M, Touré-Fall A, Thiam D, Gadji M. La drépanocytose homozygote au Sénégal: influence du taux d’hémoglobine foetal F, des facteurs socioculturels et économiques. Dakar Med. 1999; 171-4.
[14]
Makani J, Cox SE, Soka D, Komba AN, Oruo J, Mwamtemi H, et al. Mortality in Sickle Cell Anemia in Africa: A Prospective Cohort Study in Tanzania. PLoS ONE [Internet]. 16 févr 2011 [cité 5 août 2015]; 6 (2). Disponible sur: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3040170/.
[15]
Grosse SD, Odame I, Atrash HK, Amendah DD, Piel FB, Williams TN. Sickle Cell Disease in Africa. Am J Prev Med. déc 2011; 41 (6): S398-405.
[16]
VOCkaridou E, Kattamis A, Hassapopoulou E, Economou M, Kourakli A, Maragkos K. A national registry of haemoglobinopathies in Greece: Deducted demographics, trends in mortality and affected births. Ann Hematol. 2012; 91: 1451-8.
[17]
Thomas C, Bernaudin F, Feingold J, Guillou-Bataille M, Reinert P, Réseau INSERM de recherche clinique sur la drépanocytose. Drépanocytose : étude de la mortalité pédiatrique en lle de France de 1985 à 1992. Arch Pédiatrie Paris. 3: 445-51.
[18]
Kremp O, Paty AC, Suzan F. Mortalité liée à la drépanocytose en France de 0 à 18 ans. Arch Pédiatrie Paris. 2008; 15 (5): 629-32.
[19]
Powars DR, Chan LS, Hiti A, Ramicone E, Johnson C. Outcome of Sickle Cell Anemia A 4-Decade Observational Study of 1056 Patients. Medicine (Baltimore). 2005; 84 (6): 363-76.
[20]
Organisation Mondiale de la Santé. Drépanocytose: une stratégie pour la région africaine de l’OMS. OMS AFR/RC60/8. 2010;
[21]
Quinn CT, Rogers ZR, McCavit TL, Buchanan GR. Improved survival of children and adolescents with sickle cell disease. Blood. 29 avr 2010; 115 (17): 3447-52.
[22]
Quinn CT, Rogers ZR, Buchanan GR. Survival of children with sickle cell disease. Blood. 1 juin 2004; 103 (11): 4023-7.
[23]
Schog JB, Duits AJ, Muskiet FAJ, Ten Cate H, Rojer RA, Brandjes DPM. Sickle cell disease; a general overview. Neth J Med. nov 2004; 62 (10): 364-74.
[24]
Ye D, Koueta F, Dao L. Prise en charge de la drépanocytose en milieu pédiatrique : expérience du centre hospitalier universitaire pédiatrique Charles-de-Gaulle de Ouagadougou (Burkina Faso). Cah Santé Montrouge. 2008; 18 (2): 71-5.
[25]
Ekhtel BMMS. Létalité de la drépanocytose de l’enfant et de l’adolescent: expérience de la cohorte du Centre Hospitalier National Albert Royer (Dakar) [Diplôme d’Etudes Spéciales en Pédiatrie]. [Dakar (Sénégal)]: Faculté de Médecine et d’Odontostomatologie, Université Cheikh Anta Diop; 2003.
[26]
Sebastiani P, Nolan VG, Baldwin CT, Abad-Grau MM, Wang L, Adewoye AH, et al. A network model to predict the risk of death in sickle cell disease. Blood. 1 oct 2007; 110 (7): 2727-35.
[27]
Gill FM, Sleeper LA, Weiner SJ, Bellevue R, Grover R. Clinical events in the first decade in a cohort of infants with sickle cell disease. Blood. 1995; 776-83.
[28]
Lionnet F, Nadjib N, Katia H, Stojanovic S, Avellino V, Haymann J-P. Hemoglobin sickle cell disease complications: a clinical study of 179 cases. Haematologica. 2012; 97 (8): 1136-41.
[29]
Lepage P, Forget PMF, Schmitz V, Hoyoux C. Infection et prophylaxies anti-infectieuses dans la drépanocytose. Rev Médicale Liége. 2013; 59 (3): 145-8.
[30]
Bégué P, Castello-Herbreteau B. La drépanocytose : de l’enfant à l’adolescent. Prise en charge en 2001. Bull Soc Pathol Exot. 2001; 94 (2): 85-9.
[31]
Sanda A. Les principales causes de mortalité dans le service de pédiatrie B. Rev Médicale Côte Ivoire. 1987; (82): 51-2.
[32]
Diagne I, Ndiaye O, Moreira C, Signaté H, Camara B, Diouf S. Les syndromes drépanocytaires majeurs en pédiatrie à Dakar. Arch. de Pédiatrie (Paris). 7 (1) 2000; 16-24.
[33]
Chambers RL, Skinner CJ. Analysis of survey data [Internet]. 2003. Disponible sur: http://public.eblib.com/choice/publicfullrecord.aspx?p=159632.
[34]
Hyman HH. Survey Design and Analysis: Principles, Cases and Procedures [Internet]. Free Press of Glencoe; 1955. Disponible sur: https://books.google.sn/books?id=nQqPAAAAIAAJ.
[35]
Hill C, Com-Nougué C, Kramar A. Analyse statistique des données de survie [Internet]. Flammarion Médecine-Sciences; 1996. (Statistique en biologie et en médecine). Disponible sur: https://books.google.sn/books?id=xVooAAAACAAJ.
[36]
Athale UH, Chintu C. Clinical analysis of mortality in hospitalized Zambian children with sickle cell anaemia. East Afr Med J. 1994; 71 (6): 388-91.
[37]
Rogers DM, Clarke JM. Early death in Jamaica children with sickle-cell disease. Br Med J. 1978 Jun 10; 1 (6126): 515-6.
[38]
Seeler RA. Deaths in children with sickle-cell anemia. A clinical analysis of 19 fatals instances in Chicago. Clin Pediatr (Phila). 1972 Nov; 11 (11): 634-7
[39]
Kampatide N, Ategbo S, Bodjona H, Assimadi K. Hôpital et drépanocytose. In Drépanocytose et Santé publique. Coéd. INSERM/Centre International de l’Enfance, Paris, 1997, 1: 91-95.
[40]
David H, Gergen PJ, Moore Jr RM. Geographic differences in mortality of young children with sickle cell disease in United States. Public Health Rep. 1997; (152): 52-8.
[41]
Koko J, Dufillot D, M’ba-Meyo J. Mortalité des enfants drépanocytaires dans un service de pédiatrie en Afrique Centrale. Arch Pédiatrie Paris. 1998; 5 (9): 965-9.
[42]
Begue P, Castello-Herbreteau B. Infections graves chez l’enfant drépanocytaire : aspects cliniques et prévention. Arch Pédiatrie Paris. 2001; 8 (suppl4): 732-41.
[43]
Mabiala Babela JR, Nzingoula S, Senga P. Les crises vaso-occlusives drépanocytaires chez l’enfant et l’adolescent à Brazzaville, Congo. Étude rétrospective de 587 cas. Bull Soc Pathol Exo. 2005; 98 (5): 365-70.
[44]
Quinn CT, Shull EP, Naveed A, Lee NJ, Rogers ZR, Buchanan GR. Clinical Trials and Observations Prognostic significance of early vaso-occlusive complications in children with sickle cell anemia. Blood. janv 2007; 109 (1): 40-5.
[45]
Chekoury A, Laporte M, Dupy E. Les accidents vasculaires cérébraux dans la drépanocytose Stroke in sickle cell disease. Sang Thromb Vaiss. 2008; 20 (2): 77-81.
[46]
Allareddy V, Roy A, Lee MK, Nalliah RP, Rampa S, Allareddy V, et al. Outcomes of Acute Chest Syndrome in Adult Patients with Sickle Cell Disease: Predictors of Mortality. PLoS ONE [Internet]. 16 avr 2014 [cité 5 août 2015]; 9 (4). Disponible sur: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3989222/.
[47]
Castro O, Brambilla DJ, Thorington B, Reindorf CA, Scott RB, Gillette P, et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood. 15 juill 1994; 84 (2): 643-9.
[48]
Lanzkron S, Carroll CP, Haywood C. Mortality Rates and Age at Death from Sickle Cell Disease: U.S., 1979–2005. Public Health Rep. 2013; 128 (2): 110-6.
[49]
Benkerrou M, Brahimi L, Vilmer E. L’anémie chez l’enfant drépanocytaire. Ann Pédiatrie Paris. 1999; 46 (7): 479-85.
[50]
Diagne I, Diagne-Gueye NDR, Signaté H, Camara B, Lopez-Sall Ph, Diack-Mbaye A. Prise en charge de la drepanocytose chez l’enfant en afrique: experience de la cohorte de l’hopital d’enfants Albert Royer de Dakar. Médecine Trop. 2003; (63): 513-20.
[51]
Beyeme-Owono M, Chiabi A. Physiopathologie et clinique de la drépanocytose chez l’enfant. Clin Mother Child Health. 200 4 jan; 1 (1): 37-42.
[52]
Vichinsky E. Comprehensive care in sickle-cell disease: its impact on morbidity and mortality. Journal Semin Hematol. 1991 Jul; 28 (3): 220-6.
[53]
Labie D, Elion J. La drépanocytose: problème de l’Afrique. Médecine Trop. 2010; (70): 449-53.
[54]
Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, et al. Mortality in sickle cell disease. Life expectancy and risk factors for early death. N Engl J Med. 1994 june 9; 330 (23): 1639-44.
[55]
Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A, et al. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. JAMA. 2003 apr; 289 (13): 1645-51.
[56]
Strouse JJ, Heeney MM. Hydroxyurea for the Treatment of Sickle Cell Disease: Efficacy, Barriers, Toxicity, and Management in Children. Pediatr Blood Cancer. 2012 august; 59 (2): 365-71.
[57]
Montalembert MD, Tshilolo L. Les progrès thérapeutiques dans la prise en charge de la drépanocytose sont-ils applicables en Afrique subsaharienne. Médecine Trop. 2007; (67): 612-6.
[58]
Lê PQ, Gulbis B, Dedeken L, Dupont S, Vanderfaeillie A, Heijmans C, et al. Survival among children and adults with sickle cell disease in Belgium: Benefit from hydroxyurea treatment. Pediatr Blood Cancer.2015 Nov; 62 (11): 1956-61. doi: 10.1002/pbc.25608. Epub 2015 Jul 14.
[59]
Gaston MH, Verter JI, Woods GM. Prophylaxis with oral penicillin in children with sickle cell anemia. N Engl J Med. 1986; 314 (25): 1593-9.
[60]
Diallo D, Tchernia G. Sickle cell disease in Africa Current. Opin Hematol. 2002; (9): 111-6.
[61]
Williams TN, Obaro SK. Sickle cell disease and malaria morbidity: a tale with two tails. Trends Parasitol. jul 2011; 27 (7): 315-20.
[62]
Darbari DS, Wang Z, Kwak M, Hildesheim M, Nichols J, Allen D, et al. Severe Painful Vaso-Occlusive Crises and Mortality in a Contemporary Adult Sickle Cell Anemia Cohort Study. PLoS ONE [Internet]. 5 nov 2013 [cité 5 août 2015]; 8 (11). Disponible sur: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3818240/.
[63]
Ndiaye A. Syndromes drépanocytaires majeurs associés aux pathologies chroniques chez l’enfant et l’adolescent: interférences sémiologiques, évolutives et thérapeutiques [Thèse Doctorat en Médecine]. [Dakar (Sénégal)]: Université Cheikh Anta Diop; 2011.
[64]
Djembo TM, Tchiloemba M, Galacteros F, Rosa J, Lissouba P. Étude épidémiologique des hémoglobinopathies au Congo chez 2257 nouveau-nés. Nouv Rev Fr Hématologie 1986; (28): 249-51.
[65]
Steinberg MH. Predicting clinical severity in sickle cell anaemia. Br J Haematol. 2005 may; 129 (4): 465-81.
[66]
Tchokoteu PF. La drépanocytose de l’enfant: aspects cliniques et prise en charge. Clin Mother Child Health. 2004; 1 (1): 21-9.
ADDRESS
Science Publishing Group
1 Rockefeller Plaza,
10th and 11th Floors,
New York, NY 10020
U.S.A.
Tel: (001)347-983-5186