Objective: To establish the epidemiological profile of complications of major sickle cell syndrome in acute situations. Patients and Methods: This was a prospective, longitudinal, multicenter, one-year cohort study, including patients over the age of 3 years with major sickle cell syndrome presenting for an acute complication in major university hospitals. Their history, clinical, and paraclinical data were reviewed. Results: 67 patients were included, with a mean age of 19 years. The sex ratio was 1.23, with 36 men and 31 women. There was a clear predominance of the SS phenotype (87% of cases). In history, there was a clear predominance of VOC (77% of cases) and infections (malaria and pneumonia). Baseline hemoglobin was 7 g/dL. Usual analgesia included a combination of paracetamol and NSAIDs. Sixty-six percent of the included patients received no form of antimicrobial prophylaxis, and malaria prophylaxis was absent in 43% of cases. General signs were dominated by fever in 25% of patients. The main contributing factor was exposure to cold. Complications were dominated by VOC, decompensated by infection, in which malaria (53% of cases) and pneumonia (15% of these cases) were predominantly found. Conclusion: Acute complications of sickle cell syndromes affect young subjects with a history of SCD and infections. Prevention of these aggravating factors could significantly reduce the occurrence of these complications.
References
[1]
OMS, Organisation Mondiale de la Santé (2006) Cinquante-Neuvième Assemblée mondiale de la Santé. https://apps.who.int/gb/ebwha/pdf_files/WHA59REC3/A59_REC3-fr
[2]
Sundd, P., Gladwin, M.T. and Novelli, E.M. (2019) Pathophysiology of Sickle Cell Disease. Annual Review of Pathology: Mechanisms of Disease, 14, 263-292. https://doi.org/10.1146/annurev-pathmechdis-012418-012838
[3]
Haute Autorité de Santé (2010) Syndromes drépanocytaires majeurs de l’adulte: Protocole national de diagnostic et de soins pour une maladie rare. 87 p.
[4]
Kpakoutou, N.A. (2020) Complications pulmonaires chez les enfants drépanocytaires majeurs suivis dans le département de pédiatrie du CHU Gabriel. Thèse de Médecine, Université des Sciences, des Techniques et des Technologies de Bamako, 97 p.
[5]
Sonia, D., Kisito, N., Laure, T., Ismaël, T., Madibèlè, K., Fla, K., et al. (2017) Syndromes drépanocytaires majeurs et infections associées chez l’enfant au Burkina Faso. Pan African Medical Journal, 26, Article No. 7. https://doi.org/10.11604/pamj.2017.26.7.9971
[6]
Chetcha Chemegni, B., Ngouefo, G., Ngo Sack, F., Ngouadjeu, E., Kouemeni, L. and Kaptué Noche, L. (2017) Complications aigues de la drépanocytose aigues. 21, 18-23.
[7]
Dodo, R., Zohoun, A., Baglo, T., Mehou, J. and Anani, L. (2018) Urgences drépanocytaires au Service des Maladies du Sang du Centre National Hospitalier Universitaire-Hubert Koutoukou Maga de Cotonou, Benin. Pan African Medical Journal, 30, Article No. 192. https://doi.org/10.11604/pamj.2018.30.192.15931
[8]
Perignon, A., Botterel, F., Farrugia, C., et al. (2008) Paludisme et drépanocytose homozygote. Médecine et Maladies Infectieuses, 38, 140-141.
[9]
Platt, O.S., Brambilla, D.J., Rosse, W.F., Milner, P.F., Castro, O., Steinberg, M.H., et al. (1994) Mortality in Sickle Cell Disease: Life Expectancy and Risk Factors for Early Death. New England Journal of Medicine, 330, 1639-1644. https://doi.org/10.1056/nejm199406093302303
[10]
Ondo, A. and Ngou Milama, E. (1985) Evolution des protéines de l’inflammation chez le sujet drépanocytaire. Rissatma, 58, 329-338.
[11]
Thiam, L., Dramé, A., Coly, I.Z., Diouf, F.N., Seck, N., Boiro, D., et al. (2017) Profils épidemiologiques, cliniques et hématologiques de la drépanocytose homozygote SS en phase inter critique chez l’enfant à Ziguinchor, Sénégal. Pan African Medical Journal, 28, Article No. 208. https://doi.org/10.11604/pamj.2017.28.208.14006
[12]
Mbika Cardorelle, A., Okoko, A. and Mouko, A. (2010) Les crises vaso-occlusives de l’enfant drépanocytaire à Brazzaville. Archives de Pédiatrie, 17, 295-296. https://doi.org/10.1016/j.arcped.2009.11.009
[13]
Nacoulma, E.W.C., Sakande, J., Kafando, E., Kpowbié, E.D. and Guissou, I.P. (2006) Profil hématologique et biochimique des drépanocytaires SS et SC en phase stationnaire au Centre Hospitalier National Yalgado Ouedraogo de Ouagadougou. Mali Medical, 21, 8-11.
[14]
Ouakasse, S. (2015) Drépanocytose homozygote chez l’enfant à l’hôpital provincial de Tanger: À propos de 10 cas. Thèse de Médecine, Université Mohammed V de Rabat, 84 p.
[15]
Dreux, O. (2012) Education thérapeutique pour les enfants drépanocytaires: Justifications à la mise en place et initiation de ce projet au CHU de Grenoble. Thèse de Médecine, Université Joseph Fournier, 87 p.
[16]
Thuilliez, V. and Vierin, Y. (1997) Le poids de la drépanocytose en milieu pédiatrique au Gabon. Santé Publique, 1, 45-60.
[17]
Nagel, R.L., Fabry, M.E. and Steinberg, M.H. (2003) The Paradox of Hemoglobin SC Disease. Blood Reviews, 17, 167-178. https://doi.org/10.1016/s0268-960x(03)00003-1
[18]
Biaz, A., Neji, M., Ajhoun, Y., Machtani, S.E., Dami, A., Reda, K., et al. (2017) Découverte fortuite d’une drépanocytose hétérozygote composite S/C. Pan African Medical Journal, 27, Article No. 93. https://doi.org/10.11604/pamj.2017.27.93.12724
[19]
Markham, M.J., Lottenberg, R. and Zumberg, M. (2003) Role of Phlebotomy in the Management of Hemoglobin SC Disease: Case Report and Review of the Literature. American Journal of Hematology, 73, 121-125. https://doi.org/10.1002/ajh.10328
[20]
Bouchaïr, N., Manigne, P., Kanfer, A., Raphalen, P., de Montalembert, M., Hagege, I., et al. (2000) Prevention of Sickle Cell Crises with Multiple Phlebotomies. Archives de Pédiatrie, 7, 249-255. https://doi.org/10.1016/s0929-693x(00)88740-0
[21]
Darbari, D.S., Sheehan, V.A. and Ballas, S.K. (2020) The Vaso-Occlusive Pain Crisis in Sickle Cell Disease: Definition, Pathophysiology, and Management. European Journal of Haematology, 105, 237-246.
[22]
Darbari, D.S., Sheehan, V.A. and Ballas, S.K. (2020) The Vaso-Occlusive Pain Crisis in Sickle Cell Disease: Definition, Pathophysiology, and Management. European Journal of Haematology, 105, 237-246. https://doi.org/10.1111/ejh.13430
[23]
Bertholdt (2012) Les complications respiratoires chez les enfants: Le syndrome thoracique aigu. Revue Médicale de Bruxelles, 33, 138-144.
[24]
Maître, B., Mekontso-Dessap, A., Habibi, A., Bachir, D., Parent, F., Godeau, B., et al. (2011) Complications pulmonaires des syndromes drépanocytaires majeurs chez l’adulte. Revue des Maladies Respiratoires, 28, 129-137. https://doi.org/10.1016/j.rmr.2010.08.013
[25]
Bopp, T., Stephan, C., Samii, K. and Stirnemann, J. (2018) Syndrome thoracique aigu: Une complication grave de la drépanocytose. Revue Médicale Suisse, 14, 1844-1848. https://doi.org/10.53738/revmed.2018.14.623.1844
[26]
Beyeme-Owono, M. and Chiabi, A. (2004) Epidémiologie de la Drépanocytose. CMCH, 1, 6-8.
[27]
Allison, A.C. (1954) Protection Afforded by Sickle-Cell Trait against Subtertian Malarial Infection. BMJ, 1, 290-294. https://doi.org/10.1136/bmj.1.4857.290
[28]
Drépanocytose la maladie 2016. https://drepaconseils.org
[29]
Ondo, A., Nguéma Mvé, R. and Obame Edou, S. (1984) Aspects actuels du traitement des crises aigues de la drépanocytose: Formule H.A.A.T.O.P. In: Begue, P., Ed., La Maladie Drépanocytaire, Sandoz Edition, 128-139.
[30]
Délicat-Loembet, L.M., Elguero, E., Arnathau, C., Durand, P., Ollomo, B., Ossari, S., et al. (2014) Prevalence of the Sickle Cell Trait in Gabon: A Nationwide Study. Infection, Genetics and Evolution, 25, 52-56. https://doi.org/10.1016/j.meegid.2014.04.003
[31]
Chou, S.T. (2013) Transfusion Therapy for Sickle Cell Disease: A Balancing Act. Hematology, 2013, 439-446. https://doi.org/10.1182/asheducation-2013.1.439
[32]
Kple-Faget, P., Seka Seka, J., Akre Dagra, P., et al. (1996) La transfusion sanguine chez les enfants drépanocytaires au CHU d’Abidjan. Médecine d’Afrique Noire, 43, 650-653.
[33]
Steinberg, M.H., McCarthy, W.F., Castro, O., Ballas, S.K., Armstrong, F.D., Smith, W., et al. (2010) The Risks and Benefits of Long-Term Use of Hydroxyurea in Sickle Cell Anemia: A 17.5 Year Follow-Up. American Journal of Hematology, 85, 403-408. https://doi.org/10.1002/ajh.21699
[34]
Haute Autorité de Santé (2005) Prise en charge de la drépanocytose chez l’enfant et l’adolescent: Recommandations pour la pratique clinique. 32 p.
[35]
Tshilolo, L., Tomlinson, G., Williams, T.N., Santos, B., Olupot-Olupot, P., Lane, A., et al. (2019) Hydroxyurea for Children with Sickle Cell Anemia in Sub-Saharan Africa. New England Journal of Medicine, 380, 121-131. https://doi.org/10.1056/nejmoa1813598
[36]
Ondo, A., Ngou Milama, E., Obame Edou, S.Y. and Nguema Mve, R. (1984) La thérapeutique transfusionnelle chez le drépanocytaire: Expérience en zone tropicale. In: Begue, P., Ed., La Maladie Drépanocytaire, Sandoz Édition, 289-297.
[37]
Ondo, A., Obame Nguéma, R., et al. (1984) Etude des crises aigues chez les enfants drépanocytaires gabonais. In: Begue, P., Ed., La Maladie Drépanocytaire, Sandoz Édition, 48-52.
[38]
Mekontso Dessap, A., Contou, D., Dandine-Roulland, C., Hemery, F., Habibi, A., Charles-Nelson, A., et al. (2014) Environmental Influences on Daily Emergency Admissions in Sickle-Cell Disease Patients. Medicine, 93, e280. https://doi.org/10.1097/md.0000000000000280
[39]
Mohan, J., Marshall, J.M., Reid, H.L., Thomas, P.W., Hambleton, I. and Serjeant, G.R. (1998) Peripheral Vascular Response to Mild Indirect Cooling in Patients with Homozygous Sickle Cell (SS) Disease and the Frequency of Painful Crisis. Clinical Science, 94, 111-120. https://doi.org/10.1042/cs0940111
[40]
Mohan, J.S., Marshall, J.M., Reid, H.L., Thomas, P.W., Hambleton, I. and Serjeant, G.R. (1998) Comparison of Responses Evoked by Mild Indirect Cooling and by Sound in the Forearm Vasculature in Patients with Homozygous Sickle Cell Disease and in Normal Subjects. Clinical Autonomic Research, 8, 25-30. https://doi.org/10.1007/bf02267600
[41]
Koko, J. (2013) Prise en charge de l’enfant drépanocytaire au Gabon. Université de Stragbourg. 8e Congrès drépanocytose.
[42]
Bartolucci, P., Habibi, A., Khellaf, M., Roudot-Thoraval, F., Melica, G., Moutereau, S., et al. (2014) Score prédictif de survenue d’un syndrome thoracique durant une crise vaso-occlusive drépanocytaire dès l’arrivée aux urgences. La Revue de Médecine Interne, 35, A39-A40. https://doi.org/10.1016/j.revmed.2014.10.058
[43]
Chekoury, A., Laporte, M. and Dupuy, E. (2008) Les accidents vasculaires cérébraux dans la drépanocytose. Sang Thrombose Vaisseaux, 20, 77-81.
[44]
Ndiaye, M., Sow, A.D., Basse, A.M., Diagne, N.S., Seck, L.B., Sami, M.L., et al. (2015) Complications neurologiques de la drépanocytose. Revue Neurologique, 171, A130. https://doi.org/10.1016/j.neurol.2015.01.293
[45]
Bernaudin, F. and Verlhac, S. (2008) Accidents vasculaires cérébraux des drépanocytaires: Apport du Doppler transcrânien et de l’imagerie en résonance magnétique. Hématologie, 14, 25-35.
[46]
Adams, R.J., Nichols, F.T., Figueroa, R., McKie, V. and Lott, T. (1992) Transcranial Doppler Correlation with Cerebral Angiography in Sickle Cell Disease. Stroke, 23, 1073-1077. https://doi.org/10.1161/01.str.23.8.1073
[47]
Koreissi, M. (2009) Complications aigues de la drépanocytose dans le service de pédiatrie du CHU Gabriel Touré. These de Médecine, Université de Bamako, 95 p.
[48]
Mabiala-Babéla, J.R., Nkanza-Kaluwako, S.A.T., Ganga Zandzou, P.S., et al. (2005) Causes d’hospitalisation des enfants drépanocytaires: influence de l’âge (CHU de Brazzaville, Congo). Bulletin de la Société de Pathologie Exotique, 98, 392-393.
[49]
Dembele, A. (2008) prise en charge de la crise douloureuse drépanocytaire selon les critères de l’OMS en milieu pédiatrique. Thèse de Médecine, Université de Bamako, 115 p.
[50]
Latoudji, S., Anani, L., Able, E. and Zahou, I. (1991) Morbidité et mortalité drépanocytaire au Benin. Médecine d’Afrique Noire, 38, 8-9.
[51]
Purohit, P., Mohanty, P.K., Patel, S., Das, P., Panigrahi, J. and Das, K. (2018) Comparative Study of Clinical Presentation and Hematological Indices in Hospitalized Sickle Cell Patients with Severe Plasmodium Falciparum Malaria. Journal of Infection and Public Health, 11, 321-325. https://doi.org/10.1016/j.jiph.2017.08.013
