Academic Journal
Acute Neurological Events in Children With Hemoglobin SC Disease: A Multicenter Retrospective Study.
| Τίτλος: | Acute Neurological Events in Children With Hemoglobin SC Disease: A Multicenter Retrospective Study. |
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| Συγγραφείς: | Paulmin C; Le Kremlin Bicêtre, Reference Center for Rare Constitutional Disorders of the Red Blood Cell and Erythropoiesis (MCGRE), Bicêtre Hospital, Le Kremlin-Bicêtre, France., Koehl B; Sickle Cell Disease Center, Hematology Unit, Robert Debré Hospital, Université Paris Cité, Inserm BIGR-team Bahsic, Paris, France., Maudoux A; Pediatric ENT and Head and Neck Surgery, Robert Debré Hospital, Paris, France., Mansour H; Fort-de-France, Reference Center MCGRE, University Hospital of Martinique, Martinique, France., Odievre MH; Paris-Trousseau Pediatric Reference Center MCGRE, Paris, France., Garrec N; Pediatric Care Center MCGRE of the East Francilien Hospital Group, Meaux, France., Georget E; Pediatric Care Center MCGRE of Villeneuve-Saint-Georges, Villeneuve-Saint-Georges, France., Arnaud C; Créteil Pediatric Reference Center MCGRE, Créteil, France., Allali S; Necker-Enfants Malades Pediatric Reference Center MCGRE, Paris, France., Doumdo L; Guadeloupe Pediatric Reference Center MCGRE, Pointe-à-Pitre, France., Belloy M; Pediatric Care Center MCGRE of Aulnay-sous-Bois, Aulnay-sous-Bois, France., Guitton C; Bicêtre Pediatric Reference Center MCGRE, Paris, France. |
| Πηγή: | Pediatric blood & cancer [Pediatr Blood Cancer] 2026 Nov; Vol. 73 (11), pp. e70605. Date of Electronic Publication: 2026 Aug 20. |
| Τύπος έκδοσης: | Journal Article; Multicenter Study |
| Γλώσσα: | English |
| Στοιχεία περιοδικού: | Publisher: John Wiley Country of Publication: United States NLM ID: 101186624 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1545-5017 (Electronic) Linking ISSN: 15455009 NLM ISO Abbreviation: Pediatr Blood Cancer Subsets: MEDLINE |
| Imprint Name(s): | Original Publication: Hoboken, N.J. : John Wiley, c 2004- |
| Ιατρικοί όροι (MeSH): | Nervous System Diseases*/etiology , Nervous System Diseases*/epidemiology , Nervous System Diseases*/pathology , Hemoglobin SC Disease*/complications, Humans ; Retrospective Studies ; Female ; Male ; Child, Preschool ; Child ; Adolescent ; Infant ; Infant, Newborn ; Acute Disease ; Follow-Up Studies ; Prognosis |
| Περίληψη: | Introduction: Neurological manifestations in children with hemoglobin SC (HbSC) disease remain insufficiently characterized, particularly regarding acute events. The aim of this study was to describe the spectrum and frequency of acute neurological events in a multicenter cohort of children with HbSC disease. Materials and Methods: We conducted a retrospective descriptive multicenter study including children with HbSC disease aged 0-18 years who presented with at least one acute neurological event between January 1, 2010, and August 31, 2023, in Île-de-France, Martinique, and Guadeloupe. Results: Twenty-five patients from 10 centers were included. A total of 34 acute neurological events were recorded. Neuro-ENT (ear, nose, throat) manifestations were the most frequent (41%), including vestibular syndromes and sudden hearing loss, followed by central neurological deficits or altered consciousness (18%), seizures (18%), acute headaches (18%), and peripheral neuropathies (6%). Neuro-ENT manifestations showed recurrence in some patients. Cerebrovascular events occurred across all age groups and were observed in patients with a history of vaso-occlusive crises. Conclusion: Acute neurological events may occur in children with HbSC disease and show a heterogeneous clinical spectrum. Further studies are needed to better characterize their determinants and clinical significance. Clinical Trial Registration: Not applicable. (© 2026 The Author(s). Pediatric Blood & Cancer published by Wiley Periodicals LLC.) |
| References: | A. M. Thomson, T. A. McHugh, A. P. Oron, et al., “Global, Regional, and National Prevalence and Mortality Burden of Sickle Cell Disease, 2000–2021: A Systematic Analysis From the Global Burden of Disease Study 2021,” Lancet Haematology 10, no. 8 (2023): e585–e599, https://doi.org/10.1016/S2352‐3026(23)00118‐7. M. R. DeBaun, L. C. Jordan, and A. A. King, “American Society of Hematology 2020 Guidelines for Sickle Cell Disease: Prevention, Diagnosis, and Treatment of Cerebrovascular Disease in Children and Adults,” Blood Advances 4, no. 8 (2020): 1554–1588, https://doi.org/10.1182/bloodadvances.2019001142. I. A. Lagunju and B. J. Brown, “Adverse Neurological Outcomes in Nigerian Children With Sickle Cell Disease,” International Journal of Hematology 96, no. 6 (2012): 710–718, https://doi.org/10.1007/s12185‐012‐1204‐9. R. H. Fabian and B. H. Peters, “Neurological Complications of Hemoglobin SC Disease,” Archives of Neurology 41, no. 3 (1984): 289–292. K. Ohene‐Frempong, “Cerebrovascular Accidents in Sickle Cell Disease: Rates and Risk Factors,” Blood 91, no. 1 (1998): 288–294. C. Runel‐Belliard, E. Lesprit, B. Quinet, S. Wiener‐Vacher, C. Saizou, and E. Grimprel, “Syndrome Vestibulaire Chez Une Adolescente drépanocytaire SC. Le Traitement Est Urgent,” Archives de Pédiatrie 15, no. 9 (2008): 1423–1425, https://doi.org/10.1016/j.arcped.2008.06.013. N. Saito, M. Watanabe, and J. Liao, “Clinical and Radiologic Findings of Inner Ear Involvement in Sickle Cell Disease,” AJNR American Journal of Neuroradiology 32, no. 11 (2011): 2160–2164, https://doi.org/10.3174/ajnr.A2720. B. Emilie, C.‐T. Natacha, and E. Bois, “Evaluation de la fonction auditive chez les enfants drépanocytaires: résultats de l'étude prospective DADREP sur 89 patients,” Université Pierre et Marie Curie, (2017). P. Desai, M. Dejoie‐Brewer, and S. K. Ballas, “Deafness and Sickle Cell Disease: Three Case Reports and Review of the Literature,” Journal of Clinical Medicine Research 7, no. 3 (2015): 189–192, https://doi.org/10.14740/jocmr2028w. J. Schreibstein, C. B. MacDonald, L. Clarkecox, and L. Bloom, “Sudden Hearing Loss in Sickle Cell Disease: A Case Report,” Otolaryngology—Head and Neck Surgery 116, no. 4 (1997): 541–544, https://doi.org/10.1016/S0194‐59989770307‐3. F. Lionnet, N. Hammoudi, and K. S. Stojanovic, “Hemoglobin Sickle Cell Disease Complications: A Clinical Study of 179 Cases,” Haematologica 97, no. 8 (2012): 1136–1141, https://doi.org/10.3324/haematol.2011.055202. B. K. Sathi, Y. Yoshida, and M. R. Weaver, “Unusually High Prevalence of Stroke and Cerebral Vasculopathy in Hemoglobin SC Disease: A Retrospective Single Institution Study,” Acta Haematology 145, no. 2 (2022): 160–169, https://doi.org/10.1159/000519360. V. Brousse, M. Kossorotoff, and M. De Montalembert, “How I Manage Cerebral Vasculopathy in Children With Sickle Cell Disease,” British Journal of Haematology 170, no. 5 (2015): 615–625, https://doi.org/10.1111/bjh.13477. D. Fridlyand, C. Wilder, E. L. Jerome Clay, B. Gilbert, and B. S. Pace, “Stroke in a Child With Hemoglobin SC Disease: A Case Report Describing Use of Hydroxyurea After Transfusion Therapy,” Pediatric Reports 9, no. 1 (2017): 6984, https://doi.org/10.4081/pr.2017.6984. S. L. Linguet, S. Verlhac, and F. Missud, “Stroke Without Cerebral Arteriopathy in Sickle Cell Disease Children: Causes and Treatment,” Haematologica 109, no. 10 (2024): 3346, https://doi.org/10.3324/haematol.2023.283773. Haute Autorité de Santé, “Syndromes drépanocytaires majeurs de l'enfant et de l'adolescent—Protocole national de diagnostic et de soins pour une maladie rare,” Haute Autorité de Santé, 2024. C. R. Deane, D. Goss, and S. O'Driscoll, “Transcranial Doppler Scanning and the Assessment of Stroke Risk in Children With Haemoglobin Sickle Cell Disease,” Archives of Disease in Childhood 93, no. 2 (2008): 138–141, https://doi.org/10.1136/adc.2007.125799. R. P. Santiago, C. Vieira, and C. S. A. Adanho, “Laboratory and Genetic Biomarkers Associated With Cerebral Blood Flow Velocity in Hemoglobin SC Disease,” Disease Markers 2017 (2017): 6359871, https://doi.org/10.1155/2017/6359871. L. H. Pecker, B. A. Schaefer, and L. Luchtman‐Jones, “Knowledge Insufficient: The Management of Haemoglobin SC Disease,” British Journal of Haematology 176, no. 4 (2017): 515–526, https://doi.org/10.1111/bjh.14444. K. P. Guilliams, M. E. Fields, and M. L. Hulbert, “Higher‐Than‐Expected Prevalence of Silent Cerebral Infarcts in Children With Hemoglobin SC Disease,” Blood, The Journal of the American Society of Hematology 125, no. 2 (2015): 416–417. |
| Contributed Indexing: | Keywords: HbSC disease; epileptic seizures; facial nerve involvement; neurological manifestations; stroke; sudden hearing loss; vertigo |
| Entry Date(s): | Date Created: 20260820 Date Completed: 20260923 Latest Revision: 20260923 |
| Update Code: | 20260923 |
| DOI: | 10.1002/1545-5017.70605 |
| PMID: | 42622377 |
| Βάση Δεδομένων: | MEDLINE |
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