Clinical and therapeutic aspects of sickle cell disease in three clinical cases
DOI:
https://doi.org/10.15574/PP.2021.88.66.Keywords:
sickle cell disease, acute anemia, stroke, osteonecrosisAbstract
One of the reasons of high pediatric mortality in developing countries, sickle cell disease is gradually emerging and is becoming a public health problem in many countries where it is rife. In Algeria the incidence is 2.7%. The management of sickle cell disease is increasingly better codified now thanks to better knowledge of the condition. It takes into account not only currently accepted universal principles but also the realities specific to our country.
Purpose: to share with health care professionals our therapeutic attitude during main acute complications as well as during the inter3critical phase of sickle cell disease in Algerian children.
Clinical cases. In this article, we presented three clinical cases concerning two adolescents and a two3year3old infant, carriers of major sickle cell syndrome, who were hospitalized for severe forms.
Conclusions. Providing right care for children with sickle cell disease could help prevent or improve many complications associated with this disease and allow them to lead healthier and more productive lives. Our patients were presented late. These cases revealed the problematic nature of early diagnosis, regular follow-up and early detection of complications in SCD patients especially with asymptomatic osteonecrosis of the femoral head.
The research was carried out in accordance with the principles of the Helsinki declaration. The informed consent of the patients was obtained for conducting the studies.
No conflict of interest was declared by the author.
References
Adams RJ. (2005). TCD in sickle cell disease: an important and useful test. Pediatr Radiol. 35(3): 229. https://doi.org/10.1007/s00247-005-1409-7; PMid:15703904
Adams RJ, McKie VC, Hsu L, Files B, Vichinsky E, Pegelow C et al. (1998). Prevention of a First stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial Doppler ultrasonography. N Engl J Med. 339 (1): 5. https://doi.org/10.1056/NEJM199807023390102; PMid:9647873
Akinyanju OO. (1989). Profile of sickle cell disease in Nigeria. Ann N Y Acad Sci. 565: 126-136. https://doi.org/10.1111/j.1749-6632.1989.tb24159.x; PMid:2672962
Anson JA, Koshy M, Ferguson L, Crowell RM. (1991). Subarachnoid hemorrhage in sickle-cell disease. J Neurosurg. 75 (4): 552-8. https://doi.org/10.3171/jns.1991.75.4.0552; PMid:1885973
Austin H, Key NS, Benson JM, Lally C, Dowling NF et al. (2007). Sickle cell trait and the risk of venous thromboembolism among blacks. Blood. 110: 908-912. https://doi.org/10.1182/blood-2006-11-057604; PMid:17409269
Aygun B, Odame I. (2012). A global perspective on sickle cell disease. Pediatr Blood Cancer. 59: 386-390. https://doi.org/10.1002/pbc.24175; PMid:22535620
Ballas SK, Kesen MR, Goldberg MF, Lutty GA, Dampier C, Osunkwo I, Wang WC, Hoppe C, Hagar W, Darbari DS et al. (2012). Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management. Sci. World J. 2012: 949535. https://doi.org/10.1100/2012/949535; PMid:22924029 PMCid:PMC3415156
Belahnai M. (2012). Revue Sante$Mag. 03; Fevrier, 3: 9.
Beutler E. (2005). Chapter 47: the sickle cell diseases and related disorders. In: Beutler E, Lichtman MA, Coller BS, et al., eds. Williams Hematology. 7th edn. New York, NY: McGraw-Hill: 581e607.
Booth C, Inusa B, Obaro SK. (2010). Infection in sickle cell disease: a review. Int J Infect Dis. 14: e2-12. https://doi.org/10.1016/j.ijid.2009.03.010; PMid:19497774
Brousse V, Buffet P, Rees D. (2014). The spleen and sickle cell disease: the sick(led)spleen. Br J Haematol. 166: 165-176. https://doi.org/10.1111/bjh.12950; PMid:24862308
Brousse V, Elie C, Benkerrou M et al. (2012). Acute splenic sequestration crisis in sickle cell disease: cohort study of 190 paediatric patients. Br J Haematol. 156: 643-648. https://doi.org/10.1111/j.1365-2141.2011.08999.x; PMid:22224796
Brown M. (2012). Managing the acutely ill adult with sickle cell disease. Br J Nurs. 21(90-2): 5-6. https://doi.org/10.12968/bjon.2012.21.2.90; PMid:22306637
Camporesi EM, Vezzani G, Bosco G, Mangar D, Bernasek TL. (2010). Hyperbaric oxygen therapy in femoral head necrosis. J Arthroplasty. 25; 6 Suppl: 118-123. https://doi.org/10.1016/j.arth.2010.05.005; PMid:20637561
Dickerhoff R. (2002). Splenic sequestration in patients with sickle cell disease. [Article in German]. Klin Padiatr. 214: 70-73. https://doi.org/10.1055/s-2002-25266; PMid:11972313
Ellison AM, Shaw K. (2007). Management of vasoocclusive pain events in sickle cell disease. Pediatr Emerg Care. 23: 832-838 quiz 8-41. https://doi.org/10.1097/PEC.0b013e31815a05e2; PMid:18007218
Emond AM, Collis R, Darvill D et al. (1985). Acute splenic sequestration in homozygous sickle cell disease: natural history and management. J Pediatr. 107: 201-206. https://doi.org/10.1016/S0022-3476(85)80125-6
Goodman J, Newman MI, Chapman WC. (2004). Disorders of the spleen. In: Greer JP, Foerster J, Lukens JN et al. Editors Wintrobe's Clinical Hematology, 11th ed. Philadelphia, PA: Lippincott Williams & Wilkins: 1893-1909.
Joseph B, Rao N, Mulpuri K, Varghese G, Nair S. How does a femoral varus osteotomy alter the natural evolution of Perthes' disease? J Pediatr Orthop B.;14 (1):10-15. https://doi.org/10.1097/01202412-200501000-00002; PMid:15577301
Kamata N, Oshitani N, Sogawa M et al. (2008). Usefulness of magnetic resonance imaging for detection of asymptomatic osteonecrosis of the femoral head in patients with inflammatory bowel disease on longterm corticosteroid treatment. Scand J Gastroenterol. 43 (3): 308-313. https://doi.org/10.1080/00365520701676773; PMid:18938768
Kinney TR, Ware RE, Schultz WH, et al. (1990, Aug). Long$term management of splenic sequestration in children with sickle cell disease. J Pediatr. 117 (2 Pt 1): 194-199. PMID: 2380816. https://doi.org/10.1016/S0022-3476(05)80529-3
Lee MT, Piomelli S, Granger S, Miller ST, Harkness S, Brambilla DJ et al. (2006). Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results. Blood. 108 (3): 847-852. https://doi.org/10.1182/blood-2005-10-009506; PMid:16861341 PMCid:PMC1895848
Makani J, Cox SE, Soka D, Komba AN, Oruo J,Mwamtemi H et al. (2011). Mortality in sickle cell anemia in Africa: a prospective cohort study in Tanzania. PLoS ONE. 6: e14699. https://doi.org/10.1371/journal.pone.0014699; PMid:21358818 PMCid:PMC3040170
Milner PF, Kraus AP, Sebes JI, Sleeper LA, Dukes KA, Embury SH et al. (1991). Sickle cell disease as a cause of osteonecrosis of the femoral head. New England Journal of Medicine. 325 (21): 1476-1478. https://doi.org/10.1056/NEJM199111213252104; PMid:1944426
Modell B, Darlison M. (2008). Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ. 86: 480-487. https://doi.org/10.2471/BLT.06.036673; PMid:18568278 PMCid:PMC2647473
Mukisi MM, Bashoun K, Burny F. (2009). Sickle-cell necrosis and intraosseous pressure. Orthopaedics & traumatology, surgery & research. 95 (2): 134-138. https://doi.org/10.1016/j.otsr.2009.01.001; PMid:19285936
Naymagon L, Pendurti G, Billett HH. (2015). Acute splenic sequestration crisis in adult sickle cell disease: a report of 16 cases. Hemoglobin. 39: 375-379. https://doi.org/10.3109/03630269.2015.1072550; PMid:26287797
Ndugwa CM. (1992). Aseptic necrosis of the head of the femur among sickle cell anemia patients in Uganda. East African Medical Journal. 69 (10): 572-576.
Oyesiku NM, Barrow DL, Eckman JR, Tindall SC, Colohan AR. (1991). Intracranial aneurysms in sickle-cell anemia: clinical features and pathogenesis. J Neurosurg. 75 (3): 356. https://doi.org/10.3171/jns.1991.75.3.0356; PMid:1869933
Piyakunmala K, Sangkomkamhang T, Chareonchonvanitch K. (2009). Is magnetic resonance imaging necessary for normal plain radiography evaluation of contralateral non$traumatic asymptomatic femoral head in high osteonecrosis risk patient. J Med Assoc Thai. 92 (6): S147-151.
Powars RD, Welss JN, Chan LS, Schroeder WA. (1984). Is there a threshold level of fetal hemoglobin that ameliorates morbidity in sickle cell anemia? Blood. 63: 921-926. https://doi.org/10.1182/blood.V63.4.921.bloodjournal634921; PMid:6200161
Roach ES, Golomb MR et al. (2008, Sep). Management of stroke in infants and children: a scientific statement from a Special Writing Group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young. Stroke. 39 (9): 2644-2691. https://doi.org/10.1161/STROKEAHA.108.189696; PMid:18635845
Saxena P, Dhiman P, Bihari C, Rastog A. (2015). Sickle cell trait causing splanchnic venous thrombosis. Case Reports Hepatol.: 3. https://doi.org/10.1155/2015/743289; PMid:26221548 PMCid:PMC4499632
Schnog JB, Duits AJ,Muskiet FA, ten Cate H, Rojer RA, Brandjes DP. (2004). Sickle cell disease; a general overview. Neth J Med. 62: 364-374.
Theodorou DJ, Malizos KN, Beris AE, Theodorou SJ, Soucacos PN. (2001). Multimodal imaging quantitation of the lesion size in osteonecrosis of the femoral head. Clin Orthop Relat Res. (386): 54-63. https://doi.org/10.1097/00003086-200105000-00007; PMid:11347848
Tsaras G, Owusu-Ansah A, Boateng FO, Amoateng-Adjepong Y. (2009). Complications associated with sickle cell trait: a brief narrative review. Am J Med. 122: 507-512. https://doi.org/10.1016/j.amjmed.2008.12.020; PMid:19393983
Verlhac S. (2008). Doppler transcranien et protocole de prevention desaccidents vasculaires cerebraux de l'enfant drepanocytaire. Transcranial Doppler and prevention of stroke in sickle cell disease. Archives de Pediatrie. 15: 636-638. https://doi.org/10.1016/S0929-693X(08)71858-X
Ware RE, Davis BR, Schultz WH 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 (10019): 661-670.
Weatherall DJ. (2010). The inherited diseases of hemoglobin are an emerging global health burden. Blood. 115: 4331-4336. https://doi.org/10.1182/blood-2010-01-251348; PMid:20233970 PMCid:PMC2881491
Yamamoto S, Watanabe A, Nakamura J et al. (2011). Quantitative T2 mapping of femoral head cartilage in systemic lupus erythematosus patients with noncollapsed osteonecrosis of the femoral head associated with corticosteroid therapy. J Magn Reson Imaging. 34 (5): 1151-1158. https://doi.org/10.1002/jmri.22685; PMid:21953994
Downloads
Published
Issue
Section
License
Copyright (c) 2022 Ukrainian journal of Perinatology and Pediatrics
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
The policy of the Journal “Ukrainian Journal of Perinatology and Pediatrics” is compatible with the vast majority of funders' of open access and self-archiving policies. The journal provides immediate open access route being convinced that everyone – not only scientists - can benefit from research results, and publishes articles exclusively under open access distribution, with a Creative Commons Attribution-Noncommercial 4.0 international license(СС BY-NC).
Authors transfer the copyright to the Journal “MODERN PEDIATRICS. UKRAINE” when the manuscript is accepted for publication. Authors declare that this manuscript has not been published nor is under simultaneous consideration for publication elsewhere. After publication, the articles become freely available on-line to the public.
Readers have the right to use, distribute, and reproduce articles in any medium, provided the articles and the journal are properly cited.
The use of published materials for commercial purposes is strongly prohibited.