• Clinical and therapeutic aspects of sickle cell disease in three clinical cases
en To content Full text of article

Clinical and therapeutic aspects of sickle cell disease in three clinical cases

Ukrainian Journal of Perinatology and Pediatrics. 2021.4(88): 66-71; doi 10.15574/PP.2021.88.66.
Aggoune S.
Pediatric Hassan badi hospital, El Harrach, Algeria
Algiers's University

For citation: Aggoune S. (2021). Clinical and therapeutic aspects of sickle cell disease in three clinical cases. Ukrainian Journal of Perinatology and Pediatrics. 4(88): 66-71. doi 10.15574/PP.2021.88.66.
Article received: Nov 06, 2021. Accepted for publication: Dec 13, 2021.

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.
Keywords: sickle cell disease, acute anemia, stroke, osteonecrosis.

REFERENСES

1. 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

2. 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

3. 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

4. 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

5. 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

6. 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

7. 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

8. Belahnai M. (2012). Revue Sante$Mag. 03; Fevrier, 3: 9.

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.

10. 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

11. 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

12. 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

13. 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

14. 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

15. 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

16. 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

17. 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

18. 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.

19. 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

20. 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

21. 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

22. 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

23. 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

24. 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

25. 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

26. 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

27. 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

28. 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.

29. 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

30. 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.

31. 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

32. 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

33. 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

34. 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.

35. 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

36. 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

37. 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

38. 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.

39. 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

40. 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