SICKLE CELL DISEASE AND INFECTIONS IN HIGH- AND LOW-INCOME COUNTRIES
Main Article Content
Keywords
Sickle cell disease; infections; prognosis; prophylaxis; socio-economics.
Abstract
Infections, especially pneumococcal septicemia, meningitis, and Salmonella osteomyelitis, are a major cause of morbidity and mortality in patients with sickle cell disease (SCD). SCD increased susceptibility to infection, while infection leads to SCD-specific pathophysiological changes. The risk of infectious complications is highest in children with a palpable spleen before 6 months of age. Functional splenectomy, the results of repeated splenic infarctions, appears to be an important host-defense defect. Infection is the leading cause of death, particularly in less developed countries. Defective host-defense mechanisms enhance the risk of pneumococcal complications. Susceptibility to Salmonella infections can be explained at least in part by a similar mechanism. In high-income countries, the efficacy of the pneumococcal vaccine has been demonstrated in this disease. A decreased in infection incidence has been noted in SCD patients treated prophylactically with daily oral penicillin. Studies in low-income countries suggest the involvement of a different spectrum of etiological agents.
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References
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3. Piel FB, Steinberg MH, Rees DC. Sickle cell disease. N Engl J Med 2017; 376:1561-1573.
4. Weatherall DJ. The inherited diseases of hemoglobin are an emerging global health burden. Blood 2010; 115:4331-4336.
5. Wong TE, Brandow AM, Lim W, Lotenberg R. Update on the use of hydroxyurea therapy in sickle cell disease. Blood 2014; 124:3850-3857.
6. Cannas G, Poutrel S, Thomas X. Hydroxycarbamine: from an old drug in malignant hemopathies to a current standard in sickle cell disease. Mediterr J Hematol Infect Dis 2017; 9:e2017015.
7. Mulaku M, Opiyo N, Karumbi J, et al. Evidence review of hydroxyurea for the prevention of sickle cell complications in low-income countries. Arch Dis Child 2013; 98:908-14.
8. Telen MJ. Beyond hydroxyurea: new and old drugs in the pipeline for sickle cell disease. Blood 2016; 127:810-819.
9. Telfer P, Coen P, Chakravorty S, et al. Clinical outcomes in children with sickle cell disease living in England: a neonatal cohort in East London. Haematologica 2007; 92:905-912.
10. Kauf TL, Coates TD, Huazhi L, et al. The cost of health care for children and adults with sickle cell disease. Am J Heamatol 2009; 84:323-327.
11. Tshilolo L, Kafando E, Sawadogo M, et al. Neanatal screening and clinical care programmes for sickle cell disorders in sub-Saharan Africa: lessons from pilot studies. Public Health 2008; 122:933-941.
12. Grosse SD, Odame I, Atrash HK, et al. Sickle cell disease in Africa: a neglected cause of early childhood mortality. Am J Prev Med 2011; 41(suppl.4): S398-S405.
13. Ramakrishnan M, Moïsi JC, Klugman KP, et al. Increased risk of invasive bacterial infections in African people with sickle-cell disease: a systematic review and meta-analysis. Lancet 2010; 10:329-337.
14. Weatherall MW, Higgs DR, Weiss H, et al. Phenotype/genotype relationships in sickle cell disease: a pilot twin study. Clin Lab Haematol 2005; 27:384-390.
15. Lipowsky HH, Willaims ME. Shear rate dependency of red cell sequestration in skin capillaries in sickle cell disease and its variation with vasoocclusive crisis. Microcirculation 1997; 4:289-301.
16. Amjad H, Bannerman RM, Judisch JM. Sickling pain and season. BMJ 1974; 2:54.
17. Konotey-Ahulu FI. Sicklaemic human hygrometers. Lancet 1965; 1:1003-1005.
18. Smith WR, Coyne P, Smith VS, Mercier B. Temperature changes, temperature extremes, and their relationship to emergency department visits and hospitalization for sickle cell crisis. Pain Manag Nurs 2003; 4:106-111.
19. Redwood AM, Williams EM, Desal P, Serjeant GR. Climate and painful crisis of sickle-cell disease in Jamaica. Br Med J 1976; 1:66-68.
20. Seeler RA. Non-seasonality of sickle-cell crisis. Lancet 1973; 2:743.
21. Slovis CM, Talley JD, Pitts RB. Non relationship of climatologic factors and painful sickle cell anemia crisis. J Chronic Dis 1986; 39:121-126.
22. Tewari S, Brousse V, Piel FB, et al. Environmental determinants of severity in sickle cell disease.Haematologica 2015; 100:1108-1116.
23. Jones S, Duncan ER, Thomas N, et al. Windy weather and low humidity are associated with an increased number of hospital admissions for acute pain and sickle cell disease in an urban environment with a maritime temperature climate. Br J Haematol 2005; 131:530-533.
24. Nolan VG, Zhang Y, Lash T, et al. Association between wind speed and the occurrence of sickle cell acute painful episodes: results of a case-crossover study. Br J Haematol 2008; 143:433-438.
25. Cohen RT, DeBaun MR, Blinder MA, et al. Smoking is associated with an increased risk of acute chest syndrome and pain among adults with sickle cell disease. Blood 2010; 115:3852-3854.
26. West DC, Romano PS, Azari R, et al. Impact of environmental tobacco smoke on children with sickle cell disease. Arch Pediatr Adolesc Med 2003; 157:1197-1201.
27. Farber MD, Koshy M, Kinney TR. Cooperative study of sickle cell disease: demographic and socioeconomic characteristics of patients and families with sickle cell disease. J Chronic Dis 1985; 38:495-505.
28. Ballester OF, Pasad AS. Anergy, zinc deficiency and decreased nucleoside phosphorylase activity in patients with sickle cell anemia. Ann Intern Med 1983; 98:180-182.
29. Prasad AS, Back FW, Kaplan J, et al. Effect of zinc supplementation on incidence of infections and hospital admissions in sickle cell disease. Am J Hematol 1999; 61:194-202.
30. Bohnsack JF, Brown EJ. The role of the spleen in resistance to infection. Ann Rev Med 1986; 37:49-59.
31. Johnston RB Jr, Newman LS, Struth AG. An abnormality of the alternate pathway of complement activity in sickle cell disease. N Engl J Med 1973; 288:803-808.
32. Chambers JB, Forsythe DA, Bertrand SL, et al. Retrospective review of osteoarticular infections in a pediatric sickle cell age group. J Pediatr Orthop 2000; 20:682-685.
33. Sadat-Ali M. The status of acute osteomyelitis in sickle cell disease. A 15-year review. Int Surg 1998; 83:84-87.
34. Almeira A, Roberts I. Bone involvement in sickle cell disease. Br J Haematol 2005; 129:482-490.
35. Workman MR, Philpott-Howard J, Bellingham AJ. Salmonella bacteraemia in sickle cell disease at King’s College Hospital: 1976-1991. J Hosp Infect 1997; 34:195-199.
36. Wang IK, Kuo HL, Chen YM, et al. Extraintestinal manifestations of Edwardsiella tarda infection. Int J Clin Pract 2005 ; 59 :917-921.
37. Vichinsky EP, Neumayr LD, Earles AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 2000; 342:1855-1865.
38. William BM, Thawani N, Sae-Tia S, Corazza GR. Hyposplenism: a comprehensive review. Part II: Clinical manifestations, diagnosis and management. Haematology 2007; 12:89-98.
39. Tamouza R, Neonato MG, Busson M, et al. Infectious complications in sickle cell disease are influenced by HLA class II alleles. Hum Immunol 2002; 63:194-199.
40. Larcher VF, Wyke RJ. Defective yeast opsonisation and functional deficiency of complement in sickle cell disease. Arch Dis Child 1982; 57:343-346.
41. Anyaegbu CC, Okpala IE. Peripheral blood neutrophil count and candidacidal activity correlate with the clinical severity of sickle cell anaemia. Eur J Haematol 1998; 60:267-268.
42. Adewoye A, Nolan V, Ma Q, et al. Association of polymorphisms of IGF1R and genes in the TGF?/BMP pathway with baxteremia in sickle cell anemia. Clin Infect Dis 2006; 43:593-598.
43. Gaston MH, Verter JI, Woods G, et al. Prophylaxis with oral penicillin in children with sickle cell anemia – a randomized trial. N Engl J Med 1986; 314:1593-1599.
44. Quinn CT, Rogers ZR, McCavit TL, et al. Improved survival of children and adolescents with sickle cell disease. Blood 2010 ; 115 :3447-3452.
45. McCavit TL, Quinn CT, Techasaensiri C, et al. Increase in invasive Streptococcus pneumoniae infections in children with sickle cell disease since pneumococcal conjugate vaccine licensure. J Pediatr 2011; 158:505-507.
46. Castro O, Brambilla DJ, Thorington B, et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. The cooperative study of sickle cell disease. Blood 1994; 84:643-649.
47. Berger E, Saunders N, Wang L, et al. Sickle cell disease in children: differentiating osteomyelitis from vaso-occlusive crisis. Arch Pediatr Adolesc Med 2009; 163:251-255.
48. Marti-Carvajal AJ, Agreda-Perez LH. Antibiotics for treating osteomyelitis in people with sickle cell disease. Cochrane Database Syst Rev 2016; 11:CD007175.
49. Hand WL, King NL. Serum opsonization of Salmonella in sickle cell anemia. Am J Med 1978; 64:388-395.
50. Brown B, Dada-Adegbola H, Trippe C, Olopade O. Prevalence and etiology of bacteremia in febrile children with sickle cell disease at a Nigeria tertiary hospital. Mediterr J Hematol Infect Dis 2017; 9:e2017039.
51. Okuonghae HO, Nwankwo MU, Offor EC. Pattern of bacteraemia in febrile children with sickle cell anaemia.Ann Trop Paediatr 1993; 13:55-64.
52. Brown BJ, Jacob NE, Lagunju IA, Jarrett OO. Morbidity and mortality pattern in hospitalized children with sickle cell disorders at the University College Hospital, Ibadan, Nigeria. Nig J Paediatr 2013; 40:34-39.
53. Kizito ME, Mworozi E, Ndugwa C, Serjeant GR. Bacteraemia in homozygous sickle cell disease in Africa: is pneumococcal prophylaxis justified? Arch Dis Child 2007; 92:21-23.
54. Bansil NH, Kim TY, Tieu L, Barcega B. Incidence of serious bacterial infections in febrile children with sickle cell disease. Clin Pediatr 2013; 52:661-666.
55. Morrissey BJ, Bycroft TP, Almossawi O, et al. Incidence and predictors of bacterial infection in febrile children with sickle cell disease. Hemoglobin 2015; 39:316-319.
56. Serjant G. Mortality from sickle cell disease in Africa. BMJ 2005; 330:432-433.
57. Akinyanju O, Johnson AO. Acute illness in Nigerian children with sickle cell anaemia. Ann Trop Paediatr 1987; 7:181-186.
58. Akuse RM. Variation in the pattern of bacterial infection in patients with sickle cell disease requiring admission. J Trop Paediatr 1996; 42:318-323.
59. Makani J, Mgaya J, Balandya E, et al. Bacteraemia in sickle cell anaemia is associated with low haemoglobin: a report of 890 admissions to a tertiary hospital in Tanzania. Br J Haematol 2015; 171:273.
60. Bello N, Kudu ATD, Adetokun AB, et al. Characterization and antimicrobial susceptibility profile of bacteraemia causing pathogens isolated from febrile children with and without sickle cell disease in Kano, Nigeria. Mediterr J Hematol Infect Dis 2018; 10:e2018016.
61. Yanda ANA, Nansseu JRN, Awa HDM, et al. Burden and spectrum of bacterial infections among sickle cell disease children living in Cameroon. BMC Infec Dis 2017; 17:221.
62. Kateete DP, Kajumbula H, Kaddu-Mulindwa DH, et al. Nasopharyngeal carriage rate of Streptococcus pneumonia in Ugandan children with sickle cell disease. BMC Res Notes 2012; 5:28.
63. Mava Y, Bello M, Ambe JP, Zailani SB. Antimicrobiol sensitivity pattern of organisms causing urinary tract infection in children with sickle cell anemia in Maiduguri, Nigeria. J Clin Prac 2012; 15:420-423.
64. Athale UH, Chintu C. Clinical analysis of mortality in hospitalized Zambian children with sickle cell anaemia. East Afr Med J 1994; 71:388-391.
65. Smith-Whitley K, Zhao H, Hodinka RL, et al. Epidemiology of human parvovirus B19 in children with sickle cell disease. Blood 2004; 103:422-427.
66. Serjeant BE, Hambleton IR. Haematological response to parvovirus B19 infection in homozygous sickle cell disease. Lancet 2001; 358:1779-1780.
67. Servey JT, Reamy BV, Hodge J. Clinical presentations of parvovirus B19 infection. Am Fam Physician 2007; 75:373-376.
68. Owusu ED, Visser BJ, Nagel IM, et al. The interaction between sickle cell disease and HIV infection: a systematic review. Clin Infect Dis 2015; 60:612-626.
69. Godeau B, Bachir D, Schaeffer A, et al. Severe pneumococcal sepsis and meningitis in HIV-infected adults with sickle cell disease. Clin Infect Dis 1992; 15:327-329.
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