Sickle cell disease subphenotypes in patients from Southwestern Province of Saudi Arabia

Journal of Pediatric Hematology/Oncology

Volumn 34, Issue 2, 2012, Pages 79-84

  Alsultan, Abdulrahman ^a   Aleem, Aamer ^a   Ghabbour, Hazem ^b   Algahtani, Farjah H ^a   Al Shehri, Ali ^a   Osman, Mohamed Elfaki ^a   Kurban, Kadijah ^a   Alsultan, Mohammed S ^b   Bahakim, Hasan ^a   Al Momen, Abdelkareem M ^a  

^a KING SAUD UNIVERSITY   (Saudi Arabia)

^b KING SAUD UNIVERSITY   (Saudi Arabia)

Author keywords

phenotype; Saudi Arabia; sickle cell disease

Indexed keywords

GLUCOSE 6 PHOSPHATE DEHYDROGENASE; HEMOGLOBIN F; LACTATE DEHYDROGENASE;

ACUTE CHEST SYNDROME; ADOLESCENT; AGED; ALPHA THALASSEMIA; ARTICLE; BONE NECROSIS; CEREBROVASCULAR ACCIDENT; CHOLECYSTECTOMY; COMORBIDITY; CONTROLLED STUDY; DIAGNOSTIC IMAGING; DISEASE ASSOCIATION; DISEASE SEVERITY; FEMALE; GALLSTONE; GENE; GENE DELETION; GENE MUTATION; HAPLOTYPE; HBB GENE; HEMOGLOBIN BLOOD LEVEL; HUMAN; INFANT; INFECTION; LEG ULCER; LEUKOCYTE COUNT; MAJOR CLINICAL STUDY; MALE; PAIN; PHENOTYPIC VARIATION; PRIAPISM; PRIORITY JOURNAL; RISK ASSESSMENT; SAUDI ARABIA; SEX DIFFERENCE; SICKLE CELL ANEMIA; SPLEEN DISEASE; SPLEEN SEQUESTRATION; SPLENOMEGALY;

ADOLESCENT; ADULT; AGED; ANEMIA, SICKLE CELL; CHILD; CHILD, PRESCHOOL; FEMALE; HUMANS; INFANT; MALE; MIDDLE AGED; PHENOTYPE; SAUDI ARABIA; YOUNG ADULT;

EID: 84857912002     PISSN: 10774114     EISSN: 15363678     Source Type: Journal    
DOI: 10.1097/MPH.0b013e3182422844     Document Type: Article

References (46)

1. Al-Qurashi MM, El-Mouzan MI, Al-Herbish AS, et al. The prevalence of sickle cell disease in Saudi children and adolescents. A community-based survey. Saudi Med J. 2008;29:1480-1483.
2. el-Hazmi MA, Warsy AS, al-Swailem AR, et al. Sickle cell gene in the population of Saudi Arabia. Hemoglobin. 1996;20: 187-198.
3. Padmos MA, Roberts GT, Sackey K, et al. Two different forms of homozygous sickle cell disease occur in Saudi Arabia. Br J Haematol. 1991;79:93-98.
4. el-Hazmi MA. The relationship of the genetic heterogeneity of sickle cell gene to clinical manifestations. J Trop Pediatr. 1993;39:23-29.
5. Muralitharan S, Krishnamoorthy R, Nagel RL. Beta-globinlike gene cluster haplotypes in hemoglobinopathies. Methods Mol Med. 2003;82:195-211.
6. Tan AS, Quah TC, Low PS, et al. A rapid and reliable 7-deletion multiplex polymerase chain reaction assay for alphathalassemia. Blood. 2001;98:250-251.
7. Viprakasit V, Green S, Height S, et al. Hb H hydrops fetalis syndrome associated with the interaction of two common determinants of alpha thalassaemia (-MED/(alpha)TSaudi- (alpha)). Br J Haematol. 2002;117:759-762.
8. Solovieff N, Milton JN, Hartley SW, et al. Fetal hemoglobin in sickle cell anemia: Genome-wide association studies suggest a regulatory region in the 50 olfactory receptor gene cluster. Blood. 2010;115:1815-1822.
9. Perrine RP, Brown MJ, Clegg JB, et al. Benign sickle-cell anaemia. Lancet. 1972;2:1163-1167.
10. el-Hazmi MA. Heterogeneity and variation of clinical and haematological expression of haemoglobin S in Saudi Arabs. cta Haematol. 1992;88:67-71.
11. el-Hazmi MA. Clinical and haematological diversity of sickle cell disease in Saudi children. J Trop Pediatr. 1992;38:106-112.
12. Padmos A, Roberts G, Lindahl S, et al. Avascular necrosis of the femoral head in Saudi Arabians with homozygous sickle cell disease-risk factors. Ann Saudi Med. 1995;15:21-24.
13. Thein SL. Genetic modifiers of the beta-haemoglobinopathies. Br J Haematol. 2008;141:357-366.
14. Steinberg MH. Predicting clinical severity in sickle cell anaemia. Br J Haematol. 2005;129:465-481.
15. Acquaye JK, Omer A, Ganeshaguru K, et al. Non-benign sickle cell anaemia in western Saudi Arabia. Br J Haematol. 1985;60:99-108.
16. Perrine RP, Pembrey ME, John P, et al. Natural history of sickle cell anemia in Saudi Arabs. A study of 270 subjects. Ann Intern Med. 1978;88:1-6.
17. Powars DR, Chan LS, Hiti A, et al. Outcome of sickle cell anemia: A 4-decade observational study of 1056 patients. Medicine (Baltimore). 2005;84:363-376.
18. Nolan VG, Adewoye A, Baldwin C, et al. Sickle cell leg ulcers: Associations with haemolysis and SNPs in Klotho, TEK and genes of the TGF-beta/BMP pathway. Br J Haematol. 2006;133:570-578.
19. Serjeant GR. Leg ulceration in sickle cell anemia. Arch Intern Med. 1974;133:690-694.
20. Platt OS, Thorington BD, Brambilla DJ, et al. Pain in sickle cell disease. Rates and risk factors. N Engl J Med. 1991;325:11-16.
21. Milner PF, Kraus AP, Sebes JI, et al. Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med. 1991;325:1476-1481.
22. Al-Hawsawi ZM. Acute chest syndrome in sickle cell disease. Saudi Med J. 2004;25:116-117.
23. 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.
24. Gill FM, Sleeper LA, Weiner SJ, et al. Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease. Blood. 1995;86:776-783.
25. Al-Hawsawi ZM, Ismail GA. Acute splenic sequestration crisis in children with sickle cell disease. Saudi Med J. 2001;22: 1076-1079.
26. Rennels MB, Dunne MG, Grossman NJ, et al. Cholelithiasis in patients with major sickle hemoglobinopathies. Am J Dis Child. 1984;138:66-67.
27. Frempong KOSM. Clinical aspects of sickle cell anemia in adults and children. In: Steinberg MHFB, Higgs DR, Nagel RL, eds. Disorders of Hemoglobin: Genetics, Pathophysiology, and Clinical Management Cambridge. Cambridge University Press; 2001:611-710.
28. Al Hawsawi ZM, Ismail GA. Stroke among sickle cell disease patients in Madina Maternity and Children's Hospital. Ann Saudi Med. 1998;18:472-474.
29. Annobil SH, Omojola MF, Adzaku FK, et al. Cerebrovascular accidents (strokes) in children with sickle cell disease residing at high and low altitudes of Saudi Arabia. Ann Trop Paediatr. 1990;10:191-198.
30. Fowler JE Jr, Koshy M, Strub M, et al. Priapism associated with the sickle cell hemoglobinopathies: Prevalence, natural history and sequelae. J Urol. 1991;145:65-68.
31. Koshy M, Entsuah R, Koranda A, et al. Leg ulcers in patients with sickle cell disease. Blood. 1989;74:1403-1408.
32. Alsultan A, Solovieff N, Aleem A, et al. Fetal hemoglobin in sickle cell anemia: Saudi patients from the Southwestern province have similar HBB haplotypes but higher HbF levels than African Americans. Am J Hematol. 2011; 86:612-614.
33. el-Hazmi MA, Warsy AS, Addar MH, et al. Fetal haemoglobin level-effect of gender, age and haemoglobin disorders. Mol Cell Biochem. 1994;135:181-186.
34. Steinberg MH, Hsu H, Nagel RL, et al. Gender and haplotype effects upon hematological manifestations of adult sickle cell anemia. Am J Hematol. 1995;48:175-181.
35. Steinberg MH, Embury SH. Alpha-thalassemia in blacks: Genetic and clinical aspects and interactions with the sickle hemoglobin gene. Blood. 1986;68:985-990.
36. Steinberg MH, Rosenstock W, Coleman MB, et al. Effects of thalassemia and microcytosis on the hematologic and vasoocclusive severity of sickle cell anemia. Blood. 1984;63: 1353-1360.
37. Bailey K, Morris JS, Thomas P, et al. Fetal haemoglobin and early manifestations of homozygous sickle cell disease. Arch Dis Child. 1992;67:517-520.
38. De Ceulaer K, Serjeant GR. Acute splenic sequestration in Jamaican adults with homozygous sickle cell disease: A role of alpha thalassaemia. Br J Haematol. 1991;77:563-564.
39. Boyd JH, Macklin EA, Strunk RC, et al. Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia. Blood. 2006;108:2923-2927.
40. Boyd JH, Moinuddin A, Strunk RC, et al. Asthma and acute chest in sickle-cell disease. Pediatr Pulmonol. 2004;38: 229-232.
41. Nordness ME, Lynn J, Zacharisen MC, et al. Asthma is a risk factor for acute chest syndrome and cerebral vascular accidents in children with sickle cell disease. Clin Mol Allergy. 2005;3:1-5.
42. Miller ST, Sleeper LA, Pegelow CH, et al. Prediction of adverse outcomes in children with sickle cell disease. N Engl J Med. 2000;342:83-89.
43. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376:2018-2031.
44. De Franceschi L, Cappellini MD, Olivieri O. Thrombosis and sickle cell disease. Semin Thromb Hemost. 2011;37:226-236.
45. Al-Awamy B, Taha SA, Naeem MA. Priapism in association with sickle cell anemia in Saudi Arabia. Acta Haematol. 1985;73:181-182.
46. Wali YA, Al-Lamki Z, Hussein SS, et al. Splenic function in Omani children with sickle cell disease: Correlation with severity index, hemoglobin phenotype, iron status, and alphathalassemia trait. Pediatr Hematol Oncol. 2002;19:491-500.