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Hemoglobin F concentration as a function of age in Kuwaiti sickle cell disease patients.
Med Princ Pract. 2007; 16(4):286-90.MP

Abstract

OBJECTIVE

This study aimed to document the transition of hemoglobin (Hb) F levels from early childhood to adulthood in Kuwaiti sickle cell disease patients, investigating its relationship to sex, Hb genotype and coexistence of alpha-thalassemia trait.

SUBJECTS AND METHODS

The following parameters were extracted from the patients' records: age, sex, Hb, mean corpuscular volume, mean corpuscular Hb, red blood cell count, Hb F, Hb S, Hb A(2) and alpha-globin genotype. Hb quantitation was performed with cation exchange HPLC, while alpha-globin genotype was determined by PCR.

RESULTS

Records were available for 149 patients, made up of 94 SS and 55 Sbeta(0)thal; 83 males and 66 females, aged 3 months to 60 years (mean 10.5 +/- 1.8). The mean Hb F level in the whole population was 21.5 +/- 8.1% and was not significantly different between males and females, and SS or Sbeta(0)thal. When the age groups were analyzed, the Hb F level was highest (28.9 +/- 10.9%) in those below 5 years. Indeed, patients < or =2 years had a mean level of 31.9 +/- 13.0%. There was no significant difference in the Hb F levels in SS patients with or without coexistent alpha-thal trait.

CONCLUSIONS

Kuwaiti sickle cell disease patients below 5 years of age have close to 30% Hb F and this is probably a major reason why they usually do not present before this age, unlike patients elsewhere who present within the first year of life.

Authors+Show Affiliations

Department of Pediatrics, Faculty of Medicine, Kuwait University, Kuwait. adekile@hsc.edu.kwNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

17541294

Citation

Adekile, Adekunle, et al. "Hemoglobin F Concentration as a Function of Age in Kuwaiti Sickle Cell Disease Patients." Medical Principles and Practice : International Journal of the Kuwait University, Health Science Centre, vol. 16, no. 4, 2007, pp. 286-90.
Adekile A, Al-Kandari M, Haider M, et al. Hemoglobin F concentration as a function of age in Kuwaiti sickle cell disease patients. Med Princ Pract. 2007;16(4):286-90.
Adekile, A., Al-Kandari, M., Haider, M., Rajaa, M., D'Souza, M., & Sukumaran, J. (2007). Hemoglobin F concentration as a function of age in Kuwaiti sickle cell disease patients. Medical Principles and Practice : International Journal of the Kuwait University, Health Science Centre, 16(4), 286-90.
Adekile A, et al. Hemoglobin F Concentration as a Function of Age in Kuwaiti Sickle Cell Disease Patients. Med Princ Pract. 2007;16(4):286-90. PubMed PMID: 17541294.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hemoglobin F concentration as a function of age in Kuwaiti sickle cell disease patients. AU - Adekile,Adekunle, AU - Al-Kandari,Mohammed, AU - Haider,Mohammad, AU - Rajaa,Marouf, AU - D'Souza,Mark, AU - Sukumaran,Jalaja, PY - 2006/12/06/received PY - 2007/01/27/accepted PY - 2007/6/2/pubmed PY - 2007/8/2/medline PY - 2007/6/2/entrez SP - 286 EP - 90 JF - Medical principles and practice : international journal of the Kuwait University, Health Science Centre JO - Med Princ Pract VL - 16 IS - 4 N2 - OBJECTIVE: This study aimed to document the transition of hemoglobin (Hb) F levels from early childhood to adulthood in Kuwaiti sickle cell disease patients, investigating its relationship to sex, Hb genotype and coexistence of alpha-thalassemia trait. SUBJECTS AND METHODS: The following parameters were extracted from the patients' records: age, sex, Hb, mean corpuscular volume, mean corpuscular Hb, red blood cell count, Hb F, Hb S, Hb A(2) and alpha-globin genotype. Hb quantitation was performed with cation exchange HPLC, while alpha-globin genotype was determined by PCR. RESULTS: Records were available for 149 patients, made up of 94 SS and 55 Sbeta(0)thal; 83 males and 66 females, aged 3 months to 60 years (mean 10.5 +/- 1.8). The mean Hb F level in the whole population was 21.5 +/- 8.1% and was not significantly different between males and females, and SS or Sbeta(0)thal. When the age groups were analyzed, the Hb F level was highest (28.9 +/- 10.9%) in those below 5 years. Indeed, patients < or =2 years had a mean level of 31.9 +/- 13.0%. There was no significant difference in the Hb F levels in SS patients with or without coexistent alpha-thal trait. CONCLUSIONS: Kuwaiti sickle cell disease patients below 5 years of age have close to 30% Hb F and this is probably a major reason why they usually do not present before this age, unlike patients elsewhere who present within the first year of life. SN - 1011-7571 UR - https://www.unboundmedicine.com/medline/citation/17541294/Hemoglobin_F_concentration_as_a_function_of_age_in_Kuwaiti_sickle_cell_disease_patients_ L2 - https://www.karger.com?DOI=10.1159/000102151 DB - PRIME DP - Unbound Medicine ER -