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Six-year outcome of the national premarital screening and genetic counseling program for sickle cell disease and β-thalassemia in Saudi Arabia.
Ann Saudi Med. 2011 May-Jun; 31(3):229-35.AS

Abstract

BACKGROUND AND OBJECTIVES

Saudi Arabia has a high prevalence of hereditary hemoglobin disorders. Data has been collected by the Saudi Premarital Screening and Genetic Counseling Program on the prevalence of sickle cell disease and β-thalassemia but the outcomes were not quantified. We used six years of premarital screening data to estimate the burden of sickle disease and β-thalassemia over the program period and to assess the frequency of at-risk marriage detection and prevention.

DESIGN AND SETTING

Retrospective review, premarital couples attending premarital and genetic counseling clinics with marriage proposals between 2004 and 2009.

METHODS

Blood samples obtained from all couples with marriage proposals between 2004 and 2009 were tested for sickle cell disease and β-thalassemia. Test results were shared with all examinees and genetic counseling was offered for all at-risk couples. Marriage certificates were issued irrespective of the results and compliance with medical advice was voluntary.

RESULTS

Out of all men and women examined, 70,962 (4.5%) and 29,006 (1.8%) were carriers or cases of sickle cell disease and β-thalassemia, respectively. While the prevalence of sickle cell disease was constant between 2004 and 2009 (average 45.1 per 1000 examined persons, P=.803), the prevalence of β-thalassemia steadily decreased from 32.9 to 9.0 per 1000 examined persons (P<.001). The frequency of at-risk couples decreased by about 60% between 2004 and 2009 (from 10.1 to 4.0 per 1000 examined persons, P<.001). The frequency of voluntary cancellation of marriage proposals among at-risk couples showed more than 5-fold increase between 2004 and 2009 (from 9.2% to 51.9%, P<.001). The eastern region had 58% of all detected at-risk marriages and showed the greatest decline in detection and increase in prevention over time compared to other regions of Saudi Arabia.

CONCLUSION

Six years of premarital screening in Saudi Arabia markedly reduced the number of at-risk marriages, which may considerably reduce the genetic disease burden in Saudi Arabia in the next decades.

Authors+Show Affiliations

Preventive Medicine, Ministry of Health, Riyadh, Saudi Arabia. zmemish@yahoo.comNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

21623050

Citation

Memish, Ziad Ahmed, and Mohammad Y. Saeedi. "Six-year Outcome of the National Premarital Screening and Genetic Counseling Program for Sickle Cell Disease and Β-thalassemia in Saudi Arabia." Annals of Saudi Medicine, vol. 31, no. 3, 2011, pp. 229-35.
Memish ZA, Saeedi MY. Six-year outcome of the national premarital screening and genetic counseling program for sickle cell disease and β-thalassemia in Saudi Arabia. Ann Saudi Med. 2011;31(3):229-35.
Memish, Z. A., & Saeedi, M. Y. (2011). Six-year outcome of the national premarital screening and genetic counseling program for sickle cell disease and β-thalassemia in Saudi Arabia. Annals of Saudi Medicine, 31(3), 229-35. https://doi.org/10.4103/0256-4947.81527
Memish ZA, Saeedi MY. Six-year Outcome of the National Premarital Screening and Genetic Counseling Program for Sickle Cell Disease and Β-thalassemia in Saudi Arabia. Ann Saudi Med. 2011 May-Jun;31(3):229-35. PubMed PMID: 21623050.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Six-year outcome of the national premarital screening and genetic counseling program for sickle cell disease and β-thalassemia in Saudi Arabia. AU - Memish,Ziad Ahmed, AU - Saeedi,Mohammad Y, PY - 2011/5/31/entrez PY - 2011/5/31/pubmed PY - 2011/9/17/medline SP - 229 EP - 35 JF - Annals of Saudi medicine JO - Ann Saudi Med VL - 31 IS - 3 N2 - BACKGROUND AND OBJECTIVES: Saudi Arabia has a high prevalence of hereditary hemoglobin disorders. Data has been collected by the Saudi Premarital Screening and Genetic Counseling Program on the prevalence of sickle cell disease and β-thalassemia but the outcomes were not quantified. We used six years of premarital screening data to estimate the burden of sickle disease and β-thalassemia over the program period and to assess the frequency of at-risk marriage detection and prevention. DESIGN AND SETTING: Retrospective review, premarital couples attending premarital and genetic counseling clinics with marriage proposals between 2004 and 2009. METHODS: Blood samples obtained from all couples with marriage proposals between 2004 and 2009 were tested for sickle cell disease and β-thalassemia. Test results were shared with all examinees and genetic counseling was offered for all at-risk couples. Marriage certificates were issued irrespective of the results and compliance with medical advice was voluntary. RESULTS: Out of all men and women examined, 70,962 (4.5%) and 29,006 (1.8%) were carriers or cases of sickle cell disease and β-thalassemia, respectively. While the prevalence of sickle cell disease was constant between 2004 and 2009 (average 45.1 per 1000 examined persons, P=.803), the prevalence of β-thalassemia steadily decreased from 32.9 to 9.0 per 1000 examined persons (P<.001). The frequency of at-risk couples decreased by about 60% between 2004 and 2009 (from 10.1 to 4.0 per 1000 examined persons, P<.001). The frequency of voluntary cancellation of marriage proposals among at-risk couples showed more than 5-fold increase between 2004 and 2009 (from 9.2% to 51.9%, P<.001). The eastern region had 58% of all detected at-risk marriages and showed the greatest decline in detection and increase in prevention over time compared to other regions of Saudi Arabia. CONCLUSION: Six years of premarital screening in Saudi Arabia markedly reduced the number of at-risk marriages, which may considerably reduce the genetic disease burden in Saudi Arabia in the next decades. SN - 0975-4466 UR - https://www.unboundmedicine.com/medline/citation/21623050/Six_year_outcome_of_the_national_premarital_screening_and_genetic_counseling_program_for_sickle_cell_disease_and_β_thalassemia_in_Saudi_Arabia_ L2 - https://www.annsaudimed.net/doi/full/10.4103/0256-4947.81527?url_ver=Z39.88-2003&amp;rfr_id=ori:rid:crossref.org&amp;rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -