Obligatory premarital tests for ?-thalassaemia in the Gaza Strip: evaluation and recommendations

Tarazi, Issa S.; Najjar, Esmat; Lulu, N.; Sirdah, Mahmoud M.

doi:10.1111/j.1365-2257.2006.00836.x

Cited by 14 publications

(23 citation statements)

References 15 publications

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“…In our study however, informed choice was left completely to the couple at risk. 29 One option to reduce the rate of affected births may be to implement haemoglobinopathy screening among secondary school students, in parallel with an education programme, so that a decision can be made before any possible commitment to marriage. This option has been successful in some countries.…”

Section: Ivs-i-mentioning

confidence: 99%

The first five years of a preventive programme for haemoglobinopathies in Northeastern Iraq

Al-Allawi

Jalal

Ahmed³

et al. 2013

J Med Screen

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Objective: To evaluate the feasibility and effectiveness of a preventive programme for haemoglobinopathies in a single centre in Northeastern Iraq. Methods: Premarital screening, genetic counselling and prenatal diagnosis (PND) were implemented over a 5 year period. Results: Among a total of 108,264 screened individuals (54,132 couples), b-thalassaemia trait, db-thalassaemia trait, and sickle cell trait were diagnosed in 3.98%, 0.11% and 0.07%, respectively. Of 130 at risk couples (2.4/1000), 107 (82%) were available for follow up, with 105 couples (98.1%) proceeding with their marriage after counselling. In the 125 registered pregnancies in the latter couples, PND was performed in 85 (in 80 couples, uptake 76%). Selective termination was chosen in 10 of the 11 pregnancies with an affected fetus. Six affected babies were born among couples who declined PND. At the same time 30 already married couples with at least one thalassaemic child underwent PND, revealing three affected fetuses; all three pregnancies were terminated. Conclusion: The programme revealed that most at risk couples diagnosed by premarital screening chose to proceed with their marriage, with 76% seeking PND followed by selective termination of an affected fetus. A 65% reduction in number of affected births was reported over the 5 year period. This regional programme could serve as a prototype for a national haemoglobinopathy prevention programme.

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Section: Ivs-i-mentioning

confidence: 99%

The first five years of a preventive programme for haemoglobinopathies in Northeastern Iraq

Al-Allawi

Jalal

Ahmed³

et al. 2013

J Med Screen

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“…3 Nevertheless, some countries including Iran, Saudi Arabia, Palestinian Territories and Cyprus have laws in place making premarital screening for haemoglobinopathies mandatory for all couples before they are given approval to get married. 9,[20][21][22][23][24][25][26] This raises ethical questions, as couples are unable to voluntarily decide for themselves whether they would like to undergo genetic screening. Thalassaemia carrier screening has been included as part of an existing mandatory premarital blood test in Iran since 1991.…”

Section: Mandatory Screeningmentioning

confidence: 99%

“…Thalassaemia carrier screening has been included as part of an existing mandatory premarital blood test in Iran since 1991. [22][23][24] This form of mandatory premarital thalassaemia screening also began in the Gaza Strip in 2000 9 and in Saudi Arabia in 2003. 20 Carrier couples receive advice on the options available to them, one of which is cancellation of marriage, and they can then decide whether to marry each other.…”

Section: Mandatory Screeningmentioning

confidence: 99%

“…A genetic screening programme that provides the opportunity to compare different approaches taken to screen populations around the world is b-thalassaemia carrier screening, as this is one of the most common single-gene inherited conditions in the world. [8][9][10] Thalassaemias are haemoglobinopathies that are characterized by a decrease in the production of globin chains, which results in microcytic anaemia. [1][2][3][4][5] The two major types of thalassaemia are a-thalassaemia and b-thalassaemia.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Carrier screening for Beta-thalassaemia: a review of international practice

et al. 2010

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b-thalassaemia is one of the most common single-gene inherited conditions in the world, and thalassaemia carrier screening is the most widely performed genetic screening test, occurring in many different countries. b-thalassaemia carrier screening programmes provide a unique opportunity to compare the delivery of carrier screening programmes carried out in different cultural, religious and social contexts. This review compares the key characteristics of b-thalassaemia carrier screening programmes implemented in countries across the world so that the differences and similarities between the programmes can be assessed. The manner in which thalassaemia carrier screening programmes are structured among different populations varies greatly in several aspects, including whether the programmes are mandatory or voluntary, the education and counselling provided and whether screening is offered pre-pregnancy or antenatally. National and international guidelines make recommendations on the most appropriate ways in which genetic carrier screening programmes should be conducted; however, these recommendations are not followed in many programmes. We discuss the implications for the ethical and acceptable implementation of population carrier screening and identify a paucity of research into the outcomes of thalassaemia screening programmes, despite the fact that thalassaemia screening is so commonly conducted.

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“…(White et al 1986) 1.5 % (1,000) (White et al 1993) 48.5 % (7,837) (Alkindi et al 2010) 2.8 % (7,837) (Alkindi et al 2010) 58.3 % (87) (Hassan et al 2010) 2.4 % (790) (White et al 1986) Palestinian territories 4.3 % (1,650) (Sirdah et al 1998) 3.5 % in West Bank (Darwish et al 2005). 2.6 % in Gaza (21,825) (Tarazi et al 2007) Qatar 8.85 (712) (Tremblay et al 2011) Saudi Arabia 3 % (840) (Ganeshaguru et al 1987) 28 % in Al-Qatif and 16.3 % in Al-Hasa (12,220) (Nasserullah et al 1998) 3.22 % (488,315) (Alhamdan et al 2007) 43.3 % (840) (Ganeshaguru et al 1987 (Mseddi et al 1999) 2 % all Tunis and 4 % Northern Region (304) (Zorai et al 2002) 7.38 % (529) (Siala et al 2008) UAE 1.7 % (4,221) (White et al 1986) 49 % (418) (Baysal 2001;El-Kalla and Baysal 1998;Haj Khelil et al 2010) (White et al 1986) IVS-II-848 (C>A) may have Egyptian origin with a reported rate of 11 % (Hussein et al 1993).…”

Section: Introductionmentioning

confidence: 99%

Epidemiological profile of common haemoglobinopathies in Arab countries

2012

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Haemoglobinopathies including the thalassemias and sickle cell disease are known to be prevalent inherited disorders in most Arab countries with varying prevalence rates and molecular characterisation. β-thalassemia is encountered in polymorphic frequencies in almost all Arab countries with carrier rates of 1-11 % and a varying number of mutations. The most widespread mutation in Lebanon, Egypt, Syria, Jordan, Tunisia and Algeria is the IVS-I-110 (G>A). In the Eastern Arabian Peninsula, the Asian Indian mutations (IVS-I-5 (G>C), codons 8/9 (+G) and IVS-I (−25 bp del)) are more common. The α-thalassemias are encountered in the majority of Arab countries in frequencies ranging from 1 to 58 % with the highest frequencies reported from Gulf countries. The (−α 3.7 ) mutation is the most frequent followed by the non-deletional α2 polyadenylation signal mutation (AATAAA>AATAAG) and the α2 IVS1 5-bp deletion. The rates of sickle cell trait in Arab countries range from 0.3 to 30 %, with the Benin, the Arab-Indian and the Bantu haplotypes constituting the bulk of the haplotypes, leading to two major phenotypes; a mild one associated with the Arab-Indian and a severe one with the Benin and Bantu haplotypes. Public health approaches targeting prevention of haemoglobinopathies in Arab countries include newborn screening for sickle cell disease, and premarital screening for carriers of β-thalassemia and sickle cell disease. These services are still patchy and inadequate in many Arab countries recommending the upgrade of these services with strengthening of the education and training of health care providers and raising public awareness on the feasibility of prevention and care for haemoglobinopathies.

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Obligatory premarital tests for ?-thalassaemia in the Gaza Strip: evaluation and recommendations

Cited by 14 publications

References 15 publications

The first five years of a preventive programme for haemoglobinopathies in Northeastern Iraq

The first five years of a preventive programme for haemoglobinopathies in Northeastern Iraq

Carrier screening for Beta-thalassaemia: a review of international practice

Epidemiological profile of common haemoglobinopathies in Arab countries

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