Pre-marital screening for beta thalassaemia in Pakistan: an insight

Shariq, M; Moiz, Bushra; Zaidi, Nazneen; Azhar, Waleed Bin; Iqbal, Waseem; Humaira, Aiyesha; Memon, Rab Nawaz

doi:10.1177/0969141314541434

Cited by 5 publications

(6 citation statements)

References 6 publications

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“…The most common cause of temporary deferral in our study was use of therapeutic injections followed by low haemoglobin. In this study, the high deferral due to anaemia could be as a result of high prevalence of thalassaemia trait in the general population up to 6Á6% [11]. Furthermore, iron deficiency anaemia has been reported between 16% and 29% of the young population, more so in females [12].…”

Section: Discussionmentioning

confidence: 90%

Frequency and causes of deferral amongst voluntary non‐remunerated blood donors from Pakistan – a single‐centre study

Jamal

Shaikh

Sohail

et al. 2018

ISBT Science Series

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Background Amidst a system dependent on replacement donors in Pakistan, The Indus Hospital Blood Centre predominantly recruits voluntary non‐remunerated blood donors (VNRBD) to ensure provision of safe blood. Furthermore, due to unsafe practices of reuse of syringes in Pakistan, an additional criterion was added, deferring donors on recent history of therapeutic injections for medicinal purposes. Methods During the study period of 37 months (Dec 2013–Dec 2016), all potential donors visiting blood drives and the donor centre underwent a screening process via a brief physical examination and an interview. Donors were either accepted for donation, temporarily deferred or permanently deferred. Results In the study period, 63 582 potential donors visited, whereas 84% were VNRBD and 16% replacement donors. With an overall deferral of 24%, amidst VNRBD and replacement, the deferral rate was 25% and 18%, respectively. The major reasons for deferral were use of therapeutic injections followed by low haemoglobin. Furthermore, 54% of females and 21% of males visiting were deferred. Replacement donors, males and donors from underprivileged residential areas and factories had a comparatively higher proportion of deferral due to use of therapeutic injections. Conclusion The high deferral rate in this study is illustrative of the increased use of therapeutic injections amongst the Pakistani population. Additionally, due to the compulsion of providing blood donors associated with replacement donation, they are more likely to hide health conditions, hence the lower comparative deferral rate. The Pakistani population needs to be educated regarding the high risks associated with injections and replacement donors.

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Section: Discussionmentioning

confidence: 90%

Frequency and causes of deferral amongst voluntary non‐remunerated blood donors from Pakistan – a single‐centre study

Jamal

Shaikh

Sohail

et al. 2018

ISBT Science Series

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“…1 Globally it is second most common hemoglobinopathy after sickle cell disease. 2 Thalassemia has emerged as significant public health issue. According to World Health Organization (WHO) report, this is the most prevalent genetic blood disorder in the world.…”

Section: Introductionmentioning

confidence: 99%

“…Therefore prevention of thalassemia is demanding as well as challenging. 17 Since there is no permanent cure for this disease and treatment depends on lifelong blood transfusion which impose huge financial and emotional burden on families and health system of country. Best strategy to reduce burden of thalassemia is prevention and parental knowledge regarding disease is crucial in long term prevention.…”

Section: Introductionmentioning

confidence: 99%

Knowledge of thalassemia and consanguinity: A multicenter hospital based retrospective cohort study from metropolitan city of Karachi, Pakistan.

Khalid

Noreen

Qureshi

et al. 2019

TPMJ

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Objectives: To find the association between consanguinity and frequency of b-thalassemia and to assess the knowledge of parents regarding disease as well as inclination towards premarital carrier screening (PMCS), pre natal diagnosis, pre conception genetic counseling. Study Design: A retrospective cohort study. Setting: Patients with beta thalassemia attending Fatimid Foundation and PNS Shifa Hospital, Karachi. Period: Six Months July – December, 2017. Materials and Methods: Data was collected by pre tested questionnaire which include basic demographic profile, frequency of consanguineous marriages and knowledge regarding disease from parents of children suffering from thalassemia. The study was approved by ethical review committee. Data was analyzed using SPSS version 21. Chi square test was applied. Results: Data was collected from 200 study participants including parents of thalassemia. Males were 52% and 48% were the females. 78.5% were relatives (p=0.001) out of these related, first degree relatives were 61% and 17.5% were distant relatives (p 0.009). Only 25% were aware of genetic counseling, 65% know that thalassemia had genetic mode of transmission (p=0.005), 24% were aware of screening modalities available for thalassemic patients (p=0.001). About 63.55% agreed that premarital screening can prevent thalassemia (p=0.001), majority (83%) of parents were not aware of pre natal diagnosis of thalassemia (p=0.001) more than half of study participants (52%) were still in favor of cousin marriages in future. Conclusion: Thalassemia was found most prevalent among first degree familial relatives. Parents have inadequate knowledge regarding disease. Lack of knowledge and trends consanguineous marriages are strong contributory factor for causation of disease.

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“…b-Thalassaemia major is a preventable non-communicable hereditary disease, and given the high prevalence of thalassaemia in certain areas, and the lack of a certain cure (excepting bone marrow transplant), screening has been implemented in many countries. [12][13][14] Most thalassaemia minor cases have HbA2 levels higher than 3.5%. In the Isfahan thalassaemia screening programme, only 3.3% of thalassaemia minor cases had HbA2 lower than 3.5%.…”

Section: Discussionmentioning

confidence: 99%

“…β-Thalassaemia major is a preventable non-communicable hereditary disease, and given the high prevalence of thalassaemia in certain areas, and the lack of a certain cure (excepting bone marrow transplant), screening has been implemented in many countries. 12–14…”

Section: Discussionmentioning

confidence: 99%

The pros and cons of the fourth revision of thalassaemia screening programme in Iran

Moafi

Vallian

et al. 2016

J Med Screen

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Objective To evaluate the repercussions of recent changes to the cut-offs used in the first screening step of the pre-marital screening programme for thalassaemia prevention in Iran. Methods The profiles of 984 subjects referred to a genetic laboratory, and the tests of 242 parents of children with thalassaemia major were assessed for red blood cell (RBC) indices, haemoglobin (Hb) A2 levels and results of Hb electrophoresis. Results Of 407 suspected thalassaemia minor (STM) cases, 18 proved positive for thalassaemia minor on molecular analysis (18/407, confidence interval 2.6-6.9%). If the revised screening cut-offs had been used to determine who would undergo molecular analysis, two of these cases would not have been identified. Only 4.4% of suspected cases with lower than normal RBC indices (mean corpuscular volume <80 fl and mean corpuscular Hb <27 pg) and HbA2 (<3.5%) were diagnosed with thalassaemia minor. Conclusion The thalassaemia major prevention programme is performed in two separate steps. One step involves the screening of subjects and identification of β-thalassaemia minor, suspected cases for thalassaemia minor (STM), and normal subject groups. The other step concerns the identification of thalassaemia minor in the STM group. Changing the cut-offs at the first screening step does not result in significant improvement from an economic view, and is associated with significant risk at the second screening step.

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Pre-marital screening for beta thalassaemia in Pakistan: an insight

Cited by 5 publications

References 6 publications

Frequency and causes of deferral amongst voluntary non‐remunerated blood donors from Pakistan – a single‐centre study

Frequency and causes of deferral amongst voluntary non‐remunerated blood donors from Pakistan – a single‐centre study

Knowledge of thalassemia and consanguinity: A multicenter hospital based retrospective cohort study from metropolitan city of Karachi, Pakistan.

The pros and cons of the fourth revision of thalassaemia screening programme in Iran

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