Genetic Haemoglobin Disorders in Pakistan

Ahmed, Suhaib

doi:10.21089/njhs.23.0095

Cited by 8 publications

(5 citation statements)

References 17 publications

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“…South Asia has been confronting a silent epidemic of thalassemia- a life-threatening inherited hemoglobin disorder. Over 200,000 estimated thalassemia patients (45–70 million carriers) live in the Indian Subcontinent whose lives depend on regular blood transfusion with chelation therapy [ 1 – 3 ]. There is an acute crisis of availability of blood for thalassemia patients especially in South Asia.…”

Section: Letter To the Editormentioning

confidence: 99%

The forgotten people with thalassemia in the time of COVID-19: South Asian perspective

Hossain

Raheem

Siddiqee

2020

Orphanet J Rare Dis

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South Asia is the hotspot of beta-thalassemia, with an estimated 200,000 patients whose lives depend on regular blood transfusion. Due to COVID-19 pandemic, many countries have adopted unprecedented lockdown to minimize the spread of transmission. Restriction of nationwide human mobility and fear of COVID-19 infection has put thalassemia patients in a life-threatening situation because of an acute shortage of blood supply. As a public health preparedness strategy during a crisis like COVID-19 pandemic, the plights of thalassemia patients should be considered. Government-sponsored community blood-banks needs to be established or coverage expanded as a safety net for the thalassemia patients in lower- and middle-income countries.

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Section: Letter To the Editormentioning

confidence: 99%

The forgotten people with thalassemia in the time of COVID-19: South Asian perspective

Hossain

Raheem

Siddiqee

2020

Orphanet J Rare Dis

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“…There are limited facilities for thalassemia patients in the government hospitals and thalassemic patients were the primary reason for the proliferation of nongovernmental organization (NGO) sector thalassemia centers in the country. 20 These patients are mostly treated by these NGOs whose standard of services varies. Some of these centers are not adequately equipped, have poor technical expertise, and place major emphasis on getting the blood transfusions neglecting other interventions including quality-assured screening for TTIs, among others.…”

Section: Introductionmentioning

confidence: 99%

A Systematic Review and Meta-Analysis on the Epidemiology of Hepatitis B and Hepatitis C Virus among Beta-Thalassemia Major Patients in Pakistan

Waheed

Saba²,

Wazeer

et al. 2021

J Lab Physicians

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Beta-thalassemia major patients are the leading consumers of blood transfusions in Pakistan and, therefore, have a greater risk of acquiring transfusion-transmitted infections, most notably hepatitis B and C virus (HBV and HCV). The present study includes a comprehensive review on the status of HBV and HCV in beta-thalassemia major patients in Pakistan. For this purpose, we examined original articles assessing the epidemiology of HBV and HCV in transfusion-dependent thalassemia patients.We searched 10 major subscription databases from January through February 2020, that is, Medline, PakMediNet, CINAHL, Scopus, PubMed, Web of Science, Embase, Science Direct, Google Scholar, and Directory of Open Access Journals. The World Health Organization resources were also explored for relevant reports. The search criteria included published articles up to December 31, 2019, with no language restrictions. Articles identified were introduced into the Endnote version X9 software and then screened for relevance and duplication. The results were stated as the pooled prevalence for the overall study and also for region-wise subgroups.A total of 33 studies conducted from 1995 to 2019 were included in the review. All 33 articles yielded information on HCV prevalence, while 19 of them provided information on HBV prevalence. The overall sample size was 8,554 that tested the prevalence of HCV in thalassemia patients. The sample size from the 19 studies that tested the prevalence of HBV was 6,184. The overall pooled prevalence of HBV was computed to be 4.13%, while the pooled prevalence of HCV was 29.79%. The majority of the studies were obtained from the Punjab Province (33.33%), followed by Khyber Pakhtunkhwa Province (24.24%).The total sample size of 33 studies was less than 10% of the total number of estimated thalassemic patients, that is, 100,000. Further studies or a national baseline survey are imperative to confirm the actual frequency of HBV and HCV in thalassemia patients across the country.

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“…In Pakistan, Hb D is reported in approximately one percent of Punjabi population. Hb D Punjab and Hb D Iran exists as two different genotypes of Hb D. They are both clinically silent and have the same electrophoretic mobility and can be distinguished on basis of PCR 11 .…”

Section: Resultsmentioning

confidence: 99%

Unexpected Discovery of Hemoglobin D Iran Families in Punjab, Pakistan by using two different Screening Methods

Sikander¹,

Bashir²,

Tariq³

et al. 2021

PJMHS

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Background: Hemoglobin D Iran is frequently misdiagnosed as Hb E or Hb D Punjab if only one method of screening is used. The objective of our study was to highlight the importance of using two different screening techniques in diagnosis of a hemoglobin variant, Hb D Iran in our case. Hematological parameters of heterozygous Hb D Iran and compound heterozygous β/Hb D Iran were also compared. Methods: A descriptive study was carried out on results of 52,379 subjects which were part of thalassemia extended family cascade screening from 36 districts of Punjab from October 2019-March 2021. Cases of Hb D Punjab and Hb E were run on both CE-HPLC (cation exchange-high performance liquid chromatography) and CZE (capillary zone electrophoresis). Resulting Hb D Iran cases were confirmed by ARMS-PCR (Amplification refractory mutation system-polymerase chain reaction). Results: Forty cases of Hb D Iran were detected out of 160 initially suspected Hb D Punjab cases and 126 Hb E cases. Diagnosis was confirmed by molecular analysis. Statistical significance was found between RBC count, MCV, MCH, Hb F and diagnosis of “heterozygous Hb D Iran” and “compound heterozygous for β/ Hb D Iran”. Conclusion: Hb D Iran can be easily missed and misdiagnosed as Hb E or Hb D Punjab, if two screening methods are not used. This maybe a reason why Hb D Iran remains unreported in our region. CBC and HPLC indices can also be suggestive if a case is of heterozygous D Iran or compound heterozygous β/Hb D Iran. Keywords: Hb D Iran, Hb E, Hb D Punjab, Cation exchange High performance liquid chromatography, Capillary zone electrophoresis

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Genetic Haemoglobin Disorders in Pakistan

Cited by 8 publications

References 17 publications

The forgotten people with thalassemia in the time of COVID-19: South Asian perspective

The forgotten people with thalassemia in the time of COVID-19: South Asian perspective

A Systematic Review and Meta-Analysis on the Epidemiology of Hepatitis B and Hepatitis C Virus among Beta-Thalassemia Major Patients in Pakistan

Unexpected Discovery of Hemoglobin D Iran Families in Punjab, Pakistan by using two different Screening Methods

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