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Observational study on the current status of thalassaemia in Malaysia: a report from the Malaysian Thalassaemia Registry.
BMJ Open. 2020 Jun 29; 10(6):e037974.BO

Abstract

OBJECTIVE

Thalassaemia is the most common inherited blood disorder in Malaysia. This study aims to report the current status of thalassaemia in Malaysia and provide a comprehensive understanding of the disease through data obtained from the Malaysian Thalassaemia Registry.

DESIGN

Data were extracted from the Malaysian Thalassaemia Registry, a web-based system accessible to enrolled users through www.mytalasemia.net.my.

SETTING

The Malaysian Thalassaemia Registry data was recorded from reports obtained from 110 participating government and university hospitals in Malaysia.

PARTICIPANTS

The patients were those attending the 110 participating hospitals for thalassaemia treatment.

INTERVENTION

Data were collected from the Malaysian Thalassaemia Registry from 2007 until the fourth quarter of 2018.

PRIMARY OUTCOME MEASURE

7984 out of 8681 patients with thalassaemia registered in the Malaysian Thalassaemia Registry were reported alive.

RESULTS

Majority of the patients were reported in the state of Sabah (22.72%); the largest age group affected was 5.0-24.9 years old (64.45%); the largest ethnic group involved was Malay (63.95%); and the major diagnosis was haemoglobin E/β-thalassaemia (34.37%). From the 7984 patients, 56.73% were on regular blood transfusions and 61.72% were on chelation therapy. A small fraction (14.23%) has undergone splenectomy, while the percentage of patients with severe iron overload (serum ferritin ≥5000 µg/L) reduced over time. However, cardiac complications are still the main cause of death in patients with thalassaemia.

CONCLUSION

Data gathered into the registry can be used to understand the progression of the disorder, to monitor iron overload management and to improve the outcomes of treatment, to enhance preventive strategies, reduce healthcare burden and improve the quality of life. Sustainability of the Malaysian Thalassaemia Registry is important for surveillance of thalassaemia management in the country and help the national health authorities to develop more effective policies.

Authors+Show Affiliations

Division for Research and Technical Support, Ministry of Health Malaysia, Putrajaya, Wilayah Persekutuan Putrajaya, Malaysia. Department of Paediatrics, Hospital Tunku Azizah, Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur, Malaysia.Department of Paediatrics, Hospital Tunku Azizah, Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur, Malaysia.Department of Paediatrics, Hospital Tunku Azizah, Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur, Malaysia.Department of Paediatrics, Hospital Tunku Azizah, Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur, Malaysia.Department of Paediatrics, Hospital Tunku Azizah, Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur, Malaysia.Department of Paediatrics, Sabah Women & Children Hospital, Likas, Kota Kinabalu, Sabah, Malaysia.Department of Paediatrics, Sabah Women & Children Hospital, Likas, Kota Kinabalu, Sabah, Malaysia.Department of Paediatrics, Hospital Umum Sarawak, Kuching, Sarawak, Malaysia.Department of Paediatrics, Hospital Pulau Pinang, Georgetown, Pulau Pinang, Malaysia.Department of Paediatrics, Hospital Raja Permaisuri Bainun, Ipoh, Perak, Malaysia.Department of Paediatrics, Hospital Sultan Ismail, Johor Bharu, Johor, Malaysia.Department of Paediatrics, Hospital Sultanah Nur Zahirah Kuala Terengganu, Kuala Terengganu, Terengganu, Malaysia.Department of Paediatrics, UKM Medical Centre, The National University of Malaysia, Jalan Yaacob Latif, Cheras, Kuala Lumpur, Malaysia.UKM Medical Molecular Biology Institute, Jalan Yaacob Latif, Cheras, Kuala Lumpur, Malaysia.Department of Paediatrics, Hospital Universiti Sains Malaysia, Kota Bahru, Kelantan, Malaysia.Department of Paediatrics, University of Malaya Medical Centre, Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur, Malaysia.Department of Paediatrics, UKM Medical Centre, The National University of Malaysia, Jalan Yaacob Latif, Cheras, Kuala Lumpur, Malaysia midalias@ppukm.ukm.edu.my.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

32601117

Citation

Mohd Ibrahim, Hishamshah, et al. "Observational Study On the Current Status of Thalassaemia in Malaysia: a Report From the Malaysian Thalassaemia Registry." BMJ Open, vol. 10, no. 6, 2020, pp. e037974.
Mohd Ibrahim H, Muda Z, Othman IS, et al. Observational study on the current status of thalassaemia in Malaysia: a report from the Malaysian Thalassaemia Registry. BMJ Open. 2020;10(6):e037974.
Mohd Ibrahim, H., Muda, Z., Othman, I. S., Mohamed Unni, M. N., Teh, K. H., Thevarajah, A., Gunasagaran, K., Ong, G. B., Yeoh, S. L., Muhammad Rivai, A., Che Mohd Razali, C. H., Din, N. D., Abdul Latiff, Z., Jamal, R., Mohamad, N., Mohd Ariffin, H., & Alias, H. (2020). Observational study on the current status of thalassaemia in Malaysia: a report from the Malaysian Thalassaemia Registry. BMJ Open, 10(6), e037974. https://doi.org/10.1136/bmjopen-2020-037974
Mohd Ibrahim H, et al. Observational Study On the Current Status of Thalassaemia in Malaysia: a Report From the Malaysian Thalassaemia Registry. BMJ Open. 2020 Jun 29;10(6):e037974. PubMed PMID: 32601117.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Observational study on the current status of thalassaemia in Malaysia: a report from the Malaysian Thalassaemia Registry. AU - Mohd Ibrahim,Hishamshah, AU - Muda,Zulaiha, AU - Othman,Ida Shahnaz, AU - Mohamed Unni,Mohamed Najib, AU - Teh,Kok Hoi, AU - Thevarajah,Asohan, AU - Gunasagaran,Kogilavani, AU - Ong,Gek Bee, AU - Yeoh,Seoh Leng, AU - Muhammad Rivai,Aisyah, AU - Che Mohd Razali,Che Hadibiah, AU - Din,Nazzlin Dizana, AU - Abdul Latiff,Zarina, AU - Jamal,Rahman, AU - Mohamad,Norsarwany, AU - Mohd Ariffin,Hany, AU - Alias,Hamidah, Y1 - 2020/06/29/ PY - 2020/7/1/entrez PY - 2020/7/1/pubmed PY - 2020/7/1/medline KW - Malaysia KW - database KW - haemoglobinopathy KW - healthcare burden KW - registry KW - thalassaemia SP - e037974 EP - e037974 JF - BMJ open JO - BMJ Open VL - 10 IS - 6 N2 - OBJECTIVE: Thalassaemia is the most common inherited blood disorder in Malaysia. This study aims to report the current status of thalassaemia in Malaysia and provide a comprehensive understanding of the disease through data obtained from the Malaysian Thalassaemia Registry. DESIGN: Data were extracted from the Malaysian Thalassaemia Registry, a web-based system accessible to enrolled users through www.mytalasemia.net.my. SETTING: The Malaysian Thalassaemia Registry data was recorded from reports obtained from 110 participating government and university hospitals in Malaysia. PARTICIPANTS: The patients were those attending the 110 participating hospitals for thalassaemia treatment. INTERVENTION: Data were collected from the Malaysian Thalassaemia Registry from 2007 until the fourth quarter of 2018. PRIMARY OUTCOME MEASURE: 7984 out of 8681 patients with thalassaemia registered in the Malaysian Thalassaemia Registry were reported alive. RESULTS: Majority of the patients were reported in the state of Sabah (22.72%); the largest age group affected was 5.0-24.9 years old (64.45%); the largest ethnic group involved was Malay (63.95%); and the major diagnosis was haemoglobin E/β-thalassaemia (34.37%). From the 7984 patients, 56.73% were on regular blood transfusions and 61.72% were on chelation therapy. A small fraction (14.23%) has undergone splenectomy, while the percentage of patients with severe iron overload (serum ferritin ≥5000 µg/L) reduced over time. However, cardiac complications are still the main cause of death in patients with thalassaemia. CONCLUSION: Data gathered into the registry can be used to understand the progression of the disorder, to monitor iron overload management and to improve the outcomes of treatment, to enhance preventive strategies, reduce healthcare burden and improve the quality of life. Sustainability of the Malaysian Thalassaemia Registry is important for surveillance of thalassaemia management in the country and help the national health authorities to develop more effective policies. SN - 2044-6055 UR - https://www.unboundmedicine.com/medline/citation/32601117/Observational_study_on_the_current_status_of_thalassaemia_in_Malaysia:_a_report_from_the_Malaysian_Thalassaemia_Registry L2 - http://bmjopen.bmj.com/cgi/pmidlookup?view=long&pmid=32601117 DB - PRIME DP - Unbound Medicine ER -
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