Medical Journal of Indonesia
Vol 27, No 1 (2018): March

Applicability of a clinical scoring criteria for disease severity of ß-thalassemia/hemoglobin E in Indonesia

Wahidiyat, Pustika A. ( Department of Child Health, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo Hospital, Jakarta)
Sastroasmoro, Sudigdo ( Department of Child Health, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo Hospital, Jakarta)
Fucharoen, Suthat ( Thalassemia Research Center, Institute of Molecular Biosciences, Mahidol University, Bangkok)
Setianingsih, Iswari ( Eijkman Institute for Molecular Biology, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo Hospital, Jakarta)
Putriasih, Siti A. ( Department of Child Health, Faculty of Medicine, Universitas Indonesia, Cipto Mangunkusumo Hospital, Jakarta)



Article Info

Publish Date
08 May 2018

Abstract

Background: β-thalassemia/HbE presents with a variety of clinical symptoms, from asymptomatic to severe, requiring routine transfusion. However, there is currently no agreed classification system to stratify patients based on clinical severity of β-thalassemia/HbE in the Indonesian population. Thailand has already established a classification system, and this study aimed to identify the applicability of the Thailand clinical scoring criteria to determine the severity of β-thalassemia/HbE in the Indonesian population.Methods: This descriptive study was conducted by evaluating patients with β-thalassemia/HbE, who were classified into mild, moderate, or severe groups based on the Thailand clinical scoring criteria.Results: A total of 293 subjects with β-thalassemia/HbE were included. Based on this clinical scoring criteria, it was found that only 21.5% of patients were classified as mild, and the remaining 35.5% and 44% were classified as moderate and severe respectively. Approximately 68.2% of the subjects in the severe group received transfusion at <4 years old, while only 10% of those in the mild group were transfused at the same age. In the mild group, only 10% of the subjects underwent routine transfusion, compared to 98.4% of the subjects in the severe group. In addition, only 27% of the subjects in the mild group showed stunted growth, while that in the moderate and severe groups were 54.5% and 86.8%, respectively.Conclusion: Thailand clinical scoring criteria is able to determine the severity of Indonesia thalassemia patient which needs further management, i.e. transfusion and observation of stunted growth. This scoring system will help provide the provision of the most ideal management for the groups of patients based on their requirements.

Copyrights © 2018






Journal Info

Abbrev

MJI

Publisher

Subject

Medicine & Pharmacology

Description

This quarterly medical journal is an official scientific journal of the Faculty of Medicine Universitas Indonesia in collaboration with German-Indonesian Medical Association (DIGM) Indexed in: IMSEAR; CAB Abstracts; Global Health; HINARI; DOAJ; DRJI; Google Scholar; JournalTOCs; Ulrichsweb Global ...