METHODS: The searched articles were selected based on the available keywords in the title and abstract with the publication restricted between January 2010 and September 2020. Articles generated from the databases must fulfill both inclusion and exclusion criteria in the present systematic review. Our initial search retrieved 221 literature reviews. After excluding articles with irrelevant topics, a total of 23 articles were chosen for this current review.
RESULTS: The factors were classified based on three main factors: patient-related, medication-related, and healthcare workers-related factors. For patient-related factors, gender, age, number of family members, education level, post-hospitalization, comorbidities and cardiovascular disease risk, follow-up status, occupation, socio-economy, insurance, perception, ethnicity, and health plan were among the factors affecting adherence to LLDs. As for medication-related factors, timing, polypharmacy, duration of treatment, generic medication, intensity of medication, side effects, initiating dose, packaging, drug dosing, and type of drugs were revealed as contributing factors. In the light of healthcare workers, related factors shown were counseling, medication optimization, type of provider, and location of the hospital.
CONCLUSIONS: Recommendations to improve adherence include educating patients on the disease itself and the importance of the treatment, modification of the dosing, timing and type of LLDs, and effective consultations by healthcare workers. Further studies need to be done in Malaysia as there is inadequate research on this topic.
METHODS: A cross-sectional study was conducted using data collected from secondary school students in Kelantan from 2017 to 2018 who participated in a thalassemia screening program conducted by the Ministry of Health, Malaysia. Hb analysis was performed using an automated CE system (CAPILLARYS 2 Flex-Piercing System Sebia) and HPLC (VARIANT II, Bio-rad Laboratories). DNA analysis was used multiplex polymerase chain reaction and multiplex amplification refractory mutation system to detect deletion and non-deletion α-thalassemia.
RESULTS: Termination codon CS mutation was confirmed among 376 (99.5%) samples with a peak value in zone 2 of CE. Heterozygous Hb CS was the most common type, detected in 344 samples (91.5%), followed by compound heterozygous Hb CS in 31 samples (8.2%) and one sample (0.3%) of homozygous Hb CS.
CONCLUSIONS: The diagnosis of Hb CS is most accurately achieved by combining CE and HPLC methods, with confirmation by DNA molecular study, although the latter is more expensive.