INTRODUCTION: Haemoglobinopathy testing is performed for carrier screening and evaluation of microcytic anaemia. We evaluated the effectiveness of thalassaemia screening tests at our institution and suggest ways of improving the testing algorithm. MATERIALS AND METHODS: A total of 10,084 non-antenatal and 11,364 antenatal samples with alkaline gel electrophoresis (AGE), capillary electrophoresis (CE), haemoglobin H (HbH) inclusion test, mean corpuscular haemoglobin (MCH) and mean corpuscular volume (MCV) were retrospectively reviewed. A subgroup of 187 samples with genetic testing was correlated with HbH inclusions and MCH/ MCV. The effect of iron deficiency on percentage hemoglobin A2 (HbA2) was studied. RESULTS: HbH inclusion test showed low sensitivity of 21.43% for α-thalassaemia mutations but higher sensitivity of 78.95% for deletion. By receiver operating characteristic (ROC) analysis, MCH ≤28 pg or MCV ≤80 fl for non-antenatal samples and MCH ≤27 pg or MCV ≤81 fl for antenatal samples had >98% sensitivity for HbH inclusions. Above these thresholds, the probability that HbH inclusions would be absent was <99% (negative predictive value [NPV] >99%). MCH ≥28 pg had 100% sensitivity (95% CI 95.63%-100%) for α-thalassaemia mutations and 97.68% calculated NPV in the antenatal population. Detection of haemoglobin variants by CE correlated highly with AGE (99.89% sensitivity, 100% specificity). Severe iron deficiency reduced HbA2 in hemoglobin ( <0.001) and α-thalassaemia ( = 0.0035), but not in β-thalassaemia. CONCLUSION MCH/MCV thresholds have adequate sensitivity for α-thalassaemia in the antenatal population, and genotyping plays an important role as HbH inclusion test shows low sensitivity. CE without AGE, may be used as initial screening for haemoglobin variants. Our study provides contemporary data to guide thalassaemia screening algorithms in Singapore.
Blood Protein Electrophoresis ; Electrophoresis, Capillary ; Erythrocyte Inclusions ; pathology ; Erythrocyte Indices ; Female ; Genetic Testing ; Hemoglobin H ; analysis ; Humans ; Male ; Mass Screening ; Pregnancy ; Pregnancy Complications, Hematologic ; blood ; diagnosis ; Retrospective Studies ; Sensitivity and Specificity ; Singapore ; alpha-Thalassemia ; blood ; diagnosis

INTRODUCTION: To describe the maternal and fetal outcomes in systemic lupus erythematosus (SLE) pregnancies followed-up in a single tertiary referral centre. METHODS: We performed a retrospective cohort study of 75 SLE pregnancies who were followed up in Singapore General Hospital over a 16-year period from 2000 to 2016. Adverse fetal and maternal outcomes including preterm delivery, miscarriages, fetal growth restriction, congenital heart block, neonatal lupus, pre-eclampsia and SLE flares were obtained from the medical records. RESULTS: The mean age at conception was 32 years old (SD 3.8). The mean SLE disease duration was 5.9 years (SD 5.2). The majority (88%) had quiescent SLE disease activity at baseline. Most pregnancies resulted in a live birth (74.7%). The mean gestational age at birth was 37.4 weeks (SD 3.4). Adverse fetal outcomes occurred in 53.3%. Preterm delivery (33.9%), miscarriages (20%) and fetal growth restriction (17.3%) were the most frequent adverse fetal outcomes. There was 1 neonatal death and SLE flares occurred in a third (33%). In the subgroup of SLE pregnancies with antiphospholipid syndrome, there were higher SLE flare rates (40%) and adverse fetal outcomes occurred in 8 pregnancies (80%). There were no predictive factors identified for all adverse fetal and maternal outcomes. In the subgroup analysis of preterm delivery, anti-Ro (SS-A) antibody positivity and hydroxychloroquine treatment were associated with a lower risk of preterm delivery. CONCLUSION Although the majority had quiescent SLE disease activity at baseline, SLE pregnancies were associated with high rates of adverse fetal and maternal outcomes.

Introduction: Multitude studies have shown perception is an integral factor associated with smoking, However, no such tool was available in Malay language. In this study, we established a Bahasa Malaysia version of PTSQ (BM-PTSQ) and tested the validity and reliability among secondary school adolescents. Methods: The English version of PTSQ originally consists of 12 items. It was translated into Bahasa Malaysia and back-translated again into English to check for consistency. After face validity (face-to-face query) was determined among 20 secondary school adolescents, only 10 items were included in the survey. Construct validity was established from 407 school adolescents through random selection in the same locality. More than 60% of the respondents were female, a majority (67.3%) were schooling in rural areas. Then, the reliability of the questionnaire was determined with Cronbach's alpha. Results: EFA has grouped PTSQ into two components, they are associated with either knowledge or attitude towards smoking. The variance and Cronbach's alpha for the first and second component were 38.24% and 0.861 (7 items) and 21.62% and 0.661 (3 items) respectively. Conclusion The PTSQ showed good validity and reliability for measurement of perception in smoking among school adolescents in Malaysia, thus this is a viable measurement tool. More importantly this study shows an urgent need to improve the smoking education among adolescents in Malaysia.