Notes

W-TSS: A new Wavelet-Based Criteria for Discovering Occasion Sequence Shapelets.
To determine the Dermatology Life Quality Index (DLQI) among the subtypes of leprosy and to examine correlation with deformity and lepra reactions.

This was a cross-sectional study done at Dermatology Outpatient Clinic, Queen Elizabeth Hospital and two health clinics in Kota Kinabalu between 1st April 2019 and 30th November 2019. A standardised case report form was formulated to collect the demographic data and disease profile of the leprosy patients. The quality of life (QoL) was assessed using Dermatology Life Quality Index (DLQI) questionnaire.

A total of 54 patients were included with a male to female ratio of 2.41 (38 males and 16 females). The mean DLQI score was 8.31±6.15. The difference between the mean DLQI scores among the leprosy subtypes was not significant. The most affected domain was symptoms and feeling followed by daily activities and leisure. Twenty-one patients (38.9%) had facial deformity and they were found to have significantly higher DLQI score. WHO grade 1 and 2 disability were observed in 37 patients (68.5%) with higher DLQI score compared to those without any disability. More than half of patients with MB leprosy (52.2%) developed lepra reactions but the difference of mean DLQI scores were not significant.

Leprosy-related disabilities may predispose patients to develop psychosocial problems which may have negative impact on QoL. Thus, periodic assessment of QoL should be incorporated into the management of leprosy patients.
Leprosy-related disabilities may predispose patients to develop psychosocial problems which may have negative impact on QoL. Thus, periodic assessment of QoL should be incorporated into the management of leprosy patients.
Sarawak has a population that is geographically and characteristically widely varied. This study aimed to determine the demographic profile of patients in Sarawak, Malaysia. Materials and Methods - A cross-sectional study was conducted in 2019 at four major haemophilia treatment centres in Kuching, Sibu, Bintulu and Miri Hospitals, Sarawak. Demographic and clinical data were collected with consents from patients.

Ninety-six haemophilia patients were identified - 79(82.3%) haemophilia A(HA) and 17(17.7%) haemophilia B(HB). Severe haemophilia patients were noted in 45.6% (36/79) of HA and 64.7% (11/17) of HB. In all 44.3% of the HA and 52.9% of the HB population had no identifiable family history of haemophilia. Two-thirds of the patients with severe HA were on prophylaxis [24/36 (66.7%)] and only onethird [4/11 (36.4%)] in severe HB. Inhibitors developed in 9/79 (11.4%) of the HA population [3/79 (3.8%) high responders]. The median inhibitor titre was not significantly different between the different treatred responsibilities among all parties are of utmost importance to improve the care of our haemophilia population.
This study outlines the Sarawakian haemophilia landscape and offers objective standards for forward planning. Shared responsibilities among all parties are of utmost importance to improve the care of our haemophilia population.
The management of potential treatment-related complications and bleeding events in haemophilia is challenging in developing countries. Providing optimal care among these patients improve their quality of life (QOL) and life expectancy. This study explores the demographic characteristics and treatment outcome in a major haemophilia treatment centre in Malaysia.

A total of 260 patients were recruited in this retrospective cross-sectional analysis. Clinical data, including treatment regimens and outcome, were collected and analysed.

A total of 211 patients were diagnosed with haemophilia A (HA) (severe disease, 72.5%) and 49 patients had haemophilia B (HB) (severe disease, 65.3%). The median age was 31 (IQR;2-84) years. Majority of the patients had at least one episode of musculoskeletal bleeding since diagnosis. The mean annual bleeding event (ABE) was 4.91 (SD±6.07) in 2018. Target joints were identified in 80.4% of the patients. Chronic arthropathy and synovitis collectively accounted for more than halfia.Non-transfused β-thalassaemia patients develop complications related to unsuppressed ineffective erythropoiesis (IE). AMI1 Serum markers of IE would be useful for risk stratification and monitoring treatment. We studied β- thalassaemia trait (β-TT) and non-transfusion-dependent β- thalassaemia (β-NTDT) patients. Serum erythropoietin (EPO) and soluble transferrin receptor (sTfR) were correlated against markers of clinical severity (haemoglobin, LDH, retics, bilirubin, spleen size) and iron overload (ferritin, hepcidin, and MRI-T2* in NTDT patients). Eleven β-NTDT and nine β-TT subjects were studied. β- NTDT patients had significantly higher markers of haemolysis and iron overload. In β-NTDT, liver iron ranged from mild to severe, but no cardiac loading was seen. EPO and sTfR were higher in patients with β-NTDT than β-TT, and correlated significantly with each other (ρ=0.630, p=0.003). Both markers were negatively correlated with haemoglobin (sTfR ρ=-0.540, p=0.014; EPO ρ=-0.807, p less then 0.001, and positively correlated with spleen size (sTfR ρ=0.783, p less then 0.001; EPO ρ=0.654, p=0.002) and markers of iron overload. There was a strong correlation between ferritin and hepcidin (ρ=0.720, p less then 0.001), and a relatively lower increment of hepcidin for the degree of iron overload in β- NTDT compared to β-TT. EPO and sTfR appear to be reliable markers of erythropoiesis in non-transfused β-thalassaemia and correlate well with markers of disease severity. Their role in managing patients, predicting complications, and monitoring response to treatments aimed at reducing IE should be explored.
The aim of this study was to compare the postoperative refractive outcome after cataract surgery between a hospital-based and an outreach-based cataract service centre.

This study was conducted at the Hospital Selayang (HS), Selangor, Malaysia, a tertiary referral centre and an outreach-based cataract service centre (Pusat Pembedahan Katarak MAIWP, PPKM). Data was sourced from the Cataract Surgery Registry (CSR) in the National Eye Database (NED).

A total of 2318 surgeries were analysed. PPKM achieved postoperative refraction outcome within ±1.0D in 94.3% of cases compared with 88.4% in Selayang Hospital. Mean absolute prediction error was also better in PPKM (0.39±0.27D vs. 0.33±0.24D, p<0.001). Multivariate analysis showed that the tertiary hospital, persons of Chinese ethnicity, history of uveitis, previous history of ocular surgery and intraoperative complications as significant independent predictive factors for poor refractive outcomes.

The outreach-based cataract service centre, which incorporates streamlined process designs and workflows, achieved superior refractive outcomes within ±1 dioptre after cataract surgery compared to a tertiary hospital.
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