Demographics and outcome of patients with congenital haemophilia in Sarawak, Malaysia.

INTRODUCTION: 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. RESULTS AND DISCUSSION: 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 treatment groups - on demand versus prophylaxis (1.0BU versus 2.0BU; z statistic -1.043, p-value 0.297, Mann-Whitney test). None of the patients developed inhibitory alloantibodies to factor IX. Four HA patients (5.1%) underwent immune tolerance induction where one case had a successful outcome. Three severe HA patients received emicizumab prophylaxis and showed remarkable reduction in bleeding events with no thromboembolic events being reported. One female moderate HA patient received PEGylated recombinant anti-haemophilic factor. Eleven patients underwent radiosynovectomy. One mild HB patient succumbed to traumatic intracranial bleeding. Our data reported a prevalence (per 100,000 males) of 5.40 cases for all severities of HA, 2.46 cases for severe HA; 1.16 cases for all severities of HB, and 0.75 cases for severe HB. The overall incidence of HA and HB was 1 in 11,500 and 1 in 46,000, respectively. CONCLUSION: 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.

Bonding temperature optimization and property evolution of SU-8 material in metal/adhesive hybrid wafer bonding.

Bonding temperature optimization of SU-8 material for metal/adhesive hybrid bonding was investigated. The good bond quality of SU-8 adhesive can be achieved with the bonding temperature between 150 degrees C and 250 degrees C, while bond failures of SU-8 wafers are observed starting from 275 degrees C. IR transmittance spectra measurements indicate the crosslinks inside SU-8 break and further bond failure is observed due to the large decomposition of epoxy rings and phenyl in plane bending above 275 degrees C. This research provides guidelines of material selection and bonding parameters for heterogeneous integration, 3DIC and MEMS applications using metal/adhesive hybrid bonding.

The effect of pregnancy on the response to blood loss in a rat model.

STUDY OBJECTIVES: A commonly held belief is that the blunted hemodynamic response to hemorrhage observed in pregnant women is secondary to expanded blood volume. In addition to increased blood volume, pregnancy is also a vasodilated state. Vasodilatation may have deleterious effects on the response to hemorrhage by inhibiting central blood shunting after blood loss. How these conflicting variables of increased blood volume and vasodilatation integrate into a whole body model of maternal hemorrhagic shock has yet to be studied in a controlled experiment. We tested the null hypothesis that there would be no difference in the hemodynamic and metabolic responses to hemorrhage between pregnant (PRG) and non-pregnant (NPRG) rats. METHODS: Twenty-four adult female Sprague-Dawley rats (12 PRG and 12 NPRG) were anesthetized with Althesin via the intraperitoneal route. Femoral arteries were cannulated by cut-down. Twelve (six PRG and six NPRG) rats underwent controlled catheter hemorrhage of 25% of their total blood volume. Twelve rats (six PRG and six NPRG) served as non-hemorrhage controls. Mean arterial pressure (MAP) and base excess (BE) were measured pre-hemorrhage and then every 15 min post-hemorrhage for the next 90 min. Data were reported as mean+/-standard error of the mean (S.E.M.) over the 90-min post-hemorrhage observation period. Group comparisons were analyzed by ANOVA with repeated values post-hoc by Bonferroni. Statistical significance was defined by an alpha=0.05. RESULTS: PRG and NPRG rats were evenly matched for MAP (P=0.788) and BE (P=0.146) pre-hemorrhage. Post-hemorrhage there were no mortalities in either group. Post-hemorrhage both the PRG and NPRG groups experienced significant (P=0.011) drops in systolic and diastolic blood pressures as compared to their non-hemorrhage controls. Post-hemorrhage there was no significant (P=0.43) difference in MAP between the PRG (89+/-2 mmHg) and NPRG (80+/-2 mmHg) rats. BE also dropped significantly within both PRG (P=0.004) and NPRG (P=0.001) groups post-hemorrhage. No significant (P=0.672) difference was noted in BE between PRG and NPRG groups post-hemorrhage -6.1+/-0.3 mEq/l and -6.9+/-0.4 mEq/l, respectively. CONCLUSION: After a controlled hemorrhage of 25% of total blood volume we found no significant differences in MAP and BE between pregnant and non-pregnant rats. Pregnancy does not affect the response to hemorrhage.

Transcatheter embolization of a coronary arteriovenous fistula with a complex, helical-fibered platinum coil.

Transcatheter embolization was successfully performed on a 51-year-old man with a coronary arteriovenous fistula arising from the left circumflex coronary artery and draining into the superior vena cava. The patient presented with typical manifestations of angina pectoris that were first noticed during childhood but had worsened over the past 5 years. A 2 x 20 mm complex, helical-fibered platinum coil was placed in the mid-portion of the fistula and successfully occluded the fistula within 30 minutes. The patient has been free from angina pectoris since the procedure.