Haemophilia care in Asia: Learning from clinical practice in some Asian countries.

BACKGROUND: The healthcare systems in Asia vary greatly due to the socio-economic and cultural diversities which impact haemophilia management. METHODS: An advisory board meeting was conducted with experts in haemophilia care from Asia to understand the heterogeneity in clinical practices and care provision in the region. FINDINGS: The overall prevalence of haemophilia in Asia ranges between 3 and 8.58/100,000 patients. Haemophilia A was more prevalent as compared to haemophilia B with a ratio of around 5:1. There is under-diagnosis in the region due to lack of diagnosis, registries and/or lack of appropriate facilities in suburban areas. Most patients are referred to the haematologists by their families or primary care physicians, while some are identified during bleeding episodes. Genetic testing faces obstacles like resource constraints, services available at limited centres and unwillingness of patients to participate. Prophylaxis is offered for people with haemophilia (PWH) with a severe bleeding phenotype. Recombinant factors are approved in most countries across the region and are the preferred therapy. The challenges highlighted for not receiving a high standard of care include patients' reluctance to use an intravenous treatment, poor patient compliance due to frequency of infusions, budget constraints and lack of funding, insurance, availability and accessibility of factor concentrates. Prevalence of neutralizing antibodies ranged from 5% to 20% in the region. Use of immune tolerance induction and bypassing agents to treat inhibitors depends on their cost and availability. CONCLUSION: Haemophilia care in Asia has evolved to a great extent. However, some challenges remain for which a strategic approach along with multi-stakeholder involvement are needed.

The Effect of Randomized Beta-Carotene Supplementation on CKD in Men.

Introduction: Beta-carotene (BC) protects the body against free radicals that may damage the kidney and lead to the development of acute kidney injury and chronic kidney disease (CKD). Previous studies in animal models have demonstrated a potential protective effect of 30 mg/kg BC supplementation on renal ischemia or reperfusion injury and subsequently improved kidney function. The extension of these findings to humans, however, remains unclear. Methods: Our study leverages previously collected data from the Physicians' Health Study I (PHS I), a large-scale, long-term, randomized trial of middle-aged and older US male physicians testing 50 mg BC every other day for primary prevention of cardiovascular disease and cancer. We examined the impact of randomized BC supplementation on self-reported incident CKD identified by self-reports stating "yes" to kidney disease from annual follow-up questionnaires from randomization in 1982 through the end of the randomized BC intervention at the end of 1995, and on CKD defined as an estimated glomerular filtration rate (eGFR) < 60 ml/min per 1.73 m2 at the end of 1995. Analyses compared incident CKD between BC supplementation and placebo using Cox proportional hazards regression models and logistic regression. We also examined whether smoking status (current vs. former or never smoker) or other factors modified the effect of randomized BC supplementation on CKD. Results: A total of 10,966 participants were randomized to BC, and 10,952 participants were randomized to a placebo group. Baseline characteristics between randomized BC groups were similar. There was no significant benefit between BC supplementation and self-reported incident CKD after adjusting for age and randomized aspirin treatment (hazard ratio [HR] = 0.97, 95% confidence interval [CI]: 0.86-1.08, P-value = 0.56). Stratified by smoking status, there was no significant benefit of BC supplementation and self-reported incident CKD either among former or never smokers (HR = 0.95, 95% CI: 0.84-1.07, P-value = 0.41) or current smokers (HR = 1.08, 95% CI: 0.78-1.50, P-value = 0.64). Smoking status did not modify the association between BC supplementation and incident CKD (P-interaction = 0.47). In subgroup analysis among those with available serum creatinine at the study end (5480 with BC and 5496 with placebo), there was no significant benefit between BC supplementation and CKD based on eGFR < 60 ml/min per 1.73 m2 (odds ratio [OR] = 0.96, 95% CI: 0.85-1.08, P-value = 0.49). Conclusion: Long-term randomized BC supplementation did not affect the risk of incident CKD in middle-aged and older male physicians.

Sex differences on outcomes of catheter ablation of ventricular tachycardia in patients with structural heart disease: A real-world systematic review and meta-analysis.

Background: Sex differences have diversely affected cardiac diseases. Little is known whether these differences impact outcomes of catheter ablation of ventricular tachycardia (VT). Objectives: To assess the impact of sex differences on outcomes of catheter ablation of VT. Methods: Databases were searched from inception through December 2021. Effect estimates from individual studies were extracted and combined using the random-effects, generic inverse variance method of DerSimonian and Laird. The outcomes of interest included VT recurrence rates, all-cause mortality, and composite outcomes of mortality, left ventricular assistant device use, and heart transplantation following VT ablation. Results: Our analysis included 22 observational studies. There were 10,206 patients, of which 12.8% were women. We found no statistical difference between sexes for VT recurrence rate (pooled hazard ratio [HR] 1.04, P = .57, I 2 = 14.9%). Similarly, there was statistical difference in neither all-cause mortality nor composite outcomes (pooled HR 0.93, P = .75, I 2 = 59.1% and pooled HR 0.9, P = .33, I 2 = 0%, respectively). There was a trend toward an increase in women undergoing VT ablation in the recent registries (P = .071). Conclusion: Our contemporary analysis suggests that sex may have no impact on clinical outcomes of catheter ablation of VT in patients with structural heart disease, though women are the underrepresented. However, recent VT ablation registries have involved more women in their studies. Future studies with a higher proportion of women are encouraged to verify the current perception.

The effect of levocarnitine supplementation on dialysis-related hypotension: A systematic review, meta-analysis, and trial sequential analysis.

BACKGROUND: Dialysis patients have been shown to have low serum carnitine due to poor nutrition, deprivation of endogenous synthesis from kidneys, and removal by hemodialysis. Carnitine deficiency leads to impaired cardiac function and dialysis-related hypotension which are associated with increased mortality. Supplementing with levocarnitine among hemodialysis patients may diminish incidence of intradialytic hypotension. Data on this topic, however, lacks consensus. METHODS: We conducted electronic searches in PubMed, Embase and Cochrane Central Register of Controlled Trials from January 1960 to 19th November 2021 to identify randomized controlled studies (RCTs), which examined the effects of oral or intravenous levocarnitine (L-carnitine) on dialysis-related hypotension among hemodialysis patients. The secondary outcome was muscle cramps. Study results were pooled and analyzed utilizing the random-effects model. Trial sequential analysis (TSA) was performed to assess the strength of current evidence. RESULTS: Eight trials with 224 participants were included in our meta-analysis. Compared to control group, L-carnitine reduced the incidence of dialysis-related hypotension among hemodialysis patients (pooled OR = 0.26, 95% CI [0.10-0.72], p = 0.01, I2 = 76.0%). TSA demonstrated that the evidence was sufficient to conclude the finding. Five studies with 147 participants showed a reduction in the incidence of muscle cramps with L-carnitine group (pooled OR = 0.22, 95% CI [0.06-0.81], p = 0.02, I2 = 74.7%). However, TSA suggested that further high-quality studies were required. Subgroup analysis on the route of supplementation revealed that only oral but not intravenous L-carnitine significantly reduced dialysis-related hypotension. Regarding dose and duration of L-carnitine supplementation, the dose > 4,200 mg/week and duration of at least 12 weeks appeared to prevent dialysis-related hypotension. CONCLUSION: Supplementing oral L-carnitine for at least three months above 4,200 mg/week helps prevent dialysis-related hypotension. L-carnitine supplementation may ameliorate muscle cramps. Further well-powered studies are required to conclude this benefit.

The effects of omega-3 fatty acids on diabetic nephropathy: A meta-analysis of randomized controlled trials.

OBJECTIVE: To evaluate the effects of omega-3 long-chain polyunsaturated fatty acids on proteinuria, estimated glomerular filtration rate (eGFR) and metabolic biomarkers among patients with diabetes. STUDY DESIGN: Meta-analysis of randomized controlled clinical trials (RCTs). SETTING & SUBJECTS: Patients with diabetes. SELECTION CRITERIA FOR STUDIES: We conducted electronic searches in PubMed, Embase and Cochrane Central Register of Controlled Trials from January 1960 to April 2019 to identify RCTs, which examined the effects of omega-3 fatty acids on proteinuria, eGFR and metabolic biomarkers among diabetic patients. RESULTS: Ten RCTs with 344 participants were included in our meta-analysis. Omega-3 fatty acids reduced the amount of proteinuria among type 2 diabetes mellitus (type 2 DM) and type 1 diabetes mellitus (type 1 DM). This association was only significant among type 2 DM (SMD = -0.29 (95% CI: -0.54, -0.03; p = 0.03). Only studies with duration of intervention of 24 weeks or longer demonstrated a significant lower proteinuria among omega-3 fatty acids compared to control group (SMD = -0.30 (95% CI: -0.58, -0.02; p = 0.04). There was a higher eGFR for both type 1 and type 2 DM groups among omega-3 fatty acids compared to control group, however, the effect was not statistically significant. Regarding serum total cholesterol, LDL-cholesterol and HbA1C, there was no significant difference comparing omega-3 fatty acids to control group. There was a non-significant systolic blood pressure reduction in the omega-3 fatty acids supplementation group compared to control. CONCLUSION: Omega-3 fatty acids could help ameliorate proteinuria among type 2 DM who received omega-3 supplementation for at least 24 weeks without adverse effects on HbA1C, total serum cholesterol and LDL-cholesterol.

Sleep Quality of Hospitalized Patients, Contributing Factors, and Prevalence of Associated Disorders.

BACKGROUND: Data in the literature has shown poor sleep quality to be frequently observed in hospitalized patients and known to be associated with poor treatment outcome. Many factors may impact poor sleep quality, and there is currently limited available data. We aim to determine the prevalence of poor sleep quality and associated factors in patients admitted to internal medicine wards as well as the change of sleep quality over time after admission. METHODS: An analytic observational study was conducted at the internal medicine wards at the King Chulalongkorn Memorial Hospital, Bangkok, Thailand. Patients were personally interviewed to evaluate the history of sleep quality at home, sleep quality after the first and the third days of admission, and potential associated factors. The Pittsburgh Sleep Quality Index and screening questionnaires for the common diseases associated with poor sleep quality were also utilized. The logistic regression analysis was used to determine the independent factors which led to poor sleep quality. RESULTS: Data were collected from 96 patients during the period of June 2015 to February 2016. The mean age of the patients was 50.8 ± 16.7 years, and 51% were male. Infectious disease was the most common principal diagnosis accounted for 29.2%. The results show high prevalence of poor sleep quality after the first night of admission compared to baseline sleep quality at home (50% vs. 18.8%; p < 0.001). After 3 days of admission, the prevalence of poor sleep quality was reduced to the level close to baseline sleep quality at home (28.1% vs. 18.8%; p = 0.13). Multivariate analysis demonstrated that light exposure and pain were the main independent factors for poor sleep quality on the first day (odds ratio 6.68; 95% CI 2.25-19.84) and on the third day (odds ratio 3.47; 95% CI 1.24-9.71), respectively. CONCLUSIONS: This is the first study conducted on the sleep quality of hospitalized patients that included the follow-up period during hospital admission. Our study demonstrated high prevalence of poor sleep quality in hospitalized patients on the first day. Interestingly, the sleep quality was partly improved during hospitalization. Light exposure and pain were demonstrated to be the factors associated with poor sleep quality.