Maple Syrup Urine Disease Induced Grand Mal Seizures: A Case Report

Background Maple Syrup Urine Disease (MSUD) is a rare autosomal recessive metabolic error, characterized by Branched Chain α-Keto-acid Dehydrogenase Complex (BCKDC) deficiency. Mutations in 3 genes can lead to abnormal metabolism and accumulation of leucine, isoleucine, valine and corresponding keto-acids. MSUD affects 1 in 185,000 infants globally. Seizure is a common presentation among neonates. However, in intermediate MSUD, seizures have a delayed and insidious onset, along with developmental    Case Report We report a case of grand mal seizures in a patient with intermediate MSUD, presenting with multiple episodes of seizure, dystonia, spastic quadriplegia, involuntary micturition and oculogyric crisis.Seizures were managed successfully with intravenous lorazepam and other supportive measures. The patient was advised to strictly adhere to branched chain amino acid restricted diet. Conclusion This case report emphasizes on the importance of medication adherence and dietary restrictions to prevent permanent psychomotor damage or death

Health System Capacity and Access Barriers to Diagnosis and Treatment of CVD and Diabetes in Nepal

Background: Universal access to essential medicines and routine diagnostics is required to combat the growing burden of cardiovascular disease (CVD) and diabetes. Evaluating health systems and various access dimensions – availability, affordability, accessibility, acceptability, and quality – is crucial yet rarely performed, especially in low- and middle-income countries. Objective: To evaluate health system capacity and barriers in accessing diagnostics and essential medicines for CVD and diabetes in Nepal. Methods: We conducted a WHO/HAI nationally-representative survey in 45 health-facilities (public sector: 11; private sector: 34) in Nepal to collect availability and price data for 21 essential medicines for treating CVD and diabetes, during May–July 2017. Data for 13 routine diagnostics were obtained in 12 health facilities. Medicines were considered unaffordable if the lowest paid worker spends >1 day’s wage to purchase a monthly supply. To evaluate accessibility, we conducted facility exit interviews among 636 CVD patients. Accessibility (e.g., private-public health facility mix, travel to hospital/pharmacy) and acceptability (i.e. Nepal’s adoption of WHO Essential Medicine List, and patient medication adherence) were summarized using descriptive statistics, and we conducted a systematic review of relevant literature. We did not evaluate medicine quality. Results: We found that mean availability of generic medicines is low (<50%) in both public and private sectors, and less than one-third medicines met WHO’s availability target (80%). Mean (SD) availability of diagnostics was 73.1% (26.8%). Essential medicines appear locally unaffordable. On average, the lowest-paid worker would spend 1.03 (public sector) and 1.26 (private sector) days’ wages to purchase a monthly medicine supply. For a person undergoing CVD secondary-prevention interventions in the private sector, the associated expenditure would be 7.5–11.2% of monthly household income. Exit interviews suggest that a long/expensive commute to health facilities and poor medicine affordability constrain access. Conclusions: This study highlights critical gaps in Nepal’s health system capacity to offer basic health services to CVD and diabetes patients, owing to low availability and poor affordability and accessibility. Research and policy initiatives are needed to ensure uninterrupted supply of affordable essential medicines and diagnostics

Salt intake and salt‐reduction strategies in South Asia: From evidence to action

Abstract The World Health Organization recommends salt reduction as a cost‐effective intervention to prevent noncommunicable diseases. Salt‐reduction interventions are best tailored to the local context, taking into consideration the varying baseline salt‐intake levels, population's knowledge, attitude, and behaviors. Fundamental to reduction programs is the source of dietary salt‐intake. In South Asian countries, there is a paucity of such baseline evidence around factors that contribute to community salt intake. Upon reviewing the electronic literature databases and government websites through March 31, 2021, we summarized dietary salt intake levels and aimed to identify major sources of sodium in the diet. Information on the current salt reduction strategies in eight South Asian countries were summarized, namely Afghanistan, Bangladesh, Bhutan, India, Maldives, Nepal, Pakistan, and Sri Lanka. One hundred twelve publications (out of identified 640) met our inclusion‐exclusion criteria for full text review. Twenty‐one studies were included in the review. Quality of the included studies was assessed using the US National Heart, Lung, and Blood Institute assessment tool. The primary result revealed that mean salt intake of South Asian countries was approximately twice (10 g/day) compared to WHO recommended intake (< 5 g/day). The significant proportion of salt intake is derived from salt additions during cooking and/or discretionary use at table. In most South Asian countries, there is limited data on population sodium intake based on 24‐h urinary methods and sources of dietary salt in diet. While salt reduction initiatives have been proposed in these countries, they are yet to be fully implemented and evaluated. Proven salt reduction strategies in high‐income countries could possibly be replicated in South Asian countries; however, further community‐health promotion studies are necessary to test the effectiveness and scalability of those strategies in the local context

Aerosol climatology at Delhi in the western Indo-Gangetic Plain: Microphysics, long-term trends, and source strengths

We present the climatology of aerosol microphysics, its trends, and impact of potential sources based on the long term measurements (for a period of 11.5 years from December 2001 to May 2012) of aerosol optical depths (AOD) in the spectral range 340-1020 nm from an urban center Delhi (28.6 degrees N, 77.3 degrees E, 238m mean sea level) in the western Indo-Gangetic Plain (IGP). The study is the first ever long-term characterization of aerosols over the western IGP from the ground-based measurements. AODs are known to affect the air quality, visibility, radiative balance, and cloud microphysics of the region and IGP is one of the highest populated and polluted regions of the world. Our measurements show consistently high AOD during the entire period of observation. The seasonal variations of spectral AODs and Angstrom parameters are generally consistent every year. The AODs show a weak but statistically significant (in 95% confidence level) decreasing trend approximately -0.02/year at 500 nm, possibly, modulated by the pre-monsoon heavy dust loading during the first half of the observation period. The climatological monthly mean AOD at shorter wavelengths peaks twice, during June and November, while at longer wavelengths it shows only one peak in June. The annual variations of Angstrom exponent, a and its derivative, alpha' suggest the prevalence of multi-modal aerosol size distributions at Delhi. The coarse-mode aerosols dominate during summer (March-June) and monsoon (July-September) seasons, whereas fine/accumulation mode enhances during post-monsoon (October-November) and winter (December-February) seasons. Potential advection pathways have been identified using concentration weighted trajectory (CWT) analysis of the 5 day isentropic air mass back trajectories at the observation site and their seasonal variations are discussed