Socio-Economic Implications of COVID-19 Pandemic in South Asia: Emerging Risks and Growing Challenges.

The dramatic spread of COVID-19 has threatened human lives, disrupted livelihoods, and affected trade, economy and businesses across the globe. The global economy has begun to show major disruptions and is heading toward a severe recession with an unprecedented economic crisis. As the global economy is highly integrated and interdependent through the global supply chains, it has been profoundly affected by the COVID-19 pandemic. Although all countries have faced difficulties due to Covid-19, South Asian countries in particular have had to deal with a more challenging situation due to their large population, weak health facilities, high poverty rates, low socio-economic conditions, poor social protection systems, limited access to water and sanitation, and inadequate living space, necessary to maintain physical distancing and take other required measures to contain this pandemic. To contain the spread of the virus, South Asian countries have imposed stringent lockdowns, which have consequently affected the lives and livelihoods of millions of people in the region, where a third of world's poor live. Against this backdrop, this paper examines the existing and prospective impacts, risks and challenges of Covid-19 on key social and economic sectors including migration, tourism, informal sector, agriculture and rural livelihoods. The analysis revealed that COVID-19 is likely to affect economic growth, increase fiscal deficit and monetary burden, increase the risks of macroeconomic instability, decrease migration and remittance, reduce income from travel and tourism, and result in dwindling micro-small and medium industries and informal businesses. This is likely to deepen poverty and increase unemployment and the risks of hunger and food insecurity. If not addressed properly, this may reinforce existing inequalities, break social harmony, and increase tension and turbulence. The economic and social costs of the COVID-19 outbreak are therefore likely to be significant and long-lasting in South Asia.

Successful Treatment of Cardiomyopathy due to Very Long-Chain Acyl-CoA Dehydrogenase Deficiency: First Case Report from Oman with Literature Review.

Very long-chain acyl-CoA dehydrogenase deficiency (MIM 201475) is a severe defect of mitochondrial energy production from oxidation of very long-chain fatty acids. This inherited metabolic disorder often presents in early neonatal period with episodes of symptomatic hypoglycemia usually responding well to intravenous glucose infusion. These babies are often discharged without establishment of diagnosis but return by 2-5 months of age with severe and progressive cardiac failure due to hypertrophic cardiomyopathy with or without hepatic failure and steatosis. An early diagnosis and treatment with high concentration medium chain triglycerides based feeding formula can be life saving in such patients. Here, we report the first diagnosed and treated case of Very long-chain acyl-CoA dehydrogenase deficiency in Oman. This infant developed heart failure with left ventricular dilation, hypertrophy and pericardial effusion at the age of 7 weeks. Prompt diagnosis and subsequent intervention with medium chain triglycerides-based formula resulted in a reversal of severe clinical symptoms with significant improvement of cardiac status. This treatment also ensured normal growth and neurodevelopment. It is stressed that the disease must be recognized by the pediatricians and cardiologists since the disease can be identified by Tandem Mass Spectrometry; therefore, it should be considered to be included in expanded newborn screening program, allowing early diagnosis and intervention in order to ensure better outcome and prevent complications.

Detection of Inborn Errors of Metabolism using Tandem Mass Spectrometry among High-risk Omani Patients.

OBJECTIVE: This is a report on the types and patterns of inborn errors of metabolism (IEMs) of amino acids, organic acids and fatty acids oxidation detected by Tandem Mass Spectrometry for a period of 10 years (1998-2008) at Sultan Qaboos University Hospital (SQUH), the major centre for diagnosis and management of IEM in Oman. METHODS: Tandem mass spectrometry (MS/MS) was used in the initial screening and diagnosis of IEMs in high risk neonatal and pediatric populations. RESULTS: Out of 1100 patients investigated, 119 were detected positive for IEM by MS/MS spectrometry. Twenty six different metabolic diseases were detected. Patients were categorized into three major groups: a) 54 with amino acids and urea cycle disorders, b) 35 with organic acid disorders, and c) 30 with fatty acid oxidation disorders. The commonest conditions encountered were maple syrup urine disease (MSUD), phenylketonuria (PKU), propionic and isovaleric acidurias, as well as HMG-CoA lyase deficiency and glutaric aciduria type II (GA-II). Most of these IEMs were over-represented in babies born to consanguineous parents, which is consistent with the recessive autosomal inheritance. CONCLUSION: This study shows that various types of IEMs, reported elsewhere, were also prevalent in Oman, but the pattern of prevalence and distribution is different. The situation, therefore, warrants the development of a nationwide screening and prevention program.

Spectrum of paediatric lysosomal storage disorders in oman.

OBJECTIVES: The aim of this study was to look at the spectrum of paediatric lysosomal disorders in Oman. Lysosomal storage disorders (LSDs) are a heterogeneous group of inherited metabolic diseases. Few studies on the birth prevalence and prevalence of LSDs have been reported from the Arabian Peninsula. METHODS: We studied 86 children with LSDs diagnosed over a period of nine years, from June 1998 to May 2007. Detailed clinical data, including age of onset, sex, age and mode of first presentation, and presence of consanguinity were collected. RESULTS: Our data showed the combined birth prevalence for all LSDs in Oman to be around 1 in 4,700 live births. Sphingolipidoses was the most common group of disorder encountered (47.7%), followed by neuronal ceroid lipofuscinoses (NCL) (23.2%) and mucopolysaccharidoses (MPS) (23.2%). The proportion of consanguineous marriages in our series was found to be 87.5%. CONCLUSION: Our data represent the birth prevalence and clinical spectrum of such disorders in Oman, one of the highly consanguineous societies in the Middle East.

Clinical characteristics of neonates with inborn errors of metabolism detected by Tandem MS analysis in Oman.

We reviewed the clinical profile of our neonates diagnosed to have inborn errors of metabolism (IEM) by Tandem Mass Spectrometry (TMS) over a seven years period, and compared the results with published reports. We also attempted to evaluate various clinical situations wherein the screening test would yield a high pick up rate. Among the 166 neonates studied (10 aged 1 day, 79 aged 2-7 days and 77 aged 8-28 days), significant abnormalities on TMS suggestive of IEM were detected in 38 babies (23%), most common diseases diagnosed were maple syrup urine disease (10 neonates), propionic acidemia (8 neonates), urea cycle diseases (6 neonates) and isovaleric acidemia (4 neonates). The detection incidence was calculated to be one positive case out of every 4 to 5 babies tested. A high prevalence of parental consanguinity and high level of positive family history of affected siblings were the highlights of this study. The major clinical situations where testing was helpful were (a) unexplained acute neonatal encephalopathy, (b) positive family history of known or suspected IEM and (c) new born presenting with abnormal serum biochemistry suggestive of IEM.

Heterogeneity for congenital generalized lipodystrophy in seventeen patients from Oman.

Seventeen children with congenital generalized lipodystrophy or Berardinelli-Seip Congenital Lipodystrophy (BSCL) from 12 consanguineous sibships were observed in Oman. All children had widespread absence of adipose tissue from infancy together with apparent muscle hypertrophy and hepatomegaly. They did not appear to represent a single homogenous entity, and it was possible to subclassify the cases into two distinct groups. In the first group of seven cases, the features were similar to other published cases with acanthosis nigricans, raised insulin levels, and insulin resistance. In this group, there was an association between the degree of acanthosis nigricans and the severity of the disorder. Molecular analysis of these cases showed homozygosity at the BSCL2 locus on chromosome 11q13 in four of the seven cases. In the second group of ten cases, there were striking abnormalities in both skeletal and nonskeletal muscle. Reduced exercise tolerance and percussion myoxedema were observed in skeletal muscle, while infantile hypertrophic pyloric stenosis, prominent veins (phlebomegaly), disturbance of cardiac rhythm, and cardiomyopathy were observed in nonskeletal muscle. There was evidence against homozygosity in some cases for the known loci for BSCL, and this group may represent a new clinical syndrome with lipodystrophy at a different genetic location.