ABSTRACT
Gaucher's disease is a rare inborn error of metabolism with an autosomal recessive pattern of inheritance. With over 26 million births occurring per annum, extrapolation of this figure would give us an estimated burden of 17,000 babies born with lysosomal storage disorder (LSD). Given the large population of India and the high rates of consanguineous marriage that takes place in the subcontinent, LSD might not be as rare as we perceive it to be. We report a rare occurrence of type-1 Gaucher's disease in an adult female patient born of a non-consanguineous marriage, belonging to the tropical area of Chhattisgarh, India where there is a predominance of malaria, thalassemia, and sickling. The diagnosis was challenging in this case since we needed to work out all the differential diagnoses of pancytopenia with hepatomegaly and massive splenomegaly. The key part was her medical history where there was documentation of her elder brother's death due to some mental illness of undiagnosed etiology. Being a difficult time due to coronavirus disease 2019 ( COVID-19) , we were able to diagnose the patient with a bone marrow biopsy followed by glucocerebrosidase enzyme level suggestive of Gaucher's disease.
ABSTRACT
Itolizumab, an anti-CD6 monoclonal antibody, has been recently approved for the off-label indication of cytokine release syndrome in the background of COVID-19, by the Drug Controller General of India. However, this drug has not been included in the National Clinical Management Protocol for COVID-19 yet. The limited-to-no experience of the Indian health workforce with the drug urged us to conduct a situational analysis in the pre-COVID era to analyse the degree of use of the drug and the indications for which it has been employed.
ABSTRACT
With many drugs being tried in the management and treatment of COVID-19, dupilumab is one such monoclonal antibody that has come under the limelight for its possible role as an adjunct therapy in COVID-19 position. There are isolated case reports and series that document a milder course of COVID-19 infection in patients who have already been on dupilumab therapy for treatment of conditions such as atopic dermatitis and chronic rhino-sinusitis with nasal polyp. There is also an ongoing debate regarding the continuation of biologicals in the COVID patient. In this article, a non-systematic critical analysis of dupilumab was performed to delve into this hypothesis further.
ABSTRACT
With no drugs currently approved for treatment and cure of COVID-19 (coronavirus disease 2019), hydroxychloroquine is one of the many first-line drugs used in the management. However, given the life-threatening adverse effects of HCQ that have been reported, its use as a prophylactic treatment remains debated. HCQ has long been used in India for the treatment of malaria, auto-immune and inflammatory diseases, and even type 2 diabetes mellitus recently. We aimed to review existing literature and relevant Web sites regarding the safety profile of HCQ in the Indian subcontinent. A non-systematic critical analysis of all published literature/studies focused on the Indian population, recording on the use of HCQ for various indications up till April 2020 was done and frequency of occurrence of HCQ related life-threatening and cardiac side effects were noted. Results from PubMed database showed an incidence of 0.6% of cardiac-related side effects and 7.42% of other self-limiting and minor side effects among the Indian population on HCQ. Considering its minimal risk and favorable safety profile, cost-effectiveness, availability, and affordability in India, the use of hydroxychloroquine in the fight against COVID-19 appears rationale. Following the results of our study, we hypothesize that Indians might be less likely to suffer from cardiac-related side effects given their genetic make-up. However, this would need further studies, clinical trials, and a pharmacogenomic understanding of the subject.