Clinico-Hematological Profile and Management of Children With Non-Transfusion Dependent Thalassemia (NTDT) at a Pediatric Center in Northern India

Objective: To study the clinico-hematological profile, complications, and management of children with non-transfusion dependent thalassemia (NTDT) in northern India. Method: We retrieved and analyzed the data of 69 children with NTDT diagnosed between January, 2006 to December, 2018, aged under 18 years from our unit’s records. Result: The participants mean (SD) age was 4.4 (3.1) years, and they presented with anemia (29%), jaundice (13%), hemolytic facies (13%), splenomegaly (87%), thromboembolism (2.9%) and pathological short stature (28.5%). The most common cause of NTDT was ?-thalassemia (45%), followed by either compound-heterozygous or homozygous for E?-thalassemia mutation. The most frequent single genotype observed was compound heterozygous for IVS1-5 (G>C) and codon 26 (G>A). The mean (SD) follow-up duration was 3.5 (2.4) years. On follow-up, 27 children (%) remained transfusion free, and 30 (%) needed occasional transfusions. 63% of patients initially presenting with pathological short stature showed improvement in growth. Amongst children older than 10 years (n=20), subclinical hypothyroidism was detected in 6 children and impaired glucose tolerance test in 1 child. Conclusion: Eß-thalassemia was the commonest cause of NTDT in this population.

Anti-obesity and anticancer activity of Solanum xanthocarpum leaf extract: An in vitro study

Background: Solanum xanthocarpum grows in parts of India as a wild herb. The active principles of this plant are Solasodine, Carpesterol, ?-Sitosterol, and Diosgenin. Pharmacological effects such as hypoglycemic, hepatoprotective and hypotensive activity of S. xanthocarpum have been reported. Solasodine, an active component of this plant is reported to have antioxidant activity. Aims and Objectives: The objective of this study is to evaluate the anticancer and anti-obesity property of S. xanthocarpum. Materials and Methods: This study was carried out in Department of Pharmacology, Government Kilpauk Medical College, with laboratory support from Life Teck Research Center, Chennai. Both dry and fresh leaves of S. xanthocarpum were taken and evaluated for anticancer property using MCF cell line and anti-obesity activity using Pancreatic Lipase inhibition activity. Results: There was significant decrease in cell viability with increase in concentration of both dry and fresh leaves which shows anticancer activity. With increase in concentrations of leaf extracts, the inhibition of pancreatic lipase was found but in comparison to Orlistat the standard treatment, the effect was very less. Conclusion: Based on above results, it is concluded that S. xanthocarpum has good anticancer and minimal anti-obesity activity. Further investigations are required to identify the actual phytoactive component.

Correlation of Transient Elastography With MRI T2* and Serum Ferritin Levels in Children With Transfusion-Dependent Thalassemia

Objectives: We investigated the correlation of transient elastography (TE) with MRI R2* values and serum ferritin in patients with transfusion-dependent thalassemia (TDT) Methods: We reviewed hospital records of 59 patients with TDT aged ?8 years without any evidence of chronic liver disease and who had fibroscan within 3 months of MRI T2*, who seen at our center between January, 2014 and December, 2019. Spearman correlation and linear regression analysis were used to evaluate the correlation between TE liver stiffness measurements and R2* MRI values and with serum ferritin. Results: Mean (SD) age of the subjects was 13.0 (3.1) years and body mass index was 16.6 (2.3) kg/m2. Mean liver stiffness measurement, MRI T2*(3T), corresponding MRI R2*(3T), and ferritin values were 6.55 (3.10) kPa, 3.4 (4.6) milliseconds, 616.20 (383.9) Hz, and 2874.69 (1570.7) ng/ mL, respectively. TE measurements correlated with MRI R2* values (r=0.61; P=0.001) and with serum ferritin level (r=0.59, P=0.001). Conclusion: TE is a reliable tool to estimate hepatic iron overload in patients with TDT.

Suspecting and diagnosing transthyretin amyloid cardiomyopathy (ATTR-CM) in India: An Indian expert consensus

Transthyretin cardiac amyloidosis (ATTR-CM) is a rare and under-recognized disorder characterized by the aggregation of transthyretin-derived insoluble amyloid fibrils in the myocardium. Heterogeneity of symptoms at presentation, makes its diagnosis often delayed. An expert panel gathered on a virtual platform across India to conduct a meeting for developing a guiding tool for ATTR-CM diagnosis. The panel recommended younger age (40 years) for suspecting ATTR-CM and thick-walled non-dilated hypokinetic ventricle was considered as one of the important red flags. Electrocardiogram (ECG) and echocardiography (ECHO) findings were recommended as primary tests to raise the suspicion while nuclear scintigraphy and hematological tests were recommended to confirm the diagnosis and rule out amyloid light-chain (AL) amyloidosis. Cardiac magnetic resonance (CMR) and biopsy were recommended in case of ambiguity in the presence of red flags. Considering the lack of expert guidelines in the Indian scenario, a standardized diagnostic algorithm was also proposed.

Diagnosis, Treatment and Prevention of Nutritional Anemia in Children: Recommendations of the Joint Committee of Pediatric HematologyOncology Chapter and Pediatric and Adolescent Nutrition Society of the Indian Academy of Pediatrics

Justification: Anemia in children is a significant public health problem in our country. Comprehensive National Nutrition Survey 2016-18 provides evidence that more than 50% of childhood anemia is due to an underlying nutritional deficiency. The National Family Health Survey-5 has reported an increase in the prevalence of anemia in the under-five age group from 59% to 67.1% over the last 5 years. Clearly, the existing public health programs to decrease the prevalence of anemia have not shown the desired results. Hence, there is a need to develop nationally acceptable guidelines for the diagnosis, treatment and prevention of nutritional anemia. Objective: To review the available literature and collate evidence-based observations to formulate guidelines for diagnosis, treatment and prevention of nutritional anemia in children. Process: These guidelines have been developed by the experts from the Pediatric Hematology-Oncology Chapter and the Pediatric and Adolescent Nutrition (PAN) Society of the Indian Academy of Pediatrics (IAP). Key areas were identified as: epidemiology, nomenclature and definitions, etiology and diagnosis of iron deficiency anemia (IDA), treatment of IDA, etiology and diagnosis of vitamin B12 and/or folic acid deficiency, treatment of vitamin B12 and/or folic acid deficiency anemia and prevention of nutritional anemia. Each of these key areas were reviewed by at least 2 to 3 experts. Four virtual meetings were held in November, 2021 and all the key issues were deliberated upon. Based on review and inputs received during meetings, draft recommendations were prepared. After this, a writing group was constituted which prepared the draft guidelines. The draft was circulated and approved by all the expert group members. Recommendations: We recommend use of World Health Organization (WHO) cut-off hemoglobin levels to define anemia in children and adolescents. Most cases suspected to have IDA can be started on treatment based on a compatible history, physical examination and hemogram report. Serum ferritin assay is recommended for the confirmation of the diagnosis of IDA. Most cases of IDA can be managed with oral iron therapy using 2-3 mg/kg elemental iron daily. The presence of macro-ovalocytes and hypersegmented neutrophils, along with an elevated mean corpuscular volume (MCV), should raise the suspicion of underlying vitamin B12 (cobalamin) or folic acid deficiency. Estimation of serum vitamin B12 and folate level are advisable in children with macrocytic anemia prior to starting treatment. When serum vitamin B12 and folate levels are unavailable, patients should be treated using both drugs. Vitamin B12 should preferably be started 10-14 days ahead of oral folic acid to avoid precipitating neurological symptoms. Children with macrocytic anemia in whom a quick response to treatment is required, such as those with pancytopenia, severe anemia, developmental delay and infantile tremor syndrome, should be managed using parenteral vitamin B12. Children with vitamin B12 deficiency having mild or moderate anemia may be managed using oral vitamin B12 preparations. After completing therapy for nutritional anemia, all infants and children should be advised to continue prophylactic iron-folic acid (IFA) supplementation as prescribed under Anemia Mukt Bharat guidelines. For prevention of anemia, in addition to age-appropriate IFA prophylaxis, routine screening of infants for anemia at 9 months during immunization visit is recommended.

Scoping review of published research on medical education in India during the Covid-19 pandemic

BACKGROUND Medical educators in India made rapid adjustments to maintain continuity and integrity of medical education in the midst of disruption caused by the Covid-19 pandemic. However, there are concerns regarding achievement of competence by undergraduate medical students due to inadequate clinical exposure. We explored the focus of initiatives from medical educators in India by a scoping review of published articles on developments in medical education during the pandemic to map concepts, main sources and the literature available in PubMed. METHODS We did this scoping review of published articles in PubMed database in four steps: (i) identification of research questions; (ii) identification of relevant studies; (iii) selection of studies meeting inclusion and exclusion criteria, and charting of data; and (iv) collating the summary and reporting of results. Manual content analysis was done to derive frequencies of variables. RESULTS Of the 52 articles identified, 22 met the requirements. Most studies (68.2%) were published in 2020. Half of the studies were conducted among undergraduate students and the remaining among postgraduates (27.3%), faculty (18.2%) and interns (4.5%). All the studies were evaluations at Kirkpatrick level-1 (18; 81.8%) and level-2 (4; 18.2%). Most of the studies (9, 41%) focused on exploration of perspectives about online learning among students and faculty, 9 (27.3%) on teaching– learning, 4 (18.2%) on formative assessment and 3 (13.6%) on summative assessment. CONCLUSIONS Most studies were evaluations at Kirkpatrick level-1 and level-2 among undergraduate medical students with a focus on conceptual understanding.

Reappraising the Role of Eplerenone in the Management of Heart Failure

Background: In India, the prevalence of heart failure (HF) is increasing at 1.2/1,000 people according to a study in northern India, and the mortality rate at 1 year (INTERnational Congestive Heart Failure [INTER-CHF]) is 37%. Due to the diverse phenotypes of HF, nonadherence to guideline-directed medical therapy (GDMT), resistance to uptitration of medication and underuse of mineralocorticoid receptor antagonists (MRAs), such as eplerenone, a uniform management approach may not be feasible. This review is aimed at assessing the burden of HF, reasons for underutilization of MRAs in treatment, evaluating the evidence and reappraising the disease-modifying role of eplerenone in HF management. Methods: An electronic database search was performed to identify relevant literature. Results: The review details various studies that demonstrate the role of MRA eplerenone as a disease-modifying agent in patients with mild-to-moderate hypertension and those with acute myocardial infarction (MI) complicated by left ventricular dysfunction and HF. It also outlines different patient profiles for eplerenone use and ways to handle minor side-effects. Conclusions: Eplerenone shows a promising effect in selectively blocking aldosterone receptors to suppress fibrosis and reverse cardiac remodeling.

Hindi Translation and Validation of Childhood Asthma Control Test (C-ACT)

Objective: Hindi translation and validation of the Childhood Asthma Control Test (C-ACT). Methods: Children aged 5-11 years with newly diagnosed asthma were enrolled and followed every 4-weeks for 12 weeks. Asthma control was assessed with C-ACT and Global Initiative for Asthma (GINA) criteria. Results: 60 children (34 boys, 56%) were enrolled. C-ACT showed a statistically significant correlation with GINA criteria at all visits. Cronbach’s alpha to assess the internal consistency was 0.74, and the intraclass correlation coefficient to measure test-retest reliability was 0.83. The maximum area under the curve (AUC) for C-ACT was 0.95 (95% CI: 0.89-1.0; P<0.001). At a cutoff score of ?20, the sensitivity, specificity, positive predictive value, and negative predictive value of C-ACT were 97.9%, 25%, 88.7%, and 87.5%, respectively. Conclusions: Hindi version of the CACT score is valid, reliable, and correlates well with the GINA criteria for asthma control in children. It has a high sensitivity at a cutoff score of ?20, but the specificity was poor in differentiating asthma control.

Clinico-immunological Profile of Systemic Lupus Erythematosus: An Observational Study

Background: SLE is a common connective tissue disease in Indians (mostly women) which is frequently underdiagnosed due to limited awareness and knowledge regarding the disease. Methods: This is a retrospective observational study conducted in a tertiary care hospital in Western India among patients of SLE attending outpatient Rheumatology Clinic and inpatient admissions of Topiwala Medical College and BYL Nair Charitable Hospital, Mumbai. Sixty patients were recruited based on inclusion and exclusion criteria. Results: In clinical profile, arthralgia was the most common manifestation seen in 53 patients (88.3%) followed by alopecia in 46 patients (76.7%). In systemic involvement, CNS lupus was the most common manifestation seen in 27 patients (45%) followed by renal involvement in 13 cases (21%). Pulmonary hypertension (PH) was another noticeable finding seen in 24 cases (40%) of which 18 (75%) had mild PH, 6 (25%) patients had severe PH. The mean SLEDAI score was 11.85 at baseline which reduced to 2.65 at 6 months and remained 3.65 at the end of 3 years of the study. In immunological profile, ANA was positive in all patients. Speckled pattern of ANA was the most common pattern seen in 34 patients (56.7%). A titre of above 1:100 was noted in 53 patients (88.3%). ds DNA was positive in 26 patients (43.3%). Anti Ro/La was positive in 3 patients (5%) and U1RNP in 2 patients (3.3%). Autoimmune hemolytic anemia (AIHA) was the most common autoimmune association seen in 25 patients (41.7%), antiphospholipid antibody(APLA) was seen in 15 patients (25%), 7 patients (11.6%) were anti TPO antibody positive, 3 patients (5%) were Ro/La positive while only 2 patients(3.3%) were U1RNP positive. Conclusion: Clinical profile and immunological patterns of SLE are diverse. A systematic work up is needed to identify the multisystem involvement and asking for specific antibody tests to identify common autoimmune associations is recommended.

Indian Academy of Pediatrics Revised (2021) Guidelines on Prevention and Treatment of Vitamin D Deficiency and Rickets

Justification: The emerging literature on prevalence of vitamin D deficiency in India, prevention and treatment strategies of rickets, and extra-skeletal benefits of vitamin D suggest the need for revising the existing guidelines for prevention and treatment of vitamin D deficiency in India. Objectives: To review the emerging literature on vitamin D prevalence and need for universal vitamin D supplementation. To suggest optimum vitamin D therapy for treatment of asymptomatic and symptomatic vitamin D deficiency, and rickets. To evaluate the extra-skeletal health benefits of vitamin D in children. Process: A National consultative committee was formed that comprised of clinicians, epidemiologists, endocrinologists, and nutritionists. The Committee conducted deliberations on different aspects of vitamin D deficiency and rickets through ten online meetings between March and September, 2021. A draft guideline was formulated, which was reviewed and approved by all Committee members. Recommendations: The group reiterates the serum 25- hydroxy vitamin D cutoffs proposed for vitamin D deficiency, insufficiency, and sufficiency as <12 ng/mL, 12-20 ng/mL and >20 ng/mL, respectively. Vitamin D toxicity is defined as serum 25OHD >100 ng/mL with hypercalcemia and/or hypercalciuria. Vitamin D supplementation in doses of 400 IU/day is recommended during infancy; however, the estimated average requirement in older children and adolescents (400-600 IU/day) should be met from diet and natural sources like sunlight. Rickets and vitamin D deficiency should be treated with oral cholecalciferol, preferably in a daily dosing schedule (2000 IU below 1 year of age and 3000 IU in older children) for 12 weeks. If compliance to daily dosing cannot be ensured, intermittent regimens may be prescribed for children above 6 months of age. Universal vitamin D supplementation is not recommended in childhood pneumonia, diarrhea, tuberculosis, HIV and non-infectious conditions like asthma, atopic dermatitis, and developmental disorders. Serum 25-hydroxy vitamin D level of >20 ng/mL should be maintained in children with conditions at high-risk for vitamin deficiency, like nephrotic syndrome, chronic liver disease, chronic renal failure, and intake of anticonvulsants or glucocorticoids.

Neurofibromatosis type-1 with seizures and cerebrovascular malformation: a case study

Neurofibromatosis type 1 (NF1) is the most common autosomal dominant neurocutaneous among humans. Epilepsy is more prevalent in NF1 patients than in the general population. NF1 vasculopathy is also a significant but underrecognized complication of the disease, affecting both arterial and venous blood vessels. Herein, we report a 2 year old female child with seizures and multiple cafe-au-lait spots on the body. The patient was diagnosed with NF1 based on clinical findings and family history. MRI Brain revealed middle cerebral artery dysplasia. Here we discuss diagnostic and treatment challenges and briefly reviews the existing literature.

Effect of Shatavari Root Powder (Asparagus racemosus) Supplementation on Milk Composition of Sahiwal Crossbred Cows

The present investigation was conducted to assess the effect of Shatavari root powder (Asparagus racemosus) supplementation on milk composition of Sahiwal crossbred cows during summer season on twenty lactating Sahiwal crossbred (H.F. × Sahiwal) cows in early stage of lactation were selected for the experiment. All the cows were randomly divided into two groups T1 (control) and T2 (Treatment) of 10 in each group. The treatment group was provided Shatavari root powder @ 50 g/head/day for 90 days post partum. Data related to milk composition takes fortnightly interval. The overall average milk fat, Protein and Lactose percent during the experimental period in T1 and T2 groups were 3.77 ± 0.01, 3.84 ± 0.03, 3.43 ± 0.01 and 3.46 ± 0.01, 4.54 ± 0.06 and 4.69 ± 0.06 percent respectively, there was no significant (P<0.05) differences between T1 and T2 groups. The overall average solid not fat (values) and total solid (values) differed significantly (P<0.05) between T1 and T2 groups 8.84 ± 0.04, 9.11 ± 0.11, 12.66 ± 0.03 and 13.02 ± 0.14% in T1 and T2 groups was significantly (P<0.05)

Detection of 3243 A/G and 3316 G/A mitochondrial DNA mutations in Nagpur population

The present study aims to detect 3243 A/G and 3316 G/A mitochondrial DNA (mtDNA) mutations in Nagpurpopulation. Total of 142 patients of type 2 diabetes mellitus and 142 healthy control individuals were selected forthe study from Nagpur city. Selected mutations studied using restriction fragment length polymorphism methodand confirmed by DNA sequencing. Results showed that 3316 G/A mt DNA mutation found in seven patientsof type 2 diabetes mellitus with a 4.92% prevalence, however, found absent in healthy control individuals. ChiSquare and Fisher's exact test showed a significant association between healthy control individuals and type 2diabetes mellitus patients detected with 3316 G/A mutation (p ≤ 0.01). ODDS ratio found significant (for 95%CI; p = 0.05) for 3316 G/A mutation. Furthermore, we did not find 3243 A/G mtDNA mutation in the studiedpopulation. Among studied mutations, 3316 G/A mutation in the ND1 gene is a pathogenic mutation that mayresponsible for type 2 diabetes mellitus in the Nagpur population.

Comparison of Intrathecal Clonidine and Fentanyl as Adjuvant to Hyperbaric Bupivacaine in Spinal Anaesthesia for Lower Limb Orthopedic Surgery: An Hospital Based Study

Background: There are various adjuvant used with hyperbaricbupivacaine to prolong the effect of spinal anesthesia butcommonly used fentanyl and clonidine. The present study wasundertaken to compare clonidine and fentanyl as adjuvant inspinal anesthesia in terms of time to onset of sensory andmotor blockade, duration of sensory and motor blockade andduration of postoperative analgesia and complications.Methods: The present study was undertaken in thedepartment of Anaesthesia, Government Medical College,Barmer, Rajasthan, India with primary aim to compare durationof postoperative analgesia. A total of 80 patients were enrolledin the present study. Ethical approval was obtained frominstitutional ethical committee and written consent wasobtained from all the patients. Complete demographic details ofall the patients were obtained. All the results were recorded inMicrosoft excel sheet and were analyzed by SPSS software.Results: In our study we found that time for first dose ofrescue analgesic was delayed in Group C (492.32 ± 17.32min) compared to Group F (418.80 ± 19.68min) which wasstatistically significant (P < 0.0001). Duration of sensory blockin Group C was 146.17 ± 19.42 min compared to 128.24 ±18.68min in Group F and Duration of motor block was 190.12 ±25.13 min in Group C in comparison to 176.18 ± 23.54 min in.

A Study of Supraclavicular Brachial Plexus Block by Paresthesia Technique Success Rate and Complications: An Hospital Based Study

Background: The advantage of regional anaesthesia overgeneral anaesthesia are many and well documented. Brachialplexus can be blocked by various methods includingparesthesia technique, nerve stimulation technique and ultrasound guided. The present study was undertaken forassessing time taken to perform block, Successful blockade,complication of supraclavicular brachial plexus block byparesthesia technique.Methods: The present study was undertaken in thedepartment of Anaesthesia, Government Medical College,Barmer, Rajasthan, India with aim of assessing success rateand Complications of brachial plexus block. A total of 100patients were enrolled in the present study. Ethical approvalwas obtained from institutional ethical committee and writtenconsent was obtained from all the patients after explaining indetail of the entire research process. Complete demographicdetails of all the patients were obtained. All the results wererecorded in Microsoft excel sheet and were analyzed by SPSSsoftware.Results: Mean time to perform block was 5.35 minutes andsuccesfull blockade in 92% patients, in 6% patient’s partialblockade and in 2% patient’s complete failure of block.Incidence of complications were vessels puncture 8 % patientsand pneumothorax 1% patients.Conclusion: Supraclavicular brachial plexus block byparesthesia technique is an easy and relatively safe procedurefor the upper limb surgeries below mid shaft of humerus withfew complications and provide good post-operative analgesia.

Impact of antibiotic stewardship program on usage of higher range of antibiotics in patients of intensive care units in a tertiary care hospital, India

Background: The aim of the study was to monitor the changes in antimicrobial use after implementation of Antibiotic Stewardship Programmed (ASP) and pattern of use of antimicrobials in the respective ICU抯.Methods: The study was conducted in three ICU抯 Adult ICU(AICU), Paediatric ICU (PICU), Neonatal ICU (NICU) -Six bedded each) over a period of six months from September 2018 to February 2019 in a tertiary care hospital. Antibiotics monitored over total 155 patients and antibiotics selected for the study are ?-lactam inhibitors, Carbapenem derivatives and ColistinResults: Out of total 155 patients 51% were males and the definitive therapy (Implementation of antibiotics according to the antibiotic policy of the hospital) in the respective ICU抯 showed increase from 66.7% to 83.3% after implementation of ASP activity in that particular duration. Antibiotic consumption showed fluctuation in the whole duration of the study (p value <0.05).Conclusions: Analysis of the study shows a positive impact on implementation of ASP programme in intensive care units, brought an effective increase in appropriate use of antimicrobials.

Hypertension prevalence and associated risk factors among bank employees working in an urban area of Marathwada region, Maharashtra, India

Background: Epidemiologic transition of diseases is occurring in recent times throughout the world. The morbidity, disability and mortality burden is now more because of non-communicable diseases. Cardiovascular diseases are number one killer throughout the world. Hypertension has been found to be an independent and consistent risk factor for cardiovascular diseases. Bank employee’s job being sedentary in nature and involving cash management related stress makes bank employees a potential occupational group to develop hypertension. So, this study was planned to assess the prevalence of hypertension in bank employees in the urban area of Latur district, Maharashtra.Methods: A cross-sectional observational study was carried out between January 2016 to December 2016 among all the bank employees working in urban area of Latur, Maharashtra. Approval from institutional ethics committee was obtained beforehand.Results: Total 577 bank employees were surveyed for this study. 32.4% of subjects were found to be hypertensive. Association of higher age group and managerial cadre of employment were found to be statistically significant.Conclusions: Bank employees have a higher prevalence of hypertension as compared to general population. Bank employees are an occupational risk group to develop hypertension.

HIV-free Survival at the Age of 18 Months in Children Born to Women With HIV Infection: A Retrospective Cohort Study

Objective: To assess HIV-free survival and nutritional status of HIV-exposed infants.Methods: This retrospective cohort study was conducted on infants born to woman with HIVinfection born at our Institute between January 2011 to March 2016, and followed usingcurrent National guidelines. HIV transmission rate, HIV-free survival, and nutritional statuswere assessed 18 months age. Results: Of the 155 infants, 10 (6.5%) died before 18 monthsof age. Two of 145 surviving infants were confirmed HIV-positive, the remaining were HIV-negative at 18 months (HIV-free survival 92.3%). Of the 10 infants who died, one wasconfirmed HIV-positive and three negative; the rest died before their HIV status could beascertained. HIV infection rate among the 149 infants for whom the test reports were availablewas 2%. At 18 months age, 14% HIV-uninfected infants were wasted, 28% stunted, and 3%had microcephaly. Conclusions: Infants born to mothers with HIV managed as per thecurrent National guidelines have a good outcome at 18 months of age.