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Abstract Liver failure in neonates is a rare but often fatal disease. Trisomy 21 is not usually associated with significant infantile liver disease. If present, hepatic dysfunction in an infant with Trisomy 21 is likely to be attributed to transient myeloproliferative disorder with hepatic infiltration by hematopoietic elements and may be associated with secondary hemosiderosis. A less commonly recognized cause of liver failure in neonates with Trisomy 21 is neonatal hemochromatosis (NH); this association has been reported in nine cases of Trisomy 21 in literature. NH is a rare, severe liver disease of intra-uterine onset that is characterized by neonatal liver failure and hepatic and extrahepatic iron accumulation that spares the reticuloendothelial system. NH is the most frequently recognized cause of liver failure in neonates and the commonest indication for neonatal liver transplantation. Although porto-pulmonary hypertension (PPH) has been reported as a complication of liver failure in adults and older children, this has not been reported in neonates with liver failure of any etiology. This is probably due to the rarity of liver failure in newborns, delayed diagnosis and high mortality. The importance of recognizing PPH is that it is reversible with liver transplantation but at the same time increases the risk of post-operative mortality. Therefore, early diagnosis of PPH is critical so that early intervention can improve the chances of successful liver transplantation. We report for the first time the association of liver failure with porto-pulmonary hypertension secondary to NH in an infant with Trisomy 21.

Lack of sufficient observations has been an impediment for understanding the spatial and temporal variability of sea-surface pCO 2 for the Bay of Bengal (BoB). The limited number of observations into existing machine learning (ML) products from BoB often results in high prediction errors. This study develops climatological sea-surface pCO 2 maps using a significant number of open and coastal ocean observations of pCO 2 and associated variables regulating pCO 2 variability in BoB. We employ four advanced ML algorithms to predict pCO 2 . We use the best ML model to produce a high-resolution climatological product (INCOIS-ReML). The comparison of INCOIS-ReML pCO 2 with RAMA buoy-based sea-surface pCO 2 observations indicates INCOIS-ReML's satisfactory performance. Further, the comparison of INCOIS-ReML pCO 2 with existing ML products establishes the superiority of INCOIS-ReML. The high-resolution INCOIS-ReML greatly captures the spatial variability of pCO 2 and associated air-sea CO 2 flux compared to other ML products in the coastal BoB and the northern BoB., (© 2024. The Author(s).)

Background: Dermoid cyst, a common benign neoplasm of ovary in women, needs treatment because of the risk of torsion, rupture, and malignant change. Laparoscopic surgery nowadays is the preferred treatment modality, but the only issue is its safety in case of rupture and spillage of its contents with the risk of chemical peritonitis and malignant dissemination. Aim of the study was to find out the safety of laparoscopic surgery for dermoid cyst of ovary., Methods: It was a retrospective study done from January 2017 to December 2019. All the women with diagnosis of dermoid cyst of ovary managed laparoscopically either salpingoophorectomy or cystectomy were taken into study. Analysis of size of the cyst operated, the time taken, spillage rate, duration of hospital stay, and post-operative complications especially chemical peritonitis was done., Results: There were 61 women who had undergone laparoscopic surgery. Laparoscopic cystectomy was done in 68.9% (n=42), laparoscopic salpingoophorectomy in 29.5% (n=18). Mean age of the patient was 31.74±8.38 years. Mean size was 6.21±1.99 cm. Rupture and spillage were observed in 21.3% (n=13) that were > 5cm in diameter (X2= 3.62, p=0.05). Larger the size of the cyst, more the surgical time was noted (X2=6.26, p=0.04). Significant difference in mean operating time in case of cyst rupture and spillage (p=0.004) was observed. Mean hospital stay was 53.5±1.3 hours. No case of chemical peritonitis was observed with spillage. All cases had histopathology of mature cystic teratoma., Conclusions: Laparoscopic surgery is safe for dermoid cyst of ovary even with rupture and spillage of its contents.

Background: Treatment of malignant melanoma has undergone a paradigm shift with the advent of immune checkpoint inhibitors (ICI) and targeted therapies. However, access to ICI is limited in low-middle income countries (LMICs)., Patients and Methods: Histologically confirmed malignant melanoma cases registered from 2013 to 2019 were analysed for pattern of care, safety, and efficacy of systemic therapies (ST)., Results: There were 659 patients with a median age of 53 (range 44-63) years; 58.9% were males; 55.2% were mucosal melanomas. Most common primary sites were extremities (36.6%) and anorectum (31.4%). Nearly 10.8% of the metastatic cohort were BRAF mutated. Among 368 non-metastatic patients (172 prior treated, 185 de novo, and 11 unresectable), with a median follow-up of 26 months (0-83 months), median EFS and OS were 29.5 (95% CI: 22-40) and 33.3 (95% CI: 29.5-41.2) months, respectively. In the metastatic cohort, with a median follow up of 24 (0-85) months, the median EFS for BSC was 3.1 (95% CI 1.9-4.8) months versus 3.98 (95% CI 3.2-4.7) months with any ST (HR: 0.69, 95% CI: 0.52-0.92; P = 0.011). The median OS was 3.9 (95% CI 3.3-6.4) months for BSC alone versus 12.0 (95% CI 10.5-15.1) months in any ST (HR: 0.38, 95% CI: 0.28-0.50; P < 0.001). The disease control rate was 51.55%. Commonest grade 3-4 toxicity was anemia with chemotherapy (9.5%) and ICI (8.8%). In multivariate analysis, any ST received had a better prognostic impact in the metastatic cohort., Conclusions: Large real-world data reflects the treatment patterns adopted in LMIC for melanomas and poor access to expensive, standard of care therapies. Other systemic therapies provide meaningful clinical benefit and are worth exploring especially when the standard therapies are challenging to administer., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Bajpai, Abraham, Saklani, Agarwal, Das, Chatterjee, Kapoor, Eaga, Mondal, Chandrasekharan, Bhargava, Srinivas, Turkar, Rekhi, Khanna, Janu, Bal, Ostwal, Ramaswamy, Rohila, Desouza, Guha, Kumar, Menon, Rath, Patil, Noronha, Joshi, Laskar, Rangarajan, Prabhash, Gupta and Banavali.)

Rarity in reporting whole genome sequence of Dengue virus from dengue endemic countries leaves lacunae in understanding regional pattern of virus mutation and ultimately leading to non-understanding of transmission pattern and clinical outcomes emerging at regional levels. Due to inter-serotype genomic similarity and intra-serotype genomic diversity, appropriate designing of primer pairs appears as an exhaustive exercise. Present paper reports new Dengue virus type-specific primer which may help in characterizing virus specific to Asian origin. Genomes of dengue virus serotypes of Asian region were searched and using advanced bioinformatics tools, serotype specific primers were designed and tested for their targeted amplification efficiency. 19 primers sets for DENV-1, 18 primer sets for DENV-2, 17 for DENV-3 and 18 for DENV-4 were designed. In-silico and experimental testing of the designed primers were performed on virus isolated from both clinical isolates and passaged cultures. While all 17 and 18 primer sets of DENV-3 and DENV-2 respectively yielded good quality sequencing results; in case of DENV-4, 16 out of 18 primer sets and in DENV-1, 16 out of 19 primer sets yielded good results. Average sequencing read length was 382 bases and around 82% nucleotide bases were Phred quality QV20 bases (representing an accuracy of circa one miscall every 100 bases) or higher. Results also highlighted importance of use of primer development algorithm and identified genomic regions which are conservative, yet specific for developing primers to achieve efficiency and specificity during experiments., Competing Interests: Competing Interests: The authors have declared that no competing interest exists.

ROS1 rearrangement acts as a driver mutation in 1-2% of NSCLC. Crizotinib is approved in this situation both in treatment naïve and pre-treated patients. Here we report our experience with crizotinib in patients with advanced NSCLC harbouring ROS1 rearrangement. Eleven patients were included in our study. More than half of our patients had associated comorbidities and one fourth of them had a compromised performance status. Out of 11 patients, 5 of them were exposed to crizotinib .The response rates among crizotinib treated patients was 80%. With a median follow up of 9 months, median PFS and OS were 5.4 months and 8.5 months respectively for the entire population. Analyzing the outcomes separately , median PFS and OS was not reached for those who received crizotinib compared to median PFS of 2.5 months and median OS of 4.2 months in those who were not exposed to crizotinib. The difference was statistically significant. Estimated 1 year OS was 80% for those who received crizotinib compared to 18% for who did not receive crizotinib. In conclusion, crizotinib is effective with acceptable side effect profile in patients with ROS1 rearranged NSCLC in our population., Competing Interests: There are no conflicts of interest.

Ceritinib is a novel ALK inhibitor approved for advanced stage NSCLC with ALK gene rearrangement, progressed and/or intolerant to crizotinib. 13 patients were included in our study who received ceritinib. Majority of them were women and never smokers with a median age of 47 yrs. Nearly half of them had a compromised performance status and received ceritinib in third line and beyond. Ceritinib showed nearly 50% response rates. With a median follow up of 9 months for the entire cohort, median PFS and OS were not reached. However, the mean values for PFS and OS were 10.9 and 14.8 months,with an estimated 1 year PFS and OS being 56% and 78% respectively.1/3 of the patients had gastrointestinal and liver toxicities. Metabolic abnormalities were seen in 1/4 th of them. ceritinib was permanently discontinued in one patient due to pneumonitis. In conclusion, ceritinib has a favorable efficacy and side effect profile in our patient population., similar to that reported in large clinical trials. It has shown promising efficacy even in patients with compromised performance status; presence of brain metastases and heavily pre-treated disease.

Introduction: Although surgical treatment of spondylothoracic dysplasia (STD) is controversial, we have found that an expansion thoracoplasty using a Vertical Expandable Prosthetic Titanium Rib (VEPTR; DePuy Synthes) results in favorable outcomes, including 100% survivability (at an average follow-up of 6.2 years), increased thoracic spinal length, and decreased requirements for ventilation support., Step 1 Preoperative Preparation: Make anteroposterior and lateral radiographs of the spine., Step 2 Position the Patient for the Procedure: The patient is placed in the prone position., Step 3 the Incision: A curvilinear skin incision is made, starting proximally between the spine and the medial edge of the scapula., Step 4 the Osteotomy: Perform the v-osteotomy., Step 5 Placement of the Veptr Device: A number-4 VEPTR-I device is wedged in, starting laterally within the osteotomy sites, wedging the osteotomies apart, distracting the superior ribs proximally and the inferior ribs distally, lengthening the hemithorax, and stopping approximately at the posterior axillary line, when there is maximum stress on the superior and inferior ribs, to avoid fracture, and the lamina spreaders are then removed., Step 6 Wound Closure: Insert drains and local anesthetic catheters and close the wound., Step 7 Expansion and Replacement Procedures: Lengthen the devices with the standard VEPTR technique of limited 3-cm incisions every three to six months., Results: VEPTR treatment in patients with STD is associated with increased thoracic spine height and reduced thoracic width-to-height ratio, suggesting a greater gain in height than in width., What to Watch for: IndicationsContraindicationsPitfalls & Challenges.

Background: Epidemic diphtheria is still poorly understood and continues to challenge both developing and developed countries. In the backdrop of poor immunization coverage, non-existent adult boosters, weak case based surveillance and persistence of multiple foci, there is a heightened risk of re-emergence of the disease in epidemic forms in India. Investigating each outbreak to understand the epidemiology of the disease and its current status in the country is therefore necessary. Dhule a predominantly tribal and rural district in Northern Maharashtra has consistently recorded low vaccination coverages alongside sporaidic cases of diphtheria over the last years., Methods: This study reports the findings of an onsite survey conducted to assess a recent outbreak of diphtheria in Dhule district and the response mounted to it. Secondary data regarding outbreak detection and response were obtained from the district surveillance office. Clinical data were extracted from hospital records of eleven lab confirmed cases including one death case. Frequency distributions were calculated for each identified clinical and non- clinical variable using Microsoft™ Excel® 2010., Results: Our findings suggest a shift in the median age of disease to adolescents (10-15 years) without gender differences. Two cases (18%) reported disease despite immunization. Clinical symptoms included cough (82%), fever (73%), and throat congestion (64%). About 64% and 36% of the 11 confirmed cases presented with a well defined pseudomembrane and a tonsillar patch respectively. Drug resistance was observed in all three culture positive cases. One death occurred despite the administration of Anti-Diphtheric Serum in a partially immunized case (CFR 9%). Genotyping and toxigenicity of strain was not possible due to specimen contamination during transport as testing facilities were unavailable in the district., Conclusions: The outbreak raises several concerns regarding the epidemiology of diphtheria in Dhule. The reason for shift in the median age despite consistently poor immunization coverage (below 50%) remains unclear. Concomitant efforts should now focus on improving and monitoring primary immunization and booster coverages across all age groups. Gradually introducing adult immunization at ten year intervals may become necessary to prevent future vulnerabilities. Laboratory networks for genotyping and toxigenicity testing are urgently mandated at district level given the endemicity of the disease in the surrounding region and its recent introduction in remote Dhule. Contingency funds with pre- agreements to obtain ADS and DT/Td vaccines at short notice and developing standard case management protocols at district level are necessary. Monitoring the disease, emerging strains and mutations, alongside drug resistance through robust and effective surveillance is a pragmatic way forward.

A rapid differential pulse adsorptive stripping voltammetric method has been developed for the ultra-trace determination of chromium using 2,2'-bipyridine. The base electrolyte used is 0.1 M NH4Cl (pH 6.0). The peak current was found to increase substantially with the addition of nitrite ions. A well-defined peak was observed at -1.3 V. Parameters, like concentration of the ligand, concentration of nitrite ion, accumulation potential, accumulation time, rest period, drop size, scan rate, pulse amplitude etc. have been optimized. Under the optimum conditions, the 3 sigma detection limit was found to be 0.02 ppb (3.8 x 10(-10) M). The method is highly selective and sensitive, and has been applied to the determination of Cr(VI) in spiked water, effluents and ore samples.

A simple and rapid differential pulse polarographic method has been developed for the trace determination of dexamethasone sodium phosphate. A well-defined single peak with an Ep value of -1.14 V is obtained in acetate buffer (pH 5.0). The linearity is valid in the range 0.2-1.2 mg/25 mL ( r=0.9992) with minimum detection limit of 7.6x10(-6) M. The precision of the method developed is implied from the values of relative mean deviation, standard deviation and coefficient of variation, which are 2.44%, 0.014 and 3.5% respectively. Marketed formulations of dexamethasone sodium phosphate have been analysed by calibration and standard addition methods. Recovery experiments were found to be quantitative, and analysis to determine the mass per tablet was obtained within +/-0.2% of the expected market value. The studies have shown that the method is reproducible and accurate and can be used in the analysis of marketed formulations.

Metabolic antigens of Aspergillus fumigatus, soil strain 2605 and sputum isolate, were evaluated for their diagnostic applicability using hyperimmune sera and sera of adults and pediatric patients of allergic bronchopulmonary aspergillosis. An indirect ELISA was standardised by using 2-10 micrograms/ml of coating antigen for detection of specific IgG and IgE antibodies in the sera of patients. The ratios of absorbance for specific IgE and IgG antibodies by ELISA (normal to patients) were observed to be in the range of 1:2 to 1:3 to 1:8 respectively. These antigenic preparations were further analyzed to identify and characterize the individual components by immunoblotting. This analysis indicated the presence of allergenic and antigenic determinants in the antigens of molecular weights 70, 34, and 28 Kd. The utility of the antigens of soil strain for diagnostic purpose is suggested.