Familial hemiplegic migraine in Indian children-a tertiary center experience.

Familial hemiplegic migraine (FHM), an autosomal dominant subtype of hemiplegic migraine, is a channelopathy presenting with severe headache, visual field defect, paresthesia, unilateral motor deficit, encephalopathy, seizures and aphasia. This cross-sectional study was conducted over 10 months in children aged 1-18 years suspected of hemiplegic migraine at a tertiary care pediatric hospital. Fourteen children were screened and five children with genetically confirmed FHM were included. The symptoms in the study population were paroxysmal hemiparesis (5/5), headache (5/5) and focal seizures (1/5). The hemiplegia episodes lasted from 4 h to 7 days. The mean age at the onset of neurological symptoms was 6.8 ± 0.7 years and the mean age at diagnosis was 12.8 ± 1.7 years, with a mean delay of 6.1 ± 1.9 years for the diagnosis. Neuroimaging during acute episodes revealed accentuated gray, white differentiation in the contralateral cerebral hemisphere with mild effacement of sulcal spaces in T2/fluid-attenuated inversion recovery (FLAIR) images. Genetic testing revealed ATP1A2 mutations (FHM2) in 4/5 and SCN1A (FHM3) in 1/5 patients. All of them (5/5) were initiated on oral topiramate and had favorable treatment responses with a mean follow-up duration of 7 ± 1.4 months. Diagnosis of FHM is mainly clinical and can be confirmed by genetic analysis. Perfusion and diffusion-weighted MRI should be considered during acute headache episodes, as it is mostly normal in symptom-free periods. Routine MRI sequences like T1 weighted, T2 weighted, FLAIR and contrast remain normal even during acute attacks.

Caffey's disease in disguise: a child abuse mimic.

A male infant was brought in a paediatric emergency with swelling in the right hand associated with restriction of wrist movements. Examination confirmed similar findings. The history of trauma was not forthcoming. Given the ambiguous history, the possibility of child abuse was kept. X-ray showed local soft tissue swelling with periosteal thickening and raised inflammatory markers in blood tests, which on review of the literature, was consistent with Caffey disease. Due to the self-limiting nature of the condition, the child was managed conservatively. Child abuse should be suspected in children with unexplained injuries, and before labelling abuse, its mimickers should be ruled out.

First report of clinically significant bites due to Platyceps ventromaculatus (GRAY, 1834) from Thar Desert region, Rajasthan, India.

BACKGROUND: Platyceps ventromaculatus is a non-front-fanged colubrid snake of unknown medical significance. In this study, we highlight the clinical manifestations and outcomes of P. ventromaculatus bites. We also emphasise the need to create awareness among clinicians and the public for its potential to be confused with serious venomous bites such as Echis carinatus sochureki. METHODS: This series is part of an ongoing observational clinical study from our tertiary care hospital in Jodhpur, India on the profile and outcomes of snakebite envenoming. Data was collected after approval from Institute Ethics Committee. The date and time of the bite, geographical location, type of human-snake conflict, time-to-reach a healthcare facility, antivenom used (dose), and outcomes were recorded. We retrospectively examined our clinical data for images suggestive of P. ventromaculatus and present the clinical details of these patients. The photographs were identified utilising taxonomic keys for species identification. RESULTS: A total of four images and three patients with bites due to P. ventromaculatus were identified. The clinical effects included mild local erythema, pain, transient local bleeding, and edema. All bites occurred during daylight hours, 2 during agricultural activities, and one at home. Twenty-minute Whole Blood Clotting Test was persistently prolonged for 12 h after the bite in one patient. All patients were treated symptomatically, observed at the emergency department, and discharged within 24 h. None of the patients received antivenom. CONCLUSIONS: To our knowledge, P. ventromaculatus has so far not been reported to result in envenoming or medically significant bites. This study highlights that Platyceps bite can present with clinically significant local and possibly systemic findings that may lead to confusion with saw-scaled viper (Echis) envenoming. Clinicians must receive appropriate training so as to be aware and recognize regional snake species that do not require antivenom so as to avoid unnecessary antivenom administration.

An esterase-cleavable persulfide donor with no electrophilic byproducts and a fluorescence reporter.

Hydrogen sulfide (H2S) and associated sulfur species known as persulfide or sulfane sulfur are considered among the first responders to oxidative stress. However, tools that reliably generate these species without any potentially toxic byproducts are limited, and even fewer report the generation of a persulfide. Here, using a latent fluorophore embedded with N-acetylcysteine persulfide, we report a new tool that is cleaved by esterase to produce a persulfide as well as a fluorescence reporter without any electrophilic byproducts. The rate of formation of the fluorescence reporter is nearly identical to the rate of formation of the persulfide suggesting that the use of this probe eliminates the need for secondary assays that report persulfide formation. Symptomatic with persulfide generation, the newly developed donor was able to protect chondrocyte cells from oxidative stress.

Exploring geochemical distribution of potentially toxic elements (PTEs) in wetland and agricultural soils and associated health risks.

This study is carried out to understand the degree of soil pollution, transport mechanism, and distribution pattern of potentially toxic elements (PTEs), including the exposure effects on human health. Towards this, topsoil samples were collected from the Saman wetland and surrounding agricultural fields in the Gangetic plain, India. The results show that the mean concentration of Cu, Hg, Zn, Pb, Th, As, U, and Cd of both soil types exceed the natural background values. The multivariate analysis suggests the soils are moderately contaminated with As, Cd, Zn, Pb, and Hg (possibly from anthropogenic sources) and heavily contaminated with Th and U, likely ascended from geogenic sources. The GIS-based geostatistical plots coupled with principal component analysis (PCA) and hierarchical cluster analysis (HCA) apportion the sources of these toxic elements, which vary greatly and are closely correlated to the geogenic processes and local anthropogenic sources like pesticides and agrochemicals. The health risk assessment revealed that the cumulative hazard index (HI) values of PTEs are lower than the safe level, suggesting no significant noncarcinogenic effect for adults and children. However, excess cancer risk (ECR) values exceed the permissible limit (1 × 10-6), signifying that exposure to the toxic element concentration may cause cancer in the exposed population, most probably in the children subpopulation. Thus, this study highlights the importance of local compliance, ensuring the quality checks and management policies in using pesticides and other agrochemicals containing PTEs to control the imposed cancer risks.

Protocolized Sedation Utilizing COMFORT-B Scale versus Non-protocol-directed Sedation in Mechanically Ventilated Children - An Open-label, Randomized Controlled Trial.

This study aimed to determine the effect of protocolized sedation using the COMFORT-B scale on the duration of mechanical ventilation (DMV). Eighty children with anticipated Duration of mechanical ventilation (DMV) >24 h admitted to the Pediatric intensive care unit (PICU) were randomized into one group that received protocolized sedation (PS) using the COMFORT behavioural (COMFORT-B) scale, and another group that received non-protocolized sedation (NPS). The primary outcome was the impact on the DMV. The DMV was significantly lower in PS (PS: 3.5 [3-7] vs. NPS group: 8.5 [4.25-13.75] d; p = 0.008). The cumulative dose and duration of fentanyl in the PS group was significantly lower (median [IQR]; 120 [62.88-279.12] vs. 320.4 [110.88-851.52] µg/kg; p = 0.007 and 4 [2.25-7.75] vs. 8 [4-17.5] d; p = 0.009, respectively). The authors found a decrease in DMV and sedation related adverse events (SRAE) like ventilator associated pneumonia (VAP), accidental extubation, post-extubation stridor and dose and duration of sedative agents with PS.

Clinico-Epidemiological Profile and Short-Term Outcomes of Patients with Acute Poisoning Presenting to an Emergency of a Tertiary Care Center in Western Rajasthan.

Background: This research aimed to evaluate the clinico-epidemiological profile and short-term outcomes of patients admitted to the emergency department of a tertiary hospital. Materials and Methods: This prospective observational cross-sectional study was conducted between January 1, 2021, and June 30, 2022, in the Emergency Department of a tertiary hospital. Patients of either gender of any age attending the emergency department with acute poisoning were included. These cases were analyzed for their epidemiological profiles, poisoning characteristics, and various clinical variables influencing patient outcomes. Results: Out of a total of 236 patients, 190 patients (80.5%) were aged more than 18 years. Comorbidities were present in 32 (13.5%) patients, and psychiatric illness was the most common comorbidity. The intention of poisoning was suicidal in 185 patients (78.4%) and accidental in 51 patients (21.6%). Organophosphate was the most common poisoning seen in 55 patients (23.3%), followed by drug ingestion seen in 44 patients (18.6%). Twenty-eight patients (11.8%) were transferred to the intensive care unit. Nineteen patients (8.1%) required mechanical ventilation. There were ten deaths (4.2%) in the study population. Aluminum phosphide poisoning was associated with the highest mortality rate, followed by organophosphate poisoning. Conclusions: This study indicates that most poisoning cases involved young people, mainly males. Organophosphorus poisoning was one of the most common poisonings, followed by the ingestion of drugs in this part of the world. Aluminum phosphide had the highest fatality rate.

Geochemical evolution of dissolved trace elements in space and time in the Ramganga River, India.

Understanding the spatiotemporal dynamics of river water chemistry from its source to sinks is critical for constraining the origin, transformation, and "hotspots" of contaminants in a river basin. To provide new spatiotemporal constraints on river chemistry, dissolved trace element concentrations were measured at 17 targeted locations across the Ramganga River catchment. River water samples were collected across three seasons: pre-monsoon, monsoon, and post-monsoon between 2019 and 2021. To remove the dependency of trace element concentrations on discharge, we used molar ratios, as discharge data on Indian transboundary rivers are not publicly available. The dataset reveals significant spatiotemporal variability in dissolved trace element concentrations of the Ramganga River. Samples collected upstream of Moradabad, a major industrial city in western Uttar Pradesh, are characterized by ~ 1.2-2.5 times higher average concentrations of most of the trace elements except Sc, V, Cr, Rb, and Pb, likely due to intense water-rock interactions in the headwaters. Such kind of enrichment in trace metal concentrations was also observed at sites downstream of large cities and industrial centers. However, such enrichment was not enough to bring a major change in the River Ganga chemistry, as the signals got diluted downstream of the Ramganga-Ganga confluence. The average river water composition of the Ramganga River was comparable to worldwide river water composition, albeit a few sites were characterized by very high concentrations of dissolved trace elements. Finally, we provide an outlook that calls for an assessment of stable non-traditional isotopes that are ideally suited to track the origin and transformation of elements such as Li, Mg, Ca, Ti, V, Cr, Fe, Ni, Cu, Zn, Sr, Ag, Cd, Sn, Pt, and Hg in Indian rivers.

Chyluria: clearing the 'muddiness' with lipiodol lymphangiography.

Chyluria is a rare entity characterised by the presence of chyle/lymphatic fluid within the urine. It develops following an abnormal communication between the perirenal lymphatics and pelvicalyceal lymphatics. There are multiple causes of chyluria including infective (filariasis), post-traumatic, post-surgical, pregnancy and malignancy. We present a case of a 15-year-old male who presented with a complaint of the intermittent passage of milky urine for the preceding 1 year. Conventional lipiodol lymphangiography followed by cone beam computed tomography was done to look for abnormal fistulous channels. Subsequently, the patient was successfully treated with cystoscopy-guided renal pelvic instillation sclerotherapy of povidone-iodine.

Clinical Profile, Follow-up, and Role of Neuroimaging in Pediatric Guillain-Barré Syndrome in the COVID Era: An Ambispective Study.

Background: To define the varied presentations of Guillain-Barré syndrome in children in the COVID era and 6 months' follow-up outcome. Methods: Ambispective study of 15 months' duration involving children with Guillain-Barré syndrome aged 1 month to 18 years at a tertiary care pediatric hospital. They were categorized into groups A and B based on COVID-19 serology testing. Hughes Disability Scale was used for disability assessment. Modified Rankin scale was used for improvement assessment in follow-up. Results: Of 19 children with Guillain-Barré syndrome, 9 (47%) were females and 10 (53%) were males. Groups A and B had children with negative (8) and positive serology (11), respectively. The most common presentation in both groups was motor weakness. Post-COVID pediatric Guillain-Barré syndrome presented with variants of Guillain-Barré syndrome rather than the classical form (P = .03). In group B, patients with elevated inflammatory markers had poor response to intravenous immunoglobulin, and 5 of 11 patients had good response to pulse steroids, probably depicting an inflammation-predominant pathology. Conclusion: Post-COVID Guillain-Barré syndrome in children presented with Guillain-Barré syndrome variants rather than the classic form. Neuroimaging is of great value in both confirming Guillain-Barré syndrome diagnosis and excluding differentials. Patients with elevated inflammatory markers and residual weakness may be given a pulse steroid trial.

Evaluation of Diaphragmatic Thickness and Dysfunction by Ultrasonography in Mechanically Ventilated Children for Assessment of Extubation Success.

OBJECTIVES: This study aimed to evaluate diaphragm thickness (DT) and diaphragmatic thickening fraction (DTF) in mechanically ventilated children, and study the association of these measurements with extubation success. METHODS: Consecutive children aged one month to 18 years, who required mechanical ventilation (MV) for more than 24 hours at our institution, were enrolled between April, 2019 to October, 2020. Ultrasonographic measure-ments of DT were documented, and DTF was calculated from baseline (within 24 hours of MV) until 14 days of MV, and up to three days post-extubation. RESULTS: Of the 54 children-enrolled, 40 underwent planned extubation trial, of which 9 (22.5%) had extubation failure. Pre-extubation and post-extubation DTF between children in extubation-success and extubation-failure groups were comparable (P=0.074). There was no significant difference in the diaphragm atrophy rate between the two groups (P=0.819). Binary logistic regression showed significantly decreased probability of successful extubation with total ventilation duration (P=0.012) and mean DTF% before extubation (P=0.033). CONCLUSION: Despite evidence of diaphragmatic atrophy in critically ill children receiving mechanical ventilation, there was no significant difference in DTF between extubation success and failure groups.

Post-COVID-19 Immune-Mediated Neurological Complications in Children: An Ambispective Study.

BACKGROUND: The neurological manifestation following a severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is varied, and till now, only a few studies have reported the same. METHODS: We used retrospective data from May to July 2021 and prospective study data from August to September 2021, including that from children aged between one month and 18 years who presented to a tertiary care referral center with the neurological manifestation and had a history of coronavirus disease 2019 (COVID-19) infection or exposure and positive SARS-CoV-2 serology. The neuroradiological manifestations were further categorized as in a predesigned proforma. RESULTS: Case records of the 18 children who fulfilled the criteria were included in the study; among them, seven (38.8%) were male and 11 (61.1%) were female. Predominant presentation in our study group was status epilepticus (six of 18) and Guillain-Barré syndrome (five of 18). Other manifestations included stroke (two of 18), demyelinating syndromes (three of 18), and autoimmune encephalitis (two of 18). Most of the children had favorable outcomes except for one mortality in our cohort. CONCLUSIONS: Delayed complications following SARS-CoV-2 infection are seen in children. A temporal correlation was noted between the COVID-19 infection and the increasing number of neurological cases after the second wave. Steroids could be beneficial while treating such patients, especially in the presence of high inflammatory markers. Testing for SARS-CoV-2 serology during the pandemic can give a clue to the underlying etiology. Further multicentric studies are required to understand the varied neurological manifestations following SARS-CoV-2 infection in children.

Pattern of pediatric emergency visits during lockdown -Impact of COVID-19 pandemic in Jodhpur City, India.

Objectives: To study the impact of lockdown during novel coronavirus disease-19 (COVID-19) pandemic on Pediatric Emergency visits. Methods: In this retrospective study, we compared the data regarding demographic details, triage category, underlying primary organ system involvement and outcomes among pediatric emergency visits of April-May 2019 and 2020. Results are expressed in means and proportions. Chi-square test was applied to compare the proportion in two groups. Results: We observed decrease in number of patients during lockdown period as compared to previous year (792 vs 506, decrease by 36%). There was significant increase in respiratory (P = 0.001, CI 2.66-11.6), and hematological emergencies (P < 0.001, CI 2.96-9.23) Road traffic accident (RTA) were decreased significantly (P = 0.005, CI 0.6-3.06). Unintentional injuries were comparable to previous year (P = 0.14, CI -0.86-6.19), though children being at home. Conclusion: During lockdown, number of pediatric emergency visits was reduced and also lead to delayed presentation. Parents should be informed about utilizing telemedicine services available during this period and avoid delaying in going to hospitals in emergency conditions.

Point-of-Care Thoracic Ultrasound in Children with Bronchiolitis.

OBJECTIVE: To characterize thoracic (lung and diaphragm) ultrasound findings in children < 2 y with bronchiolitis, evaluate correlation between lung ultrasound severity score (USS) and bronchiolitis severity score (BSS), and study the interobserver agreement of USS between study pediatrician and radiologist. METHODS: In this prospective observational study, thoracic ultrasound was performed on children with bronchiolitis by the study pediatrician and USS score was assigned. A radiologist blinded to all clinical information, performed an independent thoracic ultrasound. Demographics, clinical course, and other relevant details were recorded. RESULTS: Fifty-three children were enrolled; 29/53 patients (54.7%) were classified as mild bronchiolitis and 24/53 (45.2%) had moderate bronchiolitis as per clinical score; 13.2% (7/53) patients had both anterior and posterior subpleural consolidation and went on to require higher respiratory support either in the form of continuous positive airway pressure in 71.4% (5/7), oxygen for > 24 h in 14.2% (1/7), or heated humidified high-flow nasal cannula in 14.2% (1/7). These results were statistically significant (p < 0.001). A statistically significant correlation was found between the USS and type and duration of respiratory support (p value 0.002) and with the mean duration of hospital stay (p value < 0.001). There was significant correlation between the BSS and USS (p < 0.001). There was a very good agreement between the ultrasound findings of study pediatrician and radiologist (kappa 0.83). CONCLUSION: The findings of lung ultrasound (LUS) are not specific for bronchiolitis. However, LUS can be used as a good prognostic tool in patients with bronchiolitis.

Nutritional Status of Under-five Siblings of Severely Wasted Children in Bhopal.

OBJECTIVE: To study the prevalence and associated factors of undernutrition in siblings of children with severe acute malnutrition (SAM). METHOD: It was a community-based cross-sectional study of under-five year siblings of children with SAM. RESULTS: A total of 128 under-five years siblings were studied, 30% had SAM whereas 20% had moderate acute malnutrition (MAM). More than 7 members in a family (OR=4.23, CI 1.9-9.6, P<0.001), underweight mothers (OR=5.2, CI 2.08-13.0, P<0.001), children who received pre-lacteal feeds (OR=3.24, CI 1.33-7.87, P=0.007), and Muslim religion (OR=4.44, CI 1.78-11.1, P<0.001) were significantly associated with finding of another child with SAM in the family. CONCLUSION: There was high proportion of severe malnutrition in siblings of children with SAM. Consideration should be given to actively screen all under-5 children in the family of a newly diagnosed child with SAM for undernutrition.

Assessment of zinc inadequacy among tribal adolescent population of central India - A cross-sectional study.

Background & objectives: Zinc is a crucial micronutrient in adolescence, required for promoting growth and sexual maturation. Adolescents of some tribes may be at high risk of zinc deficiency due to dietary inadequacy and poor bioavailability of zinc from plant-based diets. This study aimed to evaluate the risk of zinc deficiency by estimating prevalence of inadequate zinc intake, prevalence of low serum zinc and stunting among tribal adolescents. Methods: A cross-sectional community-based survey was conducted among adolescents (10-19 yr) in three purposively selected districts where Bhil, Korku and Gond tribes were in majority. Structured data collection instrument comprising information about sociodemographic characteristics and dietary recall data was used. Anthropometric assessment was conducted by standardized weighing scales and anthropometry tapes, and blood sample was collected from antecubital vein into trace element-free vacutainers. Serum zinc was estimated using an atomic absorption spectrophotometer. Results: A total of 2310 households were approached for participation in the study, of which 2224 households having 5151 adolescents participated. Out of these enlisted adolescents, 4673 responded to dietary recall (90.7% response rate). Anthropometry of 2437 participants was carried out, and serum zinc was analyzed in 844 adolescents. The overall prevalence of dietary zinc inadequacy was 42.6 per cent [95% confidence interval (CI) 41.2 to 44.1] with reference to the estimated average requirement suggested by International Zinc Nutrition Consultative Group (IZiNCG) and 64.8 per cent (95% CI 63.4 to 66.2) with Indian Council of Medical Research-recommended requirements. Stunting was observed in 29 per cent (95% CI 27.2 to 30.8) participants. According to IZiNCG cut-offs, low serum zinc was detected in 57.5 per cent (95% CI 54.1 to 60.8) of adolescents, whereas it was 34.4 per cent (95% CI: 31.2-37.5) according to the national level cut-off. Interpretation & conclusions: Risk of dietary zinc inadequacy and low serum zinc concentration amongst adolescents of the Gond, Bhil and Korku tribes is a public health concern.