Acute necrotizing encephalopathy of childhood secondary to dengue infection: A case report from Pakistan

Acute necrotizing encephalopathy of childhood (ANEC) is a rare condition mainly affecting children with a distinct clinico-radiologic pattern. Initially thought to be secondary to respiratory viral infections, there have been more insights to the pathogenesis of ANEC including genetics. We present a case of a girl who developed this condition with classical clinico-radiologic findings of ANEC secondary to severe dengue infection and could not survive. We report this case with the aim to raise awareness about this fatal complication of dengue infection as dengue has become a global health-care problem

Clinical profile and outcome of children admitted with status epileptics in picu of a developing country

Status epilepticus (SE) is a common, life-threatening neurologic disorder. The exact incidence is not known. The frequency of occurrence of status epilepticus is 17-23 per 100, 000 persons per year, with the higher incidences occurring in developing countries. In children the mortality from SE ranges from 3-10%. Objective: To describe the etiology, clinical profile and immediate-outcome of children with status epilepticus at a Tertiary Care Hospital in Karachi

Frequency and outcome of acute neurologic complications after congenital heart disease surgery

Objectives: To determine the frequency and immediate outcome of acute neurologic complications (ANCs) in children undergoing congenital heart surgery (CHS).Materials and Methods: Inthis retrospective study, all patients undergoing CHS at our hospital from January 2007 to June 2016 were included. Patients were followed up for the development of seizures, altered level of consciousness (ALOC), abnormal movements, and stroke. Results are presented as mean with standard deviation and frequency with percentages.Results: Of 2000 patients who underwent CHS at our center during the study, 35 patients (1.75%) developed ANC. Seizures occurred in 28 (80%), ALOC in 5 (14%), clinical stroke in 2, brain death in 6 patients. Antiepileptic drugs (AEDs) were started in 32 patients, of which 13 patients required more than one AED. Mean length of stay was 10 ± 7.36 days. Of 35 patients who developed ANC, 7 expired during the study.Conclusion: Neurological complications are scarce but significant morbidity after CHS at our center

Home Learning in Times of COVID: Experiences of Parents

The widespread prevalence of COVID-19 pandemic has affected academia and parents alike. Due to the sudden closure of schools, students are missing social interaction which is vital for better learning and grooming while most schools have started online classes. This has become a tough routine for the parents working online at home since they have to ensure their children’s education. The study presented was designed to explore the experiences of home learning in times of COVID-19. A descriptive qualitative study was planned to explore the experiences of parents about home learning and management during COVID-19 to get an insight into real-life experiences.  Purposive sampling technique was used for data collection.  Data were collected from 19 parents falling in the inclusion criteria. Considering the lockdown problem, the data were collected via Google docs form with open-ended questions related to COVID-19 and home learning. Three major themes emerged after the data analysis: impact of COVID on children learning; support given by schools; and strategies used by caregivers at home to support learning. It was analyzed that the entire nation and academicians around the world have come forward to support learning at home offering a wide range of free online avenues to support parents to facilitate home-learning. Furthermore, parents too have adapted quickly to address the learning gap that have emerged in their children’s learning in these challenging times. Measures should be adopted to provide essential learning skills to children at home. Centralized data dashboards and educational technology may be used to keep the students, parents and schools updated

Clinical profiles and outcomes of children admitted to the pediatric intensive care unit from the emergency department

The aim of this study was to describe clinical profiles and outcomes of children admitted directly from the Emergency Room (ER) to the Pediatric Intensive Care Unit (PICU) of academic hospital. The medical records of all children (1 month to 16 years) admitted in PICU from ER, from January 2011 to December 2012 were reviewed. Of the 26,774 patients seen in the ER during the study period, 468 (1.7%) were admitted to the PICU which constituted about 41.5% (468/1127) of all the total PICU admissions. Sixty three percent (n=294) were under-five; males were 60.9% (285), 82.3% (385) were in medical category. Neurological and respiratory illnesses were the most common groups (\u3e 50% of all ER admissions). Multi-organ dysfunction syndrome and co-morbidity were present in 25.2% (n=118) and 23.5% (n=110) respectively. The mean length of stay was 5 ± 3.7 hours. The case-fatality rate was 20.3% (n=95) as compared to the overall PICU mortality rate of 11.9% (n=135)

Neurodevelopment assessment of small for gestational age children in a community-based cohort from Pakistan

Background: Children born small for gestational age (SGA) may experience more long-term neurodevelopmental issues than those born appropriate for gestational age (AGA). This study aimed to assess differences in the neurodevelopment of children born SGA or AGA within a periurban community in Pakistan.Methods: This was a prospective cohort study in which study participants were followed from the pilot Doppler cohort study conducted in 2018. This pilot study aimed to develop a pregnancy risk stratification model using machine learning on fetal Dopplers. This project identified 119 newborns who were born SGA (2.4±0.4 kg) based on International Fetal and Newborn Growth Consortium standards. We assessed 180 children (90 SGA and 90 AGA) between 2 and 4 years of age (76% of follow-up rate) using the Malawi Developmental Assessment Tool (MDAT).Findings: Multivariable linear regression analysis comparing the absolute scores of MDAT showed significantly lower fine motor scores (β: -0.98; 95% CI -1.90 to -0.06) among SGAs, whereas comparing the z-scores using multivariable logistic regression, SGA children had three times higher odds of overall z-scores ≤-2 (OR: 3.78; 95% CI 1.20 to 11.89) as compared with AGA children.Interpretation: SGA exposure is associated with poor performance on overall MDAT, mainly due to changes in the fine motor domain in young children. The scores on the other domains (gross motor, language and social) were also lower among SGAs; however, none of these reached statistical significance. There is a need to design follow-up studies to assess the impact of SGA on child\u27s neurodevelopmental trajectory and school performance

Assessing health-related quality of life, morbidity, and survival status for individuals with down syndrome in Pakistan (DS-Pak): Protocol for a web-based collaborative registry

Background: Down syndrome is the most common chromosomal disorder, with a global incidence of 1 in 700 live births. However, the true prevalence, associated morbidities, and health-related quality of life (HRQOL) of these individuals and their families are not well documented, especially in low- and middle-income countries such as Pakistan. Disease-specific documentation in the form of a collaborative registry is required to better understand this condition and the associated health outcomes. This protocol paper describes the aims and processes for developing the first comprehensive, web-based collaborative registry for Down syndrome in a Pakistani cohort.Objective: This study aims to assess the HRQOL, long-term survival, and morbidity of individuals with Down syndrome by using a web-based collaborative registry.Methods: The registry data collection will be conducted at the Aga Khan University Hospital and at the Karachi Down Syndrome Program. Data will be collected by in-person interviews or virtually via telephone or video interviews. Participants of any age and sex with Down syndrome (trisomy 21) will be recruited. After receiving informed consent and assent, a series of tablet-based questionnaires will be administered. The questionnaires aim to assess the sociodemographic background, clinical status, and HRQOL of the participants and their families. Data will be uploaded to a secure cloud server to allow for real-time access to participant responses by the clinicians to plan prompt interventions. Patient safety and confidentiality will be maintained by using multilayer encryption and unique coded patient identifiers. The collected data will be analyzed using IBM SPSS Statistics for Windows, Version 22.0 (IBM Corporation), with the mean and SD of continuous variables being reported. Categorical variables will be analyzed with their percentages being reported and with a P value cutoff of .05. Multivariate regression analysis will be conducted to identify predictors related to the HRQOL in patients with Down syndrome. Survival analysis will be reported using the Kaplan-Meier survival curves.Results: The web-based questionnaire is currently being finalized before the commencement of pilot testing. This project has not received funding at the moment (ethical review committee approval reference ID: 2020-3582-11145).Conclusions: This registry will allow for a comprehensive understanding of Down syndrome in low- and middle-income countries. This can provide the opportunity for data-informed interventions, which are tailored to the specific needs of this patient population and their families. Although this web-based registry is a proof of concept, it has the potential to be expanded to national, regional, and international levels.International registered report identifier (irrid): PRR1-10.2196/24901

Bioethanol production from urban cellulosic waste employing Alcaligenes faecalis HI-1 isolated from gut of termite Heterotermes indicola

This study assessed the potential of termite gut inhabiting bacteria towards bioconversion of cellulosic waste into biofuel. Total seven bacterial isolates from the gut of Heterotermes indicola were isolated. Among all the isolates, HI-1 produced the largest zone upon primary screening.  Untreated paper had more cellulose content (73.03%) than acid (0.5%) treated paper that was used as a lignocellulosic substrate for saccharification. Among all the isolates tested, glucose yield (1.08mg/mL) was high for HI-1 isolate. Several factors were considered for optimizing augmented glucose yield (8.57mg/mL) and growth (8.07×108cfu/mL), such as temperature 37°C, pH 4.5, 5% (w/v) substrate concentration, 6 % bacterial inoculum size, agitation 150 rpm with PEG 0.25 % and Ca2+ ions 0.002 g/L. Overall 8-fold increase in glucose yield was achieved. Enzyme activity of HI-1 showed higher endoglucanase 0.29 ± 0.01 (U/mL/min) and exoglucanase 0.15±0.01 (U/mL/min) activity under optimum conditions, mentioned above. temperature 37°C, pH 4.5, substrate concentration 5%, inoculum size 6%, surfactants PEG 0.01%, ions Ca2+(0.002g/L) and agitation (120 rpm). Simultaneous saccharification and fermentation (SSF) of hydrolyzed office paper yielded 5.43mg/mL bioethanol. According to 16S rRNA sequence homology, the bacterial isolate H1 was identified as Alcaligenes faecalis. Bioethanol production from office paper untreated waste proved an effective strategy. Bacteria having natural tendency towards cellulosic waste consumption are promising for bioconversion of cellulosic waste to valuable products

Bioactive Metabolites from the Mariana Trench Sediment-Derived Fungus Penicillium sp. SY2107

Mariana Trench sediments are enriched in microorganisms, however, the structures and bioactivities of their secondary metabolites are not very known. In this study, a fungus Penicillium sp. SY2107 was isolated from a sample of Mariana Trench sediment collected at a depth of 11000 m and an extract prepared from the culture of this fungus in rice medium showed antimicrobial activities. Chemical investigation on this active extract led to the isolation of 16 compounds, including one novel meroterpenoid, named andrastone C. Structure of the new compound was elucidated based on high-resolution electrospray ionization mass spectroscopy (HRESIMS) data, extensive nuclear magnetic resonance (NMR) spectroscopic analyses and a single crystal X-ray diffraction. The crystal structure of a known meroterpenoid andrastone B was also reported in this study. Both andrastones B and C exhibited antimicrobial activities against methicillin-resistant Staphylococcus aureus (MRSA), Escherichia coli, and Candida albicans with minimum inhibitory concentration (MIC) values in a range from 6 to 13 μg/mL

Brown-vialetto-van laere syndrome: A novel diagnosis to a common presentation

Brown-Vialetto-Van Laere syndrome (BVVLS) or riboflavin transporter deficiency (OMIM 211530) is a rare treatable autosomal recessive neurodegenerative disorder. This condition is associated with progressive pontobulbar palsy. We describe the clinical course of a 16-month-old boy with BVVLS and a novel homozygous mutation from Pakistan. Our patient presented with stridor and respiratory insufficiency. Hearing loss which is the most common sign of this condition was absent, making it an unusual presentation of BVVLS. His examination revealed ptosis and tongue fasciculation. His riboflavin receptor mutational analysis showed the homozygous mutation in the SLC52A3 gene. Per oral riboflavin was administered, and subsequently, he was able to be weaned off the ventilator. Now the child is improving and attaining developmental milestones