Hemimegalencephaly: Seizure outcome in an infant after hemispherectomy

Isolated hemimegalencephaly (iHME) is a rare form of congenital malformation of cortical development characterized by enlargement of all or part of one cerebral hemisphere. It typically presents with intractable seizures, developmental delay, contralateral hemiparesis, and hemianopia. We report a case of an 18-month-old girl who presented first with focal seizures at tenth day of life, with no other physical or behavioral abnormality. Initial EEG showed excessive sharp EEG transients more over the right hemisphere; repeated EEG showed spikes, polyspikes, sharps, and slow wave discharges predominately over the right hemisphere. MRI of the brain showed asymmetric enlargement of the right cerebral hemisphere, suggestive of hemimegalencephaly. Initial treatment with antiepileptic drugs (AEDs) was successful in controlling her seizures, but later on the seizures became intractable even on polytherapy of AEDs; finally, functional hemispherectomy was performed and she became seizure free. Identification of this and similar cases of iHME can help us to better understand this disorder and eventually to provide better treatment options for it

Use of N-acetylcysteine in children with fulminant hepatic failure caused by acute viral hepatitis

Objective: To determine the efficacy of N-acetylcysteine (NAC) in children aged \u3e 1 month to 16 years admitted with Fulminant Hepatic Failure (FHF) secondary to Acute Viral Hepatitis (AVH) in a tertiary care center of a developing country. Study Design: Analytical study. Place and Duration of Study: Department of Paediatrics, The Aga Khan University Hospital, Karachi, Pakistan, from January 2007 to December 2011. Methodology: Medical records of children (\u3e 1 month - 16 years) with FHF admitted with AVH of known etiology who received NAC were reviewed retrospectively. Liver function tests (mean ± SD) at baseline, 24 hours after NAC and before or at the time of discharge/death were recorded and compared via using repeated measures ANOVA (r-ANOVA). Efficacy of NAC is defined in improvement in biochemical markers, liver function test and discharge disposition (survived or died). Mortality associated risk factors were identified by using logistic regression analysis. P-value and 95% confidence interval were recorded. Results: Forty children (mean age was 80 ± 40 months) with FHF secondary to AVH received NAC. Majority were males (n=25; 63%). Vomiting (75%) and jaundice (65%) were the main presenting symptoms, one-third had hypoglycemic, while 40% had altered sensorium at the time of admission. There was significant statistical difference in liver enzymes and prothrombin time on admission comparing at discharge in children received NAC (p \u3c 0.001). Fifteen (38%) children died. Severe vomiting {Odds Ratio (OR) 0.22, 95% Confidence Interval (CI) 0.05 - 0.8}, jaundice (OR 9.3, CI 1.1 - 82.6), inotropic support (OR 20.6, CI 3.5 - 118.3) and mechanical ventilation (OR 4.3, CI 1.1 - 16.6) at the time of admission are associated with risk factors for mortality in children with FHF secondary to AVH. Conclusion: NAC used in children with FHF secondary to AVH is associated with markedly improved liver function tests and recovery. FHF with complications is high risk for mortalit

Global PARITY: Study design for a multi-centered, international point prevalence study to estimate the burden of pediatric acute critical illness in resource-limited settings

Background: The burden of pediatric critical illness and resource utilization by children with critical illness in resource limited settings (RLS) are largely unknown. Without specific data that captures key aspects of critical illness, disease presentation, and resource utilization for pediatric populations in RLS, development of a contextual framework for appropriate, evidence-based interventions to guide allocation of limited but available resources is challenging. We present this methods paper which describes our efforts to determine the prevalence, etiology, hospital outcomes, and resource utilization associated with pediatric acute, critical illness in RLS globally. Methods: We will conduct a prospective, observational, multicenter, multinational point prevalence study in sixty-one participating RLS hospitals from North, Central and South America, Africa, Middle East and South Asia with four sampling time points over a 12-month period. Children aged 29 days to 14 years evaluated for acute illness or injury in an emergency department) or directly admitted to an inpatient unit will be enrolled and followed for hospital outcomes and resource utilization for the first seven days of hospitalization. The primary outcome will be prevalence of acute critical illness, which Global PARITY has defined as death within 48 hours of presentation to the hospital, including ED mortality; or admission/transfer to an HDU or ICU; or transfer to another institution for a higher level-of-care; or receiving critical care-level interventions (vasopressor infusion, invasive mechanical ventilation, non-invasive mechanical ventilation) regardless of location in the hospital, among children presenting to the hospital. Secondary outcomes include etiology of critical illness, in-hospital mortality, cause of death, resource utilization, length of hospital stay, and change in neurocognitive status. Data will be managed via REDCap, aggregated, and analyzed across sites. Discussion: This study is expected to address the current gap in understanding of the burden, etiology, resource utilization and outcomes associated with pediatric acute and critical illness in RLS. These data are crucial to inform future research and clinical management decisions and to improve global pediatric hospital outcomes

Acute symptomatic seizures in critically ill children: Frequency, etiology and outcomes

Background: Critically ill individuals have an increased risk of acute symptomatic seizures secondary to systemic illnesses; unrecognized or untreated seizures can quickly convert into status epilepticus, which is associated with high morbidity and mortality.Objective: The aim of this study was to determine frequency, etiology, and outcome of seizures in critical ill children admitted in intensive care unit of a tertiary care hospital.Materials and methods: Retrospective review of medical records of all children admitted in pediatric intensive care unit (PICU) of the Aga Khan University from January 2016 to December 2018 and who had a new-onset seizure irrespective of underlying diagnosis was carried out after ethical review committee approval. Data were collected on a structured proforma; it included demographic information as well as relevant clinical and outcome information. The data were analyzed on Statistical Package for the Social Sciences (SPSS) software program, version 19.0. The descriptive statistics frequency and percentage was computed for qualitative variable. Mean and standard deviation were computed for quantitative variable, and univariate analysis was performed.Results: During the study period, a total 2053 patients were admitted in the PICU. One hundred six (5%) had seizure. Sixty-three (59.5%) were males. Meningitis 21 (20%), sepsis 21 (20%), complicated pneumonia 18 (17%) were the major primary diagnosis in these children. Mean age of the study population was 75 months (standard deviation [SD] ± 54.4) and 72 (68%) were age, whereas 63 (59.5%) were males. The seizures lasted \u3e10min in 10 (10%) and were associated with high had neurological deficit (P = 0.001). We did not observe any correlation with electrolyte imbalance, renal failure, need of ventilator support with duration of seizure, and type of seizure (P \u3e 0.005).Conclusion: Infection was the most common etiology associated with a new-onset seizure in children admitted in our PICU. Seizures lasting for \u3e10min were observed with high neurological deficit. We did not find any association of mortality with seizure duration

High flow nasal cannula therapy in children with acute respiratory insufficiency in the pediatric intensive care unit of a resource-limited country: A preliminary experience

A retrospective study was performed in children (aged one month - 16 years) receiving HFNC to determine the frequency, efficacy and adverse effects of high flow nasal cannula (HFNC) therapy in the pediatric intensive care unit (PICU), from January to December 2017. Treatment failure was defined as clinical deterioration on HFNC therapy such that mechanical ventilation (MV) was required. Clinical parameters before and after HFNC were assessed using repeated measures analysis of variance. A total of 120 patients received HFNC therapy (21% of total admissions). Primary diagnosis were respiratory disease (50%), central nervous system diseases (14.2%), sepsis (10.8%), and postoperative care (10%). Mean duration of HFNC was 27.5 ±19.7 hours and mean PICU length of stay was 6 ± 6 days. Pneumothorax developed in four patients. MV was required in 28 patients, and subsequently, 15 deaths occurred in that group. HFNC is a frequently used, safe and effective therapy for children requiring respiratory support in PICU. Key Words: Respiratory insufficiency, Non-invasive ventilation, Paediatric intensive care unit

Assessment of Risk-Taking Behaviour of Young Motorcyclists at Un-Signalised Intersections – A Partial Least Square Structural Equation Modelling Approach

At un-signalised at-grade intersections or roundabouts, motorcyclists have to make a quick decision to manoeuvre and avoid crash. Many studies show that risk-taking behaviour is the major cause of accidents in young motorcyclists. In this study, we analysed various factors that are involved in the risk-taking behaviour of young motorcyclists at un-signalised intersections. Online questionnaires were distributed among university and college students in Islamabad and Rawalpindi. The data of 490 respondents were collected to test the research model. Partial least square structural equation modelling approach was used to evaluate the measurement model, structural model and importance-performance map analysis. In this study, we assumed that risk-taking behaviour of young motorcyclists at un-signalised intersections could be influenced by several factors, i.e., demographic, past crash involvement, and peer influence. The results revealed that past crash involvement, confidence level, and peer influence were the significant factors that affect the risk-taking behaviour. Peer influence has the highest effect on the risk-taking behaviour. The person whose friends encourage them to take risk and accept challenges is more likely to exhibit the risk-taking behaviour. Those people who are more confident while riding a motorcycle are more likely to take risks

Spectrum of complications of severe DKA in children in pediatric intensive care unit

Objectives: To describe the spectrum of complications of Diabetic Ketoacidosis (DKA) observed in children admitted with severe DKA.Methods: Retrospective review of the medical records of all children admitted with the diagnosis of severe DKA in Pediatric Intensive Care Unit (PICU) of the Aga Khan University Hospital, from January 2010 to December 2015 was done. Data was collected on a structured proforma and descriptive statistics were applied.Results: Total 37 children were admitted with complicated DKA (1.9% of total PICU admission with 1.8% in 2010 and 3.4% in 2015). Mean age of study population was 8.1±4.6 years and 70% were females (26/37). Mean Prism III score was 9.4±6, mean GCS on presentation was 11±3.8 and mean lowest pH was 7.00±0.15. Complications observed included hyperchloremia (35.94%), hypokalemia (30.81%), hyponatremia (26.70%), cerebral edema (16.43%), shock (13.35%), acute kidney injury (10.27%), arrhythmias (3.8%), and thrombotic thrombocytopenic purpura (5.4%), while one patient had myocarditis and ARDS each. 13/37 children (35%) needed inotropic support, 11/37 (30%) required mechanical ventilation while only one patient required renal replacement therapy. Two patients (5.4%) died during their PICU stay.Conclusions: Hyperchloremia and other electrolyte abnormalities, cerebral edema and AKI are the most common complications of severe DKA

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