Chin Seng Gan

Noninvasive ventilation in a tertiary pediatric intensive care unit in a middle-income country

Objective: To determine the factors that predict outcome of noninvasive ventilation (NIV) in critically ill children. Design: Prospective observational study. Setting: Multidisciplinary pediatric intensive care unit of a university hospital in Malaysia. Patients: Patients admitted to the pediatric intensive care unit from July 2004 to December 2006 for respiratory support due to acute respiratory failure and those extubated from invasive mechanical ventilation. Interventions: NIV was used as an alternative means of respiratory support for all children. In patients who had prior invasive mechanical ventilation, NIV was used to facilitate extubation, or it was used after a failed extubation. The children were assigned to the nonresponders group (intubation was needed) or responders group (intubation was avoided totally or for at least 5 days). The physiologic variables were monitored before, at 6 hrs, and 24 hrs of NIV. Measurements and Main Results: Of 278 patients, 129 were admissions for management of acute respiratory failure and 149 patients received NIV to facilitate extubation (n = 98) or for a failed extubation (n = 48). Their median age and weight were 8.7 months (interquartile range, 3.1–33.1 months) and 5.5 kg (interquartile range, 3.3–10.8 kg), respectively. Intubation was avoided for >5 days in 79.1% (n = 220). No significant difference in age or weight of responders and nonresponders was observed. The cardiorespiratory variables in all patients improved, but significant differences between the two groups were noted at 6 hrs and 24 hrs after NIV. Conclusions: NIV was a feasible strategy of respiratory support to avoid intubation in >75% of children in this study. A higher Pediatric Risk of Mortality II score, sepsis at initiation of NIV, an abnormal respiratory rate, and a higher requirement of Fio2 may be predictive factors of NIV failure.

Thirteen years of invasive and noninvasive home ventilation for children in a developing country: A retrospective study.

Home ventilation (HV) for children is growing rapidly worldwide. The aim was to describe (1) the sociodemographic characteristics of children on HV and (2) the indications for, means and outcome of initiating HV in children from a developing country.

Risk Stratification in Pediatric Acute Respiratory Distress Syndrome: A Multicenter Observational Study*

Objectives: The Pediatric Acute Lung Injury Consensus Conference developed a pediatric specific definition for acute respiratory distress syndrome (PARDS). In this definition, severity of lung disease is stratified into mild, moderate, and severe groups. We aim to describe the epidemiology of patients with PARDS across Asia and evaluate whether the Pediatric Acute Lung Injury Consensus Conference risk stratification accurately predicts outcome in PARDS. Design: A multicenter, retrospective, descriptive cohort study. Setting: Ten multidisciplinary PICUs in Asia. Patients: All mechanically ventilated children meeting the Pediatric Acute Lung Injury Consensus Conference criteria for PARDS between 2009 and 2015. Interventions: None. Measurements and Main Results: Data on epidemiology, ventilation, adjunct therapies, and clinical outcomes were collected. Patients were followed for 100 days post diagnosis of PARDS. A total of 373 patients were included. There were 89 (23.9%), 149 (39.9%), and 135 (36.2%) patients with mild, moderate, and severe PARDS, respectively. The most common risk factor for PARDS was pneumonia/lower respiratory tract infection (309 [82.8%]). Higher category of severity of PARDS was associated with lower ventilator-free days (22 [17–25], 16 [0–23], 6 [0–19]; p < 0.001 for mild, moderate, and severe, respectively) and PICU free days (19 [11–24], 15 [0–22], 5 [0–20]; p < 0.001 for mild, moderate, and severe, respectively). Overall PICU mortality for PARDS was 113 of 373 (30.3%), and 100-day mortality was 126 of 317 (39.7%). After adjusting for site, presence of comorbidities and severity of illness in the multivariate Cox proportional hazard regression model, patients with moderate (hazard ratio, 1.88 [95% CI, 1.03–3.45]; p = 0.039) and severe PARDS (hazard ratio, 3.18 [95% CI, 1.68, 6.02]; p < 0.001) had higher risk of mortality compared with those with mild PARDS. Conclusions: Mortality from PARDS is high in Asia. The Pediatric Acute Lung Injury Consensus Conference definition of PARDS is a useful tool for risk stratification.

Sulthiame-induced drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome

BACKGROUND Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare and potentially life-threatening acute drug-induced hypersensitivity reaction. Antiepileptic drugs (AEDs) predominantly aromatic AEDs are commonly reported in DRESS. To date there are no reports of sulthiame AED causing DRESS syndrome. METHOD We report a 10-year-old girl of Indian descent with AED resistant epilepsy on maintenance sodium valproate and clonazepam. Sulthiame AED was initiated to try to improve her seizure control. Five weeks after commencing sulthiame, she developed fever with a diffuse erythematous morbilliform maculopapular rash, elevated transaminases and atypical lymphocytes. At day 3 of illness, she deteriorated with worsening elevation of liver transaminases, thrombocytopenia, progression of rash, hepatosplenomegaly, pneumonitis and markedly elevated inflammatory markers. Immunomodulatory treatment of pulse methylprednisolone was given from day 7 which was associated with improvement inflammatory markers and complete resolution of rash from day 30 of illness. RESULTS The diagnosis of sulthiame-induced DRESS syndrome was made based on clinical, laboratory and skin histology findings. She was HLA-B heterozygous for HLA-B∗15:123 and 15:240 and HLA-A homozygous for HLA-A∗11:01:09. Both these HLA-A and HLA-B typing has not been reported before in cutaneous drug reactions. CONCLUSION This is the first reported case of sulthiame-induced DRESS syndrome. Our case expands the list of possible susceptible HLA alleles associated with cutaneous drug reactions. It also raises the awareness of possible DRESS syndrome among patients commenced on sulthiame who will require immediate discontinuation of sulthiame and consideration of prompt treatment of corticosteroids.

Impact of infection control training for interns on PICU-acquired bloodstream infections in a middle-income country.

INTRODUCTION The present study aimed to determine the impact of an extended infection control training programme, which was conducted for all interns posted to the Department of Paediatrics, on the incidence of paediatric intensive care unit (PICU)-acquired bloodstream infections (BSIs) in University Malaya Medical Centre, Malaysia. METHODS The development of nosocomial BSIs during the baseline period (1 January-31 October 2008) and intervention period (1 November-31 December 2009) was monitored. During the intervention period, all paediatric interns underwent training in hand hygiene and aseptic techniques for accessing vascular catheters. RESULTS A total of 25 patients had PICU-acquired BSIs during the baseline period, while 18 patients had PICU-acquired BSIs during the intervention period (i.e. infection rate of 88 per 1,000 and 41 per 1,000 admissions, respectively). The infections were related to central venous catheters (CVCs) in 22 of the 25 patients who had PICU-acquired BSIs during the baseline period and 11 of the 18 patients who had PICU-acquired BSIs during the intervention period. Thus, the incidence rates of catheter-related BSIs were 25.2 per 1,000 CVC-days and 9.3 per 1,000 CVC-days, respectively (p < 0.05). The Paediatric Risk of Standardised Mortality III score was an independent risk factor for PICU-acquired BSIs and the intervention significantly reduced this risk. CONCLUSION The education of medical interns on infection control, a relatively low-cost intervention, resulted in a substantial reduction in the incidence of PICU-acquired BSIs.

3-Methylglutaconic aciduria, a frequent but underrecognized finding in carbamoyl phosphate synthetase I deficiency

The urea cycle disorder carbamoyl phosphate synthetase I deficiency is an important differential diagnosis in the encephalopathic neonate. This intoxication type inborn error of metabolism often leads to neonatal death or severe and irreversible damage of the central nervous system, even despite appropriate treatment. Timely diagnosis is crucial, but can be difficult on routine metabolite level. Here, we report ten neonates from eight families (finally) diagnosed with CPS1 deficiency at three tertiary metabolic centres. In seven of them the laboratory findings were dominated by significantly elevated urinary 3-methylglutaconic acid levels which complicated the diagnostic process. Our findings are both important for the differential diagnosis of patients with urea cycle disorders and also broaden the differential diagnosis of hyperammonemia associated with 3-methylglutaconic aciduria, which was earlier only reported in TMEM70 and SERAC1 defect.

Abstract O-40: HIGH FREQUENCY VENTILATION IN PEDIATRIC ACUTE RESPIRATORY DISTRESS SYNDROME; A PROPENSITY SCORE ADJUSTED STUDY

HIGH FREQUENCY VENTILATION IN PEDIATRIC ACUTE RESPIRATORY DISTRESS SYNDROME; A PROPENSITY SCORE ADJUSTED STUDY J.J.M. Wong1, S. Liu2, S. Qian3, J.S.M. Ong4, C.S. Gan5, N. Anantasit6, Y.K. Chor7, R. Samransamruajkit8, H.P. Phan9, S. Phumeetham10, X. Feng11, M. Feng2, J.H. Lee1 1KK Women’s and Children’s Hospital, Pediatric Medicine, Singapore, Singapore 2National University of Singapore, Saw Swee Hock School of Public Health, Singapore, Singapore 3Beijing Children’s Hospital, Pediatric Intensive Care Unit, Beijing, China 4National University of Singapore, Pediatric Intensive Care Unit, Singapore, Singapore 5University Malaya Medical Centre, Department of Pediatrics, Kuala Lumpur, Malaysia 6Ramathibodi Hospital, Pediatric Department, Bangkok, Thailand 7Sarawak General Hospital, Department of Pediatrics, Kuching, Malaysia 8King Chulalongkorn Memorial Hospital, Department of Pediatrics, Bangkok, Thailand 9National Children’s Hospital, Pediatric Intensive Care Unit, Hanoi, Vietnam 10Siriraj Hospital, Department of Pediatrics, Bangkok, Thailand 11Children’s Hospital of Chongqing Medical University, Pediatric Intensive Care Unit, Chongqing, China

Bickerstaff’s brainstem encephalitis with overlapping Guillain-Barré syndrome: Usefulness of sequential nerve conduction studies

Bickerstaffs brainstem encephalitis (BBE) is a rare immune-mediated disorder characterized by ophthalmoplegia, ataxia and disturbance of consciousness, which may overlap with Guillain-Barré syndrome (GBS) if there is additional limb weakness. We report a 7-month-old boy presented with ophthalmoplegia followed by a rapidly ascending paralysis of all four limbs and disturbance of consciousness. The initial impression was BBE with overlapping GBS. This was supported by sequential nerve conduction study (NCS) findings compatible with an acute inflammatory demyelinating polyneuropathy (AIDP). He received intravenous pulse methylprednisolone, intravenous immunoglobulin and plasmapharesis with complete clinical recovery after 6 weeks of illness and improved NCS findings from week 16. This is the first case of paediatric BBE with overlapping GBS with an AIDP subtype of GBS. It expands the clinical spectrum of this condition in children. Our case highlights the importance of sequential NCS in paediatric BBE with overlapping GBS for accurate electrophysiological diagnosis and prognosis particularly if the first NCS findings are not informative.

Diphtheric encephalitis and brain neuroimaging features

We report a rare case of paediatric diphtheria complicated with encephalitis. A 6-year-old boy who did not receive his scheduled diptheria-tetanus-pertusis vaccination presented with one episode of generalised convulsive seizure. His illness was preceded by a 3day history of fever associated with enlarged exudative tonsils with a pseudomembrane. He was commenced on intravenous penicillin and oral erythromycin. However, he developed progressive encephalopathy with focal neurological deficit which required intubation on day 5 of illness. Throat swab polymerase chain reaction for diphtheria toxin A and B were positive and diphtheria antitoxin was given. Magnetic resonance imaging (MRI) of brain showed T2-weighted hyperintensities over the anterior cingulate gyri, insular cortex and cerebellum. This is the first reported MRI finding of diphtheric encephalitis. Our report highlights the importance of neuroimaging in diagnosing diphtheric encephalitis particularly in cases with unremarkable cerebrospinal findings.