Lunliya Thampratankul

Sleep Disordered Breathing in Young Boys with Duchenne Muscular Dystrophy

OBJECTIVES To describe sleep-disordered breathing (SDB) in young boys with Duchenne muscular dystrophy (DMD) and its relationship with pulmonary function tests (PFTs). STUDY DESIGN This retrospective study examined diagnostic polysomnogram and PFT data of boys younger than 18 years with DMD and treated with steroids. Spirometry, respiratory muscle strength, body mass index (BMI), sleep architecture variables, and indices of SDB were analyzed. We examined the effect of PFT measures on the risk of each type of respiratory event using logistic regression and have reported results as OR (95% CI). RESULTS Subjects included 110 boys with DMD, mean age 11.5 (5.6-17.9) years. Mean (±SD) percent forced vital capacity predicted was 79.5% ± 29.1%. Mean BMI for all subjects was 21.9 ± 7.0 kg/m(2), and mean BMI z-score was 0.65 ± 1.93. Seventy (63.6%) subjects had obstructive sleep apnea; 37 (33.6%) subjects had central sleep apnea; 18 (17%) subjects had hypoventilation. Median (IQR) Apnea Hypopnea Index was 2.9 (1.6-6.9) and median Obstructive Index was 1.5 (0.5-3.8). Obstructive Index during rapid eye movement sleep positively correlated with BMI (r = 0.33, P = .002), BMI z-score (r = 0.22, P = .04), and age (r = 0.31, P = .004). Lower forced vital capacity was associated with increased risk of hypoventilation (OR 0.8, P = .001). CONCLUSION SDB is common in young boys with DMD treated with steroids. It is manifest with rapid eye movement-obstructive sleep apnea, often severe, and strongly influenced by BMI.

Acute disseminated encephalomyelitis: A 10-year cohort study in Thai children

Childhood acute disseminated encephalomyelitis (ADEM) is a demyelinating disease with variable clinical courses and outcomes. Its evolution to multiple sclerosis in Asian children is yet to be determined. Medical records, investigation results and magnetic resonance imaging of brain of Thai children aged less than 15 years with initial diagnosis of ADEM at a referral university hospital in Thailand from January 1997 to December 2006 were reviewed. Clinical course and the outcome were finalized by telephone interview, self-report questionnaire, and/or neurological examination by December 2008. Modified Rankin Score was applied for determination of disability. MRI findings were categorized along with the locations and number of areas of abnormalities shown by T2-weight and FLAIR. 16 patients consisting of 5 boys and 11 girls (age-range 1-14 years, mean 6.9 ± 3.6 years, median 6 years) were identified. Nine patients had cranial nerve dysfunctions including one child with optic neuropathy. One patient died with confirmed pathological diagnosis of ADEM. Among the remaining 15, who were followed from 2 to 10 years (mean 5.8 years), 13 and 3 patients were classified into monophasic ADEM and multiple sclerosis, respectively. Ten of 13 with final diagnosis of ADEM had complete recovery. There was no association between number of lesions or location in the initial MRI and the outcome and final diagnosis. ADEM in Thai children had similar clinical presentation and outcome to previous studies in Western countries. ADEM can occasionally evolve to multiple sclerosis in Thai children as being shown in previous reports from other Asian countries.

Migraine in junior high-school students: A prospective 3-academic-year cohort study

Migraine is a common childhood illness with expected favorable outcome. A study of the long-term clinical course of childhood migraine will provide information of evolution of migraine. A cohort study for 3-academic-year was conducted in Thai junior high-school children from July 2005 to February 2008 to determine the clinical course of migraine. Two hundred and forty-eight students in four junior high schools diagnosed with migraine according to ICHD-II in July 2005 were recruited. Each student was serially evaluated twice yearly from 7th grade during each semester of the academic year until the second semester of 9th grade. Determination of the characteristics, severity, frequency, and treatment of headache were obtained by questionnaire and direct interview. At the final evaluation, clinical course of headache was categorized into seven patterns. Among enrolled students, 209 (84.3%) completed the study. Twenty-eight (13.5%) students had no recurrent headache while that of 153 (73.5%) improved. No improvement of migraine and worsened migraine were observed in four students (1.8%) and 24 students (11.2%), respectively. Spontaneous remission and avoidance of precipitating causes contributed to relief of migraine in the majority of the students. Stress-related daily school activities and inadequate rest were reported as common precipitating factors among students with non-improving or worsening outcome. Chronic daily headache and tension-type headache was observed in 6 and 30 students, respectively. This study confirms that clinical course of migraine in schoolchildren is benign. Frequency and intensity of headache can be reduced with reassurance and appropriate guidance. Early recognition and appropriate prevention of migraine attack will decrease the risk of chronic migraine and disease burden.

Efficacy and Safety of Zonisamide in Thai Children and Adolescents With Intractable Seizures

This retrospective study examined the efficacy and safety of zonisamide for Thai children and adolescents with intractable seizures. The medical records of 24 patients (13 male, 11 female), aged 2 to 18 years (median 11.5, mean 10.4) who received zonisamide were reviewed. The underlying illness, etiology of epilepsy, seizure types, previous and concomitant antiepileptic drugs, dosage, and adverse effects of the drug were collected. Zonisamide’s efficacy was evaluated on the basis of seizure reduction rates. At final evaluation, 7 patients were still taking zonisamide from 4.7 to 10.3 mg/kg/d (median 8). One patient became seizure-free and the other 6 experienced favorable seizure control. The median duration of zonisamide therapy was 23.75 months (range 20.5-25 months). Minor adverse effects were reported in 41.6% of patients during the first 3 months of therapy. Zonisamide is an option for the treatment of intractable seizures with favorable seizure control in children and adolescents.

Intravenous levetiracetam in Thai children and adolescents with status epilepticus and acute repetitive seizures

BACKGROUND Intravenous levetiracetam is an option for treatment of status epilepticus (SE) and acute repetitive seizures (ARS). However, there have been relatively few studies with children and adolescents. Also, an appropriate dosage has yet to be determined. AIM This study investigated the safety and the efficacy of levetiracetam for intravenous treatment of convulsive status epilepticus and acute repetitive seizures in children and adolescents. METHOD Retrospectively, the study reviewed the medical records of 19 male and 31 female patients under 18 years of age who had received intravenous levetiracetam treatment either for acute repetitive seizures or for convulsive status epilepticus. The patients were admitted between April 1st, 2010 and December 31st, 2011 to the Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. Data were collected on underlying illnesses, etiology of seizures, indication for levetiracetam therapy, initial dosage, rate of infusion, untoward effects during infusion and emerged complications. Efficacy of treatment was defined as the termination of seizure within 30 min of completing levetiracetam infusion and no seizure recurrence within 6 h of initial treatment. RESULTS The age range of the 50 patients was from one day to 18 years (mean 79.6 months). The analysis included 52 episodes of 34 acute repetitive seizures (63.4%) and 18 convulsive status epilepticus (34.6%). Infusion rates ranged from 2 to 66 mg/kg/min (mean 29.6). Cessation of seizure was obtained in 59.6% of 52 episodes. Patients with underlying drug resistant epilepsy did not respond to levetiracetam therapy as well as patients with other etiology of seizures. There were no adverse drug reactions or untoward effects observed during the therapy. CONCLUSION Intravenous administration of levetiracetam is safe and effective for treatment of acute repetitive seizures and convulsive status epilepticus in children and adolescents. Failure of treatment may be related to underlying drug resistant epilepsy. Further study of appropriate initial dosage and pharmacokinetic variations in the patients is needed as possible explanation of the unresponsiveness.

Incidences, risk factors and outcomes of neonatal thromboembolism

Abstract Background: The incidences of thromboembolism (TE) in neonates were reported to be around 0.51 per 10,000 live births per year for overall TE and 24 per 10,000 NICU admissions per year. As the incidences of TE in children and adults are lower in Asian populations, the incidences, risk factors, and outcomes of neonatal TE may be different to those reports from other countries. Objectives: To determine the incidences, risk factors, and outcomes of neonatal TE in a tertiary care hospital in Thailand. Materials and methods: A retrospective study between the years 1998 and 2015. Results: From a total of 2463 neonatal admissions, 28 patients were diagnosed with TE. The female/male ratio was 1:1.2. The breakdown of diagnoses of neonatal TE were arterial ischemic stroke (AIS; 36%), arterial TE (ATE; 29%), deep vein thrombosis (DVT; 14%), cerebral venous sinus thrombosis (CVST; 11%), renal vein thrombosis (RVT; 3%), and purpura fulminans (2%). Underlying diseases were identified 57.1% of patients. The most common thrombophilic risk factor was protein C (PC) deficiency (14.3%). The overall mortality rate was 14.3%. Conclusion: The most common TE was AIS. PC deficiency was the most prevalent inherited risk factor, especially in neonates without precipitating factors.

Determination of plasma Levetiracetam level by Liquid Chromatography-Tandem Mass Spectrometry (LC-MS-MS) and its application in pharmacokinetics studies in neonates

BACKGROUND Levetiracetam (LEV) is an antiepileptic drug which has good safety and efficacy in neonatal seizure (NS), a common incident in neonates with weight <1500 g. The pharmacokinetics for LEV in neonatal populations is yet to be clearly understood. In this study, we developed and validated a method for determination of LEV in plasma by liquid chromatography tandem mass spectrometry for the purpose of pharmacokinetic study. METHODS Plasma LEV was spiked with Lamivudine as internal standard before extraction by C18 solid-phase extraction (SPE) cartridge. Chromatography was performed using isocratic elution with mobile phase A: B (10: 90) for 2.0 min with flow rate 0.4 mL/min. The mobile phase was composed of 0.1% formic acid in 10.0 mM ammonium acetate (A) and 100% methanol (B). The injection volume was 1.0 μL and the total run time was 2.0 min. Multiple reaction monitoring (MRM) with electro spray in positive mode was used. The mass transition for LEV was 171.2/126.0 and 230.0/112.0 for IS with retention time of 0.73 and 0.72 min, respectively. RESULTS A calibration curve range from 0.50-80.0 μg/mL was obtained with a correlation coefficient >0.99 in the quadratic model. Precision and accuracy was within the acceptable range and the intra- and inter-day %CV for three concentrations of QCs were <10%. CONCLUSION This method was reliable, accurate and applicable for LEV pharmacokinetic study in neonates with seizure.

P24 – 2535: Long-term outcome of West syndrome treated with vigabatrin

Objective To determine the long-term outcome of children with diagnosis with West syndrome and received treatment with vigabatrin. Methods A cross-sectional study was conducted. Medical records of all patients diagnosed with West Syndrome from January 1997 to December 2010 were reviewed to collect basic clinical information related to the diagnosis. Each patients clinical courses, latest neurodevelopment assessment, physical examination, ophthalmologic examination and IQ test were gathered of analysis. Fisher exact test and Wilcoxon rank-sum test were applied for determination of favorable prognostic factors. Results 100 patients were diagnosed with West syndrome during the study period. There were 81 patients (male 55.6% female 44.4%) received vigabatrin and were included into the study. Treatment with vigabatrin was commenced at the age of 1–19 months (mean 5 months). Duration of therapy ranged from 2 to72 months (mean 27.6 months). Follow-up duration ranged from 18 to 200 months (mean 94.2 months). Forty-four (54.3%), 19 (23.5%), and 18 (22.2%) patients responded, partially responded and did not respond to vigabatrin, respectively. Seven patients (8.6%) achieved seizure-free without any medication with seizure-free duration ranging from 6 to 16 years (mean 13 year). Fourteen patients (17.2%) were seizure-free with daily antiepileptic drug therapy. Their seizure-free duration was 3 to 14 years (mean 9.7 years). Twenty-one patients (28.4%) had other type of seizure after discontinue vigabatrin. Normal ophthalmologic examinations, which included Goldman perimetry examination, were obtained in 12 patients. IQ test (WISC III) disclosed normal and subnormal IQ in 11 and 10 patients respectively. Statistical analysis did not demonstrate significant correlation between gender, etiology, age of onset, duration prior to vigabatrin treatment and type of spasms to the favorable outcome (spasm-free without medication, normal IQ). Conclusion Vigabatrin could be considered for treatment of West syndrome with a favorable long-term outcome.

PP10.6 – 2751: Correlation between neuron-specific enolase with neurological outcome in non-traumatic comatose pediatric patients

Objective To determine clinical application of serum neuron-specific enolase (NSE) in predicting the outcome in comatose pediatric patients. Methods A prospective observational cohort study was conducted at university hospital in Thailand. Comatose patients aged from 1 month to 18 years, admitted from January 2012 to February 2013, were recruited. Serial measurements of serum NSE were performed at 0, 12 h, 24 h, 48 h, and 72 hours. Non-comatose pediatric patients were randomly selected for control-group. Determination of neurological outcome according to the Pediatrics Cerebral Performance Category (PCPC) was done after the onset of coma at 7 days, 1 month and 3 months. Results Thirty-nine comatose patients were enrolled. Nineteen patients were recruited into the control group. Poor outcomes (PCPC score 3–5) were observed in 27 and 26 patients at 7 days and 3 months after acute event, respectively. NSE level was significantly elevated (p Conclusion Serum NSE level measured at 72 hr, at the cut-off value for NSE (>33 ng/mL) was useful to determine poor outcome in comatose pediatrics patients. The cut-off level of 63 ng/mL provided high specificity in comparison to the cut-off level of 33 ng/mL.