Platelet Endothelial Cell Adhesion Molecule-1 Gene Polymorphisms are Associated with Coronary Artery Lesions in the Chronic Stage of Kawasaki Disease.

BACKGROUND: Kawasaki disease is the most common cause of pediatric acquired heart disease. The role of platelet endothelial cell adhesion molecule-1 in the inflammatory process has been documented. To date, no report has investigated the relationship between coronary artery lesions of Kawasaki disease and platelet endothelial cell adhesion molecule-1 polymorphisms. METHODS: A total of 114 Kawasaki disease children with coronary artery lesions and 185 Kawasaki disease children without coronary artery lesions were recruited in this study. The TaqMan assay was conducted to identify the genotype in this case-control study. RESULTS: In three single nucleotide polymorphisms (Leu125Val, Ser563Asn, and Arg670Gly) of platelet endothelial cell adhesion molecule-1, we found that the Leu-Ser-Arg haplotype was associated with a significantly increased risk for coronary artery lesions in the chronic stage (odds ratio 3.05, 95% confidence interval 1.06-8.80, p = 0.039), but not for coronary artery lesions in the acute stage. Analysis based on the diplotypes of platelet endothelial cell adhesion molecule-1 also showed that Kawasaki disease with one or two alleles of Leu-Ser-Arg had a significantly increased risk of chronic coronary artery lesions (odds ratio 3.38, 95% confidence interval 1.11-10.28, p = 0.032) and had increased platelet counts after Kawasaki disease was diagnosed, as compared to those with other diplotypes. CONCLUSIONS: The haplotype of platelet endothelial cell adhesion molecule-1 Leu-Ser-Arg might be associated with the increased platelet counts and the following risk of chronic coronary artery lesions in a dominant manner in Kawasaki disease.

Semi-Prone Position Can Influence Variability in Respiratory Rate of Premature Infants Using Nasal CPAP.

UNLABELLED: To determine the effect of positions (supine, lateral, semiprone) on the physiological parameters (HR, RR, SpO2) of premature infants receiving NCPAP who were non-oxygen-dependent and non-BPD, and to identify significant clinical changes associated with these variables. METHODS: A crossover study design with three different positions in the assigned sequence (supine-supine, supine-lateral and supine-semiprone) was used, and each position was maintained for 1h. The subjects' vital signs were recorded 30 min after initiation of each position and measured for 30 min. RESULTS: Forty-seven infants with a median GA of 28.6 weeks (range 26-35) were studied, and their median BW was 1210g (range 776-2920). Overall, position-related effects showed significant difference in the variability in RR (OR=0.68; CI 0.51-0.89), with the variability in RR being significantly lower in the semiprone position. The lateral position was associated with increased RR (B=2.9; p=0.02). Previous use of ventilator (PUV) was associated with increased HR, whereas BW and GA were negatively associated with higher HR. Cesarean birth, use of surfactant and PUV were associated with lower SpO2, whereas BW and GA were correlated with higher SpO2. GA was identified as a protective factor, while PUV was a risk factor for the variability in both HR and SpO2. CONCLUSIONS: Premature infants receiving NCPAP sleeping in the semiprone position may have more stable RR, while the lateral position did not improve RR. Thus, the semiprone and supine positions may be considered preferable when positioning the monitored premature infants with NCPAP.

Bone age assessment: Large-scale comparison of Greulich-Pyle method and Tanner-Whitehouse 3 method for Taiwanese children.

BACKGROUND: The Greulich-Pyle (GP) and Tanner-Whitehouse 3 (TW3) methods are two common methods for assessing bone age (BA). The applicability of these methods for populations other than those in the United States and Europe has been questioned. Thus, this study tested the applicability of these methods for Taiwanese children. METHODS: In total, 1476 radiographs (654 boys, 822 girls) were analyzed. A subset of 200 radiographs was evaluated to determine intrarater and interrater reliability and the time required to yield a BA assessment. BA was determined by two reviewers using the GP method and two of the TW3 methods (the Radial-Ulnar-Short bones [RUS] method and the carpals method [Carpal]). The GP and TW3 methods were directly compared using statistical techniques. A subgroup analysis by age was performed to compare BA and chronological age using a paired t test for each age group. RESULTS: The average times required to yield an assessment using the GP and TW3-RUS methods were 0.79 ± 0.14 and 3.01 ± 0.84 min (p < 0.001), respectively. Both the intrarater and interrater correlation coefficients were higher for the GP method (0.993, 0.992) than the TW3-RUS (0.985, 0.984) and TW3-Carpal (0.981, 0.973) methods. The correlation coefficient for the GP and TW3-RUS methods was highest in the pubertal stage (0.898 for boys and 0.909 for girls). The mean absolute deviations for the GP and TW3-RUS methods in the pubertal stage were 0.468 years (boys) and 0.496 years (girls). Both the GP and TW3-Carpal methods underestimated BA for boys in the prepubertal stage. Both the GP and TW3-RUS methods overestimated BA for girls in the pubertal and postpubertal stages. CONCLUSION: The GP and TW3-RUS methods exhibit strong agreement in the pubertal and postpubertal stages for both sexes. With appropriate adjustments based on Taiwanese data, both methods are applicable to our children.

Caregiving and positioning effects on preterm infant states over 24 hours in a neonatal unit in Taiwan.

In this prospective, descriptive study, we used a repeated-measures design to explore the 24-hour effects of caregiving and positioning on preterm infants' states and the factors associated with state changes. Thirty preterm infants (gestational age 27.6-36.1 weeks) were observed for 3 days in the neonatal intensive care unit to record six states: quiet sleep (QS), active sleep, transition, active awake, quiet awake, and fussy or crying. The occurrences of QS increased when infants received no caregiving, social interaction, non-nutritive sucking (NNS), and were laterally positioned. However, QS significantly decreased and fussy or crying state increased when infants received routine and intrusive caregiving. These results suggest that caregiving, NNS, and positioning should be appropriately provided to facilitate infants' sleep, and reduce fussiness or crying.

Applicability of the Greulich and Pyle bone age standards to Taiwanese children: A Taipei experience.

BACKGROUND: The Greulich and Pyle (GP) method is one of the most common radiographic techniques for bone age (BA) assessment. The applicability of this method to ethnic populations outside of the United States has been investigated in several recent studies worldwide. Currently, limited data are available on the accuracy of the GP method for the Taiwanese population. The purpose of this study was to determine whether the GP standards are applicable to contemporary Taipei children. METHODS: Clinical data from October 1, 2010, to March 31, 2020, were retrospectively collected from a general hospital in Taipei. BA was determined by a senior pediatrician and was reviewed by a senior pediatric radiologist according to the GP standards. Comparison of BA and chronological age (CA) was performed in children with body weight and height in the 15th to the 85th percentiles of normal children. Ethnic variations in the maturation process in the ulnar bone were investigated. All data were statistically analyzed. RESULTS: In total, 2465 medical records were collected. After excluding those with diseases and unqualified data, 654 records of boys and 809 of girls were analyzed. In boys, the mean BA was significantly delayed between 6 and 9 years of age compared with the CA. In girls, the mean BA was generally advanced between 7 and 15 years of age. Ulnar bone maturation tended to be delayed in young boys. CONCLUSION: A significant discrepancy between CA and BA was observed in our population. Delayed ulnar bone maturation in young boys was confirmed. Children in Taipei exhibit a different maturation pattern than children on whom the GP standards were based.

IL-10 polymorphisms are associated with coronary artery lesions in acute stage of Kawasaki disease.

BACKGROUND: The literature regarding interleukin (IL)-10 polymorphisms and coronary artery lesions (CALs) in Kawasaki disease (KD) is limited. We investigated whether 3 IL-10 genetic polymorphisms (-1082 A/G, -819 T/C, and -592 A/C) are associated with development of CALs in KD. METHODS AND RESULTS: The genotyping of IL-10 polymorphisms was conducted for 279 KD children (172 without and 107 with CALs in acute stage). Thirty-three patients had CALs in chronic stage and 74 only with transient CALs. The homozygous variant genotype CC of IL-10-819 and IL-10-592 was associated with 80% (P=0.006) and 79% (P=0.008) reduction in risk of CALs in acute stage, respectively. The C allele of IL-10-819 and IL-10-592 was associated with 34% (P=0.034) and 33% (P=0.044) reduction in risk of CALs in acute stage, respectively. Compared with ATA haplotype (adjusted odds ratio (AOR) 0.63, P=0.029) or non-ACC haplotype (AOR 0.64, P=0.033), ACC haplotype was associated with a significantly reduced risk for CALs in acute stage, but not for CALs in chronic stage. Compared with non-ATA haplotype (AOR 1.53, P=0.034), ATA haplotype was associated with a significantly increased risk of CALs, except for CALs in the chronic stage. CONCLUSIONS: The effects of IL-10 gene polymorphism on CALs in acute KD are important. The persistence of CALs in chronic stage depends much more on other factors such as the times of intravenous immunoglobulin treatment.

Recent pubertal timing trends in Northern Taiwanese children: Comparison with skeletal maturity.

BACKGROUND: International studies have reported an early age of onset of puberty in girls and boys. However, the current situation of puberty onset in Taiwanese children is unknown. In this study, the timing of menarche and pubertal change in testicular volume (TV) in Taiwanese children was examined, and bone age (BA) was used as an internal somatic maturity scale and compared with the chronological age (CA) at pubertal timing. METHODS: Clinical data from October 1, 2010, to March 31, 2018, were retrospectively collected from a general hospital in Taipei. The data of patients who were diagnosed with endocrine/genetic disorders were excluded. Clinical data included CA, timing of menarche, and X-ray images of TV and BA. BA was determined by a senior pediatrician and a senior pediatric radiologist. The reliability and validity of BA readings were tested. Collected data were analyzed statistically. RESULTS: Overall, TV records of 241 boys and the menarche timing data of 98 girls were collected from 1823 children. CA for menarche was 11.35 ± 1.06 years (mean ± SD), and BA for menarche was 12.95 ± 0.80 years. CA and BA at TV = 15 mL in male puberty was 12.32 ± 1.22 and 13.46 ± 0.68 years, respectively. A stronger correlation was observed between TV and BA than between TV and CA during the pubertal period. CONCLUSION: The secular trend of earlier puberty timing continues. The decline rate of menarche timing was approximately 0.43 years per decade in the past 30 years. Among boys, an advance of more than 1 year in pubertal timing age was observed over the past 20 years. BA and TV showed high correlation during puberty.

Determinants of airleak flow during humming V high-frequency oscillatory ventilation in an open-compartment ex-vivo model of airleak.

High-frequency oscillatory ventilation (HFOV) using small tidal volumes and maintaining sufficient end-expiratory lung volume may be beneficial in the treatment of airleak. However, few published guidelines exist to advise clinicians on appropriate ventilator settings in this clinical scenario. The present experiment aimed to determine the effect of frequency, stroke volume (SV) and mean airway pressure (MAP) on airleak from an isolated lung model ventilated with a Humming V HFOV. We performed a crossover non-randomized experiment using the repeated measurement method to test the hypothesis that MAP is the major determinate for airleak. The lungs of 13 healthy juvenile New Zealand white rabbits were isolated and ventilated with high peak pressure to create airleak. The dataset obtained was analyzed using the generalized estimating equation method. We found that airleak flow did not change as frequency was raised from 13 to 17 Hz (P = 0.463) with MAP and SV kept constant. SV was positively correlated to the amount of change in airleak (P < 0.01, coefficients +/- SEM = 1.2 +/- 0.1 ml/min/ml). Leakage flow increased significantly from 275 +/- 168 ml/min to 1,721 +/- 552 ml/min as MAP was increased from 5 cm H(2)O to 30 cm H(2)O (P < 0.001, coefficients +/- SEM = 56.1 +/- 3.0 ml/min/cm H(2)O) while inspiratory flow increased less and amplitude pressure remained about the same. We concluded that MAP (lung volume) was the main independent factor for airleak, whilst SV (tidal volume) exerted a lesser effect. Within the operational range of the Humming V, frequency did not affect airleak.

Ingested button battery retrieved by a modified magnet endoscope.

Ingestion of button battery (BB) by toddlers has been seen with increasing frequency over the past decade. Significant morbidity may develop if the ingested BB cannot be removed in time. Herein, we describe 2 infants whose ingested BBs were smoothly and successfully retrieved, from a stenotic esophagus and stomach, by the use of a self-made modified magnet endoscope.

Breastfeeding-associated neonatal hypernatremic dehydration in a medical center: a clinical investigation.

BACKGROUND: Hypernatremic dehydration amongst exclusively breastfed neonates due to inadequate breastfeeding or underfeeding is a potentially devastating condition. We investigated the epidemiological and clinic features of hypernatremic dehydration. METHODS: We retrospectively reviewed the medical records of neonates that were less than 28 days of age, weighing > or =2000 g with gestational age of 36 weeks born between January 2002 and December 2005, who had meet the criteria of hypernatremic dehydration, including (1) exclusively breastfed; (2) loss of weight 12% or more from birth weight; and (3) serum sodium concentration of at least 150 mmol/L at presentation, that had been subsequently admitted to our institution. RESULTS: Totally nine cases were found during this four-year period, an incidence of 2.3 per 1000 live births. Involved infants' age at presentation ranged from 3 days to 14 days (mean t Standard Deviation = 7 +/- [3.8]); five presented symptoms or signs within the first week of life. Study-involved infants featured a birth-weight loss ranging from 12% to 29% (mean +/- [SD] = 17.0 +/- [5.9]), and also hypernatremia (mean +/- [SD] = 153.5 +/- [3.2] mEq/L ). Sodium concentration of breast milk ranged from 15 mEq/L to 54 mEq/L (mean +/- [SD]= 36.0 +/- [14.32]). Subsequently, all were supplemented with breast milk or, alternatively, formula. None of the study-involved neonates suffered subsequent complications or a relapse of their condition. CONCLUSIONS: Neonatal hypernatremic dehydration due to inadequate breastfeeding or underfeeding would appear to be a rather common problem. In order to avoid serious morbidity and mortality, all breastfed infants should receive regular follow-up by health-care worker. Furthermore, we advocate that all mothers should be taught the skills of breastfeeding, and warning signs of breastfeeding failure and hypernatremia, accompanied with the monitoring of the weight of infants until growth commences, in order to reduce the likelihood of this condition arising, especially for first-time mothers.

Transcranial pulsed ultrasound facilitates brain uptake of laronidase in enzyme replacement therapy for Mucopolysaccharidosis type I disease.

BACKGROUND: Mucopolysaccharidosis type I (MPS I) is a debilitating hereditary disease characterized by alpha-L-iduronidase (IDUA) deficiency and consequent inability to degrade glycosaminoglycans. The pathological accumulation of glycosaminoglycans systemically results in severe mental retardation and multiple organ dysfunction. Enzyme replacement therapy with recombinant human alpha-L-iduronidase (rhIDU) improves the function of some organs but not neurological deficits owing to its exclusion from the brain by the blood-brain barrier (BBB). METHODS: We divided MPS I mice into control group, enzyme replacement group with rhIDU 2.9 mg/kg injection, enzyme replacement with one-spot ultrasound treatment group, and enzyme replacement with two-spot ultrasound treatment group, and compare treatment effectiveness between groups. All ultrasound treatments were applied on left side brain. Evans blue was used to simulate the distribution of rhIDU in the brain. RESULTS: Transcranial pulsed weakly focused ultrasound combined with microbubbles facilitates brain rhIDU delivery in MPS I mice receiving systemic enzyme replacement therapy. With intravenously injected rhIDU 2.9 mg/kg, the IDUA enzyme activity on the ultrasound treated side of the cerebral hemisphere raised to 7.81-fold that on the untreated side and to 75.84% of its normal value. Evans blue simulation showed the distribution of the delivered drug was extensive, involving a large volume of the treated cerebral hemisphere. Two-spot ultrasound treatment scheme is more efficient for brain rhIDU delivery than one-spot ultrasound treatment scheme. CONCLUSIONS: Transcranial pulsed weakly focused ultrasound can open BBB extensively and facilitates brain rhIDU delivery. This novel technology may provide a new MPS I treatment strategy.

Bubble CPAP elicits decreases in exhaled nitric oxide in rabbits.

Endogenous nitric oxide (NO) has a key role in pulmonary function, and the application of positive end-expiratory pressure (PEEP) can increase exhaled NO (FE(NO)) in anesthetized animals and isolated lungs. The influence of continuous positive airway pressure (CPAP), which is similar to PEEP, on the FE(NO) level has not been investigated in humans or in animals. The present study was undertaken to determine whether and how the application of CPAP in spontaneously breathing rabbits influences levels of FE(NO). We performed a randomized crossover study to measure FE(NO) levels in 12 ketamine-anesthetized rabbits that were intubated via tracheostomy for bubble CPAP (B-CPAP) or ventilator-derived CPAP (V-CPAP), which are two of the most popular CPAP modes and which have different pressure sources. The baseline FE(NO) level was 23.8 +/- 2.6 ppb, which increased to 27.1 +/- 2.9 ppb (P < 0.001) during V-CPAP and decreased to 18.6 +/- 2.2 ppb (P < 0.001) during B-CPAP. We used one high-frequency oscillatory ventilator to repeat the experiment, in which the conventional ventilation function of the ventilator was used in the baseline and V-CPAP periods, and the high-frequency function was used to replace B-CPAP. Changes in FE(NO) were similar to our previous findings. This study demonstrated that the application of CPAP did influence levels of FE(NO). We speculate that the decrease in FE(NO) during B-CPAP may have been related to the bubble-associated high-frequency oscillation.

Effects of tub bathing procedures on preterm infants' behavior.

Although medical advances have increased the survival rate of preterm infants, morbidity in terms of neurodevelopmental impairment has not decreased for this population. This results in caregivers having to reconsider how neonatal intensive care unit (NICU) caregiving impacts on preterm infants. The purpose of this study was to examine the effects of different phases of a routine tub bath on preterm infants' distress and state behavior in the NICU. The study used an exploratory repeated measures design that focused on preterm infants' distress and state behavior, and evaluated the effects of three phases of a routine tub bath, which were performed according to standard unit practice in the NICU. Thirteen nurses repeatedly bathed 12 infants on different days, and 64 baths were videotaped for the purpose of assessing the variety of distress behavior. The procedures of one bath could be categorized into three phases designated to Phases I, II, and III. The variables were measured by a preterm infant behavioral coding scheme developed for this research. The inter-rater reliability of the instrument ranged from .82 to .99. Mixed effects analysis of variance was used to analyze the differences among the bath phases in the occurrences of distress and state behavior. The results showed significant statistical difference among most distress behaviors during the three phases (e.g. "startle, jerk, tremor" F ratio = 25.62, p < .001; "finger splay, grasping, fisting" F ratio = 49.99, p < .001; "grimace" F ratio = 36.55, p < .001; "fussing or crying" F ratio = 25.27, p < .001), with the exception of "extension, arching and squirming". In particular, the occurrence of distress and state behavior increased significantly in phase II. Routine tub bathing not only disrupts preterm infants' sleep but also causes an increase in distress behavior. Preterm infants' stress increases with the intrusiveness of nursing procedures. NICU caregivers should consider the effects of routine nursing activities that influence the infants, and modify handling to promote techniques to promote preterm infants' recovery, growth and development.

PRKAG3 polymorphisms associated with sporadic Wolff-Parkinson-White syndrome among a Taiwanese population.

BACKGROUND: The aim of this study was to investigate whether mutation in AMP-activated protein kinase (AMPK) subunit genes (PRKAG3-230) is associated with sporadic, isolated Wolff-Parkinson-White (WPW) syndrome. METHODS: This study consisted of 87 patients with symptomatic WPW syndrome and 93 healthy controls. PRKAG3-230 genotypes were determined using real-time polymerase chain reaction assay. Genotype and allele frequencies of PRKAG3-230 between patients with WPW syndrome and healthy controls were ascertained using chi-square test or Fisher exact test when appropriate. RESULTS: PRKAG3-230 were genotyped in 87 patients (53 men and 34 women; age=24.4±18.0 years) with WPW syndrome and 93 healthy controls (57 men and 36 women; age=16.8±4.2 years). There were no significant differences between the two groups in terms of age and sex. The patients with CG and CG+CC genotypes had a significantly increased risk of WPW syndrome compared with those with GG genotype [odds ratio (OR)=1.99, 95% confidence interval (CI)=1.01-3.89, p=0.045; OR=1.99, 95% CI=1.04-3.78, p=0.037, respectively]. The allelic types were not associated with the risk of WPW syndrome. The patients with manifest type with CG and CG+CC genotypes had a significantly increased risk of WPW syndrome compared with those with GG genotype (OR=2.86, 95% CI=1.16-7.05, p=0.022; OR=2.84, 95% CI=1.19-6.80, p=0.019, respectively). The patients with right-side accessory pathways with CG and CG+CC genotypes had a significantly increased risk of WPW syndrome compared with those with GG genotype (OR=3.07, 95% CI=1.25-7.51, p=0.014; OR=2.84, 95% CI=1.19-6.80, p=0.019, respectively). The allelic types were not associated with the risk of WPW types and locations. CONCLUSION: This study shows that PRKAG3-230 may be associated with sporadic WPW syndrome among a Taiwanese population. Further studies are warranted to elucidate the role of mutations in AMPK subunit genes other than PRKAG3-230 in sporadic WPW syndrome.

A preliminary study of the associations among preterm infant behaviors.

The purpose of this study was to examine whether relationships exist among various preterm infant behaviors. The study used an exploratory method design. Twenty infants were bathed and a total of 120 baths were video recorded and observed to measure preterm infant behaviors based on the frequency that behaviors occurred. The frequency was measured by using the preterm infant behavioral coding scheme developed for the study. Pearson Correlation Coefficients were used to analyze the behavioral data and examine whether the relationships among these behaviors were significant. The interrater reliability of the behavioral variables ranged from .82 to .99. There were highly positive correlations between the stress behaviors. There was, however, negative correlation between the stress behaviors and the stable behavior (sucking). The occurrences of the stress behaviors were associated with the state of " eyes open " and "fuss or crying". Knowing the associations may enhance NICU nurses ' abilities to identify preterm infant behaviors. While interacting with preterm infants, nurses can sensitively and actively sense preterm infant signals, prevent or ameliorate the early threats to an infant ' s life, and adjust care to support the infant ' s growth and development.

Acute intoxication of lidocaine and chlorpheniramine: report of one case.

A case of acute intoxication involving lidocaine and chlorpheniramine (an antihistamine) in a 13-month-old child after ingestion of a commercial topical agent is presented. The major toxic reaction consisted of convulsion, coma, tachycardia, fever, and fatigue. This report shows that parents and physicians should be made aware of the hazards of lidocaine and overdose of other topical agents in infants and children.

Meningitis complicating an occult infected nasal epidermoid cyst in a child.

Congenital nasal epidermoid cyst without associated facial dysmorphism is an uncommon anomaly that is often asymptomatic. We report a case of occult nasal epidermoid cyst in a 12-year-old boy with initial nonspecific symptoms of fever, headache, abdominal pain, vomiting, and diarrhea. An enhanced magnetic resonance imaging (MRI) showed a cystic lesion in the frontal skull base with expansion of the foramen cecum and the perpendicular plate of the ethmoid bone, accompanied with meningitis. An emergent surgical intervention was undertaken, and an infected epidermoid cyst was confirmed by pathology. He recovered uneventfully during a 4-week treatment with parentally-administered antibiotics. This significant case report demonstrates the importance of MRI in the early diagnosis of a life-threatening intracranial infection from an otherwise occult infected congenital nasal epidermoid cyst in children.

McCune-Albright syndrome associated with pituitary microadenoma: patient report.

McCune-Albright syndrome (MAS) is a rare disorder characterized by the classic triad of precocious puberty, polyostotic fibrous dysplasia and café-au-lait spots. Additional endocrine abnormalities may also be present, including hyperthyroidism, growth hormone excess and hyperprolactinemia. The most commonly encountered endocrine dysfunction is gonadal hyperfunction. Gonadotropin-independent precocious puberty is typically the initial manifestation of MAS in girls. Ovarian cysts may be detected on pelvic ultrasound. Our patient was also found to have pituitary microadenoma, evidenced by dynamic magnetic resonance imaging.

Early diagnosis of Prader-Willi syndrome in a newborn.

Prader-Willi syndrome (PWS) is a multiple-systemic disorder with many manifestations related to hypothalamic insufficiency, with obesity and behavioral problems as the major causes of morbidity and mortality. We describe a 2-day-old boy who initially presented with neonatal hypotonia and was diagnosed as PWS based on abnormal DNA methylation patterns in the small nuclear ribonucleoprotein polypeptide N (SNRPN) gene at the age of one week, despite the absence of other classical features. Molecular diagnosis for PWS, which has become available in recent years, should be considered for neonates with undiagnosed central hypotonia.

Moyamoya disease initially mimicking MELAS syndrome in a 14-month-old child.

Moyamoya disease is a neurological disease rarely seen in children outside Japan. It is difficult to differentiate moyamoya disease in its early stage from mitochondrial disorders in children when nontraumatic ischemic stroke is considered. We present a 14-month-old Taiwanese female child who had suffered from sudden onset of nontraumatic seizure attack, after which progressive left limb weakness was noted. Initial brain imaging and a series of laboratory studies were performed which suggested mitochondrial disorders, especially mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome. The patient underwent an oral glucose lactate stimulation test (OGLST), and mitochondrial disorders were not favored. MR angiography of the circle of Willis showed moyamoya disease. The patient then underwent an encephalo-duro-arterio-myo-synangiosis (EDAMS) operation. Evaluation of the neurodevelopmental and intelligence outcome required close long-term follow-up.