• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.32 no.1
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• pp.35-40
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• 2021

Objectives: Among the many intervention programs for children with autism spectrum disorder (ASD), the Early Start Denver Model (ESDM) is one of the few approaches that has succeeded in demonstrating clinical efficacy in randomized control trials. Here, we investigate the clinical efficacy of ESDM intervention in young children with ASD in a community setting within Japan. Methods: All subjects were children with ASD who received ESDM intervention during the study period. Each ESDM session lasted 75 min and occurred once per week for at least 12 weeks. The outcome measures consisted of the Kyoto Scale of Psychological Development (K-test), Aberrant Behavior Checklist-Japanese version (ABC-J), and the Clinical Global Impression-Severity scale (CGI-S). Results: Twenty-seven subjects (29.4±6.4 months old) received ESDM intervention that lasted for 8.0±2.6 months on average. The score on Language and Social developmental quotient on the K-test increased significantly after the intervention. The total scores on the ABC-J and CGI-S significantly decreased after completion of the ESDM intervention. Conclusion: Our results suggest that ESDM intervention could reduce the severity of distinct clinical features of ASD, such as impairments in social interaction and communication assessed by the K-test, and maladaptive behavior rated by the ABC-J and CGI-S. We believe that the ESDM adapted to each institution might become one of the standard options for children with ASD in Japan.

• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.80-84
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• 2010

Purpose : The aims of this study were to investigate the long-term outcomes in children with infantile spasms (IS) and to identify the prognostic factors influencing their neurodevelopment. Methods : We retrospectively evaluated seventy two children over five years old who were treated for IS at Asan Medical Center, Seoul, Korea, between 1994 and 2007. Forty-three children were contacted by telephone or medical follow-up to assess their current neurodevelopmental status. Multiple logistic regression was used to calculate odds ratios (ORs) and 95% confidence interval (95% CIs) of risk factors for unfavorable outcomes.Results : The mean follow-up duration for these 43 children was $7.2{\pm}1.5$ years (range, 4.5 to 13.0 years). Of these, 13 (30.2%) had cryptogenic and 30 (69.8%) had symptomatic IS. Eleven (25.6%) children were initially treated with adrenocorticotrophic hormone (ACTH) therapy, with a mean treatment lag of $1.3{\pm}1.9$ months (range; 0.1 to 7.0 months). Eighteen (41.8%) children clinically responded to initial treatment, as shown by EEG response. Overall, 22 (51.2%) children had at least moderate neurodevelopmental disorders and 2 (4.8%) died. In univariate analysis, etiology (symptomatic) and poor electroclinical response to initial treatment were related to long-term unfavorable outcomes. In multivariate analysis, response to primary treatment was the sole significant independent risk factor with a high OR. Conclusion : Overall prognosis of children with IS was poor. Electroclinical non-responsiveness to initial treatment was related to unfavorable long-term outcomes, indicating that initial control of seizures may be important in reducing the likelihood of poor neurodevelopment.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.1
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• pp.31-37
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• 2017

Carbamoyl phosphate synthetase 1 deficiency (CPS1D) is an autosomal recessive disorder of the urea cycle that causes hyperammonemia. Two forms of CPS1D are recognized: a lethal neonatal type and a less severe, delayed-onset type. Neonatal CPS1D cases often present their symptoms within the first days of life. Delayed-onset cases are predominantly adolescents or adults, and infantile delayed-onset cases are rare. Severe hyperammonemia in the neonatal period leads to serious brain damage, coma, and death if not treated promptly. Therefore, early diagnosis and acute treatment are crucial. Despite the improvement of treatments, including continuous hemodialysis, ammonia-lowering agents, and a low-protein diet, the overall outcome of severe forms of hyperammonemia often remains disappointing. As the liver is the only organ in which ammonia is converted into urea, liver transplantation has been considered as an elegant and radical alternative therapy to classical dietary and medical therapies. However, liver transplantation has many disadvantages, such as a considerable risk for technical complications and perioperative metabolic derangement, especially in neonates. Additionally, there is a lack of suitable donor organs in most countries. According to recent studies, liver cell transplantation is a therapeutic option and serves as a bridge to liver transplantation. Here, we report a Korean CPS1D patient with novel mutations in CPS1 who was treated by liver cell transplantation after being diagnosed in the neonatal period and showed a good neurodevelopmental outcome at the last follow-up at six months of age.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.27-33
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• 2005

Purpose : To determine incidence, characteristics and risk factors associated with intrauterine intraventricular hemorrhage(IU-IVH) among premature infants. Methods : The medical records of infants with intraventricular hemorrhage(IVH) admitted to the neonatal intensive care unit of Asan Medical Center from January 1999 to June 2003 were reviewed retrospectively. Infants whose IVH with cystic change were detected within five days of life were defined as the IU-IVH group. The control group included those without any IVH. Various maternal and neonatal factors were evaluated between the IU-IVH and control groups, and risk factors for IU-IVH were identified using multiple logistic regression analysis. Results : The incidence of IU-IVH was 49/1024(15.9%). Mothers who are younger, primiparous, use less antenatal steroid, and neonates with greater incidence of neonatal respiratory distress syndrome, had higher incidences of IU-IVH compared to neonates with normal neurosonography. Risk factors associated with IU-IVH included neonatal respiratory distress syndrome and placenta infarct by placenta biopsy. Most infants with IU-IVH were ${\geq}1,501g$, ${\geq}34$ weeks gestational age and had low grade IVH. The size of the cysts associated with IU-IVH remained the same or disappeared in 96 %. IU-IVH does not seem to affect short-term neurodevelopmental outcome although a longer period of follow-up is needed. Conculusion : IU-IVH occurred mostly in ${\geq}1,501g$, ${\geq}34$ weeks infants with grade I IVH without developmental delays. However, the high incidence of total IVH merits more attention in terms of awareness of its existence as an unusual IVH among premature infants.

• Clinical and Experimental Pediatrics
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• v.49 no.1
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• pp.29-33
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• 2006

Purpose : The aim of this study was to analyze the results and contributing factors of catch-up growth in very low birth weight infants(VLBWI) at the corrected age of 2 years. In addition, we looked for a relationship between the catch-up growth and the development by the bayley scales of infant development II(BSID II). Methods : A retrospective analysis by evaluating medical records was done for the 76 VLBWI whose follow-up was possible up to a corrected age of 2 years, out of the 114 who had been treated in the neonatal intensive care unit during the January of 2000 to December of 2001 at Samsung Cheil Hospital. Based on the Standard Korean Infant's Growth Curve, the catch-up growth group was defined over the 10th percentile of weight at 2 years as the corrected age and the failed catch-up growth group was defined under the 10th percentile. We investigated the clinical factors and courses of each group and compared the scores of the BSID II. Results : The catch-up growth group of the VLBWI was 51 infants and the failed catch-up growth group was 25 infants. In comparison with maternal clinical factors, use of antenatal steroids was significantly higher in the catch-up growth group. In comparisn with clinical factors and courses of VLBWI, two clinical factors were significantly different between the two groups : birth weight and intrauterine growth retardation(IUGR). Numbers of delayed development were increased in the failed catch-up growth group but statistically significant differences could not be observed. Conclusion : The catch-up growth of the VLBWI was affected by the use of antenatal steroids, birth weight and IUGR. No significant differences regarding neurodevelopmental outcome were observed between the catch-up and failed catch-up growth groups.