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Rita Yu(Rita Yu),Eun Joo Lee(Eun Joo Lee),Joon Soo Lee(Joon Soo Lee),Hoon-Chul Kang(Hoon-Chul Kang),Heung Dong Kim(Heung Dong Kim) 대한소아신경학회 2022 대한소아신경학회지 Vol.30 No.4
Purpose: In the past, the use of medium-chain triglycerides (MCTs) for a ketogenic diet (KD) was expected to improve both patients’ and caregivers’ adherence to treatment, but many gastrointestinal problems have been reported. Through a calculated partial administration of MCTs in a KD, we aimed to reduce these complications, while maintaining acceptable seizure reduction. Methods: At a tertiary referral center for pediatric patients with epilepsy, MCT oil was given in a 1:1 ratio with long-chain triglycerides to patients on KDs. Patients who began the diet from February 2019 to February 2020 were reviewed retrospectively, and 47 patients with at least 3 months of follow-up records were enrolled in the study Results: Overall, 29.8% of patients on a KD with an adjusted MCT ratio experienced complications, such as gastrointestinal symptoms and behavioral food refusal, compared to 63.0% of prior KD patients. The mean seizure reduction rate was 68.45%±40.61%, which was not significantly different from the comparison group’s rate of 64.84%±34.24%. Conclusion: Adjusted MCT incorporation into a KD showed comparable seizure control results, with better tolerability of the diet.
Tracheal Bronchus with Persistent Pulmonary Hypertension of the Newborn: A Case Report
안세환,이민주,Rita Yu,김지혜,백혜성,반지은,임경자,양승,황일태,김수영 대한신생아학회 2017 Neonatal medicine Vol.24 No.4
Tracheal bronchus is an uncommon anomaly in which an ectopic bronchus originates directly from the supracarinal trachea. It is usually an asymptomatic anatomical variant incidentally found on computed tomography or bronchoscopy. However, it can present with symptoms, such as chronic cough, wheezing, atelectasis, and recurrent pneumonia. We report a case of tracheal bronchus diagnosed in the neonatal period, in which the term baby presented with respiratory distress and persistent pulmonary hypertension of the newborn after birth, but no other congenital anomaly was found on further evaluation.
결절성 경화증 환자에서 경막하 거대세포 성상세포종의 감마나이프와 수술치료 1례
정모경(Mo Kyung Jung),이상미(Sang Mi Lee),유리타(Rita Yu),김흥동(Heung Dong Kim),이준수(Joon Soo Lee),김동석(Dong Suk Kim),강훈철(Hoon-Chul Kang) 대한소아신경학회 2013 대한소아신경학회지 Vol.21 No.3
저자들은 결절성 경화증에서 흔하게 동반될 수 있는 SEGA가 있는 환자에서 GK-SRS를 시행하여 초기 경과가 좋았으나, 재발을 하여 결국 수술적 절제를 시행하게 된 증례를 경험하여 이를 보고하였다. Subependymal giant cell astrocytoma (SEGA) is the most common brain tumor that develops in 5-20% of patients with tuberous sclerosis complex. Although these lesions are slowly growing tumor, they can cause morbidity and mortality due to acute hydrocephalus because of their prevalent location near the foramen of Monro. Surgery has been the standard care for SEGAs demonstrating serial growth and symptomatic hydrocephalus. However, not all SEGAs are treatable by complete surgical resection. Gamma Knife stereotactic radiosurgery can be considered as a second option, but it has shown highly variable responses in patients with limited data. In recent years, drugs with novel mechanism, sirolimus and other mammalian target of rapamycin (mTOR) inhibitors have been found to reduce the size of SEGAs. We report a case of treatment of SEGA with gamma knife surgery which continued for two years without progression, even though surgical resection was done, finally.
Neonatal Type 2 Gaucher Disease with Congenital Ichthyosis: A Case Report
( Hakmin Lee ),( Jiyoung Oh ),( Jungho Han ),( Min Jung Lee ),( Joonsik Park ),( Seung Hwan Baek ),( Rita Yu ),( Ho Seon Eun ),( Min Soo Park ),( Jeong Eun Shin ) 대한주산의학회 2021 Perinatology Vol.32 No.4
Gaucher disease (GD) is a rare autosomal recessive genetic disease. The symptoms and age of onset vary depending on the subtype. Type 2 GD is potentially lethal, and the mean lifespan is less than 2 years. Due to rapid disease progression, early diagnosis of type 2 GD is important. Here, we present an infant with congenital ichthyosis and rigidity with joint contracture, who later presented with bulbar involvement. Feeding difficulty, apnea, hepatosplenomegaly, and thrombocytopenia were also evident. She was diagnosed with type 2 GD via whole-exome sequencing, which showed 2 pathogenic variants in GBA as a compound heterozygote: Arg296Gln (c.887G>A) and Pro24His (c.719C>A). Congenital ichthyosis with progressive respiratory and neurologic impairment may be key clinical findings for the early diagnosis of type 2 GD.
천식 소아에서 아토피와 간접유발 기관지과민성과의 연관성
박태영 ( Tae-young Park ),이민주 ( Min-ju Yi ),최우혁 ( Woo-hyeok Choi ),김수영 ( Su-yeong Kim ),유리타미영 ( Rita Yu ),반지은 ( Ji-eun Ban ),양승 ( Seong Yang ),황일태 ( Il-tae Hwang ),백혜성 ( Hey-sung Baek ) 대한천식알레르기학회(구 대한알레르기학회) 2017 Allergy Asthma & Respiratory Disease Vol.5 No.2
Purpose: Both atopy and bronchial hyperresponsiveness (BHR) are characteristic features of asthma. Several BHR studies comparing groups of atopic and nonatopic asthmatics have reported conflicting results. The aim of this study was to compare BHR to indirect stimuli, such as mannitol or exercise, between atopic and nonatopic asthmatics in children Methods: We performed a retrospective analysis of data from 110 children with asthma, aged 6-18 years using skin prick tests, and serum total and specific IgE levels. Atopy degree was measured using the sum of graded wheal size or the sum of the allergen-specific IgE. Bronchial provocation tests (BPTs) using methacholine were performed on all subjects. BPTs using indirect simuli, including exercise and mannitol, were also performed. Results: Asthma cases were classified as atopic asthma (n=83) or nonatopic asthma (n=27) from skin prick or allergen-specific IgE test results. There was no significant difference in the prevalence of BHR to mannitol or exercise between atopic and nonatopic asthmatics. Atopic asthma had a significantly lower postexercise maximum decrease in % forced expiratory volume in 1 second (FEV<sub>1</sub>) (geometric mean [95% confidence interval]: 31.9 [22.9-40.9] vs. 14.0 [9.4-18.6], P=0.015) and a methacholine PC<sub>20</sub> (provocative concentration of methacholine inducing a 20% fall in FEV<sub>1</sub>) than nonatopic asthmatics (geometric mean [95% confidence interval]: 1.24 [0.60-1.87] ng/mL vs. 4.97 [3.47-6.47]) ng/mL, P=0.001), whereas mannitol PD<sub>15</sub> (cumulative provocative dose causing a 15% fall in FEV<sub>1</sub>) was not significantly different between the 2 groups. Conclusion: There was no significant difference in the prevalence of BHR to mannitol or exercise between atopic and nonatopic asthmatics in children. (Allergy Asthma Respir Dis 2017:5:83-91)
최우혁 ( Woo-hyeok Choi ),박태영 ( Tae-young Park ),김수영 ( Su-yeong Kim ),유리타미영 ( Rita Yu ),반지은 ( Ji-eun Ban ),양승 ( Seong Yang ),황일태 ( Il-tae Hwang ),백혜성 ( Hey-sung Baek ) 대한천식알레르기학회(구 대한알레르기학회) 2017 Allergy Asthma & Respiratory Disease Vol.5 No.2
Purpose: It was found that periostin and squamous cell carcinoma-related antigens (SCCAs) were strongly interleukin-13-inducible gene products. This study measures the serum periostin and SCCA levels in children suffering from atopic dermatitis (AD) and to evaluate the association between the severity of AD and their values. Methods: Seventy AD children aged 1 month to 10 years were included in our study. Subjects were characterized as having atopic eczema (AE; n=55) or non-AE (NAE; n=15) by atopic sensitization. Serum SCCA and periostin levels were measured. Results: The serum periostin levels were significantly higher in children with AE than in those with NAE (geometric mean [95% confidence interval]: 80.47 ng/mL [75.06-85.93 ng/mL] vs. 67.45 ng/mL [59.99-75.64] ng/mL, P=0.020). The serum concentrations of both SCCA1 and SCCA2 were significantly higher in children with AE than in those with NAE (geometric mean [95% confidence interval]: 1.401 [1.198-1.643] ng/mL vs. 0.969 [0.723-1.268] ng/mL, P=0.039 for SCCA1) (1.178 [0.974-1.455] ng/mL vs. 0.711 [0.540- 0.994] ng/mL, P=0.025 for SCCA2). The serum periostin levels were significantly correlated with disease severity and with peripheral blood eosinophil counts. The SCCA levels were not significantly correlated with disease severity. Both SCCA1 and SCCA2 were significantly correlated with serum periostin levels and blood eosinophil counts. Conclusion: Serum periostin levels may be significantly correlated with disease severity and blood eosinophil counts in children with AD. Serum SCCA levels can be significantly correlated with serum periostin levels and blood eosinophil counts in children with AD. (Allergy Asthma Respir Dis 2017:5:73-78)