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Dandy Walker 증후군이 동반된 Neurocutaneous Giant Melanosis 1례
홍수영(Su Young Hong),반지은(Ji Eun Ban),류재호(Jae Ho Rhu),최순섭(Sun Seob Choi),황규근(Kyu Geun Hwang) 대한소아신경학회 2001 대한소아신경학회지 Vol.9 No.1
저자들은 3세 남아에서 Dandy-Walker 증후군이 동반된 neurocutaneous giant melanosis 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다. Neurocutaneous melanosis is a rate congenital phacomatosis, characterized by the presence of multiple pigmented skin nevi at birth. Meningeal melanosis tending to become malignant and seizure. Dandy-Walker syndrome is a developmental disorder of brain characterized by cystic dilatation of the fourth ventricle and agenesis of hypoplasia of the cerebellat vermis. We studied a case of Dandy-Walker syndrome with neurocutaneous giant melanosis in a 3 years old boy.
증례 : 동반 심기형이 없는 수정 대혈관 전위 성인 환자에서 나타난 완전 방실 차단 1예
한동철 ( Dong Cheul Han ),김준 ( Jun Kim ),문지윤 ( Ji Yoon Moon ),반지은 ( Ji Eun Ban ),이형두 ( Hyoung Doo Lee ),홍택종 ( Taek Jong Hong ),신영우 ( Yung Woo Shin ) 대한내과학회 2007 대한내과학회지 Vol.72 No.3
Isolated CCTGA 환자는 전체 CCTGA 환자의 약 1% 가량으로 매우 드문 것으로 알려져 있는데, 이들은 방실 차단, 전신 순환 방실 판막 역류 등 다른 기능적 이상이 나타날 때까지 무증상으로 지내는 경우가 많다. 저자들은 14년 동안 무증상으로 지내던 isolated CCTGA 환자가 호흡 곤란과 어지러움증으로 내원해 완전 방실 차단 보여 영구 심박동기를 이식해 치료한 1예를 경험하였기에 보고하는 바이다. Patients with isolated congenitally corrected transposition of the great arteries (CCTGA) comprise 1% of all CCTGA patients. They are usually asymptomatic until functional abnormalities such as atrioventricular block or systemic atrioventricular valve regurgitation become complicated. We report here a case of a 33 year-old man with isolated CCTGA and complete AV block that underwent implantation of a permanent pacemaker using endocardial leads. During a 12 month follow-up period, no symptoms developed and lead-related parameters have been stable. (Korean J Med 72:311-314, 2007)
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)
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)