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- 저자 : 진장용 ( Jang Yong Jin ) (검색결과 4 건)
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A Case of Infantile Spasms in Tuberous Sclerosis with Fetal Cardiac Tumors
Jang Yong Jin(진장용),Woo Ryoung Lee(이우령),Eun Sook Suh(서은숙) 대한소아신경학회 2009 대한소아신경학회지 Vol.17 No.1
결절성경화증은 상염색체 우성의 신경피부증후군의 한 형태이다. 횡문근종은 태아, 영아 및 소아에서 가장 흔한 심장 종양으로 결절성경화증 환아의 약 60%에서 심장 종양이 관찰되고, 산전 초음파상 심장 종양이 확인된 경우에 약 40%에서 결절성경화증으로 진단된다. 결절성경화증은 뇌, 심장, 피부 및 기타 장기들을 침범하며 경련, 지능저하, 피지샘종의 3징후를 특징으로 하며, 경련이 일찍 나타날수록 심한 지능저하를 보이는 경향이 있다. 상염색체 우성으로 가족력이 있으므로, 가족 중에 결절성경화증 환자가 있는 경우 조기에 산전 검사를 시행하여야 하고 출생 후 이에 대한 철저한 조사가 이루어져야 한다. 영아 연축은 결절성경화증 환아에서 가장 흔히 관찰되는 경련중 하나이다. 저자들은 산전 초음파상 심장 종양이 관찰되고 환아 아버지가 결절성경화증을 보였던 환아를 추적 관찰하여 색소 탈실반, 망막의 과오종, 뇌 겉질의 결절 및 뇌실막밑 결절 소견 그리고 영아 연축의 특징적인 증상과 우측 손의 기형을 동반한 결절성경화증 1례를 경험하였기에 보고하는 바이다. Tuberous sclerosis(TS), a type of neurocutaneous syndrome, is inherited in an autosomal dominant manner. Approximately 60% of children with TS have rhabdomyomas of the heart, and 40% of fetuses in whom rhabdomyomas are detected by a prenatal ultrasonography eventually end up with TS. Therefore, when multiple cardiac rhabdomyomas are detected by a fetal ultrasonography, TS should be suspected and further examination should be considered after birth. Infantile spasms is a common type of seizure among young children with TS. We describe a patient with TS who showed cardiac tumors on a fetal ultrasound. Also, hypomelanotic macules, retinal tumors, brain cortical tubers, nodules in subependymal regions, and infantile spasms was detected after birth.
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한국 소아 알레르기비염 환자의 삶의 질에 대한 설문 개발 및 유용성 평가를 위한 연구
진장용 ( Jang Yong Jin ),양현종 ( Hyeon Jong Yang ),전유훈 ( You Hoon Jeon ),김경원 ( Kyung Won Kim ),김우경 ( Woo Kyung Kim ),박용민 ( Yong Mean Park ),윤혜선 ( Hae Sun Yoon ),염혜영 ( Hye Yung Yum ),편복양 ( Bok Yang Pyun ) 대한천식알레르기학회 2009 천식 및 알레르기 Vol.29 No.4
Background: A questionnaire for assessing the quality of life (QOL) of children with allergic rhinitis has not been developed in Korea. Objective: Our study was designed to develop and validate a Questionnaire for QOL Specific to Allergic Rhinitis in Korean Children (QQOL-ARK). Method: The QQOL-ARK consisted of 4 domains including 18 items with a 5-point scale: symptoms (3 items), physical factors (8 items), psychosocial factors (6 items) and prognosis (1 item). A survey with the QQOL-ARK was performed on 168 children with allergic rhinitis twice, 4 weeks apart. We analyzed their responsiveness, reproducibility and validity. Result: The mean score of QOL according to the severity showed a statistically significant difference between the first and second visits (P<0.01). The scores of QOL were decreased in the improved group (P<0.01) and increased in the worsen group (P<0.01). The differences in scores between the first and second visits showed a statistically significant difference in intergroup analysis (P<0.01). The QQOL-ARK also showed a good reproducibility (R=0.97). There was no strong correlation between QOL and clinical severity. Conclusion: The QQOL-ARK showed a good reproducibility and validity. However, the assessment of reliability on 18 items of the questionnaire could not be performed. Although the items of this questionnaire are limited, they could provide the basis for the development of the QOL questionnaire for Korean children with allergic rhinitis in conjunction with clinical evaluation. (Korean J Asthma Allergy Clin Immunol 2009;29:242-248)
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24개월에 만성 화농성 비루를 주소로 내원하여 진단된 헌터 증후군 1례
김이안,진장용,박재옥,홍용희,Kim, I An,Jin, Jang Yong,Park, Jae Ock,Hong, Yong Hee 대한유전성대사질환학회 2015 대한유전성대사질환학회지 Vol.15 No.3
Hunter syndrome(Mucopolysaccharidosis type II, MPS type II) is an X-linked disorder of glycosaminoglycans (GAGs) metabolism caused by an iduronate-2-sulfatase (IDS2) deficiency. A 24-month-old boy visited the department of pediatrics with the chief compliant of chronic purulent rhinorrhea beginning at age one. He had a history of repeated acute otitis media and chronic rhinitis. On physical examination he had a coarse face, enlarged tongue, distended abdomen, joint stiffness, and Mongolian spots at his first visit. The urine GAGs level was elevated at 66.10 mg/mmolCr (reference range, <11.1) and iduronate-2-sulfatase activity in leukocyte was decreased at 0.21 nmol/mg protein/hr (reference range, 18.7-57). Finally with an IDS gene mutational analysis, recombinant known mutation between intron 7 and distal of exon 3 in IDS2 was detected. Recombinant iduronate-2-sulfatase therapy was started without any infusion related reactions. The author highlights the importance of suspecting Hunter syndrome when pediatric patients visit with chronic purulent rhinorrhea which is a common cause of hospital visits for infants and children.
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t(921)(q21.2q11.2) 균형 전좌 어머니로부터 출생한 Partial Trisomy 9 증후군
신익순 ( Ik Soon Shin ),정우진 ( Woo Jin Jung ),진장용 ( Jang Yong Jin ),이우령 ( Woo Ryoung Lee ) 대한주산의학회 2010 Perinatology Vol.21 No.3
Trisomy 9 syndrome was first reported by Retheore in 1970 and has been rarely reported. This syndrome consists of partial and complete trisomy 9. It is characterized by growth and mental retardation, craniofacial abnormalities including microcephaly, hypertelorism, prominent nose, deep-set ears and down-slanting palpebral fissures. Congenital heart diseases and congenital dislocations of knee are also common in trisomy 9 syndrome. Here, we report a very rare case of partial trisomy 9 due to maternal balanced translocation t(9;21). He showed craniofacial abnormalities, brain malformation, cardiac defect, hydronephrosis and congenital dislocations of hip and knee joints.
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