• Clinical and Experimental Pediatrics
• /
• 제46권8호
• /
• pp.826-830
• /
• 2003

본 저자들은 출생 후 늘어지며 경구 수유를 잘 하지 못하는 증상으로 본원에서 ARCI 증후군 진단을 받고 경구용 bicarbonate, 1,25-dihydroxycholecalciferol, 비 경구 수유와 폐렴 치료를 받았으나 사망한 1례를 경험하여 이를 보고하는 바이다. 다발성 관절 구축, 신세뇨관성 산증, 담즙 정체성 간염은 ARCI 증후군의 기본적인 임상 증상이기는 하나 이들이 모두 혹은 동시에 나타나는 것은 아니므로 신생아 시기에 늘어지며 관절구축을 보이고 산증이 있는 경우 주의 깊은 추적 관찰을 통해 진단을 내리도록 해야할 것이다. 또한 이들은 성장 부진과 잦은 감염에 시달리게 되므로 일찍부터 비관 삽입 등을 통한 영양이나 감염의 증후를 잘 관찰함으로써 적절한 성장을 할 수 있도록 도와야겠다.

• 한국초등과학교육학회지:초등과학교육
• /
• 제33권2호
• /
• pp.306-321
• /
• 2014

This study aims to analyze difficulties that pre-service elementary teachers experience in investigating the life cycle of a common cabbage butterfly in person. As difficulties they face during the process of this research, they pointed out collecting eggs, observing molting, creating environments for a breeding cage, feeding, building a breeding cage, and making butterfly specimens. Out of all the environmental difficulties related to their school fields, they pointed out a difficulty of time management most of all, followed by placing a breeding cage in the classroom and the lack of microscopes for observation. In regard to difficulties related to their evaluations on students' activities, they found it difficult to evaluate students' activity with the life cycle of an insect in the aspect of knowledge and even to set evaluation criteria. Besides, many of them responded that it would be appropriate to evaluate a research on the life cycle of an insect through a portfolio or an observation journal. In regard to difficulties in terms of teachers' knowledge, they found it difficult to understand insect molting, metamorphoses, complete metamorphoses, incomplete metamorphoses, the structure of an insect body, and how to distinguish a female insect from a male one. In regard to the application of class models, they knew it is important for students to have various experiences through direct observation, so the experience-based learning model was proper for the process of observing the life cycle of a common cabbage butterfly. However, they found it difficult for students to observe each stage of the life cycle in person.

• Korean Chemical Engineering Research
• /
• 제46권3호
• /
• pp.535-539
• /
• 2008

폐EPS 열분해반응으로부터 원료인 스티렌모노머로 회수하는 열분해반응에서 오일의 생성 및 오일 중에 함유된 스티렌, 에틸벤젠, 알파메틸스티렌의 생성은 반응중에 생성되는 잔류물의 영향을 받으며 원하는 조건에서 반응의 진행이 어려우므로 반응중에 생성되는 잔류물의 영향을 받지않는 새로운 반응기를 개발하였다. 본 연구에서는 열분해 과정에서 주입되는 원료가 회분식반응기와 달리 반응 중 생성되는 잔류물의 영향을 받지 않고 일전한 온도가 유지되는 원료가 벽을 통해 흐르는 새로운 형태의 wetted-wall형 반응기를 사용하여 반응온도, 생성오일의 배출을 쉽게 하기 위해 주입되는 질소 유량 등의 변수들을 고찰하였다. 또한 반응으로부터 선정된 최적조건에서 반응기 설계를 위한 반응속도론적 연구를 수행하였다.

• Maxillofacial Plastic and Reconstructive Surgery
• /
• 제32권3호
• /
• pp.242-245
• /
• 2010

Pierre Robin sequence (PRS) describes the clinical triad of micro- and/or retrognathia, glossoptosis and cleft soft palate. Glossopexy has been demonstrated to be an effective treatment in selected cases of obstruction caused by glossoptosis (generally 6 to 10 months of glossopexy period). If radiofrequency therapy (RF) can reduce tongue volume in PRS, it will be helpful in early releasing of the glossopexy. Two-dayold patient showed a PRS triad. Intermittent cyanosis, respiratory difficulty and feeding problems were also observed. The respiration was not improved and prolonged intubation increased the possibility of respiratory complications like pneumonia. The surgical intervention- glossopexy and RF was done 20 days after birth. We applied RF combined with conventional glossopexy and could get successful results while reducing the overall treatment time to 6 weeks. The follow-up until 12 months after birth was uneventful. Considering that early recovery is highly beneficial to PRS patients by reducing risks associated with glossopexy and low energy RF application is very simple and low risk to patient, our combination therapy should be considered for the treatment of airway problem related to PRS.

• 환경영향평가
• /
• 제26권3호
• /
• pp.207-216
• /
• 2017

오염퇴적물의 생물영향평가를 위해 다양한 생물검정 연구가 수행되고 있다. 퇴적물에 직접 노출시키는 평가 방법은 실험과정 동안 섭식 및 섭식에 의한 생물의 영향을 배제할 수 없으며 관찰을 위한 실험 생물이 대형생물 또는 저서성 생물에 제한되는 등의 한계가 있다. 본 실험에서는 짧은 생활사를 가지며 많은 연구에서 독성 결과가 축적되어 있는 요각류와 퇴적물의 용출수를 이용해 생물영향평가의 가능성을 확인하고자 하였다. 오염의 정도가 다른 두 정점의 퇴적물 용출수에 해산 요각류를 노출 시켜 개체 및 분자 수준에서 관찰되는 변화를 측정한 결과 해산 요각류의 유생의 성장과 분자생체지표의 발현에서 오염된 퇴적물의 용출수에서 대조군과 유의한 차이를 보였다. 분자생체지표의 발현은 용출수의 희석 정도와 노출 시간에 의존적인 경향을 나타내 용출수를 이용한 생물영향평가의 가능성을 보여주었다. 본 논문 결과를 바탕으로 퇴적물의 오염 및 생물영향평가에 있어 용출수 노출시험이 오염물질의 정량적 분석결과를 보완할 수 있는 방법으로 이용될 수 있을 것을 확인하였으며 향후 많은 자료의 축적과 활용성에 대한 평가 및 기준이 제시되어야 한다고 제안한다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
• /
• 제34권4호
• /
• pp.460-467
• /
• 2008

Pierre Robin sequence as a symptom triad of micrognathia, glossoptosis, and cleft palate results in upper airway obstruction and feeding problems. If mild, it is often managed in the prone position. When positional treatment fails, however, surgical intervention such as tongue-lip adhesion, tracheostomy, and mandibular distraction osteogenesis is mandatory to relieve airway obstruction. There has been growing interest in the application of distraction osteogenesis for the management of craniofacial abnormalities. The mandibular distraction osteogenesis to newborns may prevent the airway obstruction, decrease the potential tracheostomy, and reduce the likehood of orthognathic surgery after growth. We experienced an infant with Pierre Robin sequence who showed mandibular hypoplasia, glossoptosis, incomplete cleft palate, intermittent cyanos is, depression of the chest, and respiratory difficulty associated with airway obstruction. We treated the airway obstruction by tongue-lip adhesion at 2 weeks of age, and treated the mandibular retrognathism and depression of the chest byusing internal mandibular distraction osteogenesis at 7 month of age. The mandible moved forwardly, the upper airway space was enlarged, and the antero-posterior distance of the mandible was elongated after the mandibular distraction. Mandibular distraction osteogenesis may be a promising technique to avoid the need of tracheostomy and orthognathic surgery, and to correct airway obstruction in infants with congenital craniofacial malformation.

• 대한전자공학회논문지TE
• /
• 제37권1호
• /
• pp.70-78
• /
• 2000

초전도체를 초고주파 소자에 응용하려고 할 경우, 초전도 전자기학의 불확실함이나 초전도 기판의 돈도 의존성은 산업 응용을 위한 안테나를 제작하는데 있어 어려움을 준다. 따라서 실제 응용을 위해서는 일반 안테나와 비교하여 정확하고 자세한 특성이 알려져야만 한다. 본 논문에서는 금 안테나와 초전도 안테나의 비교 연구를 수행하였으며 반사 손실, 특성임피던스, 효율 및 다른 다양한 특성들을 보고하였다. 본 연구에 사용된 고온초전도 박막은 $YBa_2Cu_3O_{7-x}$ (YBCO)/MgO 이며 초전도 안테나는 마이크로스트립 급전선을 방사 패치의 50 ${\Omega}$ 영역에 결합시키는 형태로 만들어졌다. 금 안테나와 초전도 안테나의 측정 결과는 이러한 구조를 이용하여 유용한 안테나가 제작될 수 있음을 보여주었다.

• Journal of Genetic Medicine
• /
• 제11권2호
• /
• pp.74-78
• /
• 2014

Sotos syndrome (SS, OMIM 117550) is characterized by prenatal and postnatal overgrowth with multiple congenital anomalies. However, there have been few cases of growth retardation caused by renal failure from infancy. We report a case of dysplasia of the bilateral kidneys with renal failure and poor postnatal growth. A 2-month-old boy visited the emergency room owing to poor oral intake and abdominal distension. He was born at the gestational age of 38 weeks with a birth weight of 4,180 g. After birth, he had feeding difficulty and abdominal distension. Upon physical examination, his height and weight were in less than the 3rd percentile, while his head circumference was in the 50th percentile on the growth curve. He also showed a broad and protruding forehead and high hairline. Blood laboratory tests showed severe azotemia; emergent hemodialysis was needed. Abdominal ultrasonography revealed bilateral renal dysplasia with multiple cysts and diffuse bladder wall thickening. A posterior urethral valve was suggested based on vesicoureterography and abdominal magnetic resonance findings. Results of a colon study to rule out congenital megacolon did not reveal any specific findings. The conventional karyotype of the patient was 46, XY. Array comparative genomic hybridization study revealed a chromosome 5q35 microdeletion including the NSD1 gene, based on which SS was diagnosed. We describe a case of SS presenting with end stage renal disease due to posterior urethral valve. The typical somatic overgrowth of SS in the postnatal period was not observed due to chronic renal failure that started in the neonatal period.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제24권3호
• /
• pp.273-278
• /
• 2021

Purpose: Percutaneous endoscopic gastrostomy (PEG) is a safe method to feed patients with feeding difficulty. This study aimed to compare the outcomes of conventional PEG and laparoscopic-assisted PEG (L-PEG) placement in high-risk pediatric patients. Methods: In our tertiary pediatric department, 90 PEG insertions were performed between 2014 and 2019. Children with severe thoracoabdominal deformity (TAD), previous abdominal surgery, ventriculoperitoneal (VP) shunt, and abdominal tumors were considered as high-risk patients. Age, sex, diagnosis, operative time, complications, and mortality were compared among patients who underwent conventional PEG placement (first group) and those who underwent L-PEG placement (second group). Results: We analyzed the outcomes of conventional PEG placement (first group, n=15; patients with severe TAD [n=7], abdominal tumor [n=6], and VP shunts [n=2]) and L-PEG placement (second group, n=10; patients with VP shunts [n=5], previous abdominal surgery [n=4], and severe TAD [n=1]). Regarding minor complications, 1 (6.6%) patient in the first group underwent unplanned PEG removal and 1 (10%) patient in the second group had peristomal granuloma. We observed three major complications: colon perforation (6.6%) in a patient with VP shunt, gastrocolic fistula (6.6%) in a patient with Fallot-tetralogy and severe TAD, and pneumoperitoneum (6.6%) caused by early tube dislodgement in an autistic patient with severe TAD. All the three complications occurred in the first group (20%). No major complications occurred in the second group. Conclusion: In high-risk patients, L-PEG may be safer than conventional PEG. Thus, L-PEG is recommended for high-risk patients.

• Journal of Genetic Medicine
• /
• 제18권1호
• /
• pp.64-69
• /
• 2021

Primary cilium has a signal transduction function that is essential for brain development, and also determines cell polarity and acts as a mediator for important signaling systems, especially the Sonic Hedgehog (SHH) pathway. TBC1D32 is a ciliary protein, implicated in SHH signaling. Biallelic mutations in the TBC1D32 gene causes a kind of ciliopathy, heterogeneous developmental or degenerative disorders that affect multiple organs, including the brain. Here we report a boy who carried compound heterozygous variants in TBC1D32. The patient showed hypotonia, respiratory difficulty, and multiple anomalies at his birth. He was diagnosed with congenital hypopituitarism and treated with T4, hydrocortisone, and growth hormone. Despite the hormonal replacement, the patient needed long-term respiratory support with tracheostomy and nutritional support with a feeding tube. His developmental milestones were severely retarded. Hydrocephalus and strabismus developed and both required surgery, during the outpatient follow-up. Whole-exome sequencing indicated compound heterozygous variants, c.2200C>T (p.Arg734^*) and c.156-1G>T, in TBC1D32 gene. This is the first Korean case of TBC1D32-related ciliopathy and we reported detailed and sequential clinical features. This case demonstrated the utility of whole-exome sequencing and provided valuable clinical data on ultra-rare disease.