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      • 수술후 10년 이상 장기 생존 담도폐쇄증 환장에서의 영양상태 및 임상적 특성에 관한 분석

        전용순,김우기,Chun, Yong-Soon,Kim, Woo-Ki 대한소아외과학회 1998 소아외과 Vol.4 No.2

        To assess the clinical and nutritional status of long-term survivors of biliary atresia, history taking, medical record review, physical examination (height, weight, MAC, TSF), blood tests (LFT, prothrombin time, platelet count, prealbumin, calcium) and liver needle biopsy were performed in 12 patients in whom Kasai procedure were performed more than 10 years ago at Department of Pediatric Surgery in Seoul National University Hospital. None were below the 5th percentile in height and weight. TSF was above the 75th percentile in all patients and showed good subcutaneous fat deposition. MAC was above the 5th percentile in all patients. Serum prealbumin level was abnormal in 2 patients with abnormal liver function and revealed visceral protein malnutrition. Serum calcium level was decreased below normal range in 4 patients with abnormal liver function. One patient had mild ascites. Five patients had abnormal liver function and 7 patients showed clinical manifestation of portal hypertension. Liver needle biopsy was performed in 5 patients and no cirrhotic change was observed. Although some patients who have survived for more than 10 years after Kasai procedure developed protein malnutrition and vitamin deficiencies, growth and development and nutritional status were generally satisfactory. Five patients(42%) showed normal liver function and no portal hypertension. In conclusion, Kasai procedure is satisfactory as a primary treatment in biliary atresia but significant portion of long-term survivors had abnormal liver function and portal hypertension. Continuous and careful follow-up is necessary to determine when liver transplantation may be indicated.

      • 기도결찰을 시행한 토끼 태자의 실험적 횡격막탈장에서 Lamellar body counts

        전용순,정수진,이정녀,Chun, Yong-Soon,Jung, Soo-Jin,Lee, Jeong-Nyeo 대한소아외과학회 2011 소아외과 Vol.17 No.2

        Experimental tracheal ligation (TL) has been shown to reverse the pulmonary hypoplasia associated with congenital diaphragmatic hernia (CDH) and to normalize gas exchange. The purpose of this study was to determine whether the TL would correct the surfactant deficiency present in the fetal rabbit model of CDH by using lamellar body count. Lamellar bodies are synthesized and secreted by the type II pneumocytes of fetal lung. The phospholipids present in these bodies constitute the major component of pulmonary surfactant. Twenty-one pregnant New Zealand rabbits underwent hysterotomy and fetal surgery on gestational day 24. Two fetuses of each pregnant rabbit were operated. In the fetus of one end of bicornuate uterus, left DH was created by excision of fetal diaphragm through open thoracotomy (DH Group). In the fetus of the other end of bicornuate uterus, left DH and TL were created (TL Group). The fetuses were delivered by Cesarean section on gestational day 31. Fourteen in control group, 12 in the DH group and 13 in TL group were born alive. En bloc excision of lungs, bronchi and trachea was done in all newborn rabbits. A five Fr catheter was inserted through trachea and repeated irrigations with 10 cc normal saline were done. The irrigated fluid was centrifuged at $280{\times}g$ for 5 minutes and the lamellar bodies were counted with the upper level fluid in platelet channel of electronic cell counter. The average lamellar body counts were $37.1{\pm}14.2{\times}10^3/{\mu}L$ in control group, $11.5{\pm}4.4 {\times}10^3/{\mu}L$ in DH group, and $6.5{\pm}0.9{\times}10^3/{\mu}L$ in TL group. Lamellar body count in DH group was lower than in control group and did not increase after TL. This study shows TL has no therapeutic effect on decreased surfactant level of CDH and the pregnant rabbit is appropriate for the animal model of CDH.

      • 제대탈장과 동반된 방관 제외번

        전용순 인제대학교 백병원 2002 仁濟醫學 Vol.23 No.5

        Abnormalities related to urachal remnants are uncommon and rarely become pathological or symptomatic. However, such a condition is still fatal when infected, and the development of late malignant changes cannot be ruled out. Most urachal anomalies are classified into five major groups: (1) a completely patent urachus, (2) urachal sinus, (3) urachal diverticulum, (4) urachal cyst, (5) alternating cyst. Umbilical evagination of the bladder(UEB) is a rare urachal anomaly. A neonate with UEB and umbilical cord hernia is reported. A full-term male with normal antenatal history and birth weight of 3.0 kg born to a mother by vaginal delivery was noted to have umbilical cord hernia and UEB at the lower edge of the umbilical cord hernia. The infraumbilical abdominal wall and external genitalia were normal, with fully descended testes. An ultrasound scan of the kidneys was normal. With the patient under general anesthesia, the sac of umbilical cord hernia was excised. A urinary catheter was inserted through the urethral orifice and appeared from the evaginated superior dome of the bladder. The interior of the bladder was normal. Excision of the evaginated part of the bladder and primary repair were done. The bladder was pushed back into the abdominal cavity. Primary closure of the umbilical cord hernia was done.

      • 선천성 처녀막 폐쇄, double vagina와 동반된 hydrometrocolpos

        전용순 인제대학교 백병원 2002 仁濟醫學 Vol.23 No.5

        Hydrometrocolpos is a pathological distension of uterus and vagina with an excessive amount of fluid in the presence of distal vaginal outflow obstruction. The incidence of hydrometrocolpos is 1 in 30,000 live births. 6-day-old girl was admitted due to feeding difficulty and abdominal distension. Abdominal ultrasound revealed left hydronephrosis, right renal hypoplasia and a huge intraabdominal cyst. On abdominal CT scan, downward extension of this cyst to the lower vagina represents hydrometrocolpos. On physical examination, imperforate hymen was observed. During operation, 70mL dark brown-colored fluid was evacuated by the needle aspiration. Parnial hymenectomy and hymenoplasty were performed. The double vagina was observed and the right vagina ended blindly 1cm above the vaginal orifice. Postoperatively, abdominal distension and feeding difficulty disappeared. Hydrometrocolpos was not observed anymore and left hydronephrosis was improved on postoperative abdominal ultrasound.

      • 외과의사의 기술적 요인과 선천성 식도폐쇄증 수술후 문합부 협착

        전용순,김우기 인제대학교 백병원 2002 仁濟醫學 Vol.23 No.5

        Background : Esophageal stricture is a common complication of anastomosis of the esophagus in esophageal atresia(EA). The factors that have been implicated in the pathogenesis in the esophageal stricture include the poor anastomotic technique(excessive tension, two-layered anastomosis, silk suture material), ischemia at the end of the esophagus, anastomotic leak, and gastroesophageal reflux. Method : To evaluate the importance of the surgeon's technique affecting anastomotic stricture after primary repair in EA, we reviewed 69 patients with EA operated at Department of Pediatric Surgery, Seoul National University Hospital from April 1978 to June 1995 and analyzed statistically the effects of such variables as sex, age at admission, age at corrective surgery, birth weight, combined anomaly, duration from admission to operation, anastomotic leakage, postoperative complication except leakage and gastroesophageal reflux, on stricture. Results : Anastomotic stricture was developed in 36(52.2%) of 69 patients who underwent primary repair of EA. There was no statistically significant factor affecting anastomotic stricture. Conclusion : The importance of cautious surgical technique should be emphasized to prevent anastomotic stricture.

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