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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.

      • 저위 쇄항의 임상적 특징 및 수술 결과 고찰

        전용순 인제대학교 백병원 2002 仁濟醫學 Vol.23 No.4

        Objectives: The majority of patients with imperforate anus are reported to have a malformation of the low variety. Despite this, much of the discussion in the literature is devoted to the repair and results of the more complex, high lesions. The purpose of this study was to review the clinical characteristics and the operative results in patients with low imperforate anus. Methods and Meterials: A retrospective review was conducted of the medical records of patients treated for imperforate anus of the low type at Inje University Pusan Paik Hospital from March, 1997 to June 1999. 11 patients were included. A follow-up telephone survey of patients' parents was conducted. Results: Twenty patients were identified who were surgically treated for imperforate anus at Inje University Pusan Paik Hospital from March 1997 to June 1999. 11 patients (55%) were diagnosed with low type. 8 patients were boys and 3 were girls. The ages at time of operation for imperforate anus ranged from less than 24 hours to 5 months. All patiens were diagnosed with anoperineal fistula. 2 patients (18%) were born with at least one associated congenital anomaly. Cardiac, genitourinary and musculoskeletal anomalies were diagnosed. One patient exibited renal hypoplasia and polydactyly, and was diagnosed as VACTERL association. Cut-back anoplasty was performed in all patients. There was no postoperative complication. Now patients' ages range from 3 years 6 months to 5 years 3 months. One patient had constipation transiently, but now no patient has constipatient and all show normal bowel function. According to the degrees of parents' satisfaction about the shape and the position of anus, no parent is worried about the shape and the position of anus. All parents consider the bowel function more importantly than the cosmetic aspect of anus. Conclusion: Low imperforate anus can be successfully treated using a cut-back anoplasty and the need according to normal bowel function and good cosmetic result can be satisfied.

      • 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.

      • 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.

      • 영아기 이후에 진단된 중장염전증

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

        Midgut volvulus secondary to intestinal malrotation usually presents within the first month of life, typically with persistent bilous vomiting. Diagnostic delay may result in midgut infarction and death. 4-year-old boy was admitted presenting bilous vomiting and abdominal pain. The patient has had periodic bilous vomiting, irritability, abdominal pain but he just has received conservative treatment such as fasting and hydration. He was malnourished state. Bowel sound was decreased and there was right upper quadrant tenderness without abdominal distension. On abdominal radiograph, there was no gas shadow beyond jejunum and stomach was distended. Ultrasound showed distended stomach, duodenum and proximal jejunum. "Whirlpool sign" on color Doppler ultrasound was observed. During operation, intestinal malrotation and counter clockwise midgut volvulus were observed and there was no intestinal necrosis. Derotation of volvulus, Ladd's procedure and appendectomy were performed. Postoperatively, bilous vomiting and abdominal pain disappeared and persistent weight gain is being observed.

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