• Title/Summary/Keyword: 탈장

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Early Onset Renal Failure in Congenital Nephrotic Syndrome associated with Congenital Diaphragmatic Hernia by WT1 Gene Mutation (WT1 유전자 돌연변이에 의해 선천성 가로막 탈장이 동반되고 조기 신부전이 초래된 선천성 신증후군 1례)

  • Park, Yong-Jun;Oh, Jin-Won;Choi, Kyong-Min;Kim, Pyung-Kil;Lee, Jong-In;Song, Ji-Sun
    • Childhood Kidney Diseases
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    • v.13 no.1
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    • pp.84-91
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    • 2009
  • We experienced a female neonate with congenital nephrotic syndrome (CNS) associated with congenital diaphragmatic hernia (CDH). Because of the rare combination of two conditions, we report this case with literature review. CDH was found immediately after birth and emergency operation was done for hernia repair. But on the next day, generalized edema and oliguria(0.59 mL/kg/hour) was found and her blood chemistry showed hypoalbuminemia (1.6 g/dL), increased BUN (27.7 mg/dL) and serum creatinine( 1.8 mg/dL) along with heavy proteinuria (4+). We started albumin infusion with a bolus of intravenous furosemide. We suspected the neonate had congenital nephrotic syndrome and her 24hr urine protein was 1,816 mg/day. In spite of immunosuppressive therapy, the nephrotic syndrome and renal failure progressed. We started peritoneal dialysis on the day of life 22 but it was not satisfactory. She was complicated by intracranial hemorrhage and multi-organ failure and expired at 34 days of age. Kidney necropsy was performed which showed diffuse mesangial sclerosis (DMS). Her chromosome study revealed 46, XX and her gene study revealed a heterozygous missense mutation, Arg366His, in Wilms tumor suppressor gene (WT1). This case deserves attention on account of the 4th case of CNS with CDH revealing the Arg366His mutation in the WT1 gene and G the 1st case of early onset renal failure without male pseudohermaphroditism and Wilms tumor with CNS, CDH and the Arg366His mutation in the WT1 gene. So, this report gives support to the hypothesis that Arg366His mutation in the WT1 gene can result in CNS and CDH.

A Case of Short Bowel Syndrome Due to Strangulated Congenital Internal Hernia in Children (교액성 선천성 내부 탈장에 의해 발생한 단장 증후군 1례)

  • Sim, Chang Eun;Moon, Jin Soo;Kim, Ki Hong;Kim, Han-Seong
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.7 no.2
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    • pp.268-273
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    • 2004
  • Short bowel syndrome in children, most commonly results after extensive bowel resection for necrosis of the bowel. It may be caused by several intestinal catastrophes such as volvulus, hernia and necrotizing enterocolitis. The risk factors on short bowel syndrome are the remaining length of the bowel, the age of onset, the absence of the ileo-cecal valve and the time after resection. Macro and micronutritional deficiencies are the most significant complications of short bowel syndrome. We report a 5 year-old girl, who had a strangulated congenital transmesenteric hernia leading to short bowel syndrome accompanied by iron deficiency anemia.

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Laparoscopic Nissen Fundoplication and Collis Gastroplasty (복강경을 이용한 니센 위저추벽성형술 및 콜리스 위성형술)

  • Song Sang-Yun;Park Jeong-Min;Jung In-Suk;Anh Byung-Hee;Na Kook-Ju
    • Journal of Chest Surgery
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    • v.39 no.9 s.266
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    • pp.733-738
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    • 2006
  • The prevalence of gastroesophageal reflux disease has been increased recently in Korea. The use of minimally invasive laparoscopic and thoracoscopic surgery has become popular in the operation of esophageal disease such as esophageal cancer or gastroesophageal reflux disorder. We experienced three cases of laparoscopic Nissen fun-doplications and one case of laparoscopic Collis gastroplasty, and we will describe the technical aspect of these surgeries.

Long-term Outcome of Laparoscopic Hernia Sac Transection and Intracorporeal Ligation in Children: A Single Center Cohort Study (복강 내 탈장낭 절개 및 봉합법을 이용한 소아 복강경 서혜부 탈장 수술의 장기 추적관찰 결과: 단일기관 코호트 연구)

  • Lee, Chang Hun;Boo, Yoon Jung;Lee, Eun Hee
    • Advances in pediatric surgery
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    • v.20 no.2
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    • pp.23-27
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    • 2014
  • Purpose: Laparoscopic hernia repair in children is still controversial. The aim of this study was to report our long-term results of the laparoscopic hernia technique, which is based on the same surgical principles as conventional open herniotomy. Methods: Five hundred fourteen pediatric patients with inguinal hernia were included in this study under informed consent. All patients underwent a laparoscopic technique of sac transection and intracorporeal ligation. The asymptomatic contralateral inguinal ring was routinely explored and repaired if a patient had patent processus vaginalis on the contralateral side. Patients were prospectively followed for 5 years. Those who were lost to follow-up were excluded from the study. Perioperative complications and recurrences were evaluated. Results: The mean follow-up period was 29 months. Mean operation time was 27.5 minutes. Forty one percent of the patients had contralateral patent processus vaginalis. Only one hernia recurred (0.19%). We had one case of contralateral metachronous hernia (0.21%) during follow-up period. Conclusion: The long-term follow-up results of our study revealed that laparoscopic hernia sac transection and ligation can be a safe and effective alternative for conventional herniorraphy.

Dehydriding Performance in a Depleted Uranium Bed (감손 우라늄 베드 수소 탈장 성능)

  • KOO, DAESEO;KIM, YEANJIN;YUN, SEI-HUN;CHUNG, HONGSUK
    • Transactions of the Korean hydrogen and new energy society
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    • v.27 no.1
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    • pp.22-28
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    • 2016
  • It is necessary to store and supply hydrogen isotopes for Tokamak operation. A storage and delivery system (SDS) is used for storing hydrogen isotopes as a metal hydride form. We designed and fabricated a depleted uranium (DU) bed to store hydrogen isotopes. The rapid storage of hydrogen isotopes is very important not only for safety reasons but also for the economic design and operation of the SDS. The delivery rate at the desorption temperatures without the operation of a dry pump was analyzed in comparison with that with the operation of the dry pump. The effect of the initial desorption temperatures on the dehydriding of the DU without the operation of the dry pump was measured. The effect of the initial desorption temperatures on the dehydriding of DU with the operation of the dry pump was also measured and analyzed. The primary pressure on the desorption temperatures without the operation of the dry pump was analyzed in comparison with that with the operation of the dry pump. The temperature gradient of the coil heater and the primary vessel was also analyzed. Our results will be used to develop pilot scale hydrogen isotope processes. It was confirmed that dehydriding of a medium-scale DU bed has enabled without the operation of the dry pump.

Infected Sclerosing Lipogranuloma after Hernioplasty: Ultrasonographic and MRI Findings (탈장성형술 후 음낭 내에 발생한 감염성 경화지방육아종의 초음파 및 자기공명영상 소견: 증례 보고)

  • Jong Soo Park;Jae Ho Cho
    • Journal of the Korean Society of Radiology
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    • v.81 no.5
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    • pp.1255-1259
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    • 2020
  • We report the ultrasonographic and MRI findings of an infected sclerosing lipogranuloma after scrotal hernioplasty. Sclerosing lipogranuloma is a rare foreign-body reaction of fat tissue, with most cases being associated with the genital and urinary tracts. To the best of our knowledge, MRI findings in sclerosing lipogranuloma in the scrotal sac have not yet been published and this is possibly the first study to report the case of an infected sclerosing lipogranuloma in the English literature.

Intrathoracic Ectopic Liver - A case report - (흉강내 이소성 간 - 1예 보고 -)

  • Yoo, Dong-Gon;Park, Chong-Bin;Chang, Yong-Jin;Eom, Dae-Woon;Kim, Young-Ki;Kim, Chong-Wook
    • Journal of Chest Surgery
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    • v.40 no.11
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    • pp.802-804
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    • 2007
  • Although intrathoracic liver in association with a congenital diaphragmatic hernia has been well documented, the finding of intrathoracic ectopic liver tissue in the presence of an intact diaphragm is an extremely rare congenital anomaly. We have experienced a case of intrathoracic ectopic liver without any diaphragmatic hernia. A 37-year-old woman was admitted for the treatment of an incidentally detected right lung mass, A chest computed tomography scan revealed a right lower lobe lung mass close to the diaphragm, and this was suspicious for bronchial carcinoid tumor. Upon surgery, 2 round solid masses $3.5{\times}3.5 cm$ and a $2.0{\times}2.0 cm$ in size were noted, with their bottoms attached to the diaphragm dome. The masses were completely resected. Histologically, they were confirmed to be intrathoracic ectopic livers. The patient had an uncomplicated postoperative course.

Clinical Review of Diaphragmatic Hernia (횡경막 탈장의 임상적 고찰)

  • 장기경
    • Journal of Chest Surgery
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    • v.28 no.9
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    • pp.837-841
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    • 1995
  • Between June 1981 and April 1994, 15 patients underwent surgical repair of diaphragmatic hernia. The ages ranged from 1 day to 60 years, with a mean age of 34. There were 5 cases of congenital diaphragmatic hernia; Bochdalek hernia in 4 cases and Morgagni hernia in 1 case. There were 10 cases of traumatic diaphragmatic hernia;blunt trauma in 8 cases and stab wounds in 2 cases. The chest X-ray findings were abnormal in 10 cases. Operations were performed in all patients and there was only one death, who was a newborn with left Bochdalek hernia and pulmonary hypoplasia.

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