• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제7권1호
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• pp.98-101
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• 2004

Congenital hepatic fibrosis is an inherited, congenital disorder of the liver characterized by portal hypertension and hepatic fibrosis. We experienced a case of congenital hepatic fibrosis with esophageal varix in a 9-year-old male. He complained hematemesis, hematochezia, dizziness. In laboratory examination, AST/ALT was slightly increased. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and hypoechoic lesion of periportal area were seen by abdominal CT scanning. Histologic finding of liver biopsy showed fibrous tracts containing dilated bile ductules connecting adjacent portal spaces that were widened by mature fibrosis. Endocopic sclerotherpy and ligation was done. We summarized a case with review of literatures

• Journal of Yeungnam Medical Science
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• 제28권1호
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• pp.99-104
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• 2011

Essential thrombocythemia (ET), a subcategory of chronic myeloproliferative disorder, is characterized by absolute thrombocytosis due to excessive clonal proliferation of platelets, hyperaggregability of platelets, and increased incidence of thrombosis and hemorrhage. We consider a diagnosis of ET when an unexplained and persistent thrombocytosis is observed. It is difficult to consider ET first when we meet a patient with esophageal varix bleeding or unusual multiple thromboses like mesenteric vein, splenic vein, and portal vein. This article reports a patient who presented initially with esophageal varix bleeding and unusual multiple thromboses, thereafter, she was diagnosed with ET after testing positive for the Janus Tyrosine Kinase 2 (JAK2) V617F mutation. In conclusion, in patients with varix bleeding and unusual multiple thromboses, myeloproliferative disorders like essential thrombocythemia should be considered as a potential cause and testing for the JAK2 mutation is warranted.

• Clinical and Experimental Pediatrics
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• 제56권11호
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• pp.500-504
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• 2013

Here we present the case of an 11-year-old female patient diagnosed with Caroli syndrome, who had refractory esophageal varices. The patient had a history of recurrent bleeding from esophageal varices, which was treated with endoscopic variceal ligation thrice over a period of 2 years. However, the bleeding was not controlled. When the patient finally visited the Emergency Department, the hemoglobin level was 4.4 g/dL. Transhepatic intrajugular portosystemic shunt was unsuccessful. Subsequently, the patient underwent percutaneous transhepatic variceal obliteration. Twenty hours after this procedure, the patient complained of aphasia, dizziness, headache, and general weakness. Six hours later, the patient became drowsy and unresponsive to painful stimuli. Lipiodol particles used to embolize the coronary and posterior gastric veins might have passed into the systemic arterial circulation, and they were found to be lodged in the brain, kidney, lung, and stomach. There was no abnormality of the portal vein on portal venography, and blood flow to the azygos vein through the paravertebral and hemiazygos systems was found to drain to the systemic circulation on coronary venography. Contrast echocardiography showed no pulmonary arteriovenous fistula. Symptoms improved with conservative management, and the esophageal varices were found to have improved on esophagogastroduodenoscopy.

• Journal of Chest Surgery
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• 제36권1호
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• pp.51-54
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• 2003

환자는 53세 남자로 식도 정맥류 파열로 Sengstaken-Blaceore관을 삽관하였다. Sengstaken-Blaccore관 삽관 이틀 후 좌측에 혈흉이 생겨 본원으로 전원되었다. 식도내시경 소견상 길이 8cm에 이르는 거대 흉부 식도 파열이 관찰되었다. 반복되는 식도 정맥류 출혈과 전신 상태의 악화 등으로 좌측 개흉술을 식도 파열 후 33 일째에 실시하였다 좌측 농흉과 8cm크기의 위아래로 파열된 식도를 관찰할 수 있었다. 파열부위는 변연절제 후 단순 봉합하고, 파열된 식도 부위의 위 아래 경계부위에서 각각 2cm 거리를 두고 비흡수성 스테플러(TA stapler 60 H 4.8)를 이용하여 배제시킨 뒤 흉관을 위치시켰다. 흉부 식도 배제술 6일 후 실시한 식도 조영술에서 위쪽 스테플링한 부위와 연하여 누출이 관찰되었다. 경부식도 배제술을 같은 방법으로 실시하였다. 환자는 파열된 흉부 식도 배제술 137일 후, 이차적인 식도 재건술없이 경구로 음식의 섭취가 가능하였다.

• Journal of Chest Surgery
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• 제12권4호
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• pp.371-378
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• 1979

The authors have performed operations on vascular system of 108 cases from 1972 through 1979, and analyzed the diseases, surgical procedures and results. They were 1. Arterial system; 45 cases P.D.A. : 20 Occlusive diseases : 13 Coarctation of aorta : 4 Aneurysm : 4 A-V fistula : 2 Trauma : 2 2. Venous system; 6 cases Esophageal varix : 4 S.V.C. syndrome : 1 Varicose vein : 1 3. Arteriovenous shunt for hemodialysis; 57 cases Of the arterial diseases, the worst results came from Burger`s disease. For the bleeding esophageal varices, we have performed ligations of varices or collateral circulations rather than emergency splenorenal shunt with good results. External A-V shunt for hemodialysis had much more complications than the A-V fistula.

• Tuberculosis and Respiratory Diseases
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• 제44권3호
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• pp.639-648
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• 1997

배 경 : 환자에서 동맥혈 저산소증이 생길 수 있다는 보고가 있는데 기관지혈관 확장에 의해서 확산 장애에 의해 동맥혈 산소분압이 감소한다고 한다. Cabanes 등은 좌심실부전 환자에서 메타콜린에 기관지 반응이 증가함을 보고하면서 기관지혈관 확장이 한 기전이라고 주장하였다. 이에 저자들은 간경변증 환자에서 문맥고혈압에 의하여 메타콜린에 대한 기관지반응이 증가할 것이라는 가설하에 간경변증 환자에서 기관지수 축유발검사를 시행하고, 간경변증정도 및 식도 정맥류 정도와의 기관지반응과의 관계를 알아보기 위해 본 연구를 시행하였다. 방 법 : 임상적 문맥고혈압을 가진 간경변증 환자 22명에게 메타콜린 기관지수축유발검사를 시행하였고 또한 간경변 정도를 Pugh-Child 분류법에 따라 분류하고 식도내시경 검사를 시행하였다. 성적 : 총 22명의 임상적으로 문맥고혈압을 가진 간경변증 환자중 남자는 17명, 여자는 5명이었고, 연령분포는 19세에서68세 까지이며 평균연령은 48세였으며, 간경변증 원인은 알콜성 간염에 의한 경우가 9예, B형 바이러스성 간염에 의 한것이 12예였고 C형 바이러스성 간염에 의한것이 1예였다. 22명의 환자중 15예(68.2%)가 메타콜린 기관지 수축유발검사 양성이였고, 양성인 환자 15명의 $PC_{20}$은 $0.3mg/m{\ell}$에서 $16.6mg/m{\ell}$로 다양하였으며 평균값은 $6.5{\pm}5.1mg/m{\ell}$ 이다. 메타콜린 기관지 수축유발검사 $PC_{20}FEV_1$값과 Pugh-Child 분류법에 의한 간경변의 정도 사이에는 통계학적으로 상관관계가 없었다(p>0.05). 메타콜린 기관지 수축유발검사 $PC_{20}FEV_1$ 값과 식도 내시경상 식도정맥류의 심한 정도 사이에도 통계적으로 유의한 상관관계가 없었다(p>0.05). 결 론 : 이상의 결과에서 간경변증 환자에게서 메타콜린 기관지 반응이 증가(68.2%)함을 수 있었고 간경변증 정도나 식도정맥류 정도 사이에는 유의한 상관관계가 없었다.

• Clinical Endoscopy
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• 제55권3호
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• pp.381-389
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• 2022

Background/Aims: The treatment of superficial esophageal neoplasms (SENs) in cirrhotic patients is challenging and rarely investigated. We evaluated the outcomes of endoscopic submucosal dissection (ESD) to determine the efficacy and safety of treating SENs in patients with liver cirrhosis. Methods: The baseline characteristics and treatment outcomes of patients who underwent ESD for SENs between November 2005 and December 2017 were retrospectively reviewed. Results: ESD was performed in 437 patients with 481 SENs, including 15 cirrhotic patients with 17 SENs. En bloc resection (88.2% vs. 97.0%) and curative resection (64.7% vs. 78.9%) rates were not different between the cirrhosis and non-cirrhosis groups (p=0.105 and p=0.224, respectively). Bleeding was more common in cirrhotic patients (p=0.054), and all cases were successfully controlled endoscopically. The median procedure and hospitalization duration did not differ between the groups. Overall survival was lower in cirrhotic patients (p=0.003), while disease-specific survival did not differ between the groups (p=0.85). Conclusions: ESD could be a safe and effective treatment option for SENs in patients with cirrhosis. Detailed preprocedural assessments are needed, including determination of liver function, esophageal varix status, and remaining life expectancy, to identify patients who will obtain the greatest benefit.

• Journal of Chest Surgery
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• 제17권2호
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• pp.269-274
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• 1984

Since tuberculosis was a common pulmonary disease in Korea, Aspergillosis was easily misdiagnosed as tuberculosis and an acute form of Aspergillosis was misinterpreted as pneumonia because of their similarities in the X-ray findings. This investigation is designed to illustrate the clinical features and preoperative diagnosis and surgical role in the management of this disease. In a retrospective review of operative cases from Jan. 1963 through Dec. 1983, 36 cases were analyzed. Peak age incidence lies in the 3rd decade [41.7%]. All cases had a history of treatment with antituberculous drugs under diagnosis of pulmonary tuberculosis and the most common chief complaint was hemoptysis [69.5%]. Only nine cases [25%] showed cavitary lesions with mycetoma and preoperative sputum study for fungus showed low positive valve [42.3%]. Anatomical location of lesion was located mainly upper lobe [66.7%] and most of cases were managed by lobectomy. We experienced 7 cases of complication; they were postoperative empyema, hepatic failure, esophageal varix bleeding. Postoperative pathologic findings showed that 29 cases [80.5%] were combined with tuberculosis 3 cases were combined with bronchiectasis and 4 cases were not combined with other disease. In conclusion, when the patient has a longstanding history of pulmonary tuberculosis and has a hemoptysis, he must be suspected fungus super infection. Resectional surgery is the treatment of choice for symptomatic localized disease and needed resection in asymptomatic patient to prevent possible fatal sequelae in the future.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제10권2호
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• pp.221-225
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• 2007

저자들은 혈변을 호소하였던 3세된 여아에서 소아에서 비교적 드문 식도 정맥류와 비종대가 합병된 특발성 문맥 고혈압 1예를 경험하였기에 문헌 고찰과 더불어 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제45권7호
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• pp.923-927
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• 2002

우연히 발견된 간비장 종대를 주소로 내원한 3세 여아에서 신낭종을 동반한 선천성 간섬유화증을 진단한 예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.