• 보험의학회지
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• 제2권1호
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• pp.218-232
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• 1985

Congenital hereditary disease is in devided into Infantile type and Adult type, Adult type is hidden for many years and keeps normal renal function till middle age. Cyst is stimultaneously made in both sides and becomes lowered in renal function in 30's to 40's. Infantile type is generally born with the big kidneys, renal failure, undergrowth of intrahepatic bile duct. Both infantile and childhood type have ureteral dilatation and portal hypertension In infantile type, it is mostly developed into renal failure, but generally faces death as a result of hepatic disease. The reason of death is that an abnormal condition of recessive autosome affects the liver and kidneys. While the incidence of infantile type is rare as $0.017{\sim}0.07%$ and it is autosomal recessive heredity, Adult type can rarely exist in infantile period. Though it exists in middle period, 50% of patients can live for 2-4 years after the first symptom incidence and 25% can less than 2 years. It is hard to cure completely in medicine and surgery. Three difficulties in familial incidence are comparative decrease of the donor who have no affection on renal transplantation. For another consideration it is to show the family history for several generations. We, the Med. Dept. of Dae Han Kyouk Life Insurance Co. Ltd., used the ultrasonic apparatus in diagnosing the one case of adult type bilateral polycystic kidney and then doubted the family history. As a result of inspecting the family we experienced bilateral polycystic kidney from 3 persons out of 4 who can be inspected. The results are as follows: 1) We could confirm the polycystic kidney from 3 persons out of 4(75%). 2) Then when they came for check up, chief complaint was the pain in all 3 cases(100%). 3) Accompanying disease was hypertension in 2 cases(67%). 4) In early disease incidence, we couldn't observe the specific change in pathological opinion. 5) All 3 cases are not accompanied with cystic lesion in liver, spleen, pancreas.

• Advances in pediatric surgery
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• 제4권2호
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• pp.156-162
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• 1998

저자들은 1983년 1월부터 1998년 1월까지 총담관낭 환아 28례 중 4례(14.2 %)에서 자연적으로 발생한 총담관낭 천공에의한 담즙성 복막염을 경험하였고 이 중 3례에서 일차적인 낭종 절제와 간관공장 문합술을 시행하였으며 이 모든 예에서 합병증 없이 회복되었기에 문헌 고찰과 함께 보고하는 바이다. 총담관낭 천공에의한 답즙성 복막염치료는 일차적 낭종 절제와 간관 공장 문합술이 안전하고 효과적인 방법이라고 생각된다.

• Journal of Yeungnam Medical Science
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• 제33권2호
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• pp.155-158
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• 2016

Sorafenib (Nexavar) has been regarded as a treatment for unresectable hepatocellular carcinoma (HCC), with side effects that include hand-foot skin reaction, diarrhea, rash, fatigue, hypertension, nausea, anorexia, weight loss, and alopecia. Thyroid disorder, such as endocrine side effect, has also been reported. However no case involving adrenal insufficiency has been reported. Here, we report a case of adrenal insufficiency which occurred after taking sorafenib in a patient with HCC. A 56-year-old man visited our hospital due to right upper quadrant abdominal pain and he was diagnosed as multiple disseminated and unresectable HCCs with portal vein invasion; therefore transarterial chemoembolization was performed and sorafenib administration was started. Two months later, he was admitted to the hospital complaining of severe fatigue. The laboratory results showed cortisol of <$0.2{\mu}g/dL$ and adrenocorticotropic hormone of <1.00 pg/mL. The patient had no history of taking steroids or herbal medications. Secondary adrenal insufficiency was diagnosed and prednisolone 10 mg per day was started immediately; as a result, fatigue remarkably improved. This may be the first report indicating a possible association between sorafenib and adrenal insufficiency and it implies that the possibility of adrenal insufficiency should be considered in patients taking sorafenib who complain of severe fatigue.

• 대한소아치과학회지
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• 제27권1호
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• pp.70-76
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• 2000

Intra- & extra-hepatic duct system의 경화를 야기하는 Biliary atresia는 출생시 1 : 10,000의 발생빈도를 보이며, 5년 이하의 생존율을 나타내는 예후가 좋지 않은 질환이다. 담도폐쇄증의 원인은 아직 명확하지 않으나, 유전적인 요소나 발육성 원인으로 믿고 있다. 임상적 특징으로 두드러진 황달, 간비증대, 가려움증, 지방변, 황색종, 성장지연, 문맥압항진증, 출혈, 골연하증, 복수 그리고 호흡기계 감염 등을 나타낸다. 구강내 소견으로 녹색을 띄는 변색치아, 유치의 확장된 치수강과 치근관, 치아맹출지연, 법랑질 형성부전, 그리고 심한 치아 우식 등이다. 담도폐쇄증은 조기진단과 수술에 의해 사망률이 감소되고, 성장과 발달이 정상으로 돌아오며 생존율도 향상되었으나 여전히 그 원인과 치료에 대한 연구가 계속되어야 할 것이다. 이에 저자는 전북대학교 치과병원에 내원한 2명의 담도폐쇄증환아에 대해 임상, 방사선학적 특징 및 적합한 처치에 대하여 보고하고자 한다.

• 대한핵의학회지
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• 제25권2호
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• pp.200-206
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• 1991

Arterial fraction of total hepatic blood flow was estimated by a new method, slope method, on radionuclid angiogram using $^{99m}Tc-DISIDA$ and was compared with that from $^{99m}Tc-Phytate$ radionuclide angiogram. This study included 11 of normal subjects, 37 of intermediate group with various liver diseases, and 25 patients with liver cirrhosis. We analyzed the datas with slope method from radionuclide angiograms and the results were compared with hepatic arterial fractions from uptake method, introduced by Lee et al. at 1986. The hepatic arterial fractions from radionuclide angiograms using $^{99m}Tc-DISIDA$ and $^{99m}Tc-Phytate$ were $0.32{\pm}0.09\;and\;0.31{\pm}0.11$ respectively in normal subjects, and $0.75{\pm}0.18\;and\;0.77{\pm}0.21$ respectively in patients with liver cirrhosis. The hepatic arterial fractions by the slope method was well correlated with those of the uptake method on $^{99m}Tc-DISIDA$ scan. There was high correlation between the hepatic arterial fractions from $^{99m}Tc-DISIDA$ and $^{99m}Tc-Phytate$ scans. Hepatic arterial fraction estimated by the slope method is a useful index for the diagnosis of liver cirrhosis and the evaluation of status of portal hypertension.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제18권2호
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• pp.121-127
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• 2015

Purpose: To evaluate clinical and laboratory profile of Wilson's disease (WD) in children. Methods: This cross sectional study was conducted at Bangabandhu Sheikh Mujib Medical University Hospital. Bangladesh, over a period of 3 years. One hundred consecutive children of WD between 3 to 18 years of age were evaluated. Results: Mean age was $8.5{\pm}1.5years$. Male female ratio was 2:1. Ninety-one percent of patients were Muslim and 9% Hindu. A total of 53% cases of hepatic WD presented between 5 to 10 years of age and most of the neurologic WD manifested in 10-15 years age group. Sixty-nine children presented only with hepatic manifestations, 6 only with neurological manifestations, 14 with both hepatic and neurological manifestation, 10 children was asymptomatic and 1 patient presented with psychiatric features. WD presented as chronic liver disease (CLD) in 42%, CLD with portal hypertension in 34%, acute hepatitis in 20% and fulminant hepatic failure in 4% cases. Stigmata of CLD were found in 18% patients. Keiser-Fleischser ring was found in 76% total patients. Elevated serum transaminase was found in 85% cases, prolonged prothrombin time in 59% cases and hypoalbuminaemia in 53% cases. A total of 73% patients had low serum ceruloplasmin, basal urinary copper of >$100{\mu}g/day$ was found in 81% cases and urinary copper following penicillamine challenge of >$1,200{\mu}g/day$ was found in 92% cases. Conclusion: Majority of studied WD children presented with hepatic manifestation of which 76% presented with CLD. Any child presented with jaundice after the age of 3 years should be investigated for WD.

• 대한핵의학회지
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• 제22권1호
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• pp.47-53
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• 1988

Budd-Chiari syndrome (BCS) is a rare clinical entity characterized by post-sinusoidal portal hypertension caused by the obstruction to the hepatic vein outflow The diagnosis is suggested by hepatic scintigraphy and is usually confirmed by hepatic venography, inferior vena cavography and biopsy. The scintigraphic finding of BCS caused by the obstruction of main hepatic vein has been reported to consist typically of hypertrophy of the caudate lobe with increased radionuclide accumulation. Such a typical finding has been accounted for by the fact that the venous outflow from the caudate lobe is preserved when the main hepatic vein is obstructed. But usually, the hepatic venous outflow from the caudate lobe is also obstructed in BCS due to inferior vena caval obstruction. So hepatic scintigraphic findings of BCS due to inferior vena caval obstruction show different findings as compared with the BCS due to hepatic vein obstruction. We evaluate the hepatic scintigrams of the 13 cases of BCS due to inferior vena caval obstruction and review the literatures. The results are as follows : 1) We cannot observe the caudate lobe hypertrophy with increased uptake, which is known as a classic finding in BCS due to hepatic vein obstruction. 2) The most prominent hepatic scintigraphic findings of BCS are nonhomogenous uptake in the liver with extrahepatic uptake in the all cases. 3) We can see cold areas at the superior aspect of right hepatic lobe in 7 cases (54%). This is a useful finding suggesting BCS due to inferior vena caval obstruction.

• 동의생리병리학회지
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• 제23권4호
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• pp.919-924
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• 2009

The liver is one of the principal organs involved in glucose metabolism. Diabetes mellitus(DM) commonly develops in patients with liver cirrhosis as a result of hepatocyte dysfunction. Hepatic encephalopathy(HE) is a major neuropsychiatric complication of liver cirrhosis. HE develops frequently in advanced stage of liver cirrhosis due to portal hypertension. We experienced a case of liver cirrhosis with DM and HE in 67 years old male patient. The patient's symptoms were lethargy, general malaise, asterixis, dizziness, and heavy headedness. Hwangryunjihwang-tang, Saengkankunbi-tang, Injinchija-tang and Injinho-tang were prescribed to the patient. Finally, the symptoms had been improved, however significant change was not observed from serum ammonia, fasting blood sugar and postparandial blood sugar. So we hope that this case report will be helpful in treating patient of liver cirrhosis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제7권1호
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• pp.92-97
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• 2004

저자들은 간 이식 후 지속적 비기능 항진에 발생된 내과적 치료에 반응하지 않는 림프세포증식성질환의 6세된 남아에서 부분적 비동맥 색전술을 치료의 한 방법으로 선택하였다. 비기능항진의 억제 효과로 면역력 증강을 통해 혈액학적 호전을 유도하고자 하였으며 치료 후 PTLD의 호전을 보인 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
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• 제43권5호
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• pp.525-528
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• 2010

선천성 하대정맥의 중단은 간울혈과 간변성을 유발하며, 비장비대와 복수와 복통, 하지부종이 동반된다. 49세 여자환자가 자궁근종과 월경과다, 그리고 철결핍성빈혈로 본원 산부인과에 입원하여 수술 전 복부컴퓨터단층촬영검사 및 복부 및 흉부 대정맥조영술을 시행하여 하대정맥의 중단의 소견이 관찰되었다. 환자는 우측 후측방 개흉술을 시행하여 흉강 내로 접근하였고, 횡격막을 절개하여 후복막 접근을 이용하여 하대정맥을 박리하여 16 mm PTFE graft를 이용하여 우회수술을 시행하였다. 수술 후 graft의 꺾임 현상이 관찰되어 혈관성형술 및 스텐트 삽입을 시행받았으며, 이후 환자는 특별한 문제 없이 회복되었다. 하대정맥의 선천성 중단이 관찰되는 환자에게서는 PTFE 인조혈관을 이용한 후방 대정맥-우심방 우회수술이 적절한 선택이며, 후측방 개흉술을 통한 복막뒤공간 접근법이 적절한 시야를 제공할 수 있을 것이라 생각된다.