• The Journal of the Korean life insurance medical association
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• v.2 no.1
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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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• v.4 no.2
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• pp.156-162
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• 1998

Choledochal cyst is rare in the western countries, but common in oriental countries. Complicatioins include ascending cholangitis, recurrent pancreatities, progressive biliary cirrhosis, portal hypertension, stone formation and later malignant transformation. Bile peritonitis secondary to rupture is one of the rarest complications, with an incidence of 1.8 % to 18 %. The anomalous arrangement of the pancreatobiliary ductal system with a long common channel may cause inflammation leading to perforation of the cyst. The authors found 4 cases (14.2 %) of bile peritonitis among 28 cases of choledochal cyst treated from Jan. 1983 to Jan. 1998. The patients ages ranged from 6 months to 3 years and three were female. The perforation sites were located on the common bile duct at its junction with the cystic duct in 2 cases, the distal cyst wall in 1 case and the left hepatic duct at its junction with cyst in 1 case. The types of choledochal cysts by Todani's classification were Type IVa in 3 cases and type I in 1 case. By the new Komi's classification utilizing operative cholangiogram there were 2 cases of Type Ia, 1 case of type IIb and 1 case of type III. One stage cyst excision and hepaticojejunostomy(Roux-en Y type) was done in 3 cases, and two staged operation in 1 case. All patients had an uneventful course postoperatively. The average day of discharge was 9.8th postoperatively. In conclusion, primary excision of the choledochal cyst and biliary reconstruction is a safe and effective treatment of ruptured choledochal cyst in infants.

• Journal of Yeungnam Medical Science
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• v.33 no.2
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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.

• Journal of the korean academy of Pediatric Dentistry
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• v.27 no.1
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• pp.70-76
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• 2000

Congenital biliary atresia with progressive sclerosis of the intra- and extra-hepatic duct system occurs in 1 : 10,000 live births, and has a poor prognosis with an expected survival of less than 5 years. Etiology of biliary atresia is unclear, however, it is believed a genetic or developmental cause. The clinical characteristics include pronounced jaundice, hepatosplenomegaly, pruritus, steatorrhea, xanthomas, growth retardation, portal hypertension, bleedings, ascites and respiratory infections. Oral manifestations have seldom been reported in patients with biliary atresia, but there may be enamel hypoplasia, delayed tooth eruption, and green teeth. Early diagnosis and surgical intervention have decreased morbidity. returned growth and development to normal and improved the prognosis for survival. Authors report the clinical and radiologic characteristics, proper managements about two cases with biliary atresia.

• The Korean Journal of Nuclear Medicine
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• v.25 no.2
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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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• v.18 no.2
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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.

• The Korean Journal of Nuclear Medicine
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• v.22 no.1
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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.

• Journal of Physiology & Pathology in Korean Medicine
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• v.23 no.4
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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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• v.7 no.1
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• pp.92-97
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• 2004

Posttranplantations lymphoproliferative disease (PTLD) is a common and life-threatening complication for soid organ transplantation associated with the use of chronic immunosuppression and Epstein-Barr virus. There is no standardized treatment algorithm, but numerous management strategies are vaiable. Partial splenic embolization (PSE) had been demonstrated to be an effetive alternatie to splenectomy for patients hypersplenism and portal hypertension. PSE has the advantages of non-invasive intervention and resolution of the complications of hypersplenism. We report the effect of the PSE in a 6-year old male liver transplantation recepient with PTLD who has undergone persistent hypersplenism post-transplant. We reduced immunosuppression agent, started antiviral agent. We started with interferon and IV globulin one month after admission. Hepatosplenomegaly and cervical lymphadenopathy were improved. But fever was not subside. We selectively embolized the lower pole of the spleen to achieve a 50~60% reduction in flow as determined by angiography. After embolization, fever subside and peripheral blood findings were improved. Follow up abdominal CT revealed reduced volume of spleen due to ischemic change and there was no multiple enlarged mesenteric lymphnode compared to preembolization state. We thick that PSE is a safe an effetive treatment modality of PTLD with persistent hypersplenism in patients twho failed to medical treatment.

• Journal of Chest Surgery
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• v.43 no.5
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• pp.525-528
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• 2010

Congenital interruption of the inferior vena cava (IVC) can lead to secondary hepatic congestion, portal hypertension, and liver cirrhosis. A 49-year-old woman was admitted to the gynecology department with symptoms of menorrhalgia, known uterine myoma, and anemia. Abdominal computed tomography (CT) and venography performed at our hospital revealed congenital interruption of the IVC. The patient underwent retrohepatic cavoatrial bypass surgery with a polytetrafluoroethylene (PTFE) 16-mm ringed graft via posterolateral thoracotomy, and recovered without major complications. A retroperitoneal approach via posterolateral thoracotomy provides appropriate visualization during dorsal cavoatrial bypass in treating patients with congenital interruption of IVC.