• Journal of The Korean Society of Inherited Metabolic disease
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• v.12 no.1
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• pp.35-41
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• 2012

Since the causative gene, SLC25A13 which encodes citrin, was discovered in 1999, over 500 cases with citrin deficiency have been identified. Two phenotypes can occur by citrin deficiency, neonatal intrahepatic cholestasis by citrin deficiency (NICCD) and adult-onset type II citrullinemia (CTLN2). Some patients with NICCD develop CTLN2 in their later lives. Although cholestatic jaundice is spontaneously resolved within the first year of life in most cases with NICCD, a few cases experience progressive hepatic failure. In this report, two neonates with severe type of NICCD were described. Both cases exhibited neonatal cholestatic jaundice, hyperammonemia and severe coagulopathy. Of note, plasma citrulline and blood galactose levels were extremely high. Serum ${\alpha}$-fetoprotein, plasma methionine, arginine, and threonine-to-serine ratio were elevated as well. SLC25A13 mutations were found in all the four alleles of both patients. With the commencement of lactose-free formula, coagulopathy and hyperammonemia were resolved, and galactose level was normalized. Currently, no factor has been identified to predict the prognosis of NICCD. More experiences are needed to build up the adequate therapeutic strategies for severe type of NICCD. Our experience, however, indicates that the degree of citrullinemia and galactosemia might reflect the severity.

• Journal of Chest Surgery
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• v.32 no.1
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• pp.75-79
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• 1999

Budd-Chiari syndrome is a state of hepatic failure caused by impairment of blood flow anywhere from the inferior vena cava to the right atrium. In this case, a 45 year old patient had undergone membranotomy and dilatation with autogenous pericardial graft due to obstruction of the inferior vena cava caused by a congenital membrane in 1987. Ten years after the operation, restenosis occurred. Although a noninvasive method with a Gianturco stent dilatation was performed, a satisfactory result was not obtained. A reoperation was performed. The stenotic segment of inferior vena cava was excised and after augmentation with a prepared pentagon shaped Gore-Tex artificial graft allowing passage of two fingers. The patient's postoperative course was uneventful without signs of rebleeding or any other complications and the patient was discharged at postoperative two weeks without the use of anticoagulants. An excellent result was obtainable after operation using a prepared Gore-Tex graft and such a result. Reoperational case of Budd-Chiari syndrome may require rapid and excellent the operative techenic by prevention of massive bleeding under use of extracorporeal circulation.

• Journal of Chest Surgery
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• v.35 no.3
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• pp.239-243
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• 2002

In this case, a 39 year-old man was admitted with Budd-Chiari syndrome associated with complete superior vena cava(SVC) obstruction causing general edema and hepatic failure. Conservative medical therapy was failed. And after the radiologist failed to invasive procedure of balloon dilatation, we attempted the inferior vena cava to right atrium bypass graft. Operation was done through median sternotomy and extended vertical oblique abdominal incision. A 24 mm Dacron tube was placed from the inferior vena cava just below the left renal vein to the right atrium without using the cardiopulmonary bypass pump. The patient's postoperative course was uneventful without signs of bleeding or any other complications. We used anticoagulants at the postoperative first day. At the postoperative 26th day, we performed abdominal Doppler sonography and we confirmed that the graft patency was good. The patient was discharged with SVC obstructive symptoms but we noticed relief of SVC obstructive symptoms in the course of follow-up.

• Advances in pediatric surgery
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• v.11 no.2
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• pp.141-149
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• 2005

Ulcerative colitis, an inflammatory bowel disease, is primarily managed medically with a combination of 5-ASA and steroids. However, this chronic disease requires surgical management if symptoms persist or complications develop despite medical management. The clinical course, indications and outcome of surgical management of 21 patients under the age of 15 who were endoscopically diagnosed with ulcerative colitis at the Seoul National University Children's Hospital between January, 1988 and January, 2003 were reviewed. Mean follow up period was 3 years and 10 months. The mean age was 10.3 years old. All patients received medical management after diagnosis and 8 patients (38 %) eventually required surgical management. Of 13 patients who received medical management only, 7 patients (53 %) showed remission, 4 patients are still on medical management, and 2 patients expired due to congenital immune deficiency and hepatic failure as a result of sclerosing cholangitis. In 8 patients who received surgical management, the indications for operation were, 1 patient sigmoid colon perforation and 7 patients intractability despite medical management. The perforated case had a segmental colon resection and the other 7 patients underwent total colectomy with ileal pouch-anal anastomosis. One patient expired postoperatively due to pneumonia and sepsis. and 1 is still on medical management because of mild persistent hematochezia after surgery. Six other operated patients are doing well without medical therapy. Pediatric ulcerative colitis patients can be surgically managed if the patient is intractable to medical management or if complications such as perforation are present. Total colectomy & ileal pouch-anal anastomosis is thought to be the adequate surgical method.

• Journal of Veterinary Clinics
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• v.25 no.1
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• pp.48-51
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• 2008

A ten-year-old Yorkshire Terrier developed serious abdominal distension and respiratory distress. Radiography and ultrasonography revealed a hyperechoic mass around the aorta that was contiguous with the right atrium and main pulmonary artery. It was resulted in failure of the right side of the heart including tricuspid regurgitation, hepatomegaly with dilation of the hepatic vein and severe ascites due to a large, expansile mass. Computed tomography(CT) identified a large mass originating at the cardiac hilar region and spanning from the cranial vena cava to the caudal vena cava. The tumor had invaded the cranial vena cava, caudal vena cava, heart and pleural wall. A tentative diagnosis of chemodectoma was assigned to the tumor through a fine needle aspiration. This report focuses on the typical features of imaging diagnosis of heartbase tumors by radiography, ultrasonography and CT.

• Molecules and Cells
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• v.38 no.10
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• pp.843-850
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• 2015

The1hepatic cell death induced by acetaminophen (APAP) is closely related to cellular adenosine triphosphate (ATP) depletion, which is mainly caused by mitochondrial dysfunction. Adenosine monophosphate (AMP)-activated protein kinase (AMPK) is a key sensor of low energy status. AMPK regulates metabolic homeostasis by stimulating catabolic metabolism and suppressing anabolic pathways to increase cellular energy levels. We found that the decrease in active phosphorylation of AMPK in response to APAP correlates with decreased ATP levels, in vivo. Therefore, we hypothesized that the enhanced production of ATP via AMPK stimulation can lead to amelioration of APAP-induced liver failure. A769662, an allosteric activator of AMPK, produced a strong synergistic effect on AMPK Thr172 phosphorylation with APAP in primary hepatocytes and liver tissue. Interestingly, activation of AMPK by A769662 ameliorated the APAP-induced hepatotoxicity in C57BL/6N mice treated with APAP at a dose of 400 mg/kg intraperitoneally. However, mice treated with APAP alone developed massive centrilobular necrosis, and APAP increased their serum alanine aminotransferase and aspartate aminotransferase levels. Furthermore, A769662 administration prevented the loss of intracellular ATP without interfering with the APAP-mediated reduction of mitochondrial dysfunction. In contrast, inhibition of glycolysis by 2-deoxy-glucose eliminated the beneficial effects of A769662 on APAP-mediated liver injury. In conclusion, A769662 can effectively protect mice against APAP-induced liver injury through ATP synthesis by anaerobic glycolysis. Furthermore, stimulation of AMPK may have potential therapeutic application for APAP overdose.

• The Journal of Internal Korean Medicine
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• v.22 no.2
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• pp.239-243
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• 2001

Alcoholism is a chronic behavior disorder that disturbs the health, social, and economical functions by intaking alcohol repeatedly. Alcoholism includes some habituation, dependency, and addiction. It may be clinically silent or severe enough to lead to the rapid development of hepatic, renal and gastrointestinal failure. Alcoholism can also cause death. In this case, we administrated saenggangunbitang and sungjoocheonggantang to a patient suffering from alcohoism and its withdrawal symptoms. After administration of saenggangunbitang-sungjoocheonggantang medication, clinical symptoms, including liver function with diabetes mellitus and splenomegaly improved. saenggangunbitang-sungjoocheonggantang showed desirable effect on alcoholism symptoms.

• Journal of Yeungnam Medical Science
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• v.31 no.2
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• pp.117-121
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• 2014

Transarterial chemoembolization (TACE) is a widely accepted nonsurgical modality used for the treatment of multinodular hepatocellular carcinoma (HCC). The careful selection of the candidate is important due to the risk of developing various side effects. Fever, nausea, abdominal pain, and liver enzyme elevation are commonly known side effects of TACE. Hepatic failure, ischemic cholecystitis, and cerebral embolism are also reported, although their incidence might be low. Pulmonary complication after TACE is rare, and the reported cases of lipiodol pneumonitis are even rarer. A 53-year-old man was treated with TACE for ruptured HCC associated with hepatitis B virus infection. On day 19 after the procedure, the patient complained of dyspnea and dry cough. Chest computed tomography showed diffuse ground glass opacities in the whole-lung fields, suggesting lipiodol-induced pneumonitis. After 2 weeks of conservative management, the clinical symptoms and radiologic abnormalities improved. Reported herein is the aforementioned case of lipiodol-induced pnemonitis after TACE, with literature review.

• Journal of Korean Medical Science
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• v.33 no.51
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• pp.324.1-324.6
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• 2018

Oxysterol $7{\alpha}$-hydroxylase deficiency is a very rare liver disease categorized as inborn errors of bile acid synthesis, caused by CYP7B1 mutations. As it may cause rapid progression to end-stage liver disease even in early infancy, a high index of suspicion is required to prevent fatal outcomes. We describe the case of a 3-month-old boy with progressive cholestatic hepatitis and severe hepatic fibrosis. After excluding other etiologies for his early liver failure, we found that he had profuse urinary excretion of $3{\beta}$-monohydroxy-${\Delta}^5$-bile acid derivatives by gas chromatography/mass spectrometry analysis with dried urine spots on filter paper. He was confirmed to have a compound heterozygous mutation (p.Arg388Ter and p.Tyr469IlefsX5) of the CYP7B1 gene. After undergoing liver transplantation (LT) from his mother at 4 months of age, his deteriorated liver function completely normalized, and he had normal growth and development until the current follow-up at 33 months of age. We report the first Korean case of oxysterol $7{\alpha}$-hydroxylase deficiency in the youngest infant reported to undergo successful living donor LT to date.

• Molecules and Cells
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• v.46 no.9
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• pp.527-534
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• 2023

Liver ischemia-reperfusion injury (IRI) is the main cause of organ dysfunction and failure after liver surgeries including organ transplantation. The mechanism of liver IRI is complex and numerous signals are involved but cellular metabolic disturbances, oxidative stress, and inflammation are considered the major contributors to liver IRI. In addition, the activation of inflammatory signals exacerbates liver IRI by recruiting macrophages, dendritic cells, and neutrophils, and activating NK cells, NKT cells, and cytotoxic T cells. Technological advances enable us to understand the role of specific immune cells during liver IRI. Accordingly, therapeutic strategies to prevent or treat liver IRI have been proposed but no definitive and effective therapies exist yet. This review summarizes the current update on the immune cell functions and discusses therapeutic potentials in liver IRI. A better understanding of this complex and highly dynamic process may allow for the development of innovative therapeutic approaches and optimize patient outcomes.