• Journal of Chest Surgery
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• v.48 no.3
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• pp.180-186
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• 2015

Background: Extracorporeal membrane oxygenation (ECMO) has recently attracted interest as a treatment for severe acute respiratory distress syndrome (ARDS). However, the outcomes of this procedure in post-surgical settings have not yet been characterized. In this study, we evaluated the outcomes of ECMO in patients with severe postoperative ARDS. Methods: From January 2007 to December 2012, a total of 69 patients (aged $58.3{\pm}11.5$ years, 23 females) who underwent venovenous ECMO to treat severe postoperative ARDS were reviewed. Of these patients, 22 (31.9%) had undergone cardiothoracic surgery, 32 (46.4%) had undergone liver transplantation, and 15 (21.7%) had undergone other procedures. Results: Thirty-four patients (49.3%) were successfully weaned from ECMO, while the other 35 patients (50.7%) died on ECMO support. Among the 34 patients who were successfully weaned from ECMO, 21 patients (30.4%) eventually died before discharge from the hospital, resulting in 13 hospital survivors (18.8%). Multivariable analysis showed that the duration of pre-ECMO ventilation was a significant independent predictor of death (odds ratio [OR], 2.25; 95% confidence interval [CI], 1.29 to 3.90; p=0.004), whereas the concomitant use of continuous venovenous hemodialysis (CVVHD) was associated with improved survival (OR, 0.55; 95% CI, 0.31 to 0.97; p=0.038). Conclusion: Although the overall survival rate of patients treated with ECMO for postoperative ARDS was unfavorable, ECMO offered an invaluable opportunity for survival to patients who would not have been expected to survive using conventional therapy. CVVHD may be beneficial in improving the outcomes of such patients, whereas a prolonged duration of pre-ECMO ventilator support was associated with poor survival.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.6 no.1
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• pp.15-23
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• 2006

Purpose: Glycogen storage disease type III (GSD-III), is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme is amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), which is responsible for the debranching of the glycogen molecule during catabolism. The disease has been demonstrated to show clinical and biochemical heterogeneity, reflecting the genotype-phenotype heterogeneity among different patients. In this study, we analyzed mutations of the AGL gene in three unrelated Korean GSD-III patients and discussed their clinical and laboratory implications. Methods: We studied three GSD-III patients and the clinical features were characterized. Sequence analysis of 35exons and part exon-intron boundaries of the AGLgene in patients were carried out by direct DNA sequencing method using genomic DNA isolated from patients' peripheral leukocytes. Results: The clinical features included hepatomegaly (in all patients), seizures (in patient 2), growth failure (in patients 1), hyperlipidemia (in patients 1 and 3), raised transaminases and creatinine kinase concentrations (in all patients) and mild EKG abnormalities (in patients 2). Liver transplantation was performed in patient 2due to progressive hepatic fibrosis. Administration of raw-corn-starch could maintain normoglycemia and improve the condition. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 with c.1510_1511insT (p.Y504LfsX10), and patient 3 with c.3416 T>C (p.L1139P) and c.l735+1 G>T (Y538_R578delfsX4) mutations. Except R428K mutation, 4 other mutations identified in3 patients were novel. Conclusion: GSD-III patients have variable phenotypic characteristics resembling GSD-Ia. The molecular defects in the AGL gene of Korean GSD-III patients were genetically heterogeneous.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.2
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• pp.178-184
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• 1999

Purpose: The aims of this study were to evaluate the clinical manifestations and prognosis of the syndromic and nonsyndromic intrahepatic bile duct paucity (IHBDP). Methods: We studied histology of 42 infants with neonatal cholestasis. Fourteen patients were diagnosed as IHBDP. We evaluated the clinical manifestations, courses and prognosis retrospectively. Results: Underlying disease of the 42 infants with neonatal cholestasis were biliary atresia in 23, intrahepatic bile duct paucity in 14 (Alagille syndrome in 4 and nonsyndromic IHBDP in 10), neonatal hepatitis in 5 infants. The mean ratio of the bile ducts per portal tract was 0.087 (range: 0~0.5). The manifestations in 4 patients with Alagille syndrome demonstrated as follows: characteristic face in 3, chronic cholestasis in 4, posterior embryotoxon in 2, vertebral anomalies in 2, peripheral pulmonary stenosis in 2. One of 4 patients of Alagille syndrome improved cholestasis and the other 3 patients were remained their cholestasis and growth retardation. All patients of the nonsyndromic IHBDP were idiopathic. Seven out of 8 patients of nonsyndromic IHBDP showed improvement of cholestasis, and one patient received liver transplantation due to cirrhosis. Conclusion: This study suggested that IHBDP should be considered in the differential diagnosis of neonatal cholestasis. The outcome of idiopathic IHBDP was better than predicted.

• Radiation Oncology Journal
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• v.29 no.1
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• pp.36-43
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• 2011

Purpose: To evaluate the effectiveness of radiation therapy (RT) for hepatocellular carcinoma (HCC) with portal vein tumor thrombosis (PVTT) and to analyze the prognostic factors. Materials and Methods: From December 2004 to April 2009, 70 patients who had HCC with PVTT were treated with RT at Keimyung University Dongsan Medical Center. Nineteen patients whose total dose was below 30 Gy and one patient who underwent liver transplantation were excluded. The remaining 50 patients (45 males, 5 females; median age 55 years) were analyzed. According to the LCSGJ TNM stage, there were 27 patients (54.0%) with stage III and 23 (46.0%) with stage IV. Total dose of 30~54 Gy was administered (median 45). Thirty patients (60.0%) were treated with concurrent chemoradiation therapy (CCRT). The median follow-up duration was from 13.5 months (range, 3 to 70 months). Results: The median survival time from the start of RT was 9 months. One-year and 2-year overall survival rates were 24.9% and 11.2%, respectively. At the follow-up time, three patients (6.0%) displayed no evidence of disease. Seven patients (14.0%) were alive with disease, and 40 (80.0%) patients had expired due to disease progression. CCRT was associated with worse survival than RT alone (p=0.034), Response to RT (p=0.037), CLIP stage (p=0.017), and TNM stage (p=0.041) were statistically significant prognostic factors. There was no radiation-induced liver disease. Conclusion: RT is an effective and safe modality for HCC with PVTT. Further studies such as prospective randomized trials are needed to confirm the role of RT for HCC with PVTT.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.213-221
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• 2005

Purpose: Hemophagocytic syndrome (HPS) is characterized by persistent high fever, hepatosplenomegaly, cytopenias, hypertriglyceridemia, and/or hypofibrinogenemia. Hepatic manifestations including overt hepatic failure and fulminant hepatitis are common in HPS. Liver transplantation (LT) should be considered in a case of fulminant hepatitis by other than HPS, but LT is contraindicated and complete cure is possible by chemotherapy in HPS. Therefore, we conducted this study to define the characteristics of HPS presenting as severe acute hepatitis. Methods: Among the total of 23 patients diagnosed as HPS by bone marrow examination between 1994 and 2005 in Asan Medical Center, 11 cases presented as severe acute hepatitis were enrolled in this study. We analyzed the clinical features, laboratory findings and outcome retrospectively. Results: Seven (64%) of the 11 children with HPS and hepatitis were referred to pediatric gastroenterologist at first. The mean age of onset was 50 months. There was no case with family history of primary HPS. Epstein-Barr virus was positive in 4, and herpes Simplex virus was positive simultaneously in 1 case. As the presenting symptoms and signs, fever was present in 10, hepatosplenomagaly was noted in all and jaundice in 10. Anemia was observed in 10, thrombocytopenia in 10, leukopenia in 8, hypertriglyceridemia in 9, hypofibrinogenemia in 8 and hyperferritinemia in 7 cases, respectively. Nine children received chemotherapy including etopside. The overall mortality rate was 72% (8/11). Conclusion: HPS, which needs chemotherapy, should be considered as a cause of severe acute hepatitis especially when accompanied with prolonged high fever and cytopenias.

• Journal of Yeungnam Medical Science
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• v.24 no.2
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• pp.170-178
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• 2007

Background : The incidence of acute hepatitis A in adults has recently been increasing. This study was conducted to investigate the epidemiology and clinical characteristics of acute hepatitis A in Daegu province over the past 10 years. Materials and Methods : We reviewed the medical records of 55 patients (male/female: 34/21), who were diagnosed with acute hepatitis A by confirmation of the IgM anti-HAV between January 1998 and June 2007. Results : The mean age was $29.7{\pm}10.3$ years (range; 17-65 years). The incidence was most common between March and June (56.1%), in the third and fourth decades of life (78.2%) and 90.9% (50/55) of the patients were diagnosed from 2003 to present. The common symptoms included anorexia, nausea or vomiting (69.1%), fever and chills (49.1%), myalgia (47.3%), weight loss (47.3%), fatigue (40.0%), abdominal pain (36.4%), diarrhea (9.1%) and pruritus (5.5%). The mean duration of hospital stay was $8.6{\pm}3.4$ days (range; 3-20 days). The route of transmission was identified in only 11 patients (20.0%); 7 patients (12.7%) traveled (abroad or domestic), 2 patients (3.6%) ingested raw food and 2 patients (3.6%) had friends with acute hepatitis A. Fifty four patients recovered without complication; one patient developed fulminant hepatitis and recovered after a liver transplantation. Conclusion : The incidence of acute hepatitis A in adults is increasing. Because of the cost of treatment and potential for serious disease, persons, under 40 are recommened to have hepatitis A vaccination and confirmation of IgG anti-HAV.

• Journal of Genetic Medicine
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• v.4 no.1
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• pp.72-79
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• 2007

Purpose : Glycogen storage disease type III (GSD-III) is a rare autosomal recessive disorder of glycogen metabolism. The affected enzyme, amylo-1,6-glucosidase, 4-alpha-glucanotransferase (AGL, glycogen debranching enzyme), is responsible for the debranching of the glycogen molecule during catabolism. The disease shows clinical and biochemical heterogeneity, reflecting genotype-phenotype heterogeneity among different patients. In this study, we aim at analyzing mutations of the AGL gene in three unrelated Korean GSD-III patients, and characterizing their clinical and laboratory findings. Methods : We characterized the clinical features of three unrelated Korean GSD-III patients by biochemical, histological and imaging studies. The 35 exons and part of exon-intron boundaries of AGL were analyzed by direct sequencing using genomic DNA extracted from the peripheral leukocytes of patients. Results : Diverse clinical features were observed in these patients including hepatomegaly (all patients), seizures (patient 2), grow th failure (patients 1 and 2), hyperlipidemia (patients 1 and 3), raised transaminase and creatine kinase concentrations (all patients), and mild cardiomyopathy (patient 2). Liver transplantation w as performed in patient 2 due to progressive hepatic fibrosis. A dministration of uncooked corn starch maintained normoglycemia and improved biochemical and growth profiles. DNA sequence analysis revealed mutations in 5 out of 6 alleles. Patient 1 was a compound heterozygote of c.1282 G>A (p.R428K) and c.1306delA (p.S603PfsX6), patient 2 had c.1510_1511insT (p.Y 504L fsX 10), and patient 3 had c.3416 T >C (p.L 1139P) and c.1735+1 G>T (p.Y 538_R578delfsX 4) mutations. A part from the p.R428K mutation, the 4 other substitutions identified w ere nov el. Conclusion : GSD-III patients display variable phenotypic characteristics resembling those of GSD-Ia. Molecular defects in the AGL gene of Korean GSD-III patients are genetically heterogeneous.