• Advances in pediatric surgery
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• v.4 no.2
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• pp.125-130
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• 1998

To assess the clinical and nutritional status of long-term survivors of biliary atresia, history taking, medical record review, physical examination (height, weight, MAC, TSF), blood tests (LFT, prothrombin time, platelet count, prealbumin, calcium) and liver needle biopsy were performed in 12 patients in whom Kasai procedure were performed more than 10 years ago at Department of Pediatric Surgery in Seoul National University Hospital. None were below the 5th percentile in height and weight. TSF was above the 75th percentile in all patients and showed good subcutaneous fat deposition. MAC was above the 5th percentile in all patients. Serum prealbumin level was abnormal in 2 patients with abnormal liver function and revealed visceral protein malnutrition. Serum calcium level was decreased below normal range in 4 patients with abnormal liver function. One patient had mild ascites. Five patients had abnormal liver function and 7 patients showed clinical manifestation of portal hypertension. Liver needle biopsy was performed in 5 patients and no cirrhotic change was observed. Although some patients who have survived for more than 10 years after Kasai procedure developed protein malnutrition and vitamin deficiencies, growth and development and nutritional status were generally satisfactory. Five patients(42%) showed normal liver function and no portal hypertension. In conclusion, Kasai procedure is satisfactory as a primary treatment in biliary atresia but significant portion of long-term survivors had abnormal liver function and portal hypertension. Continuous and careful follow-up is necessary to determine when liver transplantation may be indicated.

• Tuberculosis and Respiratory Diseases
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• v.66 no.1
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• pp.47-51
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• 2009

Hepatopulmonary syndrome (HPS) is characterized by a defect in arterial oxygenation that's induced by pulmonary vascular dilatation in the setting of liver disease. Some studies have shown the relationship between the presence of the HPS and the severity of liver disease, but there are only rare cases of HPS inpatient with Child-Pugh class A liver cirrhosis. We report here on a case of a 58 years-old male who suffered from progressive dyspnea for the previous few years. He was diagnosed with alcoholic liver cirrhosis 5 years previously. There was no significant abnormality on the chest radiograph and transthoracic echocardiography, but the arterial blood gas analysis revealed severe hypoxemia. Contrast-enhanced transesophageal echocardiograpy with agitated saline demonstrated a delayed appearance of microbubbles in the left cardiac chambers. Thus, he was finally diagnosed with HPS. This case suggests that we should consider HPS when a patient with compensated liver cirrhosis has unexplained dyspnea.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.1
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• pp.32-38
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• 2003

Purpose: Hepatic allografts from donors with hepatitis B core antibody have been demonstrated to transmit hepatitis B virus (HBV) infection to recipients after liver transplantation (LT). The efficacy of hepatitis B immune globulin (HBIg) to prevent de novo hepatitis B was investigated by comparing active immunization in the early phase to HBIg monotherapy in the late phase of pediatric liver transplants at Samsung Medical Center. Methods: Among pediatric liver transplants, from May, 1996 to June, 2002, 15 recipients who were hepatitis B surface antigen (HBsAg) (-) received an allograft from a donor with hepatitis B core antibody (HBcAb) (+). Except two who died from unrelated causes, eleven of 13 recipients were HBsAb (+), and 2 were naive (HBsAb(-), HBcAb(-)). All patients were vaccinated for HBV before LT. In the early phase (January, 1997~November, 1997, 3 patients), HBsAb (+) recipients received booster vaccination after LT. In the late phase (December, 1997~, 10 patients), all recipients were given booster vaccination and received HBIg therapy in order to maintain HBsAb titer greater than 200 IU/L. Lamivudine was given in one case because of severe side effect of HBIg. We retrospectively analyzed the effect of the preventive therapy for de novo hepatitis B through medical records. Results: De novo hepatitis B developed in three of 13 recipients (23.1%). All of 3 patients who received active immunization in the early phase became HBsAg (+) at 7~19 months after transplantation. One of them was naive before LT and the other two were HBsAb (+). All of 10 recipients who were given HBIg in the late phase remained HBsAg (-) at 7~55 months' follow-up. Conclusion: Passive immunization with HBIg was effective for prevention of de novo hepatitis B in HBsAg (-) recipients of hepatic allografts from HBcAb (+) donors.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.4 no.1
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• pp.63-70
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• 2001

Purpose: The aim of this study is to evaluate the effective role of living-related liver transplantation (LRLT) on posttransplant linear growth in children. Methods: Thirty six children were enrolled who received LRLT at Asan Medical Center from December, 1994 to February, 1999 and showed more than one-year postoperative survival. Mean height standard deviation score (zH) was analyzed according to medical records including heights during pretransplant and posttransplant follow-up periods. Results: zH of total children showed significant linear growth after LRLT from -1.58 to 0.33 at 24 posttransplant month (p<0.05). zH in children under 6 years of age, to exclude the effect of adolescent linear growth spurt, showed increment in height (p<0.05). Linear growth of children with liver cirrhosis improved and that with fulminant hepatitis was matained same. While stunted children (mean zH=-2.30) achieved good catch-up growth after transplantation, children with normal growth remained same. Children with significant hepatic dysfunction after LRLT such as chronic rejection or posttransplant lymphoproliferative disorder showed retarded posttrasplant linear growth. There was no statistical difference according to the type of immunosuppressants. Conclusion: LRLT resulted in adequate or catch-up linear growth in children with acute, chronic and metabolic liver disease. Successful LRLT suggested to be a promising option not only in long term survival but also in normal linear growth.

• 대한미세수술학회:학술대회논문집
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• 1996.10a
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• pp.158-158
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• 1996

• Journal of Korean Public Health Nursing
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• v.19 no.1
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• pp.129-138
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• 2005

As the patients who need to undergo liver transplant operation continues to grow. the number of livers that are donated can not keep pace with the demand. With the development of surgery skills, the necessity for operations from living donors is increasing. Nevertheless, satisfactory research has been conducted on the factors which generally affect the living donors. In this article. therefore. researchers focused on the factors which generally affect the donating liver donor in order to design a plan for recommending liver donation from living donors. The subjects were 91 living liver donors in C university hospital from October 1. 2000 to December 31. 2003. The results on the uncertainty of living donor, by test sheet. were analyzed with SAS program. The final results were as follows: 1. The uncertainty of the living donors was 51.54 marks per full credit 100. 2. The factor with the greatest effect on donation was the possibility of survival of the donor, followed by the admission period. marriage status and age. In recommending the living donation, the rate of donor survival after the operation was 5.2 times higher than death, 5.2 times higher when the admission period was under 20 days. 5.0 times higher when married. and 27.3 times higher when the family-related donation was very active at the age of 20s than in the 50s. These results suggest that all medical staffs should care for living donors with more interest and activity to give them the least complaints in admission and the lowest possibilities for complication. To enhance the survival rate and improve the surgical success rate. on-going monitoring should include regular health-checks. and continual efforts and education should be made to care for the health condition of the living donors after donation.

• Advances in pediatric surgery
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• v.13 no.2
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• pp.144-154
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• 2007

Liver tumors in children are rare, relatively complex, and encompass a broad spectrum of disease processes. This study reviews our experience of liver tumors during the last 10 years. Medical records of 36 cases of liver tumors in children, treated at Samsung Medical Centers, from October 1994 to December 2005, were reviewed in this study. We analyzed disease characters and survival rates as a whole and by specific disease. The median age was 3.6 years. Male and female ratio was 1:1. The most common symptom was the palpable mass in 15 cases. Others were abdominal distension in 9 cases, jaundice in 2, vomiting in 2, weight loss in 2, and pubic hair growth in 1. CT or US and liver biopsy were performed for diagnosis. There were 28 malignant tumors: malignant rhabdoid tumor (1 case), hepatocellular carcinoma (3 cases), hemangioendothelioma type II (3 cases), angiosarcoma (1 case), and hepatoblastoma (20 cases). Eight tumors were benign; hepatic adenoma (1 case), focal nodular hyperplasia (2 cases), hemangioendothelioma type I (2 cases), mesenchymal hamartoma (3 cases). In this study the clinical characteristics were not different from the other reports. Liver transplantation was performed in 3 cases-1 with hepatoblastoma and 2 with hepatocelleular carcinoma. Accurate and early diagnosis, and individualized multimodality therapeutic approaches might be important for better outcome.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.5
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• pp.353-375
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• 2022

No systematic review to date has examined histopathological parameters in relation to native liver survival in children who undergo the Kasai operation for biliary atresia (BA). A systematic review and meta-analysis is presented, comparing the frequency of native liver survival in peri-operative severe vs. non-severe liver fibrosis cases, in addition to other reported histopathology parameters. Records were sourced from MEDLINE, Embase, and CENTRAL databases. Studies followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines and compared native liver survival frequencies in pediatric patients with evidence of severe vs. non-severe liver fibrosis, bile duct proliferation, cholestasis, lobular inflammation, portal inflammation, and giant cell transformation on peri-operative biopsies. The primary outcome was the frequency of native liver survival. A random effects meta-analysis was used. Twenty-eight observational studies were included, 1,171 pediatric patients with BA of whom 631 survived with their native liver. Lower odds of native liver survival in the severe liver fibrosis vs. non-severe liver fibrosis groups were reported (odds ratio [OR], 0.16; 95% confidence interval [CI], 0.08-0.33; I²=46%). No difference in the odds of native liver survival in the severe bile duct destruction vs. non-severe bile duct destruction groups were reported (OR, 0.17; 95% CI, 0.00-63.63; I²=96%). Lower odds of native liver survival were documented in the severe cholestasis vs. non-severe cholestasis (OR, 0.10; 95% CI, 0.01-0.73; I²=80%) and severe lobular inflammation vs. non-severe lobular inflammation groups (OR, 0.02; 95% CI, 0.00-0.62; I²=69%). There was no difference in the odds of native liver survival in the severe portal inflammation vs. non-severe portal inflammation groups (OR, 0.03; 95% CI, 0.00-3.22; I²=86%) or between the severe giant cell transformation vs. non-severe giant cell transformation groups (OR, 0.15; 95% CI, 0.00-175.21; I²=94%). The meta-analysis loosely suggests that the presence of severe liver fibrosis, cholestasis, and lobular inflammation are associated with lower odds of native liver survival in pediatric patients after Kasai.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.sup2
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• pp.35-43
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• 2008

Jaundice is common in breast-fed infants. Any infant noted to be jaundiced at 2 weeks of age need to be evaluated for cholestasis with measurement of total and direct serum bilirubin. The most common causes of cholestatic jaundice in infants are biliary atresia and neonatal hepatitis. Genetic causes of the neonatal hepatitis syndrome are increasingly recognized and idiopathic neonatal hepatitis is decreasing. Cholestasis should be investigated using a structured protocol. Early detection and timely, accurate diagnosis is important for successful treatment and a favorable prognosis. In particular, a Kasai portoenterostomy for biliary atresia has the best outcome if performed before the infant is 8 weeks of age. The management of cholestasis is mainly supportive, including nutritional support and alleviation of symptoms to improve the quality of life. Specific treatments are available for some causes of neonatal hepatitis syndrome and should be started as soon as possible. For decompensated liver disease, liver transplantation yields a better outcome.

• Journal of Chest Surgery
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• v.55 no.2
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• pp.171-173
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• 2022

A 65-year-old man underwent right trisectionectomy of the liver and reconstruction of the chest wall and diaphragm with a 2-mm Gore-Tex membrane due to recurrent hepatocellular carcinoma. After 3 years, the Gore-Tex membrane in the diaphragm migrated to the abdominal cavity and perforated the colon. We report a rare complication of a Gore-Tex membrane after diaphragm repair.