• 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.

• The Korean Journal of Nuclear Medicine
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• v.28 no.1
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• pp.62-68
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• 1994

This study was aimed to compare the density of the functional microcirculation of hepatocellular carcinoma (HCC) with normal liver and to investigate the effect of hepatic-arterial oily chemoembolization (HAE) by radionuclide examination. Methods : Eight patients with HCC proven by biopsy in five, and clinically and radiologically in three were included. The mixture of 2 cc normal saline with three to four mCi of $^{99m}Tc$-MAA was infused through a hepatic-arterial catheter for a minute. Dynamic images were obtained at a rate of 4 sec per frame for a minute, and static images and SPECT were followed. Results : In three patients who underwent hepatic arterial angiography (HAA) alone, radioactivity was markedly increased in tumors compared to the adjacent liver immediately after infusion of $^{99m}Tc$-MAA. The ratios of tumoral and extratumoral up-take (T/E ratio) were above 6.5 (range; $6.5{\sim}l9$, mean; 12.5). In four of the five patients who under-went superselective HAE, T/E ratio were remark-ably decreased ($0.5{\sim}1.3$). The areas of embolization were better delineated in radionuclide study than in postembolization HAA. In the other one who was considered to be embolized completely on HAA, strong radiouptake in the tumor was disclosed (T/E ratio; 7.0). Conclusions : Therefore hepatic-arterial flow study with radionuclide imaging using $^{99m}Tc$-MAA can be a valuable method to assess the accurate embolization effect in HCC.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.1
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• pp.56-62
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• 2000

Purpose: The aim of this study was to evaluate the efficacy and histologic changes of interferon-alfa therapy on chronic hepatitis B virus infection in children. Patients and Methods: Thirty five children aged 3~16 years who were seropositive for HBV DNA, HBsAg and HBeAg were enrolled. Interferon-alfa 2a ($3.4\;MU/m^2$) were given for 6 months. Serologic markers of viral replication was evaluated 1 year after therapy. Post treatment liver biopsy was performed in 18 patients who showed serologic response. Results: Serum HBeAg and viral DNA became negative in 22 (63%) of treated children at 12 months after therapy. Serum aminotransferase levels normalized in all of the responders and HBsAg became negative in one responder. Horizontal transmission, serum aminotransferase levels more than twice normal, and active inflammation on liver biopsy were predictive factors for response to interferon therapy. Periportal piecemeal necrosis, lobular activity, portal inflammation, fibrosis, and total histologic activity index were reduced in responders. Conclusion: In children with chronic hepatitis B, interferon alfa promotes loss of viral replication and improves aminotransferase. Serologic response is associated with improvement in hepatic histology.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.77-81
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• 1998

The author exprienced a case of glycogen storage disease type Ia(GSD-I) in an 18-year-old male patient who was admitted to our hospital due to proteinuria and hypertension. he was suspected to have GSD when 12 years old because of his family history of short stature and hepatomegaly. On admission, physical examination revealed short stature, heparomegaly, and The diagnosis of GSD-I was confirmed by compatible liver biopsy finding and enzyme assay which erealeddeficiency of glcose-6-phosphatase if hepatocyte. Sympromatic treatment was done using antihypertensive drugs and allopurinol with diet control. The authors report a case of glycogen storage disease type Ia completely confirmed by typical clinical manifestation, pathologic findings of the liver and the kidney, and the result of enzyme assay which revealed deficiency of glucose-6-phosphatase in hepatocytes with brief review fo related literatures.

• Journal of the Korean Society of Radiology
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• v.13 no.4
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• pp.539-546
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• 2019

Liver biopsy is invasive and it is a risk of complications. Nevertheless, liver biopsy is gold standard for predicting liver fibrosis. To compensate for these shortcomings, in this study, the liver fibrosis stage was divided using Fibroscan(R) in 200 chronic hepatitis C patients. And, the usefulness and cut-off values of fibrosis index based on four factors(FIB-4), AST to platelet ratio index(APRI) and AST/ALT ratio(AAR) calculated as serum tests were investigated by analyzing ROC curve. As a result, using FIB-4 and APRI rather than AAR is appropriate for evaluation of liver fibrosis. And using APRI to predict significant Fibrosis(F2) and FIB-4 is considered useful for predicting cirrhosis(F4). By applying the advantages of the serum based liver fibrosis marker, which are convenient and repeatable, liver fibrosis follow-up term can be reduced, and furthermore, the prevalence of liver cirrhosis and hepatocellular carcinoma(HCC) can be reduced.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.1 no.1
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• pp.56-78
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• 1998

Objective: Chronic hepatitis B infection (CHB) occurs in 6% to 10% of population in Korea. In ethinic communities where prevalence of chronic infection is high such as Korea, transmission of hepatitis B infection is either vertical (ie, by perinatal infection) or by close family contact (usually from mothers or siblings) during the first 5 years of life. The development of chronic hepatitis B infection is increasingly more common the earlier a person is exposed to the virus, particularly in fetal and neonatal life. And it progress to cirrhosis and hepatocellular carcinoma, especially in severe liver damage and perinatal infection. Histopathology of CHB is important when evaluating the final outcomes. A numerical scoring system which is a semiquantitatively assessed objective reproducible classification of chronic viral hepatitis, is a valuable tool for statistical analysis when predicting the outcome and evaluating antiviral and other therapies. In this study, a numerical scoring system (Ludwig system) was applied and compared with the conventional histological classification of De Groute. And the comparative analysis of cinical findings, family history, serology, and liver function test by histopathological findings in chronic hepatitis B of children was done. Methods: Ninety nine patients [mean age=9 years (range=17 months to 16 years)] with clinical, biochemical, serological and histological patterns of chronic HBV infection included in this study. Five of these children had hepatocelluar carcinoma. They were 83 male and 16 female children. They all underwent liver biopsies and histologic evaluation was performed by one pathologist. The biopsy specimens were classified, according to the standard criteria of De Groute as follows: normal, chronic lobular hepatitis (CLH), chronic persistent hepatitis (CPH), mild to severe chronic active hepatitis (CAH), or active cirrhosis, inactive cirrhosis, hepatocellular carcinoma (HCC). And the biopsy specimens were also assessed and scored semiquantitatively by the numerical scoring Ludwig system. Serum HBsAg, anti-HBs, HBeAg, anti-HBe, anti-HBc (IgG, IgM), and HDV were measured by radioimunoassays. Results: Male predominated in a proportion of 5.2:1 for all patients. Of 99 patients, 2 cases had normal, 2 cases had CLH, 22 cases had CPH, 40 cases had mild CAH, 19 cases had moderate CAH, 1 case had severe CAH, 7 cases had active cirrhosis, 1 case had inactive cirrhosis, and 5 cases had HCC. The mean age, sex distribution, symptoms, signs, and family history did not differ statistically among the different histologic groups. The numerical scoring system was correlated well with the conventional histological classification. The histological activity evaluated by both the conventional classification and the scoring system was more severe as the levels of serum aminotransferases were higher. In contrast, the levels of serum aminotransferases were not useful for predicting the degree of histologic activity because of its wide range overlapping. When the histological activity was more severe and especially the cirrhosis more progressing, the prothrombin time was more prolonged. The histological severity was inversely related with the duration of seroconversion of HBeAg. Conclusions: The histological activity could not be accurately predicted by clinical and biochemical findings, but by the proper histological classification of the numerical scoring system for the biopsy specimen. The numerical scoring system was correlated well with the conventional histological classification, and it seems to be a valuable tool for the statistical analysis when predicting the outcome and evaluating effects of antiviral and other therapies in chronic hepatitis B in children.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.2
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• pp.166-172
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• 2007

Purpose: It has recently been reported that de novo HBV infection following liver transplantation is caused by grafts from HBcAb positive donors, and this phenomenon has been observed in one third of the liver transplant patients in our center. Therefore, we investigated the presence of HBV virus DNA in liver tissues obtained from HBcAb positive donors to determine the mechanism by which de novo HBV infection occurs. Methods: This study was conducted on 6 patients that were HBsAg negative, HBsAb positive, and HBcAb positive who were donors for liver transplantation between November 1997 and November 1998 at Asan Medical Center. We isolated DNA from a portion of liver biopsy tissues that were obtained during the operation, and then identified the surface and core region of HBV DNA using nested PCR. In addition, four children who received liver grafts from these donors were monitored to determine if they became afflicted with non-HBV related diseases while receiving prophylaxis consisting of short-term HBIG treatment and long-term treatment with an antiviral agent. Results: The surface antigen region was identified in all 6 donors and the core antigen region was observed in 4 of the 6 donors. However, no episodes of de novo HBV infection with prophylaxis were observed. Conclusion: The results of this study support the results of previous studies, which indicated that HBV infection may be the main cause of de novo HBV infection in patients that receive HBsAb positive and HBcAb positive donor grafts.