• Journal of Yeungnam Medical Science
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• 제34권1호
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• pp.88-90
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• 2017

Although Dieulafoy lesion can occur in any part of the gastrointestinal tract, its occurrence in the rectum is rare. Rectal Dieulafoy lesions have been associated with advanced age, renal failure, burns, liver transplantation and cirrhosis. Here, we report on a case of massive bleeding from a rectal Dieulafoy lesion after lung decortication surgery in a 57-year-old male patient with alcoholic cirrhosis. Although rare, a rectal Dieulafoy lesion should be included in the differential diagnosis of massive lower gastrointestinal bleeding in a patient with cirrhosis.

• Advances in pediatric surgery
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• 제5권2호
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• pp.111-115
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• 1999

The results of hepatic portojejunostomy in 34 patients with biliary atresia operated upon by one surgeon between May 1989 and December 1997 were analyzed. Eleven (32.3 %) patients were 60 days or younger, 14 patient (41.2 %) were between 60 and 90 days, and 9 (26.5 %) were over 90 days of age. Jaundice cleared in 20 cases (58.8 %). Three patients died of liver insufficiency, 2 were anicteric but died from esophageal variceal bleeding. Three patients died as a result of sepsis, heart failure and left kidney agenesis. Five patients were lost to follow-up. The five-year survival rate was 73.8 %. Two patients over 90 days of age, survived more than 5 years. Survival rates were not significantly related to the age at operation. We conclude that hepatic portojejunostomy should be considered as a primary surgical modality for biliary atresia, even at age 90 days or more. Early detection of esophageal varices and sclerotherapy may be necessary. Liver transplantation is necessary if hepatic failure develops.

• Nuclear Medicine and Molecular Imaging
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• 제42권sup1호
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• pp.60-65
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• 2008

Hepatocellular carcinoma is the most common primary tumor in the liver. FDG PET has been applied for staging and treatment planning of hepatocellular carcinoma. It could reflect tumor prognosis because glucose metabolism assessed by FDG PET is known to have correlations with the differentiation and aggressiveness of the tumor. Although the ability of FDG PET to detect well-differentiated or low grade tumors and intra-hepatic lesions is not good, it is expected to playa major role in pre-surgical assessments for liver transplantation because it is useful in detecting extra-hepatic lesions and unexpected distant metastases with a better diagnostic performance than other conventional imaging modalities. Additionally, FDG PET has an advantage to screen other cancers through whole body scanning. As a new tracer for PET, Acetate demonstrates higher sensitivity and specificity to FDG in evaluating hepatocellular carcinoma. It thus seems that simultaneous use of Acetate PET with FDG PET could be helpful in diagnosis, especially detecting extra-hepatic metastases.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권5호
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• pp.479-486
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• 2019

Progressive familial intrahepatic cholestasis (PFIC) is a group of severe genetic disorders, inherited in an autosomal recessive manner, causing cholestasis of hepatocellular origin, later progressing to biliary cirrhosis and liver failure. This is the first report of PFIC type 1 with novel compound heterozygous mutations in Korea. The patient was presented with intrahepatic cholestasis, a normal level of serum ${\gamma}-glutamyl$ transferase, steatorrhea, and growth failure. Genetic testing of this patient revealed novel compound heterozygous mutations (p.Glu585Ter and p.Leu749Pro) in the ATP8B1 gene. After a liver transplantation at age 19 months, the patient developed severe post-transplant steatohepatitis.

• Molecules and Cells
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• 제44권11호
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• pp.851-860
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• 2021

Label-free optical diffraction tomography (ODT), an imaging technology that does not require fluorescent labeling or other pre-processing, can overcome the limitations of conventional cell imaging technologies, such as fluorescence and electron microscopy. In this study, we used ODT to characterize the cellular organelles of three different stem cells-namely, human liver derived stem cell, human umbilical cord matrix derived mesenchymal stem cell, and human induced pluripotent stem cell-based on their refractive index and volume of organelles. The physical property of each stem cell was compared with that of fibroblast. Based on our findings, the characteristic physical properties of specific stem cells can be quantitatively distinguished based on their refractive index and volume of cellular organelles. Altogether, the method employed herein could aid in the distinction of living stem cells from normal cells without the use of fluorescence or specific biomarkers.

• Clinical and Experimental Pediatrics
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• 제60권3호
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• pp.86-93
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• 2017

Purpose: To evaluate the clinical spectrum of posttransplantation lymphoproliferative disorder (PTLD) after solid organ transplantation (SOT) in children. Methods: We retrospectively reviewed the medical records of 18 patients with PTLD who underwent liver (LT) or kidney transplantation (KT) between January 1995 and December 2014 in Seoul National University Children's Hospital. Results: Eighteen patients (3.9% of pediatric SOTs; LT:KT, 11:7; male to female, 9:9) were diagnosed as having PTLD over the last 2 decades (4.8% for LT and 2.9% for KT). PTLD usually presented with fever or gastrointestinal symptoms in a median period of 7 months after SOT. Eight cases had malignant lesions, and all the patients except one had evidence of Epstein-Barr virus (EBV) involvement, assessed by using in situ hybridization of tumor tissue or EBV viral load quantitation of blood. Remission was achieved in all patients with reduction of immunosuppression and/or rituximab therapy or chemotherapy, although 1 patient had allograft kidney loss and another died from complications of chemotherapy. The first case of PTLD was encountered after the introduction of tacrolimus for pediatric SOT in 2003. The recent increase in PTLD incidence in KT coincided with modification of clinical practice since 2012 to increase the tacrolimus trough level. Conclusion: While the outcome was favorable in that all patients achieved complete remission, some patients still had allograft loss or mortality. To prevent PTLD and improve its outcome, monitoring for EBV infection is essential, which would lead to appropriate modification of immunosuppression and enhanced surveillance for PTLD.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제17권4호
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• pp.239-247
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• 2014

Purpose: There are no studies of hepatic glycogen storage diseases (GSDs) other than type I and III in Korea. We aimed on investigating the characteristics of hepatic GSDs in Korea diagnosed and followed at a single center. Methods: We retrospectively analyzed patients who were diagnosed as GSD and followed at Samsung Medical Center from January, 1997 to December, 2013. Clinical manifestations, laboratory results, treatment, and prognosis were investigated. Results: Twenty-one patients were included in the study. The types of 17 patients were confirmed by enzyme activity tests and/or gene analysis. GSD Ia was diagnosed in 7 patients (33.3%), Ib in 1 patient (4.8%), III in 2 patients (9.5%), IV in 1 patient (4.8%), and IX in 6 patients (28.6%). Types other than GSD I constituted 52.9% (9/17) of the patients diagnosed with a specific type of hepatic GSD. The median age at presentation was 2 years. Hepatomegaly was observed in 95.2%, elevated liver transaminases in 90.5%, and hyperlactacidemia in 81.0% of the patients. The duration for follow-up was $77{\pm}62.0$ months. Uncooked corn starch was initiated in all the patients. No mortality was observed during the follow-up period, and liver transplantation was performed in 14.3%. Conclusion: Types other than GSD I comprised more than half of the patients diagnosed with a specific type of hepatic GSD. Clinical suspicion and thorough evaluation of hepatic GSDs in Korea should be focused not only on GSD I, but also on other types.

• IMMUNE NETWORK
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• 제12권3호
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• pp.126-128
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• 2012

We report on a case of severe hepatotoxicity in a 52-year-old male with multiple myeloma (MM) who had received bortezomib therapy. At patient presentation, liver enzymes were normal, but started to markedly increase 3 days after the patient's second dose of bortezomib was administered, when free kappa light chains were noticeably reduced in the serum. After discontinuation of bortezomib, liver enzymes recovered gradually to baseline. Then, the patient was started on a thalidomide-containing regimen, which he was able to tolerate well. The patient achieved complete remission prior to autologous stem cell transplantation (ASCT). The patient underwent ASCT without occurrence of further liver toxicity.

• 한국임상약학회지
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• 제28권1호
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• pp.57-64
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• 2018

Background: Patients experience significant differences in aspects of mortality, quality of life, and costs between during the year of receiving liver transplant (LT) and the subsequent years (post-LT). This study aimed to estimate the medical utilization and cost of LT for patients compared to post-LT patients by using a recent National Patient Sample (NPS) data provided by the Korean Health Insurance Review and Assessment Service (HIRA). Methods: This study used a subset of the 2015 HIRA-NPS. Patient claims data that included Z944 (Korean Standard Classification of Diseases code for LT status) were selected. Within the selected data, LT patients were identified based on whether the national health insurance number code of Q80 (procedure code for LT surgery) was included, and they were compared to post-LT patients. Results: In the analysis, 330 patients were included. The average cost per patient was $90,066{\pm}36,959$ thousand KRW and $10,557{\pm}9,668$ thousand KRW for LT and post-LT patients, respectively. Especially, LT patients' costs for injection/procedure, surgery/treatment, and examination were higher than other costs, being $35,983{\pm}18,115$ thousand KRW, $28,246{\pm}9,408$ thousand KRW, and $12,131{\pm}6,604$ thousand KRW, respectively. For inpatients, the average number of hospitalized days was $63.5{\pm}66.0$ days for LT patients and $22.3{\pm}35.1$ days for post-LT patients. Conclusion: Compared to post-LT patients, LT patients had higher costs, especially for injection/procedure, surgery/treatment, and examination. Additionally, the LT group had longer hospitalization duration and higher costs for their hospital admission, whereas they did not show a significant difference in number of visits and medical costs for outpatient-care.

• Advances in pediatric surgery
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• 제14권2호
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• pp.153-163
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• 2008

Inflammatory myofibroblastic tumor (IMT) is a rare reactive lesion characterized by the feature of myofibroblasts and a mixed inflammatory infiltrate that rarely undergoes malignant transformation. Extrapulmonary IMTs in children have been described involving the mesentery, omentum, retroperitoneum, abdominal soft tissues, liver, bladder, mediastinum, head and neck, extremity, appendix, and kidney. Medical records of children treated with abdominal IMT between 1985 and 2005 were reviewed retrospectively. Seven children were treated for IMT with the mean age of 3 y 2 m (range, 1 y 1 m to 14 y). Tumors were located in transverse mesocolon (n=2), omentum (n=1), porta hepatis (n=2), complex site (antrum, duodenum, common bile duct, porta hepatis) (n=2). The symptoms included abdominal mass, fever, jaundice, abdominal pain and anemia. The masses were excised totally in transverse mesocolon, omentum IMT and there is no evidence of recurrence (follow-up periods: 6 y 8 m, 8 y 9 m, 4 y 10 m). In porta hepatis IMT, liver transplantations were performed and there is no evidence of recurrence (follow period: 6 y 8 m, 8 y 7 m). In one case of complex site IMT, partial excision of mass was performed and he still survived with no change of the residual tumor during follow-up period. The other one of complex site IMT denied further treatment after the biopsy. In conclusion, complete surgical excision including liver transplantation and close follow-up are mandatory for the abdominal IMT in child.