• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제20권2호
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• pp.114-123
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• 2017

Purpose: The goal of this study was the early diagnosis of ABCB11 spectrum liver disorders, especially those focused on benign recurrent intrahepatic cholestasis and progressive familial intrahepatic cholestasis. Methods: Fifty patients presenting neonatal cholestasis were evaluated to identify underlying etiologies. Genetic analysis was performed on patients suspected to have syndromic diseases or ABCB11 spectrum liver disorders. Two families with proven ABCB11 spectrum liver disorders were subjected to genetic analyses to confirm the diagnosis and were provided genetic counseling. Whole exome sequencing and Sanger sequencing were performed on the patients and the family members. Results: Idiopathic or viral hepatitis was diagnosed in 34%, metabolic disease in 20%, total parenteral nutrition induced cholestasis in 16%, extrahepatic biliary atresia in 14%, genetic disease in 10%, neonatal lupus in 2%, congenital syphilis in 2%, and choledochal cyst in 2% of the patients. The patient with progressive familial intrahepatic cholestasis had novel heterozygous mutations of ABCB11 c.11C>G (p.Ser4*) and c.1543A>G (p.Asn515Asp). The patient with benign recurrent intrahepatic cholestasis had homozygous mutations of ABCB11 c.1331T>C (p.Val444Ala) and heterozygous, c.3084A>G (p.Ala1028Ala). Genetic confirmation of ABCB11 spectrum liver disorder led to early liver transplantation in the progressive familial intrahepatic cholestasis patient. In addition, the atypically severe benign recurrent intrahepatic cholestasis patient was able to avoid unnecessary liver transplantation after genetic analysis. Conclusion: ABCB11 spectrum liver disorders can be clinically indistinguishable as they share similar characteristics related to acute episodes. A comprehensive genetic analysis will facilitate optimal diagnosis and treatment.

• 대한암한의학회지
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• 제16권2호
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• pp.19-23
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• 2011

Objectives : The aim of this study was to evaluate safety of Rhus Verniciflua STOKES as a combination therapy of Oriental and conventional cancer treatment. So, We investigated liver function test(especially total bilirubin, AST, ALT, and ALP) levels in outpatients at an oriental medical clinic, to know whether Rhus Verniciflua STOKES decotion (Chijongtang) was hepatotoxic. The outpatients were treated with Rhus Verniciflua STOKES decotion(Chijongtang) during intravenous or oral chemotherapy. Methods : We surveyed whether Chijongtang caused liver injury in patients, who visited and took Chijongtang at Hana oriental medical clinic, from March, 2008 to November 2011. We looked over all the liver function tests of those patients, done during medication. Patients who had no records of liver function tests or with basal liver disease were excluded. And patients were classified into normal and abnormal liver function groups. Results and Conclusions : 48 patients were enrolled, and their mean dosage was 300ml/day, and the mean medication period was 9.3 (2-42) months. During and after medication, there were no abnormal liver function test results at all, in the normal liver function group. and 10 out of 11 patients in the abnormal liver function group showed normal liver function test levels. Roughly speaking, according to the above results, it seems that taking Chijongtang did not cause hepatotoxicity. And it seems Chijongtang has hepatoprotective effect. But we still need more laboratory and clinical research to reach a more definitive conclusion.

• Radiation Oncology Journal
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• 제35권2호
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• pp.172-179
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• 2017

Purpose: To evaluate the outcomes of stereotactic body radiation therapy (SBRT) for patients with liver oligo-recurrence and oligo-progression from various primary tumors. Materials and Methods: Between 2002 and 2013, 72 patients with liver oligo-recurrence (oligo-metastasis with a controlled primary tumor) and oligo-progression (contradictory progression of a few sites of disease despite an overall tumor burden response to therapy) underwent SBRT. Of these, 9 and 8 patients with uncontrollable distant metastases and patients immediate loss to follow-up, respectively, were excluded. The total planning target volume was used to select the SBRT dose (median, 48 Gy; range, 30 to 60 Gy, 3-4 fractions). Toxicity was evaluated using the Common Toxicity Criteria for Adverse Events v4.0. Results: We evaluated 55 patients (77 lesions) treated with SBRT for liver metastases. All patients had controlled primary lesions, and 28 patients had stable lesions at another site (oligo-progression). The most common primary site was the colon (36 patients), followed by the stomach (6 patients) and other sites (13 patients). The 2-year local control and progression-free survival rates were 68% and 22%, respectively. The 2- and 5-year overall survival rates were 56% and 20%, respectively. The most common adverse events were grade 1-2 fatigue, nausea, and vomiting; no grade ${\geq}3$ toxicities were observed. Univariate analysis revealed that oligo-progression associated with poor survival. Conclusion: SBRT for liver oligo-recurrence and oligo-progression appears safe, with similar local control rates. For liver oligo-progression, criteria are needed to select patients in whom improved overall survival can be expected through SBRT.

• Journal of Digestive Cancer Reports
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• 제7권2호
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• pp.61-64
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• 2019

We report two cases of patients with unresectable pancreatic cancer treated with neoadjuvant chemotherapy and surgical resection. In the first case, main mass was located at the neck of the pancreas, encasing superior mesenteric artery and peritoneal seeding was suspected. In the second case, main mass was located at the body of pancreas and superior mesenteric artery was encased. Both patients received FOLFIRINOX chemotherapy regimen, consisting of 5-FU, folinic acid, irinotecan and oxaliplatin. In both cases, tumor size decreased and vascular involvement regressed in response to chemotherapy. After subsequent chemoradiation therapy, both patients underwent surgical resection with negative resection margin. The pathological stages were ypT1cN0 and ypT1aN0, respectively. Both patients received postoperative adjuvant chemotherapy with 6 cycles of 5-FU/folinic acid and remained without evidence of disease for more than 6 months after the surgery.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제11권sup2호
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• pp.50-58
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• 2008

급성 간부전은 여러 분야의 전문가 및 이식 센터의 협조가 필요한 질환으로 급성 간염을 보이는 모든 환자에서 급성 간부전으로 진행할 가능성에 대해 고려하여야 한다. 소아에서 급성 간부전은 원인 미상인 경우가 가장 흔하며, 영아기에는 대사성 간질환도 염두에 두어야 한다. 급성 간부전의 예후는 간이식이 도입된 이후 획기적으로 향상되었으나, 간성 뇌증, 혈액응고 장애, 감염, 신부전 등 주요 합병증에 대한 치료는 여전히 예후를 좌우하는 중요한 부분이며, 여기에 더하여 간이식의 여부 및 시기에 대한 결정이 소아과 의사의 중요한 역할 중 하나가 되었다. 원인이 밝혀진 경우에는 예후 예측에 도움이 되고, 원인에 대한 치료를 시도해볼 수 있으나 소아 환자는 원인 미상인 경우가 많아 예후 예측이 어렵고, 주 사망 원인인 간성 뇌증에 대한 평가가 어려워 특히 세심한 모니터가 필요하다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제3권1호
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• pp.47-55
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• 2000

Purpose: The aim of this research is to examine the abnormal liver function complicated with Kawasaki disease (KD) and to analyze its clinical characteristics and complications. Methods: Ninty eight cases were diagnosed as having KD, among which thirty four cases had abnormal alanine aminotransferase (ALT) (>30 IU/L). These abnormal ALT cases were evaluated in terms of age and sex distribution, major symptoms, complications, laboratory and histopathologic findings. Results: Male to female ratio was 2.4:1 and most patients (91.2%) were under 5 years of age. Cervical lymphadenopathy was observed in 3 cases (8.8%); and recurred cases were two (5.8%). Average fever duration was 8 days; average length of hospitalization was 9 days; and average recovery period was 13 days. Immediately after admission, positive CRP was observed in 31 cases (91.2%), leukocytosis (> $10,000/mm^3$) in 26 cases (76.0%), thrombocytosis (> $450,000/mm^3$) in 7 cases (20.6%), and anemia (<10 gm/dl) in 7 cases (20.6%), respectively. GB hydrops or cholecystitis were noted in 3 cases (8.8%), abnormal ECG finding in 1 case (2.9%), coronary dilatation or aneurysm in 2 cases (5.9%). Liver biopsy was done in four cases and revealed mild infiltration of lymphocytes on the portal area and mild bile duct proliferations. Conclusion: The abnormal liver function was noted in 34.7% of KD patients, and subsided all within one month. But the liver function test should be checked closely in patients of the abnormal liver function test complicated with KD despite of its good prognosis.

• 대한한의학회지
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• 제37권4호
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• pp.36-44
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• 2016

Objectives: Granulocyte-colony-stimulating factor (G-CSF) mobilized bone marrow (BM)-derived hematopoietic stem cells could contribute to improvement of liver function. In addition, liver fibrosis can reportedly be prevented by the Rg 1 component of red ginseng. This study investigated the combined effect of G-CSF and red ginseng on decompensated liver cirrhosis. Methods: Four patients with decompensated liver cirrhosis were injected with G-CSF to proliferate BM stem cells for 4 days ($5{\mu}g/kg$ bid subcutaneously) and followed-up for 3 months. The patients also received red ginseng for 4 days (2 tablets tid per os). We analyzed Child-Pugh scores, Model for End-Stage Liver Disease (MELD) scores and cirrhotic complications. Results: All patients showed marked increases in White blood cell (WBC) and CD34+ cells in the peripheral blood, with a peak time of 4 days after G-CSF injection. Spleen size also increased after G-CSF injection, but not severely. At end of the study, 2 patients showed improvement in Child-Pugh scores, hepatic encephalopathy, and refractory ascites. During the clinical trial period, none of the 4 patients showed any other adverse events or deterioration of liver function. Conclusions: We conclude that G-CSF/red ginseng combination therapy is relatively effective in improving liver function and major complications of decompensated liver cirrhosis without adverse effects. Further clinical trials are warranted to assess the clinical effects of G-CSF for decompensated liver cirrhosis.

• 대한핵의학회지
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• 제19권2호
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• pp.81-86
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• 1985

To evaluate the significance of serum $beta_2-microglobulin$ in patients with various liver diseases, serum $\beta_2m$ levels were measured in 44 cases of normal controls, 32 cases of asymptomatic HBsAg carriers and 134 patients with various liver diseases, by radioimmunoassay using Phadebas $Beta_2-micro$ test kits. The following results were obtained: 1) The mean level of serum $\beta_2m$ was $1.39{\pm}0.25mg/l(Mean{\pm}S.D.)$ in normal controls ($1.39{\pm}0.23mg/l$ in 24 males, $1.38{\pm}0.27mg/l$ in 20 females). 2) The serum levels of $\beta_2m$ in patients with various liver diseases and asymptomatic HBsAg carriers were as follows; $1.40{\pm}0.27mg/l$ in asymptomatic HBsAg carriers, $2.42{\pm}0.37mg/l$ in 45 patients with acute viral hepatitis, $2.10{\pm}0.26mg/l$ in 46 patients with chronic persistent hepatitis, $2.60{\pm}0.34mg/l$ in 23 patients with chronic active hepatitis, and $2.60{\pm}0.49mg/l$ in 20 patients with liver cirrhosis. Serum $\beta_2m$ levels of each disease group were significantly higher than that of normal controls(p<0.001). 3) There was significant correlation between the levels of serum $\beta_2m$ and the degrees of lymphocytic infiltration in patients with chronic active hepatitis(p<0.001). 4) Significant correlations were observed between the levels of serum $beta_2-microglobulin$ and serum alanine aminotransferase(r=0.68, p<0.05) and bilirubin(r=0.63, p<0.05) in 15 patients with acute viral hepatitis. In conclusion, the serum $beta_2-microglobulin$ levels were increased in patients with various liver diseases, and it may serve as a new index of liver disease activity.

• 한국산학기술학회논문지
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• 제13권12호
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• pp.5931-5936
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• 2012

3.0T 고자기장 자기공명분광법에서 비알콜성 지방간 환자 평가를 위해 PRESS 기법과 Biopsy결과의 상관관계를 토대로 PRESS 기법의 임상적 유용성을 알아보았다. 본 실험은 3.0T 고자지장 자기공명영상 장비를 이용하여 실험을 하였고 분광스펙트럼을 적분(peak area integration) 방법으로 면적을 구하여 간지방량에 상대비율에 따른 공명피크의 면적을 이용하여 계산하였다. 비알콜성 지방간환자에서 MR 스펙트럼 피크가 모두 0.9 - 1.6 ppm에서 나타났으며 비알콜성 환자에 대하여 MRS와 Biopsy 결과 민감도 94%와 특이도 80%로 높은 값을 얻었다(p=0.000). 정상(r=0.79, p=0.04) 및 비알콜성 지방간질환 대한 결과( r=0.89, p=0.002) 역시 높은 상관관계를 보여주었다. 따라서 3.0T 고자장 MRS을 적용했을 때 PRESS 기법은 비알콜성 지방간 환자를 평가하는데 있어서 정상부분과 지방간부분의 구별을 가능하게 하였으며 생검검사와 MRS의 상관관계 분석에서 간지방량 비율(%)은 r값이 0.7 이상의 높은 Lipid를 제공하여 PRESS 기법의 임상적 유용성을 권고 할 만하였다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권2호
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• pp.146-153
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• 2020

Purpose: Alpha-1 antitrypsin deficiency (A1ATD) in one of the most common genetic causes of liver disease in children. We aimed to analyze the clinical characteristics and outcomes of patients with A1ATD. Methods: This study included patients with A1ATD from five pediatric hepatology units. Demographics, clinical findings, genetics, and outcome of the patients were recorded (n=25). Results: Eight patients (32.0%) had homozygous PiZZ genotype while 17 (68.0%) had heterozygous genotype. Patients with PiZZ genotype had lower alpha-1 antitrypsin levels than patients with PiMZ genotype (37.6±7.7 mg/dL vs. 66.5±22.7 mg/dL, p=0.0001). Patients with PiZZ genotype were diagnosed earlier than patients with PiMZ genotype, but this was not significant (13±6.8 months vs. 23.7±30.1 months, p=0.192). Follow-up revealed the death of one patient (12.5%) with a homozygous mutation, and revealed that one patient had child A cirrhosis, five patients (62.5%) had chronic hepatitis, and one patient (12.5%) was asymptomatic. Nine of the 17 patients with a heterozygous mutation had chronic hepatitis (52.9%), two (11.7%) had child A cirrhosis, and six (35.2%) were asymptomatic. Overall, 18 (72%) of the 25 children had liver pathology in the long-term. Conclusion: Although prevalence is rare, patients with liver disorders should be checked for alpha-1 antitrypsin levels. Moreover, long-term follow-up is essential because most patients have a liver pathology.