• Biomolecules & Therapeutics
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• 제31권6호
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• pp.674-681
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• 2023

Bile pigment, bilirubin, and biliverdin concentrations may change as a results of biliary tract cancer (BTC) altering the mechanisms of radical oxidation and heme breakdown. We explored whether changes in bile pigment components could help distinguish BTC from benign biliary illness by evaluating alterations in patients with BTC. We collected bile fluid from 15 patients with a common bile duct stone (CBD group) and 63 individuals with BTC (BTC group). We examined the bile fluid's bilirubin, biliverdin reductase (BVR), heme oxygenase (HO-1), and bacterial taxonomic abundance. Serum bilirubin levels had no impact on the amounts of bile HO-1, BVR, or bilirubin. In comparison to the control group, the BTC group had considerably higher amounts of HO-1, BVR, and bilirubin in the bile. The areas under the curve for the receiver operating characteristic curve analyses of the BVR and HO-1 were 0.832 (p<0.001) and 0.891 (p<0.001), respectively. Firmicutes was the most prevalent phylum in both CBD and BTC, according to a taxonomic abundance analysis, however the Firmicutes/Bacteroidetes ratio was substantially greater in the BTC group than in the CBD group. The findings of this study showed that, regardless of the existence of obstructive jaundice, biliary carcinogenesis impacts heme degradation and bile pigmentation, and that the bile pigment components HO-1, BVR, and bilirubin in bile fluid have a diagnostic significance in BTC. In tissue biopsies for the diagnosis of BTC, particularly for distinguishing BTC from benign biliary strictures, bile pigment components can be used as additional biomarkers.

• 한국간담췌외과학회지
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• 제27권4호
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• pp.366-371
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• 2023

Backgrounds/Aims: Biliary surgery in patients with extrahepatic portal vein obstruction with portal cavernoma (PC) is technically challenging, and associated with the risk of bleeding. Therefore, prior portal vein decompression is usually recommended before definitive biliary surgery. Only a few studies have so far reported the safety of isolated laparoscopic cholecystectomy. We aimed to evaluate our experience of laparoscopic cholecystectomy in patients with PC without prior portal decompression. Methods: Prospectively maintained data for patients with PC who underwent laparoscopic cholecystectomy for symptomatic gallstone disease without portal decompression were analyzed. Clinical features, imaging, intraoperative factors, conversion rate, complications of surgery, and long-term outcomes were assessed. Results: Sixteen patients underwent cholecystectomy without portal decompression from 2012 to 2021, of which interventions 14 were laparoscopic cholecystectomies. One patient required conversion (7.1%) to open surgery. Jaundice was present in 5 patients (35.7%), and underwent endoscopic stone clearance before surgery. Median intraoperative blood loss, operative time, and hospital stay were 100 mL (20-400 mL), 105 min (60-220 min), and 2 days (1-7 days), respectively. Blood transfusion was required in two patients (14.2%). Prior endoscopic or percutaneous intervention was associated with significant blood loss and prolonged intraoperative time. Conclusions: In centers with experience, prior portal decompression can be avoided in patients with PC requiring isolated cholecystectomy to treat gallstones or their complications. Laparoscopic surgery is safe and feasible for these patients, and gives excellent outcomes in the selected group.

• 한국간담췌외과학회지
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• 제27권3호
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• pp.322-327
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• 2023

Situs inversus totalis (SIT) is a rare condition in which cardiac and abdominal organs are inverted from their normal left-sided orientation. Mirizzi syndrome, characterized by the obstruction of the common hepatic duct or the common bile duct by gallstone, is a rare condition. Mirizzi syndrome co-occurrence in SIT patients is rare. Gallbladder in sinistroposition is extremely uncommon in SIT patients. We report a known case of diabetes, ventricular septal defect with transposition of the great arteries in a 32-year-old female who presented with jaundice, cholangitis, chills, and fever that had lasted for 10 days. She was confirmed to have SIT with type III Mirizzi syndrome following a series of diagnostic procedures. Primarily, endoscopic retrograde cholangiopancreatography along with common bile duct stenting was performed to initially reduce cholangitis. After an eight-week follow-up after the reduction of cholangitis, surgery was conducted. Mirror-imaged ports were used for the laparoscopic procedure, and the surgeon was on the patient's right side rather than the usual left side. The patient was discharged from the hospital following two days of uneventful healing.

• Korean Journal of Radiology
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• 제21권12호
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• pp.1367-1373
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• 2020

Objective: To assess diffusion tensor imaging (DTI) parameters of the hepatic parenchyma for the differentiation of biliary atresia (BA) from Alagille syndrome (ALGS). Materials and Methods: This study included 32 infants with BA and 12 infants with ALGS groups who had undergone DTI. Fractional anisotropy (FA) and mean diffusivity (MD) of the liver were calculated twice by two separate readers and hepatic tissue was biopsied. Statistical analyses were performed to determine the mean values of the two groups. The optimum cut-off values for DTI differentiation of BA and ALGS were calculated by receiver operating characteristic (ROC) analysis. Results: The mean hepatic MD of BA (1.56 ± 0.20 and 1.63 ± 0.2 × 10^-3 mm²/s) was significantly lower than that of ALGS (1.84 ± 0.04 and 1.79 ± 0.03 × 10^-3 mm²/s) for both readers (r = 0.8, p = 0.001). Hepatic MD values of 1.77 and 1.79 × 10^-3 mm²/s as a threshold for differentiating BA from ALGS showed accuracies of 82 and 79% and area under the curves (AUCs) of 0.90 and 0.91 for both readers, respectively. The mean hepatic FA of BA (0.34 ± 0.04 and 0.36 ± 0.04) was significantly higher (p = 0.01, 0.02) than that of ALGS (0.30 ± 0.06 and 0.31 ± 0.05) for both readers (r = 0.80, p = 0.001). FA values of 0.30 and 0.28 as a threshold for differentiating BA from ALGS showed accuracies of 75% and 82% and AUCs of 0.69 and 0.68 for both readers, respectively. Conclusion: Hepatic DTI parameters are promising quantitative imaging parameters for the detection of hepatic parenchymal changes in BA and ALGS and may be an additional noninvasive imaging tool for the differentiation of BA from ALGS.

• 한국간담췌외과학회지
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• 제28권2호
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• pp.125-133
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• 2024

Undifferentiated carcinoma with osteoclast-like giant cells (UC-OGCs) of the pancreas is a rare neoplasm that accounts for less than 1% of all pancreatic malignancies. The aim of this study was to review the literature regarding UC-OGC, and to highlight its biological behavior, clinicopathologic characteristics, prognosis, and therapeutic options. A systematic review of the literature in PubMed/Medline and Scopus databases was performed (last search October 31st, 2023) for articles concerning pancreatic UC-OGC in the adult population. Fifty-seven studies met the inclusion criteria, involving 69 patients with a male-to-female ratio of 1.1:1 and a mean age of 62.96. Main symptoms included abdominal pain (33.3%), jaundice (14.5%), weight loss (8.7%), while fourteen patients (20.3%) were asymptomatic. Surgical resection was performed in 88.4% of cases. Survival rates at one, three, and five years were 58%, 44.7%, and 37.3% respectively. Sex, age, size (cut-off of 4 cm), location, and adjuvant treatment did not significantly affect patient survival. UC-OGC of the pancreas is a rare subtype of undifferentiated pancreatic carcinoma with a better prognosis than conventional pancreatic ductal adenocarcinoma or undifferentiated carcinoma without giant cells. The establishment of a dedicated patient registry is imperative to further delineate the optimal treatment for this uncommon clinical entity.

• 한국간담췌외과학회지
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• 제28권1호
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• pp.53-58
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• 2024

Backgrounds/Aims: Double duct sign (DDS) (dilated common bile and pancreatic duct) is synonymous with pancreatic head/peri-ampullary tumor (PHPAT). There is limited evidence on whether incidental DDS (I-DDS) is associated with an increased risk of malignancy. This study aimed to evaluate 5-year outcomes of I-DDS. Methods: Patients were categorized according to their risk of malignancy. 'Low-risk' patients, including those with I-DDS between 2010 and 2015, were analyzed in this study. The primary outcome was incidence of PHPAT within five years of identification of DDS. Histology results from endoscopic ultrasound-guided biopsy were considered diagnostic. Secondary outcomes were incidence of benign causes, extent of follow-up investigations, and clinical indicators of malignancy in patients with DDS. Results: Among 103 patients with DDS, 20 had I-DDS. Subsequent follow-up of these 20 patients found no patient with PHPAT, two (10%) patients with chronic pancreatitis, and 18 (90%) patients with no cause found. The median follow-up duration for 'low-risk' patients was 7.3 years (range, 6-11 years). The mean number of follow-up investigations per patient was two (range, 0-9). Investigations included computed tomography (n = 27), magnetic resonance cholangiopancreatography (n = 23), endoscopy (n = 16), and ultrasound (n = 14). Patients with jaundice were more likely to have malignancy (p < 0.01). Those with abdominal pain were more likely to have a benign cause (p < 0.01). Hyperbilirubinemia and/or deranged liver enzymes and raised CA19-9 were more likely to be associated with PHPAT (p < 0.01). Conclusions: Patients with I-DDS have a low risk of developing PHPAT within five years.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제27권5호
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• pp.298-312
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• 2024

Purpose: Cytomegalovirus (CMV) infection affects the hepatic, neurologic, hematopoietic, respiratory, gastrointestinal, and other organs, resulting in a high mortality rate and longterm sequelae. It may cause acute or chronic hepatitis, or even lead to hepatic cirrhosis. Valganciclovir (VGCV) is an effective, safe, and well-tolerated treatment for congenital CMV infection, without any serious adverse effects. This study was conducted to evaluate the clinical, biochemical, and virological profiles of infants with CMV with intrahepatic cholestasis and to determine the outcomes with or without treatment with VGCV. Methods: Twenty infants aged <6 months diagnosed with congenital CMV infection with evidence of intrahepatic cholestasis were included in this study. Randomization was used to divide the study participants into 2 groups. The control group (n=10) was treated with only supportive management, and the intervention group (n=10) was treated with oral VGCV at 16 mg/kg/dose 12 hours a day for 6 weeks plus supportive treatments. Physical examinations and biochemical, serological, and virological tests were performed at the time of diagnosis and at the end of 6 weeks and 6 months. Results: The control and intervention groups were compared in terms of clinical and laboratory parameters such as jaundice, dark urine, pale stool, hepatomegaly, total bilirubin, aminotransferases, gamma-glutamyl transferase, alkaline phosphatase, and CMV polymerase chain reaction load, which showed a significant reduction after treatment in the intervention group (p<0.05) with oral VGCV, with very few side effects, whereas the control group showed no significant changes. Conclusion: Oral VGCV can be used to effectively treat CMV infection with intrahepatic cholestasis without notable side effects.

• Journal of Digestive Cancer Research
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• 제7권1호
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• pp.18-21
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• 2019

Serine protease inhibitor Kazal-type 1 (SPINK1) is a gene expressed from pancreatic acinar cell which its mutation is known to be associated with chronic pancreatitis (CP) and pancreatic cancer. We report a case of a 47-years-old female with nausea and weight loss with yellow discoloration of skin. Initial imaging and endoscopic study led us to an impression of chronic pancreatitis with pancreatic cancer with common bile-duct dilation. Biopsy result was confirmed with pancreatic adenocarcinoma and additional imaging revealed lymph node and bone metastasis. Our genetic analysis revealed 194+2T>C mutation of SPINK1. Biliary obstruction was successfully decompressed by stent insertion and underwent chemotherapy and radiotherapy. Although there is accumulating evidence of association between SPINK1 mutation and CP, the relationship between SPINK1 mutation and pancreatic cancer in CP patient is an emerging concept. Genetic analysis should be considered in patients with young age especially when diagnosed with both CP and pancreatic cancer.

• 한국간담췌외과학회지
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• 제26권1호
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• pp.69-75
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• 2022

Backgrounds/Aims: Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive inherited disease requiring liver transplantation (LT). The objective of this study was to investigate the clinicopathological features and posttransplant courses of seven LT recipients with PFIC. Methods: This was a retrospective single-center study of patients with PFIC who underwent LT from January 2013 to June 2020. Results: Two and five patients were diagnosed with PFIC type 1 and type 2, respectively. For all seven patients, age of PFIC onset was at birth. Jaundice was present in all cases. Mean pretransplant total and direct bilirubin levels were 16.1 ± 8.1 mg/dL and 12.4 ± 6.2 mg/dL, respectively. Median patient age and body weight at LT were 10 months and 7 kg, respectively. Types of donors were mothers of patients in four and deceased donors in three. All five patients with PFIC type 2 recovered uneventfully. One patient each with PFIC type 1 underwent retransplantation due to graft failure or died due to multi-organ failure. Overall graft and patient survival rates at five years were 66.7% and 83.3%, respectively. Bile salt export pump immunohistochemical staining showed normal canalicular expression in two patients with PFIC type 1, focal loss in two patients with PFIC type 2, and total loss in three patients with PFIC type 2. Conclusions: LT is currently the only effective treatment for PFIC-associated end-stage liver diseases. It is mandatory to perform regular follow-up due to the risk of complications including steatohepatitis, especially for patients with PFIC type 1.

• 한국간담췌외과학회지
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• 제28권3호
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• pp.358-363
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• 2024

Backgrounds/Aims: Pancreaticoduodenectomy (PD) is being performed more frequently. A pancreaticojejunostomy (PJ) leak is the major determinant of patient outcomes. An omental flap around PJ might improve postoperative outcomes. Methods: A prospective randomized controlled trial was planned at PGIMER, Chandigarh. Fifty-eight patients meeting the criteria were included in the study. Group A underwent PD with omental roll-up and group B underwent standard PD. Results: The mean age of patients in group A was 57.1 ± 14.3 years and 51.2 ± 10.7 in group B. Jaundice (p = 0.667), abdominal pain (p = 0.69), and co-morbidities were equal among the groups. The body mass index of patients in group B was higher at 24.3 ± 5.4 kg/m² (p = 0.03). The common bile duct diameter (12.6 ± 5.3 mm vs. 17.2 ± 10.3 mm, p = 0.13) and the pancreatic duct diameter (4.06 ± 2.01 mm vs. 4.60 ± 2.43 mm, p = 0.91) were comparable. The intraoperative blood loss (mL) was significantly higher in group B (233.33 ± 9.57 vs. 343.33 ± 177.14, p = 0.04). Drain fluid amylase levels on postoperative day (POD) 1 (p = 0.97) and POD3 (p = 0.92) were comparable. The rate of postoperative pancreatic fistula (POPF) grade A (p ≥ 0.99) and grade B (p = 0.54) were comparable. The mean postoperative length of stay among was similar (p = 0.89). Conclusions: An omental wrap can be performed without increase in complexity of the procedure. However, its utility in preventing POPFs and morbidity remains unclear.