No Prognostic Impact of Graft-to-Recipient Weight Ratio on Hepatocellular Carcinoma Recurrence Following Living Donor Liver Transplantation.

BACKGROUND The effects of a low graft-to-recipient weight ratio (GRWR) on the prognosis of patients with hepatocellular carcinoma (HCC) are unclear. The present study examined whether the GRWR had an impact on the rate of HCC recurrence following living donor liver transplantation (LDLT). MATERIAL AND METHODS This retrospective observational single-center study included 856 patients who underwent LDLT for HCC between January 2006 and December 2016 at Asan Medical Center and evaluated the association between GRWR and post-transplant tumor recurrence. RESULTS Of the 856 patients who underwent LDLT for HCC, 54 (6.3%), 272 (31.8%), 274 (32.0%), and 256 (29.9%) had GRWR <0.8%, 0.8-0.99%, 1.0-1.19%, and ≥1.2%, respectively. Analysis of all patients revealed that the disease-free survival (DFS; P=0.545) and overall survival (OS; P=0.313) rates were not different in these 4 groups. Subgroups analyses also showed that GRWR did not influence survival rates in patients within (DFS: P=0.398; OS: P=0.676) and beyond (DFS: P=0.602; OS: P=0.649) the Milan criteria, or in patients with alpha-fetoprotein-des-γ-carboxyprothrombin-tumor volume scores <5log (DFS: P=0.633; OS: p=0.285) and ≥5log (DFS: P=0.674; OS: P=0.906). CONCLUSIONS GRWR less than 0.8% did not demonstrate a noteworthy prognostic influence on the oncological results among patients who had undergone LDLT for HCC. High-volume multi-center studies are necessary to validate these findings.

Complex Hypersensitivity and Irritation Reaction (CHAIR) Phenomenon after Cyanoacrylate Closure of Varicose Vein.

Cyanoacrylate glue is a non-thermal, non-tumescent agent used to treat saphenous reflux. It was introduced to overcome heat-related discomfort and complications. Multiple randomized controlled trials using this therapy have demonstrated excellent clinical outcomes at long-term follow-up. However, diffuse injection-site inflammation and systemic urticaria are worrisome complications. In preclinical studies, serial histopathological findings demonstrated acute inflammatory reaction, subacute vasculitis, chronic granulomatous foreign body reaction, fibrotic changes with partial vascular recanalization, and chronic foreign body-type inflammatory response. While the exact nature of this unique complication remains undefined, complex hypersensitivity and irritation reaction phenomena have been suggested based on reported clinical presentations. The incidence of this complication has been reported as ranging from 0.3%-25.4%. Typically, erythematous reactions can occur near treatment sites, with symptoms ranging from mild pruritus and/or erythema that resolves without treatment to recurrent severe inflammation and pruritus requiring nonsteroidal anti-inflammatory drugs, antihistamines, and/or corticosteroids. Surgical excision has been rarely reported in patients with severe intractable inflammation or treatment-site infections. Although several anecdotal studies reported on using antihistaminics or corticosteroids, no effective strategies have been established to prevent this complication.

Non-Invasive Diagnosis for Acute Rejection Using Urinary mRNA Signature Reflecting Allograft Status in Kidney Transplantation.

Urine has been regarded as a good resource based on the assumption that urine can directly reflect the state of the allograft or ongoing injury in kidney transplantation. Previous studies, suggesting the usefulness of urinary mRNA as a biomarker of acute rejection, imply that urinary mRNA mirrors the transcriptional activity of the kidneys. We selected 14 data-driven candidate genes through a meta-analysis and measured the candidate genes using quantitative PCR without pre-amplification in the cross-sectional specimens from Korean kidney transplant patients. Expression of 9/14 genes (CXCL9, CD3ϵ, IP-10, LCK, C1QB, PSMB9, Tim-3, Foxp3, and FAM26F) was significantly different between acute rejection and stable graft function with normal pathology and long-term graft survival in 103 training samples. CXCL9 was also distinctly expressed in allografts with acute rejection in in situ hybridization analysis. This result, consistent with the qPCR result, implies that urinary mRNA could reflect the magnitude of allograft injury. We developed an AR prediction model with the urinary mRNAs by a binary logistic regression and the AUC of the model was 0.89 in the training set. The model was validated in 391 independent samples, and the AUC value yielded 0.84 with a fixed manner. In addition, the decision curve analysis indicated a range of reasonable threshold probabilities for biopsy. Therefore, we suggest the urine mRNA signature could be used as a non-invasive monitoring tool of acute rejection for clinical application and could help determine whether to perform a biopsy in a recipient with increased creatinine.

Corrigendum: Non-Invasive Diagnosis for Acute Rejection Using Urinary mRNA Signature Reflecting Allograft Status in Kidney Transplantation.

[This corrects the article DOI: 10.3389/fimmu.2021.656632.].

Clinicopathologic analysis of intraductal papillary neoplasm of bile duct: Korean multicenter cohort study.

BACKGROUND: IPNB is very rare disease and most previous studies on IPNB were case series with a small number due to low incidence. The aim of this study is to validate previously known clinicopathologic features of intraductal papillary neoplasm of bile duct (IPNB) based on the first largest multicenter cohort. METHODS: Among 587 patients previously diagnosed with IPNB and similar diseases from each center in Korea, 387 were included in this study after central pathologic review. We also reviewed all preoperative image data. RESULTS: Of 387 patients, 176 (45.5%) had invasive carcinoma and 21 (6.0%) lymph node metastasis. The 5-year overall survival was 80.9% for all patients, 88.8% for IPNB with mucosal dysplasia, and 70.5% for IPNB with invasive carcinoma. According to the "Jang & Kim's modified anatomical classification," 265 (68.5%) were intrahepatic, 103 (26.6%) extrahepatic, and 16 (4.1%) diffuse type. Multivariate analysis revealed that tumor invasiveness was a unique predictor for survival analysis. (p = 0.047 [hazard ratio = 2.116, 95% confidence interval 1.010-4.433]). CONCLUSIONS: This is the first Korean multicenter study on IPNB through central pathologic and radiologic review process. Although IPNB showed good long-term prognosis, relatively aggressive features were also found in invasive carcinoma and extrahepatic/diffuse type.

Predictor of abnormal gallbladder ejection fraction in patients with atypical biliary pain: Histopathological point of view.

Cholescintigraphy has traditionally been used as a tool to select patients with biliary pain for elective cholecystectomy. However, atypical biliary pain presents a clinical challenge and there is no literature evaluating the factors of the gallbladder (GB) wall related to abnormal ejection fraction of cholescintigraphy in such patients. Therefore, we aimed to evaluate characteristics of the GB wall in patients with abnormal gallbladder ejection fraction (GBEF) on cholescintigraphy and atypical biliary pain. Patients who underwent cholescintigraphy for atypical biliary pain and subsequent cholecystectomy were initially recruited for this study. Medical records and pathologic findings of these patients were retrospectively reviewed. Parameters that were significant on univariate analysis, including factors of GB wall and cholescintigraphy, were subsequently tested by multivariate analysis to identify independent predictors for abnormal GBEF. Abnormal or low GBEF was defined as GBEF <35%. A total of 41 adult patients were divided into a low GBEF (n = 15) and a high GBEF group (n = 26) based on the cutoff value of 35%. In univariate analysis mean muscle thickness, muscle to total layer ratio, and muscle to fibrosis layer ratio were significantly higher in the low GBEF group than in the high GBEF group. In multivariate analysis, the muscle to fibrosis layer ratio was found to be an independent risk factor for abnormal GBEF (odds ratio = 3.514, 95% confidence interval = 1.058-11.673, P = .04). The fibrosis to total layer ratio was negatively correlated with GBEF in the low GBEF group (r = -0.657, P < .01). Muscle to fibrosis layer ratio was significantly associated with decreased GBEF. The fibrosis thickness ratio also seems to play an important role in patients with decreased GBEF.

Who experiences endoscopic retrograde cholangiopancreatography after laparoscopic cholecystectomy for symptomatic gallstone disease?

PURPOSE: Laparoscopic cholecystectomy (LC) has become a standard treatment of symptomatic gallstone disease. But, some patients suffer from retained common bile duct stones after LC. The aim of this study is to analyze the predicting factors associated with subsequent postoperative endoscopic retrograde cholangiopancreatography (ERCP) after LC. METHODS: We retrospectively reviewed a database of every LC performed between July 2006 and September 2012. We classify 28 patients who underwent ERCP within 6 months after LC for symptomatic gallstone disease as the ERCP group and 56 patients who underwent LC for symptomatic gallstone disease during same period paired by sex, age, underlying disease, operation history, and body mass index as the control group. To identify risk factor performing postoperative ERCP after LC, we compared admission route, preoperative biochemical liver function test, number of gall stones, gallstone size, adhesion around GB, wall thickening of GB, and existence of acute cholecystitis between the 2 groups. RESULTS: Admission route, preoperative AST, ALT, and ALP, stone size, longer operation time, and acute cholecystitis were identified as risk factors of postoperative ERCP in univariate analyses. But, longer operation time (P = 0.004) and acute cholecystitis (P = 0.048) were identified as independent risk factors of postoperative ERCP in multivariate analyses. CONCLUSION: The patient who underwent ERCP after LC for symptomatic gallstone disease are more likely experienced longer operation time and acute cholecystitis than the patient who did not undergo ERCP after LC.

Spilled gallstones mimicking a retroperitoneal sarcoma following laparoscopic cholecystectomy.

Laparoscopic cholecystectomy has become a standard treatment of symptomatic gallstone disease. Although spilled gallstones are considered harmless, unretrieved gallstones can result in intra-abdominal abscess. We report a case of abscess formation due to spilled gallstones after laparoscopic cholecystectomy mimicking a retroperitoneal sarcoma on radiologic imaging. A 59-year-old male with a surgical history of a laparoscopic cholecystectomy complicated by gallstones spillage presented with a 1 mo history of constant right-sided abdominal pain and tenderness. Computed tomography and magnetic resonance imaging demonstrated a retroperitoneal sarcoma at the sub-hepatic space. On open exploration a 5 cm × 5 cm retroperitoneal mass was excised. The mass contained purulent material and gallstones. Final pathology revealed abscess formation and foreign body granuloma. Vigilance concerning the possibility of lost gallstones during laparoscopic cholecystectomy is important. If possible, every spilled gallstone during surgery should be retrieved to prevent this rare complication.

Blood levels related to the Z-score of bone mineral density in young males and females.

[Purpose] The purpose of this study was to investigate the blood levels related to the bone mineral density by using the dual energy X-ray absorption for females before menopause and males younger than 50 years old. [Subjects and Methods] Between August 1, and September 15, 2013, the Z-score was measured in females before menopause and males younger than 50 years old using a bone mineral density measuring instrument. After the measurement, the subjects were classified into two groups, that is, the below expectations and within expectations groups. Next, we analyzed and compared the differences in age, body mass index, and blood levels between the 2 groups. [Results] The results showed a correlation of 0.212 for total protein, -0.317 for alanine aminotransferase, -0.199 for gamma-glutamyl transferase, -0.358 for alkaline phosphatase, 0.266 for uric acid, -0.313 for lactate dehydrogenase, 0.244 for creatinine, -0.234 for the red blood cell count, and -0.230 for the red cell distribution width in patients with less than expected level for their age. [Conclusion] In conclusion, osteoporosis may occur in females before menopause and males younger than 50 years old, and aggressive attention is required for prevention and treatment.

Outcome of conservative percutaneous cholecystostomy in high-risk patients with acute cholecystitis and risk factors leading to surgery.

BACKGROUND: Laparoscopic cholecystectomy (LC) is the treatment of choice for acute cholecystitis. However, the morbidity and mortality rates are high in elderly patients or in those with co-morbidities at the time of surgery. Percutaneous cholecystostomy (PC) is a safe treatment for acute inflammation of the gall bladder. This study aimed to evaluate the safety and efficacy of PC for acute cholecystitis and investigate the post-PC factors leading to subsequent LC. MATERIALS AND METHODS: Ninety-three patients with acute cholecystitis who underwent PC between August 2006 and December 2012 were retrospectively reviewed for clinical course, outcomes, and prognosis. We evaluated patient age, the presence of co-morbidities, American Society of Anesthesiologists (ASA) score, duration of drainage of the PC tube, performance of LC, conversion rate, hospital stay, recurrence, and 30-day mortality. We compared these characteristics in two study groups: 31 were treated with only conservative PC (group I) and 62 with PC followed by elective LC (group II). RESULTS: Patients in group I were older than those in group II (80.38 ± 10.05 vs. 70.50 ± 11.81 years, p < 0.001). More group I patients had an ASA score of III or IV (deemed high risk for surgery) compared to group II patients (80.6 %, n = 25 vs. 37.0 %, n = 23, p = 0.0012). Age, ASA score, and cerebrovascular accident (CVA) were significantly correlated when analyzing factors used to decide surgery (R (2) = 0.15, p < 0.001; R (2) = 0.21, p < 0.001; R (2) = 0.05, p = 0.05, respectively). Two patients in group I died within 30 days. Six patients (19.3 %) in group I experienced recurrent cholecystitis after PC tube removal. CONCLUSIONS: PC is a safe and effective therapeutic option in high-risk patients with acute cholecystitis, or for preoperative management. The decisive risk factors for surgery after PC were age, ASA score, and CVA.

Osteopontin Expression in Patients with Hepatolith.

To investigate the role of osteopontin (OPN) in the formation of hepatolith, we have immunohistochemically studied the involvement of OPN in hepatolithiasis in the intrahepatic bile ducts and the intramural and extramural glands, and in stones. In our hospital, 17 hepatic resections for hepatolithiasis were carried out from June 2006 to October 2009. Immunohistochemistry was performed on 17 liver specimens with hepatolith and on 24 control liver specimens without hepatolith. We compared the osetopontin expression in the epithelia of the intrahepatic bile ducts and peribiliary glands or periglandular macrophages. Staining for OPN was positive in the cytoplasm of the epithelial cells of stone-containing intrahepatic bile ducts and the intramural and extramural glands, and in stones. But there were no significant differences between the hepatolithiasis group and the control group for the OPN immunoreactivity of the luminal surface of the epithelial cells of the intrahepatic bile ducts and intramural and extramural glands. The stone-containing intrahepatic bile ducts were infiltrated by macrophages that showed intense staining for OPN. The core and matrix of the stones showed OPN immunoreactivity. The degree of OPN from the cytoplasm of the epithelia of the intrahepatic bile ducts and peribiliary glands or periglandular macrophages was different between hepatolithiasis and control groups. Our result suggests that the OPN from the intrahepatic bile ducts and peribiliary glands plays a role in the formation of intrahepatic stone.

Simultaneous hybrid revascularization for symptomatic lower extremity arterial occlusive disease.

Multilevel revascularization, using a combination of endovascular and open (hybrid) surgery, is increasingly being used. Hybrid surgery allows complex anatomy to be treated by minimally invasive procedures in medically high risk patients. The aim of the present study was to report a novel hybrid surgery for lesions in the multilevel lower extremity arteries and to evaluate the clinical outcomes. Consecutive patients who presented at a single institution between March 2009 and Feburary 2012 were selected for inclusion in the study. The patients had disabling claudication or critical limb ischemia and underwent treatment for revascularization by open surgery or by a combination of open surgery and endovascular procedure. Retrospective analysis was conducted from a prospectively collected database. All procedures were performed by a vascular surgeon in an operating room. Postoperative surveillance in outpatient clinics was conducted at 3 and 6 months and every 6 months thereafter. A total of 76 patients were included in the study with a mean age of 67.1±11.3 years (range, 42-94 years) and the male to female ratio was 67:9. The most common indication for revascularization was Rutherford category IV (resting pain). The immediate technical success rate of hybrid surgery was 90.5%, with an overall limb salvage rate of 97.4%. The primary patency rates of the hybrid and open groups were 100 and 90.9%, respectively (P=0.441). Therefore, the results of the present study indicate that hybrid surgery is a feasible option for the treatment of multilevel peripheral arterial occlusive disease, showing favorable patency and limb salvage rates. These observations indicate that femoral endarterectomy plays a vital role in hybrid surgery.

Laparoscopic cholecystectomy in patients with anesthetic problems.

Laparoscopic cholecystectomy is a standard operation for benign gallbladder disease. As experience with laparoscopic cholecystectomy has increased, the procedure has become possible in patients with anesthetic problems. Patients with ankylosing spondylitis or severe kyphosis represent a challenging group to anesthesiologists and laparoscopic surgeons since these diseases are associated with difficult intubation, restrictive ventilatory defects, and cardiac problems. The relatively new approach of awake fiberoptic intubation is considered to be the safest option for patients with anticipated airway difficulties. Laparoscopic cholecystectomy is usually performed under general anesthesia but considerable difficulties in anesthetic management are encountered during laparoscopic surgery; for example, hemodynamic instability may develop in patients with cardiopulmonary dysfunction due to pneumoperitoneum and position changes during the operation. Nonetheless, regional anesthesia can be considered as a valid option for patients with gallbladder disease who are poor candidates for general anesthesia due to cardiopulmonary problems. We report three cases of laparoscopic cholecystectomy successfully performed in patients with anesthetic problems that included cardiopulmonary disease, severe kyphosis, and ankylosing spondylitis.

Intrahepatic biliary intraductal papillary mucinous neoplasm with gallbladder agenesis: case report.

We report here on a case of intraductal papillary mucinous neoplasm (IPMN) of the bile duct, associated with gallbladder agenesis. A 65-year-old woman was admitted to the hospital with epigastric pain, anorexia, and nausea. Abdominal computed tomography scan and magnetic resonance imaging showed a 5 × 2.5 cm lobulated cystic lesion in the lateral lobe of the liver. The gallbladder was not seen on both imaging modalities. Endoscopic retrograde cholangiopancreatography suggested the diagnosis of biliary IPMN due to abundant mucin that protruded from the papilla and the lobulated cystic lesion. Laparoscopic wedge resection of the liver was performed. The final pathology was consistent with biliary IPMN. The specimen showed multifocal high-grade dysplasia with negative resection margins. The patient experienced no postoperative complications and was discharged 5 days after the operation.

Intrahepatic biliary intraductal papillary mucinous neoplasm with gallbladder agenesis: case report.

We report here on a case of intraductal papillary mucinous neoplasm (IPMN) of the bile duct, associated with gallbladder agenesis. A 65-year-old woman was admitted to the hospital with epigastric pain, anorexia, and nausea. Abdominal computed tomography scan and magnetic resonance imaging showed a 5 × 2.5 cm lobulated cystic lesion in the lateral lobe of the liver. The gallbladder was not seen on both imaging modalities. Endoscopic retrograde cholangiopancreatography suggested the diagnosis of biliary IPMN due to abundant mucin that protruded from the papilla and the lobulated cystic lesion. Laparoscopic wedge resection of the liver was performed. The final pathology was consistent with biliary IPMN. The specimen showed multifocal high-grade dysplasia with negative resection margins. The patient experienced no postoperative complications and was discharged 5 days after the operation.

Splenectomy affects the balance between hepatic growth factor and transforming growth factor-ß and its effect on liver regeneration is dependent on the amount of liver resection in rats.

PURPOSE: Small-for-size syndrome (SFSS) is a major problem in liver surgery, and splenectomy has been used to prevent SFSS. However, it is unknown whether splenectomy has the same effect on liver regeneration in both standard and marginal hepatectomy. The aim of this study is to see a difference in effect of splenectomy on liver regeneration according to the amount of liver resection. METHODS: Thirty male Sprague-Dawley rats (220 to 260 g) were divided into the following five groups: control (n = 6), 70% hepatectomy (n = 6), 70% hepatectomy with splenectomy (n = 6), 90% hepatectomy (n = 6), and 90% hepatectomy with splenectomy (n = 6). The animals were euthanized 24 hours after surgery and liver specimens were obtained. To assess liver regeneration, we performed immunohistochemistry of liver tissue using 5-bromo-2-deoxyuridine (BrdU) labeling and Western blot analysis of hepatic growth factor (HGF) and transforming growth factor-ß (TGF-ß) in the liver tissue. RESULTS: The splenectomized subgroup had a higher BrdU-positive cell count in the 90% hepatectomy group, but not in the 70% hepatectomy group (P < 0.001). Splenectomy significantly decreased TGF-ß expression (P = 0.005) and increased the HGF to TGF-ß ratio (P = 0.002) in the 90% hepatectomy group, but not in the 70% hepatectomy group. CONCLUSION: The positive effect of splenectomy on liver regeneration was greater in the group with the larger liver resection. This phenomenon may be related to the relative balance between HGF and TGF-ß in the liver.

Post-transplant lymphoproliferative disorder presented as small bowel intussusception in adult liver transplant patient.

Intestinal obstruction after liver transplant is a rare complication, with diverse clinical manifestations. Intestinal adhesion is the most common cause. However, internal hernia, abdominal wall hernia, and neoplasm are also reported. Intussusception is another rare cause of intestinal obstruction, which has been reported primarily in pediatric patients. Herein, we report a case of intestinal obstruction from intussusception in an adult liver transplant patient associated with post-transplant lymphoproliferative disorder.

Multivariable analysis of cholecystectomy after gastrectomy: laparoscopy is a feasible initial approach even in the presence of common bile duct stones or acute cholecystitis.

BACKGROUND: When performing cholecystectomy after gastrectomy, we often encounter problems, such as adhesions, nutritional insufficiency, and bowel reconstruction. The purpose of this study was to identify the factors related to surgical outcome of these associated procedures, with emphasis on the use of a laparoscopic approach. METHODS: We retrospectively analyzed data from 58 patients who had a history of cholecystectomy after gastrectomy. Differences between subgroups with respect to operation time, length of postoperative hospital stay, and complications were analyzed. To identify the factors related with outcomes of cholecystectomy after gastrectomy, we performed multivariable analysis with the following variables: common bile duct (CBD) exploration, laparoscopic surgery, gender, acute cholecystitis, history of stomach cancer, age, body mass index, period of surgery, and interval between cholecystectomy and gastrectomy. RESULTS: We found one case (2.9%) of open conversion. The CBD exploration was the most significant independent factor (adjusted odds ratio (OR), 45.15; 95% confidence interval (CI), 4.53-450.55) related to longer operation time. Acute cholecystitis also was a significant independent factor (adjusted OR, 14.66; 95% CI, 1.46-147.4). The laparoscopic approach was not related to operation time but was related to a shorter hospital stay (adjusted OR, 0.057; 95% CI, 0.004-0.74). Acute cholecystitis was independently related to the occurrence of complications (adjusted OR, 27.68; 95% CI, 1.15-666.24); however, CBD exploration and laparoscopic surgery were not. A lower BMI also was an independent predictor of the occurrence of complications (adjusted OR, 0.41; 95% CI, 0.2-0.87). CONCLUSIONS: The laparoscopic approach is feasible for cholecystectomy after gastrectomy, even in cases with CBD stones or acute cholecystitis. This approach does not appear to increase operation time or complication rate and was shown to decrease the length of postoperative hospital stay.

Clinical review of laparoscopic cholecystectomy in acute cholecystitis.

BACKGROUNDS/AIMS: Laparoscopic cholecystectomy is the best treatment choice for acute cholecystitis. However, its higher conversion rate and postoperative morbidities remain controversial. The purpose of this retrospective study is to evaluate the clinical significance of laparoscopic cholecystectomy that is performed at our institution in patients with acute cholecystitis. METHODS: Between January 2003 and December 2009, a retrospective study was carried out for 190 cases of acute cholecystitis undergoing laparoscopic cholecystectomy at our institution. They were divided into 2 groups, based on the time of operation from the onset of the symptom and other previous abdominal operation history. These groups were compared in the conversion rate and perioperative clinical outcomes, such as sex, age, accompanied disease, operation time, complications, postoperative hospital stay, total hospital stay and total costs. RESULTS: We compared the two groups based on the timing of laparoscopic cholecystectomy and history of previous abdominal operation. There were no significant differences in the open conversion rate, postoperative complications and postoperative hospital stay, total hospital stay and total costs. The sex ratio, female in the previous abdominal operation group, was larger than the non-previous abdominal operation group (70.2% vs. 43.2%, p=0.003, OR=0.32 [95% CI, 0.15-0.70]). Early operation group was larger than delayed operation group, at previous abdominal operation history (26.1% vs. 13.3%, p=0.026, OR=0.43 [95% CI, 0.20-0.91]) and closed suction drain use (79.3% vs. 66.3%, p=0.044, OR=0.51 [95% CI, 0.27-0.99]). CONCLUSIONS: Although this study was limited, early laparoscopic cholecystectomy for acute cholecystitis with previous abdominal operation history seems to be safe and feasible for patients, having a benefit of decrease in total hospital stay.

Auxiliary partial orthotopic liver transplantation for adult onset type II citrullinemia.

Adult-onset type II citrullinemia (CTLN2) is a disorder caused by an inborn error of metabolism affecting the liver. CTLN2 is an autosomal recessive disorder characterized by recurrent encephalopathy with hyperammonemia due to highly elevated plasma levels of citrulline and ammonia, caused by a deficiency of argininosuccinate synthetase in the liver. A small number of patients have undergone liver transplantation with favorable results. In Korea, the limitations of the deceased donor pool have made living donor liver transplantation a common alternative treatment option. We report the case of a patient with type II citrullinemia who was treated successfully with auxiliary partial orthotopic liver transplantation (APOLT) from a living donor. This is the first description of an APOLT for a patient with adult onset type II citrullinemia in Korea.