A rare case of granulomatosis with polyangiitis with involvement of the gastrointestinal system.

We report a rare case of a 37-year-old man with granulomatosis with polyangiitis (GPA) with gastrointestinal system (GIS) involvement who needed 526 units of blood and blood product transfusions and was followed up in the intensive care unit (ICU). GIS involvement due to GPA is a rare condition that increases morbidity and mortality of patients. Patients may require ultramassive blood product transfusions. Thus, patients with GPA can be admitted to ICUs because of massive hemorrhage due to multisystem involvement, and survival is achievable with meticulous care through a multidisciplinary approach.

Comparison of Mortality Rates in Patients Waiting for Liver Transplant and Patients With Colorectal Metastatic Tumors.

OBJECTIVES: We aimed to identify outcomes of liver surgery in patients with hepatocellular carcinoma and colorectal cancer, which result in primary and secondary liver tumors. MATERIALS AND METHODS: Our study included 51 patients with colorectal cancer and liver metastases and 63 patients with hepatocellular carcinoma who were prepared for liver transplant due to cirrhosis who underwent hepatic resection or local ablation treatments; patients were seen between January 2011 and December 2021. RESULTS: Most patients with colorectal cancer were men (58.8%). Mean age was 65.76 ± 13.818 years (range, 27-88 y). Most patients had planned, elective surgery (86.3%). Neoadjuvant chemotherapy was administered to 58.8% of patients. The most common location of metastasis in the liver was in the right lobe (43.1%), and the most common surgery was low anterior resection (17 patients). During simultaneous liver surgery, 31 patients required metastasectomy and 7 patients required radiofrequency ablation plus metastasectomy. No deaths occurred in the early posttransplant period, and cumulative survival was 82.624 ± 7.962 months. Disease-free survival was 45.2 ± 7.495 months. Most patients with hepatocellular carcinoma were men (82.5%). Mean age was 58.73 ± 17.428 years. Hepatocellular carcinoma lesions were mostly located in both the right and left lobes (23.8%). In the hepatocellular cancer group, 60.3% had transarterial chemoembolization and 42.9% had radiofrequency ablation. The primary surgical resection was metastasectomy (17.9%) because of multiple localized lesions. Median follow-up was 22 months (range, 1-126 mo). Overall survival was 101.898 ± 7.169 months, with 10-year overall survival of 38%. Disease-free survival was 74.081 ± 8.732 months, with 1-year and 5-year disease-free survival of 90.5% and 54%. CONCLUSIONS: Better survival was shown in patients with hepatocellular carcinoma than in patients with colorectal cancer.Therefore, more aggressive treatment options, as used in hepatocellular carcinoma, including liver transplant, may be options for patients with colorectal cancer.

Effect of Ozone Added to University of Wisconsin Solution on Preservation Damage in Perfused Liver.

OBJECTIVES: Ischemia is defined as the inability of the tissue to provide oxygen and other metabolites by the circulation and the removal of residual products. The University of Wisconsin solution is widely used to preserve ischemia and to preserve organs for transplant. Ozone is used in various areas of ischemia damage due to its antioxidant properties. The aim of our study was to investigate the effects of ozone added to University of Wisconsin solution on perfused liver preservation injury. MATERIALS AND METHODS: Our study included 24 Sprague Dawley rats with an average weight of 300 to 350 g. Animals were divided into 4 groups: group 1 (Ringer lactate), group 2 (Ringer lactate + ozone), group 3 (University of Wisconsin solution), and group 4 (University of Wisconsin + ozone). Solutions were perfused from the liver portal vein and aorta. After perfusion, rats were killed and liver biopsies were taken at 0, 6, and 12 hours of storage for pathological examination. For biochemical analysis, samples were collected from liver specimen storage solutions at 0, 6, and 12 hours. RESULTS: Mean alanine aminotransferase/aspartate aminotransferase levels in group 3 were 77/82 U/L at hour 0, 680/461 U/L at hour 6, and 1027/682 U/L at hour 12. In group 4, these levels were 35/31 U/L at hour 0, 415/295 U/L at hour 6, and 546/372 U/L at hour 12. CONCLUSIONS: In terms of liver function values, we observed favorable result with University of Wisconsin solution with added ozone. Therefore, we suggest that the addition of ozone to the University of Wisconsin solution may be effective in preventing liver preservation damage.

Pure 3-Dimensional Laparoscopic Living-Donor Nephrectomy: First Case In Kazakhstan.

The available scientific literature has described the tangible benefits of operations using new 3-dimensional laparoscopic systems. The purpose of this report was to describe the first experience of pure 3-dimensional laparoscopic living-donor nephrectomy for transplant in the Republic of Kazakhstan. A living-donor kidney transplant was performed in a 21-year-old male patient with the father as the donor. The operation was performed with general anesthesia using a 3-dimensional endo-videoscopic stance with flexible camera (Olympus, Tokyo, Japan). The time of warm ischemia was 130 seconds, and the total operation time was 280 minutes. The postoperative period proceeded smoothly, without any complication. The patient was discharged on day 3 after transplant with normal levels of creatinine and urea. The recipient's surgery was typical, and no complications or difficulties in perfor-ming anastomosis were encountered. With further accumulation of experience, 3-dimensional laparoscopic nephrectomy from living donors could become a new criterion standard.

Diffusion-Weighted Imaging of Breast Cancer: Correlation of the Apparent Diffusion Coefficient Value with Pathologic Prognostic Factors.

OBJECTIVE: The aim was to evaluate relationship between apparent diffusion coefficient (ADC) values with pathologic prognostic factors in breast carcinoma (BC). MATERIALS AND METHODS: 83 patients were enrolled in this study. Prognostic factors included age, tumor size, expression of estrogen receptor (ER) and progesterone receptor (PR), human epidermal growth factor receptor 2 (HER2), nuclear grade (NG), lymph node involvement and histologic type. The relationship between ADC and prognostic factors was determined using Independent sample t-test, ANOVA, Pearson correlation and relative operating characteristics (ROC) analysis. RESULTS: There was no significant difference between ADC and prognostic factors, including age, tumor size, ER, HER2 and histologic type. The PR-positive tumors (p=0.03) and axillary lymph node involvement (p=0.000) showed a significant association with lower ADC values. The ADC values were significantly lower in high-grade tumors than low-grade tumors (p=0.000). ROC analysis showed an optimal ADC threshold of 0.66 (×10-3 mm2/s) for differentiating low-grade tumors from high-grade tumors (sensitivity, 85.5%; specificity, 81%; area under curve, 0.90). CONCLUSION: The lower ADC values of BC were significantly associated with positive expression of PR, LN positivity and high-grade tumor. Especially, ADC values were valuable in predicting NG subgroups.

Effect of Right Posterior Bile Duct Anatomy on Biliary Complications in Patients Undergoing Right Lobe Living Donor Liver Transplant.

OBJECTIVES: Our aim was to evaluate the influence of the localization of right posterior bile duct anatomy relative to portal vein of the donors on posttransplant bile duct complications. MATERIALS AND METHODS: We retrospectively investigated 141 patients who had undergone living donor liver transplant using right hemiliver grafts. The patients were classified based on the pattern of the right posterior bile duct and divided into infraportal and supraportal types. Clinical donor and recipient risk factors and surgical outcomes were compared for their relationship with biliary complications using logistic regression analyses. RESULTS: The 2 groups were similar according to demographic and clinical features. The biliary complication rate was 23.7% (9/38) in the infraportal group and 47.4% (37/78) in the supraportal group (P = .014). An analysis of risk factors for the development of anastomotic bile leak using logistic regression showed that a supraportal right posterior bile duct anatomy was a statistically significant positive predictor, with odds ratio of 18.905 (P = .012; confidence interval, 1.922-185.967). The distance of the right posterior bile duct from confluence was significantly lower in patients with biliary complications than in those without (mean of 7.66 vs 0.40 mm; P = .044). According to receiver operating characteristic analyses, the cut-off point for the length of right bile duct to right posterior bile duct from the hepatic confluence was 9.5 mm regarding presence of complications. CONCLUSIONS: Factors influencing bile duct anastomosis leakage were supraportal-type donor bile duct anatomy and length of the right main bile duct from biliary confluence. Hepatic arterial complications were similarly a risk factor for biliary strictures. Because of the multiple factors leading to complications in living donor liver transplant, it is challenging to group these patients by operative risk; however, establishing risk models may facilitate the prediction of complications.

Long-term cosmetic results of single-incision vs. conventional laparoscopic appendectomy A prospective observational cohort study.

AIM: The purpose of this study was to compare cosmetic, along with surgical, results between single incision laparoscopic appendectomy (SILA) and conventional laparoscopic appendectomy (CLA), particularly from patients' points of view. MATERIALS AND METHODS: All of the patients who underwent surgery for suspected acute appendicitis and were eligible for laparoscopic surgery were evaluated prospectively in our center between June 2013 and January 2015. Patients were underwent CLA or SILA were compared for operative results and cosmetic outcomes by Body Image Questionnaire. Nonparametric tests were used in the intergroup comparisons of quantitative data. Chi-square test was used in the comparison of qualitative data. RESULTS: A total of consecutive 166 patients were underwent SILA (55) or CLA (111) were included to the study. There was no conversion to another procedure. DURATION: of operation was significantly longer in SILA group (36.69±12.79 vs. 42.64±15.15; p= 0.009). There were no significant differences in length of stay, complications. SILA patients had more postoperative pain at first day after operation (p=0.002). After 12 months, body image and cosmetic appearance were excellent for both groups and indistinguishable by most measures (55.79±2.31 vs. 55,76±2,13; p= 0,937). CONCLUSIONS: SILA resulted in more pain and longer operative times without improving short-term recovery or complications. Long-term body image and cosmetic appearance were similar and excellent in both groups. KEY WORDS: Acute appendicitis, Cosmesis, Emergency surgery Minimally invasive.

Role of preoperative C-reactive protein value and neutrophil ratio in the determination of conversion from laparoscopic appendectomy to open appendectomy.

BACKGROUND: In this study, we aimed to investigate the factors causing conversion from laparoscopic appendectomy (LA) to open appendectomy (OA) in patients with acute appendicitis and to investigate the role of preoperative C reactive protein (CRP) and neutrophil ratio in this conversion and determine a cut-off point for these parameters. METHODS: Records of patients who underwent LA due to acute appendicitis at our general surgery department between January 2011 and January 2017 were retrospectively evaluated. The preoperative American Society of Anesthesiology (ASA) scores, Alvarado scores, white blood cell count, C-reactive protein level, and neutrophil ratio were evaluated. RESULTS: LA was performed in 394 patients with an initial diagnosis of acute appendicitis. A conversion to OA (cOA) was performed in 17 patients (4.31%). A CRP value of ≥108.5 mg/L and a neutrophil ratio of ≥81.5% were found to be statistically significant for the cOA (p<0.001). CONCLUSION: Our study results showed that male gender, age, elevated neutrophil ratio, and CRP value were the main risk factors for cOA in patients who were scheduled for LA due to acute appendicitis.

Gastrointestinal Stromal Tumors: A Clinicopathological and Immunohistochemical Study of 65 Cases.

OBJECTIVE: The clinical behavior of gastrointestinal stromal tumors is divergent. The aim of the present study was to define the clinicopathological features that determine the patient's outcome. MATERIAL AND METHOD: Sixty-five gastrointestinal stromal tumors were reviewed with their histological, immunohistochemical and clinical features and compared with their clinical outcome statistically. RESULTS: Tumors were located in the stomach (n=39, 60%), small intestine (n=22, 33.8%) and large intestine (n=4, 6.2%). Immunohistochemically, CD 117 positivity was found in 90.8%, whereas CD34, Smooth muscle actin, Desmin and S100 positivity was found in 73.3%, 61.7%, 11.7% and 28.3% of tumors respectively. All six ''CD 117-negative'' cases expressed DOG-1. The mean Ki-67 proliferation index was 8.69%±12.76. Liver metastasis was detected in seven cases. A significant association was detected between decreased mean survival time and increased tumor size (p < 0.001), large bowel localization (p=0.047), mitosis (p < 0.001), the presence of necrosis (p=0.001), metastasis (p=0.033), Ki-67 proliferation index (p=0.002) and risk category (p < 0.001). CD 34 positivity was mostly seen in the stomach (p=0.001), and CD 34 positive tumors had longer overall survival (92.85.±5.77 months versus 67.21±13.68 months) (p=0.046). Higher Ki-67 proliferation index (≥6%) was also correlated with the presence of metastases (p=0.015). CONCLUSION: Our study indicates that in addition to well-known risk factors such as increased tumor size, high mitotic activity and metastasis; higher Ki-67 proliferation index, the presence of necrosis, and CD34 negativity also correlate with shorter survival time.

Long-term and Perioperative Outcomes of Laparoscopic and Open Surgery for Rectal Cancer.

OBJECTIVES: The necessity of comparing oncologic results with the use of minimally invasive surgery in rectal cancer has arisen. The aim of the present study was to evaluate the treatment approach in rectal cancer and to compare the outcomes of laparoscopic and open surgery. METHODS: Patients who underwent surgery for rectal carcinoma between January 2006 and January 2016 in our institution were evaluated. The results were compared between the two groups according to open or laparoscopic surgery. Clinical characteristics, preoperative and postoperative results, pathological examination results, and disease-free survival rates were compared after the surgical procedure. RESULTS: A total of 121 patients were included in the study. Of the patients, 50 underwent open, and 71 underwent laparoscopic surgery. The median follow-up times were 56.75 months in the open surgery group and 55.2 months in the laparoscopic surgery group. Pathological examination revealed similar numbers of lymph nodes in both groups (p>0.05). The duration of hospital stay was statistically significantly lower in the open surgery group than in the laparoscopic group (p<0.05). The rates of disease-free survival were 74% in the open surgery group and 82.5% in the laparoscopic group, and no statistically significant difference was found (p>0.05). CONCLUSION: There was no significant difference in complication and recurrence between laparoscopic and open surgery for rectal cancer in our study. The duration of hospital stay of patients was statistically significantly lower in the laparoscopic group than in the open surgery group. Laparoscopic or open surgical options could be preferred according to the clinical suitability of the patient, experience of the surgeon, and resources of the center in rectal cancer treatment.

Laparoscopic spleen-preserving distal pancreatectomy for a primary hydatid cyst mimicking a mucinous cystic neoplasia.

Pancreatic hydatid cysts are fairly rare. The disease can be encountered concurrently with systemic involvement or as an isolated pancreatic involvement. We report the first case of spleen-preserving laparoscopic distal pancreatectomy for a pancreatic hydatid cyst. There was no complication or recurrence. A 55-year-old woman was admitted to our centre with epigastric and back pain. Upper abdominal magnetic resonance imaging revealed a solitary cystic lesion with septations at the pancreatic tail level measuring 24 mm × 18 mm, which was initially thought to be a pancreatic mucinous cystic neoplasia. She underwent laparoscopic spleen-preserving distal pancreatectomy and cholecystectomy. Her post-operative course was uneventful and histopathological examination revealed a hydatid cyst in the pancreatic tail.

Can strain elastography combined with ultrasound breast imaging reporting and data system be a more effective method in the differentiation of benign and malignant breast lesions?

PURPOSE: To investigate whether a novel method that combines breast imaging reporting and data system (BI-RADS) with strain elastography contributes to diagnostic performance in differentiation of malignant and benign breast lesions. METHODS: In 81 patients, 81 breast lesions were prospectively investigated. Breast lesions were separately evaluated with ultrasonography and strain elastography. While evaluations with ultrasonography were based on 2003 BI-RADS-US, strain elastography evaluations were based on a 5-point scale and strain ratio. Diagnostic performances of ultrasonography, strain elastography, and the combined method were compared. RESULTS: Among 81 lesions, 43 (53.1%) were benign and 38 (46.9%) were malignant. When a cutoff point of category 3 was used, sensitivity, specificity, positive and negative predictive values, and accuracy for BI-RADS were 100, 11.6, 50, 100, and 53%, respectively. When BI-RADS and strain ratio were combined, sensitivity, specificity, positive and negative predictive values, and accuracy were 89.5, 93, 91.9, 90.9, and 91.3%, respectively. When BI-RADS and elastography scores were combined, sensitivity, specificity, positive and negative predictive values, and accuracy were 86.8, 97.7, 97.1, 89.4, and 92.5%, respectively. CONCLUSIONS: The combination of strain elastography and BI-RADS was found to have better diagnostic performances to diagnose breast lesions than BI-RADS alone.

Arterial complication of irreversible electroporation procedure for locally advanced pancreatic cancer.

Irreversible electroporation (IRE) is a non-thermal ablation technique used especially in locally advanced pancreatic carcinomas that are considered surgically unresectable. We present the first case of acute superior mesenteric artery (SMA) occlusion secondary to pancreatic IRE procedure that has not been reported before in the literature. A 66-year-old man underwent neoadjuvant chemoradiotherapy for locally advanced pancreatic ductal adenocarcinoma. IRE procedure was applied to the patient during laparotomy under general anesthesia. After finishing the procedure, an acute intestinal ischemia was detected. A conventional vascular angiography was performed and a metallic stent was successfully placed to the SMA and blood flow was maintained. It is important to be careful in such cases of tumor involvement of SMA when evaluating for IRE procedure of pancreatic tumor.

The Outcomes of Ultralow Anterior Resection or an Abdominoperineal Pull-Through Resection and Coloanal Anastomosis for Radiation-Induced Recto-Vaginal Fistula Patients.

PURPOSE: The purpose of this study was to evaluate the outcomes of patients who underwent colorectal resections and coloanal anastomosis for radiation-induced recto-vaginal fistulas (RVFs). The effect of the surgical treatment technique on bowel function, fecal continence, and quality of life of patients was also evaluated. METHODS: Twenty-one female patients, who received adjuvant chemotherapy and external beam pelvic radiation for cervix carcinoma after radical hysterectomy + pelvic/paraaortic lymph node dissection, having RVF but without tumor recurrence, were included. All patients underwent an ultralow anterior resection (n = 11) or an abdominoperineal pull-through resection and straight coloanal anastomosis (n = 10). A bowel functions questionnaire and a Fecal Incontinence Quality of Life (FIQLI) questionnaire were applied to patients pre-operatively and also 6 months after the ileostomy closure procedures. RESULTS: No recurrent RVF was observed in a mean follow-up period of 20 months after ostomy reversal procedures. The FIQLI depression, lifestyle, and embarrassment scores were significantly improved on the follow-up questionnaire. The mean pre- and post-operative incontinence scores were not significantly different. CONCLUSIONS: The spontaneous closure rate after a simple diverting stoma is quite low and local repair procedures usually result in failure. In selected patients, performing a nearly total rectum resection and maintaining the intestinal continuity with a coloanal anastomosis may be accepted as a safe and curative option. Recurrence-free outcome and the improvement of the quality of life of the patients represent the efficiency of this treatment modality.

Outcomes of Patients With Hepatocellular Carcinoma After Liver Transplant.

OBJECTIVES: Liver transplant is one of the few effective treatments for hepatocellular carcinoma. Our aim in this study was to evaluate the risk factors for hepatocellular carcinoma recurrence after liver transplant. MATERIALS AND METHODS: In this retrospective study, conducted between October 1988 and March 2015, four hundred seventy-three liver transplants were performed at our institution. Of these, 231 were pediatric and 242 were adult. Among these patients, liver transplant was performed in 58 patients (12.3%) for treatment of hepatocellular carcinoma. RESULTS: Hepatocellular carcinoma recurrence was detected in 14 patients (24.1%). Overall 5-year and 10-year survival rates of patients underwent liver transplant beyond the Milan criteria for hepatocellular carcinoma were 50.3% and 43.1%. Overall, 5- and 10-year survival rates of patients underwent liver transplant within the Milan criteria for hepatocellular carcinoma were 78.4% and 72.6%. The main predictive variable was whether the tumor had expensed beyond the Milan criteria. CONCLUSIONS: As expected, outcomes were significantly better in the Milan criteria group. Although the overall- and disease-free survival rates were promising in such a group of patients who had no better chance, it could be asserted that liver transplant is a safe and effective treatment option with promising results, even if the tumor expanse is beyond the Milan criteria.

Anorectal function and outcomes after transanal minimally invasive surgery for rectal tumors.

BACKGROUND: Transanal endoscopic microsurgery is a minimally invasive technique that allows full-thickness resection and suture closure of the defect for large rectal adenomas, selected low-risk rectal cancers, or small cancers in patients who have a high risk for major surgery. Our aim, in the given prospective study was to report our initial clinical experience with TAMIS, and to evaluate its effects on postoperative anorectal functions. MATERIALS AND METHODS: In 10 patients treated with TAMIS for benign and malignant rectal tumors, preoperative and postoperative anorectal function was evaluated with anorectal manometry and Cleveland Clinic Incontinence Score. RESULTS: The mean distance of the tumors from the anal verge was 5.6 cm, and mean tumor diameter was 2.6 cm. All resection margins were tumor free. There was no difference in preoperative and 3-week postoperative anorectalmanometry findings; only mean minimum rectal sensory volume was lower at 3 weeks after surgery. The Cleveland Clinic Incontinence Score was normal in all patients except one which resolved by 6 weeks after surgery.The mean postoperative follow-up was 28 weeks without any recurrences. CONCLUSION: Transanal minimally invasive surgery is a safe and effective procedure for treatment of rectal tumors and can be performed without impairing anorectal functions.

Brown Tumor of the Thoracic Spine: First Manifestation of Primary Hyperparathyroidism.

Brown tumors also called as osteoclastomas, are rare nonneoplastic lesions that arise in the setting of primary or secondary hyperparathyroidism. Parathyroid adenomas or hyperplasia constitute the major Brown tumor source in primary hyperparathyroidism while chronic renal failure is the leading cause in secondary hyperparathyroidism. Most of the patients with the diagnosis of primary hyperparathyroidism present with kidney stones or isolated hypercalcemia. However, nearly one third of patients are asymptomatic and hypercalcemia is found incidentally. Skeletal involvement such as generalized osteopenia, bone resorption, bone cysts and Brown tumors are seen on the late phase of hyperparathyroidism. The symptoms include axial pain, radiculopathy, myelopathy and myeloradiculopathy according to their locations. Plasmocytoma, lymphoma, giant cell tumors and metastates should be ruled out in the differential diagnosis of Brown tumors. Treatment of Brown tumors involve both the management of hyperparathyroidism and neural decompression. The authors report a very rare spinal Brown tumor case, arisen as the initial manifestation of primary hyperparathyroidism that leads to acute paraparesis.

Efficacy of cell saver use in living-donor liver transplant.

OBJECTIVES: Liver transplant currently is the best treatment option for end-stage liver disease. During liver transplant, there is major blood loss due to surgery and primary disease. By using a cell saver, the need for blood transfusion is markedly reduced. In this study, we aimed to evaluate the efficacy of cell saver use on morbidity and mortality in living-donor liver transplant. MATERIALS AND METHODS: We retrospectively evaluated 178 living-donor liver transplants, performed from 2005 to 2013 in our center. Child-Turcotte-Pugh A patients, deceased-donor liver transplants, and liver transplants performed for fulminant hepatic failure were not included in this study. Intraoperative blood transfusion was done in all patients to keep hemoglobin level between 10 and 12 g/dL. Cell saver was used in all liver transplants except in patients with malignancy, hepatitis B, and hepatitis C. RESULTS: We included 126 patients in the study. Cell saver was used in 84 liver transplants (66%). In 42 patients (34%), liver transplant was performed without a cell saver. In living-donor liver transplant with cell saver use, 10 mL/kg blood (range, 2-50 mL/kg blood) was transfused from the cell saver; in addition, 5 to 10 mL/kg allogeneic blood was transfused. In living-donor liver transplant without cell saver, 20 to 25 mL/kg allogeneic blood was transfused. CONCLUSIONS: During liver transplant, major blood transfusion is needed because of surgery and primary disease. Cell saver use markedly decreases the need for allogeneic blood transfusion and avoids adverse events of massive transfusion.

Results of pediatric liver transplant: a single-center experience.

OBJECTIVES: Liver transplant is an established curative therapy for children with chronic end-stage liver disease or acute liver failure. In this study, we aimed to evaluate pediatric liver transplant in terms of outcomes, complications, and long-term followup results. MATERIALS AND METHODS: Pediatric patients who had liver transplant in our institution were included. We retrospectively evaluated demographic features including body weight, Child-Pugh score, etiology of liver disease, graft source, perioperative outcomes, perioperative complications, postoperative complications, and long-term results. Outcomes of treatment of complications and revision transplant were evaluated. RESULTS: Between September 2001 and December 2013, there were 188 pediatric liver transplants performed in our institution. Most grafts (90.9%) were obtained from living-related donors. There were 13 patients (6.9%) who had an intervention because of a hemorrhage postoperatively. Biliary leakage was observed in 33 patients (17.5%) and biliary stricture during follow-up was observed in 32 patients (17%). Thrombosis rates in the hepatic artery and portal vein were 12.3% and 0.5%. Revision transplant was performed in 11 patients (5.8%); reason for revision transplant was rejection in 50% patients. The remaining children were alive with good graft functioning after treatment of complications and revision transplant. The overall 5- and 10-year survival rates were 82.3% and 78.9%. CONCLUSIONS: The overall outcomes of pediatric liver transplant at our center are very promising. With improved care of younger children and the combined efforts of the parents and medical team, the number of the children receiving transplants will increase in the future.

Liver and kidney transplant in primary hyperoxaluria: a single center experience.

OBJECTIVES: Primary hyperoxaluria, especially type 1, is a severe disease with multisystem morbidity and high mortality. We present 3 primary hyperoxaluria type 1 patients who underwent liver transplant, including living-donor liver transplant or combined liver and kidney transplant in our institution. CASE REPORTS: Patients who underwent liver transplant or combined liver/kidney transplant at our institution were evaluated, retrospectively. Between January 2002 and 2013, there were 3 patients who underwent transplant for primary hyperoxaluria. All 3 patients had disease onset in childhood, and the definitive diagnosis was established at age < 1, 6, and 8 years. Although early diagnosis was made, primary hyperoxaluria resulted in end-stage renal disease in 2 patients, and hemodialysis was introduced before liver transplant. All 3 patients underwent living-donor liver transplant. Case 1 was a 10-year-old girl who had an uneventful course after living-donor liver transplant, and she received a living-donor kidney transplant from the same donor 4 months after living-donor liver transplant. Case 2 was a 7-yearold boy who was the younger brother of the first patient; he did not have end-stage renal disease or any renal disorder after successful living-donor liver transplant. Case 3 was a 3-year-old boy who was diagnosed at age 2 months with renal disorders; although he was discharged from the hospital after living-donor liver transplant, he was readmitted because of unconsciousness that developed 1 day after discharge, and he died because of intracranial hemorrhage 2 months after liver transplant, unable to receive a kidney transplant. CONCLUSIONS: Primary hyperoxaluria is a rare disorder that is difficult to diagnose until end-organ damage is severe. Outcomes may be improved with early and accurate diagnosis, aggressive supportive treatment, and correction of the enzyme defect by liver transplant before systemic oxalosis develops. However, kidney transplant or combined liver and kidney transplant is required in many primary hyperoxaluria type 1 patients because of the delayed diagnosis or long organ waiting time.