Predictors of Death in the Liver Transplantation Adult Candidates: An Artificial Neural Networks and Support Vector Machine Hybrid-Based Cohort Study.

Background: Model for end-stage liver disease (MELD) is currently used for liver transplantation (LT) allocation, however, it is not a sufficient criterion. Objective: This current study aims to perform a hybrid neural network analysis of different data, make a decision tree and finally design a decision support system for improving LT prioritization. Material and Methods: In this cohort follow-up-based study, baseline characteristics of 1947 adult patients, who were candidates for LT in Shiraz Organ Transplant Center, Iran, were assessed and followed for two years and those who died before LT due to the end-stage liver disease were considered as dead cases, while others considered as alive cases. A well-organized checklist was filled for each patient. Analysis of the data was performed using artificial neural networks (ANN) and support vector machines (SVM). Finally, a decision tree was illustrated and a user friendly decision support system was designed to assist physicians in LT prioritization. Results: Between all MELD types, MELD-Na was a stronger determinant of LT candidates' survival. Both ANN and SVM showed that besides MELD-Na, age and ALP (alkaline phosphatase) are the most important factors, resulting in death in LT candidates. It was cleared that MELD-Na <23, age <53 and ALP <257 IU/L were the best predictors of survival in LT candidates. An applicable decision support system was designed in this study using the above three factors. Conclusion: Therefore, Meld-Na, age and ALP should be used for LT allocation. The presented decision support system in this study will be helpful in LT prioritization by LT allocators.

Nonalcoholic Steatohepatitis Is the Most Rapidly Growing Indication for Liver Transplantation in Iranian Patients.

OBJECTIVES: Nonalcoholic fatty liver disease is a rapidly growing disease and is hypothesized to become the most common cause of liver cirrhosis in the near future. This study aimed to investigate trends of nonalcoholic steatohepatitis as an indication for liver transplant in Iranian patients. MATERIALS AND METHODS: Liver transplant data from all adult patients (age > 18 y) who had undergone liver transplant between 1993 and 2017 at the Shiraz Organ Transplant Center (Shiraz, Iran) were reviewed. Underlying liver diseases leading to liver transplant were stratified according to year of transplant, and trends of increase or decline were analyzed. Kaplan-Meier curves were used for analysis of posttransplant survival of patients with nonalcoholic steatohepatitis and patients with modified nonalcoholic steatohepatitis. RESULTS: We evaluated 3184 liver transplant patients. Of these, 112 patients with biopsy-proven nonalcoholic steatohepatitis underwent liver transplant up to the end of 2017. Mean age of patients was 52.86 ± 9.01 years in those with nonalcoholic steatohepatitis and 51.73 ± 7.91 years in those with modified nonalcoholic steatohepatitis (P > .05).The prevalence of nonalcoholic steatohepatitis as an indication for liver transplant was 0.8% in 2011, 0.36% in 2012, 1.9% in 2013, 4.01% in 2014, 2.89% in 2015, 6.65% in 2016, and 9.97% in 2017. The prevalence of modified nonalcoholic steatohepatitis was 2.4% in 2011, 2.88% in 2012, 2.71% in 2013, 2% in 2014, 2.17% in 2015, 2.13% in 2016, and 2.28% in 2017. We found that nonalcoholic steatohepatitis as a cause of liver transplant increased significantly during recent years (P < .001). CONCLUSIONS: Nonalcoholic steatohepatitis is a rapidly growing indication for liver transplant among Iranian patients. Health care providers should consider programs for prevention and early diagnosis of patients with nonalcoholic steatohepatitis for proper treatment.

The Largest Single Center Report on Pediatric Liver Transplantation: Experiences and Lessons Learned.

OBJECTIVE: We described our experiences on pediatric liver transplantation (LT) from the largest LT center in the world termed the Shiraz Transplant Center. BACKGROUND: After the first successful pediatric LT in 1967, pediatric LT has become the routine treatment for children with liver failure worldwide. METHODS: Data on a total of 1141 pediatric cases of LT were collected. Specifics on baseline and anthropometric characteristics, clinicopathology, prognosis of recipients of LT, and donor characteristics are reported. RESULTS: Mean age of patients was 7.83 ±â€Š5.55 years old. Most common etiologies for LT were biliary atresia (15.9%), progressive familial intrahepatic cholestasis (13.4%), and Wilson's disease (13.3%), respectively.Whole organs, living donor grafts, and split grafts were used in 47.9%, 41%, and 11.1% of patients, respectively. In-hospital complications were seen among 34.7% of patients and the most common complications were infections (26.8%), bleeding (23.4%), and vascular complications (18%).Median (interquartile range) model for end stage liver disease score was 20 (15, 25). Main causes of death among patients were sepsis (35.2%), followed by post-transplantation lymphoproliferative diseases (10.5%), and primary nonfunction of liver (9%).Patient survival showed improvement over the years (1-year survival of 73.1%, 83.4%, and 84.4%, 2-year survival of 65.2%, 77.1%, and 78.7%, 5-year survival of 58.2%, 72%, and 77.8% for 1997-2007, 2007-2013, and 2013-2019, respectively; P < 0.001). CONCLUSIONS: This is the largest single-center report on pediatric LT in literature which provides valuable experiences in pediatric LT.

Portal vein thrombosis in patients with liver cirrhosis and its impact on early and long-term outcomes after liver transplantation.

AIM: Portal vein thrombosis (PVT) is a common complication amongst patients with liver cirrhosis. The PVT risk factors and its impact on post liver transplant outcome has not been well defined, yet. This study aimed to investigate PVT prevalence, its risk factors and influence on early and long-term outcomes after liver transplantation. METHODS: Adult (>18 years) patients with liver cirrhosis undergoing liver transplantation between March 2013 to March 2015 were included. Presence or absence of PVT was recorded at transplant. PVT risk factors in patients with liver cirrhosis and its impact on early and long-term outcomes were analysed. RESULTS: Portal vein thrombosis was diagnosed in 174 patients (17.3%). Large oesophageal varices (grade II and III vs grade I) (OR: 2.5; 95% CI: 1.46-4.26; P = 0.001), diabetes mellitus before transplantation (OR: 2.03; 95% CI: 1.13-3.64; P = 0.017) and cryptogenic-NASH (OR: 1.36; 95% CI: 1.08-1.72; P = 0.008) as a cause of underlying liver disease were the independent risk factors for PVT. PVT (OR: 2.05; 95% CI: 1.10-3.81; P = 0.023) was an independent predictor of early (within 90 days) posttransplant mortality, but did not influence long-term survival. CONCLUSION: Portal vein thrombosis prevalence is high in pretransplant period. NASH related cirrhosis and diabetes mellitus might be risk factors for PVT. More intense screening of these patients for PVT is warranted.

Liver Transplantation Status in Iran: A Multi-center Report on the Main Transplant Indicators and Survival Rates.

BACKGROUND: Iran's experience with liver transplantation (LT) began more than two decades ago. The purpose of this article is to present the status of LT in Iran, review specific characteristics of the programs, their outcomes, and their growth to become one of the largest LT programs in the world. METHODS: A questionnaire, asking for data on the number of transplants performed and specifics of the recipients and type of donors with focus on indications and outcomes was sent to LT programs. RESULTS: During a period of 23 years, 4,485 LTs were performed at 6 centers in the country. Of these, 4106 were from deceased donors and 379 were from living donors. There were 3553 adults and 932 pediatric recipients. Hepatitis B and biliary atresia were the most common etiologies in adult and pediatric patients, respectively. Overall survival rates at 1, 5, and 10 years were 85%, 77%, and 71% for adults and 76%, 67% and 56% for pediatric patients, respectively. CONCLUSION: Approval of the brain death law in Iran and coordinated efforts by the transplant centers to build comprehensive LT programs has resulted in the ability to procure more than 700 deceased donors per year with acceptable long-term survival.

Gastrotracheal Fistula as a Result of Transhiatal Esophagectomy for Esophageal Cancer: An Unusual Complication.

Gastrotracheal fistula following open transhiatal esophagectomy (Orringer's technique) for esophageal cancer is an unusual but lethal complication. Surgical intervention with resection of the fistula tract and primary interrupted suturing of gastric and tracheal orifices using a muscle flap interposition has proved to be a successful method. We report the case of a 73-year-old male with an adenocarcinoma of the distal part of the esophagus, who underwent open transhiatal esophagectomy (Orringer's technique) with gastric tube reconstruction and cervical anastomosis. The patient did not receive induction chemoradiotherapy before the esophagectomy. Two attempts of surgical repair of fistula failed and the patient died. Being aware of warning signs such as dyspnea and respiratory distress accompanied by bilious content in the tracheal tube is helpful in the early detection and treatment of this type of fistula.

Liver transplantation as a definitive treatment for familial hypercholesterolemia: A series of 36 cases.

FH is a genetic disorder characterized by an increase in serum LDL and total cholesterol values. The afflicted patients are at increased risk of premature atherosclerosis and myocardial infarction. Different treatment modalities are present, including pharmacological agents and surgical procedures. The most effective method of therapy in refractive cases is liver transplantation. Herein, we report our experience on 36 cases of patients with FH undergoing liver transplantation in our center, the main referral center of liver transplantation in Iran. The clinical findings, hospital courses, post-operative complications, and patient follow-up are also described.

Adrenal cystic lymphangioma presented with hypertension: a case report.

INTRODUCTION: Adrenal cystic lymphangioma is a rare benign tumor, which is nearly always asymptomatic and incidentally discovered during routine imaging work-ups or investigating other pathologies. CASE REPORT: A 43-year-old female presented hypertension, which during routine work-up of her newly diagnosed hypertension an adrenal mass was discovered and after operation the pathologic diagnosis was lymphangioma of adrenal. CONCLUSIONS: In evaluation of adrenal masses cystic lymphangioma should be considered as a differential diagnosis.

A comparative study of the classic and piggyback techniques for orthotopic liver transplantation.

BACKGROUND: The classic technique of hepatectomy with venovenous bypass may cause a longer anhepatic phase and increase the rate of some complications, such as post-operative renal failure and thromboembolic events. But, in some cases, such as tumors and anatomic difficulties, the surgeon is obligated to use the classic technique even though there is some controversy about the safety of this technique without venovenous bypass in liver transplantation. The aim of this study was to compare the results of using the classic technique without venovenous bypass and the piggyback technique for liver transplantation. METHODS: A retrospective case-series study was conducted on 227 consecutive successful liver transplants, including 55 cases in which the classic technique was used and 172 cases in which the piggyback technique was used. The transplants were performed from March 2010 through June 2011 in the Visceral Transplantation Ward at Namazi Hospital in Shiraz, Iran. The piggyback method was the preferred approach for hepatectomy, but the classic technique without venovenous bypass was performed in cirrhotic cases with anatomic difficulties, when there was a tumor, or when the surgeon preferred it. RESULTS: There were no significant differences in post-operative rise in creatinine, decreases in intraoperative blood pressure, transfused packed red blood cells (RBC), or survival rates between the groups. Warm ischemic time (duration that donor liver is out of ice until it's blood reperfusion in the recipient) was approximately seven minutes longer in the classic group (P = 0), but it was less than 52 minutes, which is an acceptable time for this phase. Hospital stays were shorter in the classic group than in the piggyback group (P = 0.024). CONCLUSION: Although the piggyback technique is the preferred technique for hepatectomy in liver transplantation, the classic technique without venovenous bypass can be used safely in cirrhotic livers when necessary or if the physician prefers it.

Hepatocellular carcinoma in explanted livers of patients with genotype d HBV cirrhosis: report of the first experience from Iran.

BACKGROUND: This study was conducted to determine the impact of hepatitis B virus (HBV) as a cause of hepatocellular carcinoma (HCC) in a single liver transplant center in Iran.  METHODS: We included all hepatectomy specimens from patients with HBV-related cirrhosis who underwent transplants from May 1993 until January 2012 in this study. From these, we determined the number that had HBV-induced HCC. Nested PCR results were used to determine the HBV genotype from sections of the hepatectomy pathology specimens. RESULTS: During this time period there were 1361 cirrhotic livers transplanted in our center. Of these, 249 were attributed to HBV cirrhosis. Overall, HCC was detected in 40 (2.9%) subjects, of which 29 (1.2%) had HBV-related HCC.  Genotype D was only genotype observed in all HBV subjects.  CONCLUSIONS: The results revealed that although HBV-related cirrhosis was the most frequent single cause for liver transplant, the frequency of HBV-induced HCC was very low among transplant recipients. Out of 1361 transplant recipients, only 29 (2.1%) were diagnosed with HBV-related HCC. All HBV subjects had genotype D.

Liver transplant in a case of arthrogryposis-renal tubular dysfunction-cholestasis syndrome with severe intractable pruritus.

Arthrogryposis-renal tubular dysfunction-cholestasis syndrome (MIM No. 208085) is a rare multisystem disorder involving the liver, kidney, skin, and central nervous and musculoskeletal systems. The syndrome is an autosomal-recessive trait, associated with germ-line mutations in the VPS33B gene. We report an Iranian boy of consanguineous cousin parents who had congenital deformities of the upper and lower extremities, severe ichthyosis, cholestasis, intractable pruritus, metabolic acidosis, and failure to thrive. Owing to cholestasis, severe intractable pruritus, and poor quality of life, he underwent a living-related liver transplant from his mother, and his ichthyosis and pruritus dramatically improved. To the best of our knowledge, this is a first case of someone with arthrogryposis-renal tubular dysfunction-cholestasis syndrome who underwent a liver transplant and is in good condition more than 5 years after surgery.

Clinical experience in organ transplant from the Shiraz Transplant Center: 2011.

OBJECTIVES: The Shiraz Organ Transplant Center, the largest transplant center in Iran, has expanded its program of organ transplant during recent years. This article seeks to summarize organ transplantation over the last 2 decades and evaluate its status as of 2011. MATERIALS AND METHODS: We retrospectively analyzed the clinical records of all organ transplants performed in our center in 2011. We reviewed the patients' demographics, underlying disease, operation details as well as postoperative complications. RESULTS: During this period, 655 organ transplants including 345 liver, 297 kidney, 29 pancreas, and 11 intestine and multivisceral transplants were done. Among 345 liver transplants, 291 patients received a deceased-donor graft including 18 cases of split liver transplants while 54 patients received living-donor liver transplants. The 1-year graft and patient survival rates were 90.1% and 91%. CONCLUSIONS: In recent years, our program in organ transplants has expanded in number and variety of organs transplanted. This improvement is related to our multidisciplinary strategies to expand the donor pool and the experiences obtained during our transplant activities.

Primary pure squamous cell carcinoma of gallbladder presenting as acute cholecystitis.

Squamous cell carcinoma of the gallbladder is rare and constitutes only 0.5-3% of all malignancies of this organ. Most of the reported cases have had a component of adenocarcinoma. We report a 70-year-old man who presented with acute onset right upper quadrant pain. He operated on based on a presumptive diagnosis of acute cholecystitis according to clinical and ultrasonographic findings. Histopathological examination of the infiltrating mass of the gallbladder revealed well differentiated keratinized squamous cell carcinoma invading full wall thickness. Thorough evaluations revealed no other primary site for the tumor. Pure primary squamous cell carcinoma of the gallbladder is rarely reported. Clinicians and pathologists must be aware of its vague clinical presentations.

Reducing Social Disparity in Liver Transplantation Utilization through Governmental Financial Support.

BACKGROUND: A high proportion of patients suffering from end stage liver disease are from low socioeconomic classes , which limits their access to liver transplantation as the most effctive treatment of this condition because of cost barrier. OBJECTIVES: one of the most challenging aspects of liver transplantation is its affordability and utilization by those who need it the most. PATIENTS AND METHODS: Since November 2005, Iran Ministry of Health had covered 100% of the costs of in-patient liver transplantation care. To determine the effects of this policy, patterns of utilization of liver transplantation were compared before and after implementation of the policy. Group one included 112 and group two included 120 individuals who received transplantation before (from early January 2003 to November 2005) and after (from November 2005 to the end of December 2007) the legislation entered into the effect, respectively. Socioeconomic characteristics of these patients were evaluated by data collected about house and car ownership, education level, employment status, and place of residence. RESULTS: Coverage of the costs allowed more illiterate and semiliterate people (P = 0.032) as well as more unemployed or unskilled workers to receive transplantation (P = 0.021). The number of transplantations also increased in children and geriatric age group. This legislation also led to greater countrywide regional coverage of indigent patients. CONCLUSIONS: This survey provides evidence that coverage of the costs by Ministry of Health was effective in reducing social discrimination in utilization of liver transplantation, and narrowed the gap between low and high socioeconomic classes in Iranian society.

Primary Involvement of Allografted liver in Post-Transplant Lymphoproliferative Disorders, Report of Two Pediatric Cases and Review of the Literature.

Post-transplant lymphoproliferative disorder is a lymphocyte proliferating disease, usually of B cell origin, and rarely of T cell. Involvement of liver itself in liver transplant recipients as the primary organ is not common. Herein we report our experience in two patients who primarily presented in the allografted liver, both of whom were promptly diagnosed after liver biopsy and treated successfully .Now after a few months; both of the patients are alive with normal liver function tests and negative imaging studies.

Liver abscess in children: a 10-year single centre experience.

BACKGROUND/AIM: Although liver abscess is more prevalent in developing countries than in developed countries, there is scant data about the characteristics of pediatric liver abscess in our region. We aimed to analyze the characteristics of pediatric liver abscess in our region and compare these with those of developed countries. MATERIALS AND METHODS: The clinical features, laboratory, imaging, microbiologic findings, management strategy, and final outcome were extracted from the patients' records retrospectively. RESULTS: There were 18 cases of liver abscess including 16 pyogenic liver abscess, one amebic liver abscess and one candida liver abscess. Fever and abdominal pain were the most common clinical findings and leukocytosis was the most common laboratory finding. The most predisposing factors of liver abscess were immune deficiency, minor thalassemia. Origin of liver abscess was appendicitis in two patients, the rest were considered as cryptogenic. While one patient was treated with antibiotics alone, five cases were taken for open drainage, and 12 cases were treated with percutaneous aspiration. Percutaneous aspiration failed in two patients who were later taken for open drainage, with an overall mortality rate of 5.5%. CONCLUSION: The overall characteristics of liver abscess in children in our society are not so different from developed countries. However, in contradiction to cases reported in developed countries, most cases of liver abscess were seen in healthy patients in our centre. Moreover, liver abscess was reported in our patients at a younger age and was more commonly seen in male children. Mortality rate was similar to that of developed countries.

Postrenal transplant urinary leakage caused by segmental infarction of a renal allograft treated by partial nephrectomy.

Kidney transplant is the final treatment for patients with end-stage renal disease. Urinary leakage is the most-common surgical complication early after transplant. Another complication in the early posttransplant period is segmental allograft infarction. We report a kidney recipient who developed urinary leakage secondary to a segmental infarction of the upper pole of the transplanted kidney 2 months after transplant. The patient was treated successfully by a partial nephrectomy of the infracted upper lobe of the kidney. Three months after the partial nephrectomy of the allograft, serum blood urea nitrogen and creatinine were normal, and the patient was able to partake in her daily activities. Partial nephrectomy in the context of infarction of a kidney allograft is safe and can be used in similar cases.

Hepatic angiosarcoma of childhood: a case report and review of literature.

Angiosarcomas are rare tumors that predominantly affect adults. Hepatic angiosarcoma in a child is an extremely rare event and is associated with a poor prognosis. Herein, we report our experience in a case of hepatic angiosarcoma in a child who presented with a huge unresectable mass that underwent liver transplantation.

Outcomes of kidney transplantation in patients with systemic lupus erythematosus: a single-center study.

INTRODUCTION. This study aimed to compare outcomes of kidney transplantation in patients with systemic lupus erythematosus (SLE) and a matched control group of non-SLE kidney recipients. MATERIALS AND METHODS. In a case-control study, 33 patients with kidney transplantation due to end-stage renal disease caused by SLE were matched to a control group consisted of 33 non-SLE patients who had been transplanted during the same period of time in our center. The clinical characteristics, complications, and patient and graft survival were compared between the two groups. RESULTS. In each group, 12 patients (36.4%) received a kidney from a deceased donor, 15 (45.4%) from a living unrelated donor, and 6 (18.2%) from a living related donor. There was no significant difference between the outcome in SLE patients and duration of dialysis before transplantation. The mean duration of hospital stay was 23.4 ± 18.1 days in the SLE group, while it was 13.0 ± 7.3 days in the controls (P = .006). One-year graft survival was 79.0% in patients with SLE and 90.9% in non-SLE patients (P = .17). One-year patient survival was 93.9% in patients with SLE versus 81.8% in the controls (P = .26). Nine patients in the SLE group versus 11 patients in the control group developed posttransplant complications (P = .59). CONCLUSIONS. Although hospital stay after transplantation was longer in the SLE kidney recipients than controls, safety of kidney transplantation was comparable. Graft failure in the SLE patients was not significantly different between patients with different sources of kidneys.