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Background: Short bowel syndrome (SBS) is considered a low prevalence disease. In Argentina, no registries are available on chronic intestinal failure (CIF) and SBS. This project was designed as the first national registry to report adult patients with this disease. Methods: A prospective multicenter observational registry was created including adult patients with CIF/SBS from approved centers. Demographics, clinical characteristics, nutrition assessment, home parenteral nutrition (HPN) management, surgeries performed, medical treatment, overall survival, and freedom from HPN survival were analyzed. Results: Of the 61 enrolled patients, 56 with available follow‐up data were analyzed. At enrollment, the mean intestinal length was 59.5 ± 47.3 cm; the anatomy was type 1 (n = 41), type 2 (n = 10), and type 3 (n = 5). At the end of the interim analysis, anatomy changed to type 1 in 31, type 2 in 17, and type 3 in 8 patients. The overall mean time on HPN before enrollment was 33.5 ± 56.2 months. Autologous gastrointestinal reconstruction surgery was performed before enrollment on 21 patients, and afterward on 11. Nine patients (16.1%) were weaned off HPN with standard medical nutrition treatment; 12 patients received enterohormones, and 2 of them suspended HPN; one patient was considered a transplant candidate. In 23.7 ± 14.5 months, 11 of 56 patients discontinued HPN; Kaplan‐Meier freedom from HPN survival was 28.9%. The number of cases collected represented 19.6 new adult CIF/SBS patients per year. Conclusion: The RESTORE project allowed us to know the incidence, the current medical and surgical approach for this pathology, as well as its outcome and complications at dedicated centers. [ABSTRACT FROM AUTHOR]

Background: Short-bowel syndrome remains the primary cause of intestinal failure (IF) in adult patients. We aim to report the long-term results of medical and surgical rehabilitation in a cohort of patients with type III IF (III-IF) and develop a formula to predict parenteral nutrition (PN) independency.Methods: We used a retrospective analysis of a prospective database for III-IF patients undergoing autologous gastrointestinal reconstruction surgery (AGIRS) from March 2006 to August 2018. Analyzed variables included demographic data, postsurgical intestinal length (PSIL), postsurgical anatomy, teduglutide (TED) treatment, and PN volume reduction. Univariate analysis, Cox regression, logistic regression forward stepwise models, and receiver operating characteristic (ROC) curve were done using SPSS v20.Results: AGIRS was performed in 88 patients. The most frequent anatomy at first visit was type 1. Prevailing anatomy after surgery was type 3. Eight patients started TED; 6 achieved freedom from PN. At a mean follow-up time of 1606.1 ± 1190.25 days, freedom from PN survival was achieved in 83%. Variables identified at the logistic regression analysis led to a novel formula to predict intestinal rehabilitation, including PSIL, presence of ileocecal valve, and use of TED as part of postsurgical treatment.Conclusions: AGIRS in this group of patients enabled intestinal length increase and also intestinal anatomy conversion into a more favorable type for intestinal rehabilitation. TED treatment was useful to discontinue PN in patients with classical negative anatomical predictors. The novel predicting formula has an ROC area under the curve = 0.82. Further studies are necessary to validate this formula. [ABSTRACT FROM AUTHOR]

Portal vein malformations might occur during the embryonic period, as a consequence of abnormal remodeling of vitelline veins during embryonic life. Patients suffering from biliary atresia are particularly prone to have vascular malformations; although being the most frequent indication for liver transplantation in the pediatric age, portal vein duplication has not been so far associated with biliary atresia, and to the best of our knowledge, there is no‐written evidence describing how to manage it when it is first diagnosed while performing a pediatric liver transplant. Therefore, we present a recent case from our group, describing the intraoperative diagnosis of a double portal system in a patient with biliary atresia and failed Kasai. We aim to describe its surgical management, understanding that it is a real challenge to find them unexpectedly during the surgical procedure in the setting of cirrhosis and portal hypertension, particularly in small patients; therefore, by reporting this case, we aim to make readers aware about the chance of finding it, and how to managed it, to include this approach as part of the surgical armamentarium. [ABSTRACT FROM AUTHOR]

Pancreatic intraductal papillary-mucinous neoplasm is a rare primary neoplasm of unknown pathogenesis. This kind of tumor represents 0.2–2.7% of all pancreatic cancers and they may proceed to malignant lesions. In this study, we describe a case of pancreatic intraductal papillary-mucinous tumor (4.3 cm) with normal tumoral markers and nuclear atypia. We perform also a systematic review of the literature on MEDLINE and find only one relevant study that used microwave ablation for the palliative treatment of pancreatic tumor. We describe the case of a 70-year-old Caucasian male who was diagnosed with a pancreatic tumor with biliary tree dilatation. The patient underwent computed tomography (CT), percutaneous biopsy, and an endoscopic positioning of prosthesis in the biliary tree. Due to the worsening of jaundice and cholestasis, and considering the severe systemic disease status, palliative surgery with microwave thermoablation in the head of pancreas was performed. No complications were observed. The hospitalization lasted for 11 days after surgery, with normal liver and pancreatic lab tests at discharge. The patient followed a line of chemotherapy for 6 months with a complete response for 8 months. One month after the treatment, a staging CT scan was performed showing the size of the cephalopancreatic lesion had decreased from 43 to 35 mm with signs of complete ablation. The patient had a total response at the imaging of 10 months. One year later, a CT scan follow-up showed progression of the pancreatic disease. The disease remained stable for 18 months. The patient died due to cardiovascular complications with an overall survival of 30 months. Microwave ablation in our case report has been demonstrated to be feasible and safe without complications. It can be used as a phase of multimodality treatment in patients with severe systemic disease status and advanced intraductal papillary-mucinous neoplasm. [ABSTRACT FROM AUTHOR]

Abstract: Collateral circulation secondary to liver cirrhosis may cause the development of large PSSs that may steal flow from the main portal circulation. It is important to identify these shunts prior to, or during the transplant surgery because they might cause an insufficient portal flow to the implanted graft. There are few reports of “steal flow syndrome” cases in pediatrics, even in biliary atresia patients that may have portal hypoplasia as an associated malformation. We present a 12‐month‐old female who received an uneventful LDLT from her mother, and the GRWR was 4.8. During the early post‐operative period, she became hemodynamically unstable, developed ascites, and altered LFT. The post‐operative ultrasound identified reversed portal flow, finding a non‐anatomical PSS. A 3D CT scan confirmed the presence of a mesocaval shunt through the territory of the right gonadal vein, draining into the right iliac vein, with no portal inflow into the liver. The patient was re‐operated, and the shunt was ligated. An intraoperative Doppler ultrasound showed adequate portal inflow after the procedure; the patient evolved satisfactorily and was discharged home on day number 49. The aim was to report a case of post‐operative steal syndrome in a pediatric recipient due to a mesocaval shunt not diagnosed during the pretransplant evaluation. [ABSTRACT FROM AUTHOR]

The 2015 meeting of the Intestinal Transplant Association was held in Buenos Aires, Argentina. This was the 14th International Small Bowel Transplant Symposium, and it was the first meeting organized as a joint venture of the Transplantation Society, the Intestinal Transplant Association, and the Argentinean Transplant Society (Sociedad Argentina de Trasplantes). Innovative aspects of the classic meeting format included workshops sessions, debates, and multicenter studies. This report highlights the most prominent scientific contributions and results of the first such symposium in a Latin American country. [ABSTRACT FROM AUTHOR]

Background and Aims Acute cellular rejection ( ACR) and infections are leading causes of graft loss and death in intestinal transplant patients. Our aim was to evaluate the impact of maintenance immunosuppressive therapies on the expression of pro-inflammatory mediators in small bowel at ACR diagnosis. Materials and Methods We analyzed expression levels of Th1-associated genes, IFNG, CXCL10, and CXCL11 by qPCR in 46 selected graft biopsies unequivocally assigned to mild ACR (n = 14) or normal histopathology and clinical condition (n = 32) from 15 patients receiving two different immunosuppressive (IS) schemes. Double treatment: corticosteroids and tacrolimus (n = 17) and triple treatment: sirolimus or mycophenolate mofetil in addition to the basal therapy (n = 29). Results IFNG, CXCL10, and CXCL11 were induced during rejection (p < 0.05; p < 0.005, and p < 0.05, respectively). However, when rejection and control groups were classified according to immunosuppressive treatment, in the rejection group, significant differences of IFNG, CXCL10, and CXCL11 expression (p < 0.001; p < 0.005, and 0.01, respectively) were detected, whereas no differences were observed in the control group. Conclusion Gene expression of Th1 response mediators is higher during ACR. Triple IS group showed significantly lower expression of pro-inflammatory Th1 mediators during mild ACR indicating that use of these markers to monitor rejection can be affected by the IS treatment used. [ABSTRACT FROM AUTHOR]

Intestinal transplantation has evolved from being considered an experimental procedure into a clinically accepted therapy for patients with intestinal failure and parenteral nutrition life-threatening complications. Early referral, advances in immunosuppression therapy, standardization of surgical techniques, prophylactic therapy of infections, early diagnosis of rejection, and better posttransplant patient management are some of the changes that have allowed more patients to receive transplants, thus recovering intestinal sufficiency, and at the same time allowing the procedure to spread worldwide. Over the last 2 decades, transplant centers have focused on improving short-term patient survival, which has consequently increased by >20%. It is now clear that even though isolated intestinal-transplant recipients have lower mortality risk on the waiting list, they are at higher risk for long-term graft loss. Mortality is higher on the waiting list and early posttransplant in recipients whose intestinal transplants are associated with liver grafts; however, they have better long-term patient and graft survival. Nevertheless, 3-year actuarial patient survival has not changed over the same period of time, and therefore this is our challenge for the next decade. Mt Sinai J Med 79:246-255, 2012. © 2012 Mount Sinai School of Medicine [ABSTRACT FROM AUTHOR]

Objectives: The human caliciviruses, which include Norwalk-like viruses (or Noroviruses) and Sapporo viruses, commonly cause epidemic and endemic viral gastroenteritis of short duration in healthy individuals. However, the impact of human calicivirus in immunosuppressed populations has not been established. The authors report five pediatric patients who developed human calicivirus enteritis after intestinal transplantation.Methods: Infection was documented with repetitive reverse transcription polymerase chain reaction testing with nucleotide sequencing of tissue and lumen fluid specimens.Results: A single strain, type Miami Beach, affected all patients in the hospital with an apparent index case. A potential mode of transmission was not defined. Severe osmotic or secretory diarrhea necessitated intravenous fluid therapy for 40 days or more in three of the five infants. Concurrent or recent subclinical allograft infection with adenovirus in two patients was associated with more severe symptoms. Virus excretion exceeded 80 days in two patients. Differentiation of human calicivirus enteritis from allograft rejection was difficult, as both disorders were associated with increased enterocyte apoptosis and inflammation. Intensification of immunosuppressive therapy because of suspected rejection appeared to prolong symptoms.Conclusion: These findings demonstrate that human calicivirus can be a significant pathogen in intestinal transplant recipients and potentially in other immunocompromised patients. [ABSTRACT FROM AUTHOR]

Paramesh AS, Roayaie S, Doan Y, Schwartz ME, Emre S, Fishbein T, Florman S, Gondolesi GE, Krieger N, Ames S, Bromberg JS, Akalin E. Post-liver transplant acute renal failure: factors predicting development of end-stage renal disease. Clin Transplant 2004: 18: 94–99. © Blackwell Munksgaard, 2004 Acute renal failure (ARF) occurs in 5–50% ofpatients undergoing orthotopic liver transplantation (OLT). The aim of this study was to determine factors that might predict the development of end stage renal disease (ESRD) in patients who had ARF after OLT. We studied all OLT recipients between 9/1/1988 through 12/31/2000. A total of 1602 patients underwent OLT during the study period. About 350 patients (22%) developed ARF requiring dialysis post-operatively. One hundred and twenty-three (39.8%) died within a year after OLT. Median follow up was 5.8 yr (range 1–12 yr). Forty-three patients (23%) developed ESRD over median of 3.79 yr (range 1–8 yr). Multivariate logistic regression analysis revealed creatinine levels >1.7 mg/dL at 1 yr (p < 0.001), cyclosporine as immunosuppression (p = 0.026), and the presence of diabetes pre-OLT (p < 0.001) to be associated with the development of ESRD. The development of ESRD did not decrease patient survival (p = 0.111). ESRD patients who received subsequent kidney transplantation had significantly improved survival rates (p = 0.005). Serum creatinine levels at 1 yr, cyclosporine as immunosuppression, and the presence of diabetes pre-OLT are independent predictive factors for the development of ESRD. ESRD patients who received kidney transplantation had higher10-yr survival rates when compared with patients maintained on dialysis. [ABSTRACT FROM AUTHOR]

Abstract: The hepatopulmonary syndrome has been described in as many as 5–29% of patients with liver disease. Patients with this syndrome may suffer from chronic hypoxemia, and mortality rates of liver patients with this syndrome are as high as 41%. Early diagnosis of such patients is essential. Currently, liver transplantation is the only effective therapy for such patients, and reversal of this syndrome is seen in up to 80% of patients post-transplant. Transjugular intrahepatic portasystemic shunting (TIPS) as a therapeutic maneuver for this syndrome has been described in five patients to date with mixed results. Reduction in portal hypertension with consequent redistribution of blood flow and altered synthesis of vasodilatory chemicals have been postulated to help resolve this disease. In this report, we describe an 11-yr-old female with biliary atresia and hepatopulmonary syndrome. Her disease was complicated with recurrent variceal bleeding. TIPS achieved a therapeutic response of both her bleeding and respiratory complications. [ABSTRACT FROM AUTHOR]

An end-to-side portocaval shunt has been classically described as the standard procedure that needs to be performed to obtain adequate venous drainage of the foregut in combined liver–intestinal transplant. The transplanted organs are placed en bloc in a piggyback fashion on the recipient's vena cava, and graft's porto-mesenteric venous circulation is left intact. Recurrent portal hypertension with hemorrhage has been reported as a complication after such a procedure. In an effort to improve the technical aspects of this complex operation, we began to perform an end-to-end shunt from the recipient portal vein to the infrahepatic donor cava. Here, we describe this new surgical technique and discuss its potential advantages. The new technique is described in detail. Of the 70 intestinal transplants performed in 67 patients at The Mount Sinai Hospital from 1998 to December 2004, three were multivisceral transplants (MVTx) and 28 were combined liver intestine transplants (LITx), post-surgical outcome including recurrent portal hypertension and the presence of splenomegaly and significant thrombocytopenia was retrospectively review between the group that received the classic end–side vs. the new end–end portocaval shunt. Of the 28 LITx, 23 were done with the classic end–side portocaval shunt and in five (one adult, four pediatric) the end-to-end portocaval shunt described in the present study. One patient in this group died on post-operative day 31 (adult); after a mean follow up of 19.5 months there is a 100% patient and graft survival for the pediatric population. Post-operatively there have been no complications related to this technique. Few modifications have been made to the original description of MVTx. This is the first description of this technique that is relatively simple to perform, creating a large vascular conduit with high flow rates and theoretically less turbulence compared with the end-to-side shunt. [ABSTRACT FROM AUTHOR]

The author discusses the study which describes the post liver transplantation experience of pediatric patients using biological mesh for delayed abdominal closure. The author agrees to the study that a tension-free primary abdominal wall closure should be performed whenever feasible. He believes that biological mesh provides simplicity and resistance to infection.