Late Onset Neuromyelitis Optica Spectrum Disorders (LONMOSD) from a Nationwide Portuguese Study: Anti-AQP4 Positive, Anti-MOG Positive and Seronegative Subgroups

Introduction: Several neuroimmunological disorders have distinct phenotypes according to the age of onset, as in multiple sclerosis or myasthenia gravis. It is also described that late onset NMOSD (LONMOSD) has a different phenotype. Objective: To describe the clinical/demographic characteristics of the LONMOSD and distinguish them from those with early onset (EONMOSD). Methods: From a nationwide Portuguese NMOSD study we analyzed the clinical/demographic characteristics of the LONMOSD. Results: From the 180 Portuguese patients 45 had disease onset after 50 years old, 80% were female. 23 had anti-AQP4 antibodies (51.1%), 13 anti-MOG antibodies (28.9%) and 9 were double seronegative (20.0%). The most common presenting phenotypes in LONMOSD were transverse myelitis (53.3%) and optic neuritis (26.7%), without difference from EONMOSD (p = 0.074). The mean EDSS for LONMOSD was 6.0 (SD=2.8), after a mean follow-up time of 4.58 (SD=4.47) years, which was significantly greater than the mean EDSS of EONMOSD (3.25, SD=1.80)(p = 0.022). Anti-AQP4 antibodies positive LONMOSD patients had increased disability compared to anti-MOG antibodies positive LONMOSD (p = 0.022). The survival analysis showed a reduced time to use a cane for LONMOSD, irrespective of serostatus (p<0.001). Conclusions: LONMOSD has increased disability and faster progression, despite no differences in the presenting clinical phenotype were seen in our cohort.info:eu-repo/semantics/publishedVersio

Neuromyelitis Optica Spectrum Disorders: a Nationwide Portuguese Clinical Epidemiological Study

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is a rare disorder in which astrocyte damage and/or demyelination often cause severe neurological deficits. Objective: To identify Portuguese patients with NMOSD and assess their epidemiological/clinical characteristics. Methods: This was a nationwide multicenter study. Twenty-four Portuguese adult and 3 neuropediatric centers following NMOSD patients were included. Results: A total of 180 patients met the 2015 Wingerchuk NMOSD criteria, 77 were AQP4-antibody positive (Abs+), 67 MOG-Abs+, and 36 seronegative. Point prevalence on December 31, 2018 was 1.71/100,000 for NMOSD, 0.71/100,000 for AQP4-Abs+, 0.65/100,000 for MOG-Abs+, and 0.35/100,000 for seronegative NMOSD. A total of 44 new NMOSD cases were identified during the two-year study period (11 AQP4-Abs+, 27 MOG-Abs+, and 6 seronegative). The annual incidence rate in that period was 0.21/100,000 person-years for NMOSD, 0.05/100,000 for AQP4-Abs+, 0.13/100,000 for MOG-Abs+, and 0.03/100,000 for seronegative NMOSD. AQP4-Abs+ predominated in females and was associated with autoimmune disorders. Frequently presented with myelitis. Area postrema syndrome was exclusive of this subtype, and associated with higher morbidity/mortality than other forms of NMOSD. MOG-Ab+ more often presented with optic neuritis, required less immunosuppression, and had better outcome. Conclusion: Epidemiological/clinical NMOSD profiles in the Portuguese population are similar to other European countries.info:eu-repo/semantics/publishedVersio

Recurrence of primary sclerosing cholangitis after liver transplantation – analysing the European Liver Transplant Registry and beyond

Liver transplantation for primary sclerosing cholangitis (PSC) can be complicated by recurrence of PSC (rPSC). This may compromise graft survival but the effect on patient survival is less clear. We investigated the effect of post-transplant rPSC on graft and patient survival in a large European cohort. Registry data from the European Liver Transplant Registry regarding all first transplants for PSC between 1980 and 2015 were supplemented with detailed data on rPSC from 48 out of 138 contributing transplant centres, involving 1,549 patients. Bayesian proportional hazards models were used to investigate the impact of rPSC and other covariates on patient and graft survival. Recurrence of PSC was diagnosed in 259 patients (16.7%) after a median follow-up of 5.0 years (quantile 2.5%-97.5%: 0.4–18.5), with a significant negative impact on both graft (HR 6.7; 95% CI 4.9–9.1) and patient survival (HR 2.3; 95% CI 1.5–3.3). Patients with rPSC underwent significantly more re-transplants than those without rPSC (OR 3.6, 95% CI 2.7–4.8). PSC recurrence has a negative impact on both graft and patient survival, independent of transplant-related covariates. Recurrence of PSC leads to higher number of re-transplantations and a 33% decrease in 10-year graft survival

Trattamento delle lesioni iatrogene maggioridella via biliare

The appropriate treatment of major bile duct injuries is mandatory in order to avoid serious complica- tions, such as bile peritonitis or secondary biliary liver cirrhosis. In the last fourthy years, surgical, endoscopic or radiologic techniques of cure have been proposed, but in our opinions, the preferred option is given by Roux-en-Y choledochojejunostomy or hepaticojejunostomy. Creating an anastomosis on narrow bile duct could be difficult; in the- se really rare cases, the jejunal loop could be secured by a second suture to the hilar plate with satisfactory long-term results

Perforazione della vena porta in corso di pancreatite emorragica.

La pancreatite acuta rappresenta una malattia che si manifesta con un quadro clinico ed istopatologico ampiamente variabile. Nel 20% dei casi, assume una gravita tale da mettere seriamente a rischio la vita dei pazienti principalmente per le complicanze della malattia, precoci (ipotensione grave, sconvolgimento metabolico, sequestro di liquidi) e tardive (sepsi, deficit multiplo d'organo). Il caso riportato è quello di un uomo che giunge alla nostra osservazione con un quadro di pancreatit

Successful Transplant of a Nonagenarian Liver Graft With Fully Replaced Right Hepatic Artery Reconstruction.

Organ shortage and increasing donor age in liver transplant are stimulating transplant centers to accept otherwise discarded grafts due to donor age or vascular abnormalities; nevertheless, the use of nonagenarian donor grafts is uncommon because advanced age is associated with a higher risk of ischemic-type biliary lesions and worse long-term graft survival. We herein report the case of a 90-year-old donor with fully replaced right hepatic artery. After back-table vascular assessment, the donor right hepatic artery was anastomosed end-to-end with the gastroduodenal artery with 2 polypropylene 8/0 running sutures. Even if the back-table reconstruction of a replaced right hepatic artery is not associated with an enhanced risk of posttransplant vascular complications, vascular abnormalities might discourage the use of otherwise acceptable elderly grafts. The present case underscores that elderly liver grafts should not be discarded per se even in the presence of vascular variants

Incidental intra-ductal papillary mucinous neoplasms (IPMNs) of the pancreas in deceased organ donors: are such donors unfit for donation?

Use of extended criteria donors (ECD) may lead to increased transmission of donor-derived malignancies. Intra-ductal papillary mucinous neoplasms (IPMNs) of the pancreas are being recognised more frequently due to a higher threshold of awareness and improved diagnosis, but use of deceased donors (DD) with incidental IPMNs may be questionable. Based on the literature, we sought to define an algorithm for use of DD with incidental IPMNs. Four subtypes of IPMNs have been identified: gastric, intestinal, pancreatobiliary and oncocytic. The gastric type consists of small cystic lesions in peripheral branch ducts with low grade atypia, while the other subtypes presenta s larger lesions involving the main duct and connecting branch ducts with marked atypia. These latter types may yeald carcinoma in situ to invasive adenocarcinoma. The progression to carcinoma occurs quickly once moderate dysplasia is found. Approximately 30% of resected IPMNs may harbor synchronous or metachronous tumors in the stomach, colon and rectum. Once an incidental IPMN is discovered, it is imperative to retrieve the whole pancreas and proceed with gross inspection of the gland, with regard to communication of the lesion(s) with the main duct, the presence of mucin, site of the cysts, and size of the involved ducts. Sample coloration with EE allows for identification of IPMN subtypes. Such procedures will help allocate IPMNs to low risk (<3 cm; branch-duct; no main duct dilatation; no mural nodules; gastric type; low dysplasia) or high risk (≥3 cm; main duct(s) involved; other than gastric; other than low dysplasia). Incidental IPMNs in DDs require careful evaluation of potential benefits and risks. Smaller lesions (<3 cm) of the branch ducts, with low dysplasia and of gastric type might be considered, provided careful evaluation of abdominal organs and patient’s consent. A strict follow-up is needed to assess risk in the long term

Implementing a robotic liver resection program does not always require prior laparoscopic experience

Background: Preliminary experience in laparoscopic liver surgery is usually suggested prior to implementation of a robotic liver resection program. Methods: This was a retrospective cohort analysis of patients undergoing robotic (RLR) versus laparoscopic liver resection (LLR) for hepatocellular carcinoma at a center with concomitant initiation of robotic and laparoscopic programs Results: A total of 92 consecutive patients operated on between May 2014 and February 2019 were included: 40 RLR versus 52 LLR. Median age (69 vs. 67; p = 0.74), male sex (62.5% vs. 59.6%; p = 0.96), incidence of chronic liver disease (97.5% vs.98.1%; p = 0.85), median model for end-stage liver disease (MELD) score (8 vs. 9; p = 0.92), and median largest nodule size (22 vs. 24&nbsp;mm) were similar between RLR and LLR. In the LLR group, there was a numerically higher incidence of nodules located in segment 4 (20.0% vs. 16.6%; p = 0.79); a numerically higher use of Pringle’s maneuver (32.7% vs. 20%; p = 0.23), and a shorter duration of surgery (median of 165.5 vs. 217.5&nbsp;min; p = 0.04). Incidence of complications (25% vs.32.7%; p = 0.49), blood transfusions (2.5% vs.9.6%; p = 0.21), and median length of stay (6 vs. 5; p = 0.54) were similar between RLR and LLR. The overall (OS) and recurrence-free (RFS) survival rates at 1 and 5&nbsp;years were 100 and 79 and 95 and 26% for RLR versus 96.2 and 76.9 and 84.6 and 26.9% for LLR (log-rank p = 0.65 for OS and 0.72 for RFS). Conclusions: Based on our results, concurrent implementation of a robotic and laparoscopic liver resection program appears feasible and safe, and is associated with similar oncologic long-term outcomes

Use of liver grafts ≥ 80 years. The lessons learned after over 150 transplants

Background: We have previously demonstrated that elderly (≥ 80 years) grafts may provide favorable long-term results after liver transplantation (LT). However, they are associated with an increased risk for ischemic-type biliary lesions (ITBL) and hepatitis C virus (HCV) recurrence after compared to standard donor grafts. Methods: This was a retrospective, case-control analysis on use of elderly liver grafts (≥ 80 years) for LT at a single institution. From January 2003 thru June 2014, 154 LT were performed with deceased donors ≥ 80 years vs. 131 with donors 18–39 years. Patients were matched on a 1:1 basis as per indication to LT, model for end-stage liver disease (MELD) score at transplantation, cold (CIT) and warm ischemia time (WIT). Finally,a total of 106 recipients of grafts ≥ 80 years (Group A) were compared against 106r recipients of standard donor grafts (18–39 years, Group B). The primary end-point was graft and patient survival rate between the groups. The secondary end-point was assessment of ITBL and HCV-related graft loss. Graft survival was censored at time of re-listing or re-transplantation. Patient survival was censored at time of death, lost to follow-up or as of December 2014. Survival rates were according to Kaplan-Meier and the level of statistical significance was set at 5%. Results: In Group A vs. Group B, HCV-related graft loss was 20/106 (18.9%) vs. 6/106 (5.7%) (p = 0.0059; OR = 3.87); incidence of ITBL was 16/106 (15.1%) vs. 6/106 (5.7%) (p = 0.040; OR = 2.96),and incidence of ITBL-related graft loss was 5/106 (4.7%) vs. 0 (0%) (p = 0.059). ITBL-related patient death was 3/106 (2.8%) vs.0 (0%) (p = 0.246) in Group A and B, respectively. Graft survival was 90.9%, 84.9%, and 75.8% at 1, 3 and 5 years in Group A vs. 92.5%, 88.4%, and 87.2% in Group B (p = 0.03,log rank). Conclusions: Although associated with a 5-year graft survival rate of 75.8%, liver donor grafts ≥ 80 years have a 4-fold increased odds for HCV-related graft failure and a 3-fold increased odds for ITBL, respectively. Novel antiviral treatments and continued management of ITBL will likely contribute to further improvements of these results, reducing the gap between elderly and standard donor grafts