Colon cancer in ulcerative colitis patients with liver transplant (UCCOLT) due to primary sclerosing cholangitis carries significant treatment challenges. Aim of this literature search is to review ...management strategies and provide a framework to facilitate the decisional process in this clinical setting.
PRISMA-compliant systematic search was followed by critical expert commentary of the results and development of a surgical management algorithm. Endpoints included surgical management, operative strategies, functional and survival outcomes. Technical and strategics aspects with particular regard to the choice of reconstruction were evaluated to tentatively develop an integrated algorithm.
Ten studies reporting treatment of 20 UCCOLT patients were identified after screening. Nine patients underwent proctocolectomy and end-ileostomy (PC) and eleven had restorative ileal pouch-anal anastomosis (IPAA). Reported results for perioperative outcomes, oncological outcomes, and graft loss were comparable for both procedures. There were no reports of subtotal colectomies and ileo-rectal anastomosis (IRA).
Literature in the field is scarce and decision-making is particularly complex. PC and IPAA have been reported with good results. Nevertheless, IRA may also be considered in UCCOLT patients in selected cases, reducing the risks of sepsis, OLT and pouch failure; furthermore, in young patients, it has the advantage of preserving fertility or sexual function. The proposed treatment algorithm may represent a valuable support in guiding surgical strategy.
Towards tolerance in liver transplantation Toti, L.; Manzia, T.M.; Sensi, B. ...
Baillière's best practice & research. Clinical gastroenterology,
October-December 2021, 2021-10-00, 20211001, Letnik:
54-55
Journal Article
Recenzirano
Life-long immunosuppression has always been considered the key in managing liver graft protection from recipient rejection. However, it is associated with severe adverse effects that lead to ...increased morbidity and mortality, including infections, cardiovascular diseases, kidney failure, metabolic disorders and de novo malignancies.
This explains the great interest that has developed in the concept of tolerance in recent years. The liver, thanks to its marked tolerogenicity, is to be considered a privileged organ: up to 60% of selected patients undergoing liver transplantation could safely withdraw immunosuppression.
•Long-term survival in liver transplant patients is still low: the side effects of immunosuppression are the one main reason for this deficiency.•The liver is immunologically privileged over other organs: for this reason, many studies have been carried out in recent decades to understand the best way to achieve complete tolerance.•Withdrawal of immunosuppression can be safely achieved in up to 40% of selected liver transplant recipients.•There is a need to define valid and safe biomarkers able to predetermine tolerance.
Steel ladle refractory lining plays an important role in steelmaking refinement as its thermal properties are strictly related to the ladle ability to keep the molten steel temperature constant. This ...work developed a transient numerical analysis, considering temperature dependent refractory properties. A holistic view of the process was proposed to help the refractory design of steel ladles, according to their saving energy capability and overall costs. The numerical description of the ladle cycle used the finite element method (FEM) via commercial software (Abaqus). The average heat flux in the hot face while holding the liquid steel determined the steel ladle energy losses and was compared to evaluate the lining efficiency. The investigations considered the application of distinct working refractories and the presence of an insulating layer. The developed thermal and energy balance tool provided a more inclusive and integrated approach for investigating the energy consumption and thermal history of the steel ladle process, regarding the refractory lining design.
Cirrhosis for biliary atresia (BA) is associated with risk of gastrointestinal bleeding (GB) from gastroesophageal varices due to portal hypertension. Primary prophylaxis of GB is controversial in ...children who are candidates for liver transplantation (LT). The aim of the study was to define the management of gastroesophageal varices and to identify the benefit of primary prophylaxis for GB in BA children waiting for LT.
A retrospective single-center study including all BA children listed for LT in 2008–2016. Clinical, endoscopical, and biochemical data were analyzed.
Of 82 children, 50 (61%) did not receive primary prophylaxis and did not present any episode of bleeding, 16 (19.5%) underwent primary prophylaxis, and 16 (19.5%) presented spontaneous GB and received secondary prophylaxis. Children without primary prophylaxis and GB were younger than patients with primary prophylaxis and those with GB (7.7 years range, 4.1–37.9 years vs 11.2 years range, 5.1–43 years; P = .03 vs 10.7 years range, 6.9–39.9 years, respectively; P = .004). Seventy-five percent of GB occurred in children older than 8 months. Fifteen (93.8%) children with GB presented esophageal varices (grade III = 10 62.5%) and 10 (62.5%) required endoscopic treatments, consisting mainly of sclerotherapy. Median time to LT was similar for children with or without bleeding (2 months range, 0–17.7 months vs 2.2 months 0–17.9 months, respectively; P = .89). After 45.5 months (range, 13.7–105.5 months) of follow-up, the overall patient survival was 97.6%. At the intention-to-treat analysis, the survival rate was 100% for patients without bleeding episode and 87.5% for children with GB (P = .16).
Despite the risk of GB being not clinically predictable in children with BA waiting for LT, our experience suggests that primary prophylaxis of GB might be unnecessary in children younger than 6 months, while it should be considered in older children. Thus, the occurrence of GB does not delay the timing of transplantation.
•Risk of gastrointestinal bleeding is not clinically predictable in biliary atresia.•Esophageal varices screening in children younger than 6 months who are waiting for liver transplant is unnecessary.•Primary prophylaxis should be always considered in selected children older than 6 months.•Occurrence of gastrointestinal bleeding does not delay the time of transplantation.
Fabry's disease (FD) is a genetic disorder of lysosomal storage characterized by the intralysosomal accumulation of globotriaosylceramide (Gb3). This genetic mutation causes a total or partial ...deficit of the α-galactosidase (GAL) enzyme activity. FD has an incidence of 1:40000-60000 born alive. Its prevalence is higher in specific pathological conditions like chronic kidney disease (CKD). The aim of this study was to evaluate the FD prevalence in Italian renal replacement therapy (RRT) patients from Lazio region.
485 patients in RRT (hemodialysis, peritoneal dialysis, and kidney transplantation) were recruited. The screening test was performed on venous blood sample. The latter was analyzed using specific FD diagnostic kit, based on the analysis of dried blood spots on filter paper.
We found 3 cases of positivity to FD (1 female and 2 males). In addition, 1 male patient was identified with biochemical alteration indicative of GAL enzyme deficiency with a genetic variant of the GLA gene of unknown clinical significance. The FD prevalence in our population was 0.60% (1 case out 163), it rises to 0.80% (1 case out of 122) if the genetic variant of unknown clinical significance is considered. Comparing the three subpopulations, we observed a statistically significant difference in GAL activity in transplanted patients compared to dialysis patients (p<0.001).
Considering the presence of an enzyme replacement therapy able to modify FD clinical history, it is essential to try to implement FD early diagnoses. However, the screening is too expensive to be extended on large scale, due to the low prevalence of the pathology. The screening should be performed on high-risk populations.
The aim of the present work is to illustrate and discuss an application of rheo-NMR techniques in the investigation of the flow micro-morphology of a rheo-thinning fluid. The viscoelastic material is ...composed by weakly hydrated nonionic Wormlike Micelles (WM), stabilized by the biocompatible phospholipid in an organic solvent (lecithin organogel). By applying rheo-NMR techniques, such as micro-imaging and flow velocimetry in Couette flow, to lecithin organogels in the concentrated isotropic phase, a new phase nucleating inhomogeneously at the inner rotating cylinder showing periodic fluctuations in space in some cases, has been identified for applied shear rates within the isotropic-nematic stress plateau. On the other hand, evident slippage phenomena have been found in flow regimes consistent with a full shear-induced nematic state. Bulk rheometric investigations executed in oscillatory, steady state and transient mode have been finally carried out to bridge different flow micro-heterogeneities detected by rheo-NMR with a variety of mechanical responses manifested by lecithin WM.
Abstract Glycogen storage diseases (GSD) are inherited metabolic disorders of glycogen metabolism due to intracellular enzyme deficiency resulting in abnormal storage of glycogen in tissues. GSD ...represents an indication for liver transplantation (OLT) when medical treatment fails to control the metabolic dysfunction and/or there is an high risk of malignant transformation of hepatocellular adenomas (HCA). Herein we have reported two cases of GSD, type Ia and type VI, which were both associated with rapidly growing HCA, and underwent OLT because of suspect changes in their radiological features. Final histological findings in the explanted liver showed the presence of hepatocellular carcinoma (HCC) in both cases. In GSD type Ia and VI, OLT is considered to be the treatment of choice when a liver neoplasm is suspected. While the association of HCC with GSD type Ia is well known, this is the first case of HCC in GSD type VI so far reported to the best of our knowledge.
We designed a retrospective case-control study to determine the efficacy and feasibility of everolimus (EVR) combined with low-dose tacrolimus (Tac) ab initio versus standard-dose Tac after liver ...transplantation (LT).
Seventy-one adult LT patients, receiving EVR and low-dose Tac without corticosteroids or induction therapy from postoperative day 1 (EVR group) were compared with a well-matched control group of 61 recipients treated with standard-dose Tac in association with antimetabolite.
Baseline characteristics for the two groups were comparable. The overall patient and graft survival rates were similar (P = .908). Liver function was stable during the follow-up. In the EVR group, biopsy-proven acute rejection occurred in two cases (2.8%), whereas chronic rejection occurred in one (1.4%). The EVR group experienced a better renal function already after 2 weeks (estimated glomerular filtration rate: 89.85 36.46 to 115.3 mL/min/1.73 m2 vs. 68.77 16.11 to 115.42 mL/min/1.73 m2; P = .013), which was also observed after a median time of 27 months (range, 0 to 82 months) from LT (estimated glomerular filtration rate: 80 45 to 118.3 mL/min/1.73 m2 vs. 70.9 45 to 88.4 mL/min/1.73 m2; P = .04). After a median time of 27 months, the EVR group showed lower incidence of arterial hypertension and insulin-dependent diabetes mellitus.
Ab initio EVR-based immunosuppression could be a valid option immediately after surgery in recipients at high-risk for post-LT renal impairment.
•A limited experience of everolimus ab initio regimen post–liver transplantation (LT) is reported.•An everolimus-based regimen reduces long-term calcineurin inhibitor (CNI)–side effects after LT.•Everolimus ab initio has been associated with improved renal function post-LT.•Everolimus ab initio is a valid option in recipients with risk of CNI side effects.