Porto-sinusoidal vascular disorder (PSVD): Application of new diagnostic criteria in a multicenter cohort of patients.

BACKGROUND AND AIM: The term porto-sinusoidal vascular disorder (PSVD) was recently proposed to replace that of idiopathic non-cirrhotic portal hypertension (INCPH) to describe patients with typical histological lesions in absence of cirrhosis, irrespective of the presence/absence of portal hypertension (PH), and new diagnostic criteria were defined. The study aimed to compare the applicability between the diagnostic criteria of PSVD and those of INCPH. MATERIALS AND METHODS: 53 patients affected by PSVD were enrolled. Biochemical, clinical, ultrasound and histological data, the presence and type of associated diseases were recorded in a database. According to the new criteria, histological data and signs of PH were divided into specific and non-specific. Percutaneous and transjugular biopsies were compared to establish the usability of the two methods for diagnostic purposes. RESULTS: In 85% of the patients the diagnosis of PSVD was obtained by applying the first criterion (25 had specific histological signs with specific signs of PH); one patient presented with specific histological signs but no PH. In 8 patients the diagnosis was obtained by applying the second criterion. 19% of patients had portal vein thrombosis. Finally, the prevalence of the various histological lesions was similar between the patients submitted to percutaneous and transjugular liver biopsy. CONCLUSIONS: The study confirms that the diagnostic criteria of PSVD lead to the inclusion of a greater number of patients than INCPH.

Serological response and breakthrough infection after COVID-19 vaccination in patients with cirrhosis and post-liver transplant.

BACKGROUND: Vaccine hesitancy and lack of access remain major issues in disseminating COVID-19 vaccination to liver patients globally. Factors predicting poor response to vaccination and risk of breakthrough infection are important data to target booster vaccine programs. The primary aim of the current study was to measure humoral responses to 2 doses of COVID-19 vaccine. Secondary aims included the determination of factors predicting breakthrough infection. METHODS: COVID-19 vaccination and Biomarkers in cirrhosis And post-Liver Transplantation is a prospective, multicenter, observational case-control study. Participants were recruited at 4-10 weeks following first and second vaccine doses in cirrhosis [n = 325; 94% messenger RNA (mRNA) and 6% viral vaccine], autoimmune liver disease (AILD) (n = 120; 77% mRNA and 23% viral vaccine), post-liver transplant (LT) (n = 146; 96% mRNA and 3% viral vaccine), and healthy controls (n = 51; 72% mRNA, 24% viral and 4% heterologous combination). Serological end points were measured, and data regarding breakthrough SARS-CoV-2 infection were collected. RESULTS: After adjusting by age, sex, and time of sample collection, anti-Spike IgG levels were the lowest in post-LT patients compared to cirrhosis (p < 0.0001), AILD (p < 0.0001), and control (p = 0.002). Factors predicting reduced responses included older age, Child-Turcotte-Pugh B/C, and elevated IL-6 in cirrhosis; non-mRNA vaccine in AILD; and coronary artery disease, use of mycophenolate and dysregulated B-call activating factor, and lymphotoxin-α levels in LT. Incident infection occurred in 6.6%, 10.6%, 7.4%, and 15.6% of cirrhosis, AILD, post-LT, and control, respectively. The only independent factor predicting infection in cirrhosis was low albumin level. CONCLUSIONS: LT patients present the lowest response to the SARS-CoV-2 vaccine. In cirrhosis, the reduced response is associated with older age, stage of liver disease and systemic inflammation, and breakthrough infection with low albumin level.

New Indications for TIPSs: What Do We Know So Far?

Since 1988, transjugular intrahepatic portosystemic shunt (TIPS) has been an effective therapy for portal hypertension in many settings. Thanks to continuous technical improvements and a wiser selection of patients, excellent results have been achieved with this therapeutic strategy. The historical indications for TIPS placement, in the context of liver cirrhosis, such as refractory ascites and variceal bleeding are now well established and known. However, in recent years, new indications are emerging. These have been investigated and approved in some studies but are not yet included in guidelines and clinical practice. This review aims to highlight what is new for the role of TIPS in portal vein thrombosis (especially in patients awaiting liver transplantation), in recurrent ascites and not only refractory ascites, as a neoadjuvant therapy before abdominal surgery and, finally, in the setting of noncirrhotic portal hypertension. All these new aspects are addressed in this review with a critical approach based on the literature revision and clinical practice. Future research is needed to explore and validate the new role of TIPS in these scenarios.

Diabetes and Metabolic Disorders: Their Impact on Cardiovascular Events in Liver Transplant Patients.

Cardiovascular diseases are currently one of the most important causes of morbidity and mortality in liver transplant patients over the long term. Therefore, evaluating prognostic factors for cardiovascular events (CVEs) in this population is essential for taking preventive measures. The aim of this study was to identify the impact of diabetes and other metabolic disorders on CVEs in liver transplant patients. Three hundred fifty-six liver transplant recipients who survived at least 6 months after surgery were enrolled. Patients were followed for a median time of 118 months (12-250 months). All cardiovascular events were carefully recorded and detailed in the patients' charts. Demographic data, diabetes, hypertension, dyslipidemia, weight changes, and a diagnosis of metabolic syndrome both before and after transplantation were noted to assess their possible relationship with CVE. The presence of a diagnosis of metabolic-associated fatty liver disease (MAFLD) was also evaluated. Immunosuppressive therapy was included in the analysis. Diabetes mellitus (DM), especially when present before transplantation, was strongly associated with CVEs (hazard risk HR 3.10; 95% confidence interval CI: 1.60-6.03). Metabolic syndrome was found to be associated with CVEs in univariate analysis (HR 3.24; 95% CI: 1.36-7.8), while pretransplantation and de novo MAFLD were not. Immunosuppressive therapy had no influence on predisposing transplanted patients to CVEs during follow-up. Further prospective studies may be useful in investigating the risk factors for CVEs after liver transplantation and improving the long-term survival of transplant patients.

Sarcopenia in chronic advanced liver diseases: A sex-oriented analysis of the literature.

Sarcopenia, defined as progressive and generalized loss of muscle mass and strength, is common in chronic liver disease. It significantly impacts the quality of life and increases the risk of liver-related complications and mortality in cirrhotic patients. Moreover, recent studies showed a negative impact of sarcopenia on patients awaiting liver transplantation (LT), on post-LT outcomes, and on response to hepatocellular carcinoma therapies. Data about the influence of sex on the incidence, prevalence, diagnosis and treatment of sarcopenia in chronic liver diseases are poor and conflicting. The aims of this review of the literature are to define sex differences in sarcopenic cirrhotic patients and to highlight the necessity of a sex stratified analysis in future studies. This analysis of the literature showed that most of the studies are retrospective, with a higher prevalence of sarcopenia in males, probably due to anatomical differences between the sexes. Moreover, diagnostic criteria for sarcopenia are different between studies, as there is not a defined cut-off and, as a consequence, no comparable results. In conclusion, sex seems to have an impact on sarcopenia, and future studies must accurately investigate its role in identifying and treating high-risk patients, reducing the negative impact of sarcopenia on the survival and quality of life of cirrhotic patients.

Severe rectal prolapse and solitary rectal ulcer in a young anorectic woman with myxoid degeneration of visceral adipose tissue.

Rectal prolapse is a disorder in which the rectum protrudes from the anal canal. Solitary rectal ulcer may coexist. Both conditions have been associated with chronic constipation and excessive straining during defecation. Rectal prolapse has been rarely reported in women suffering from anorexia nervosa. Lack of rectal support because of loss of ischiorectal fat has been proposed as one of the possible mechanisms in this condition, together with chronic constipation and abuse of laxative. We report the case of an anorexic woman with a severe rectal prolapse and bleeding requiring urgent Altmeier's procedure. Surgery was complicated by dehiscence of the anastomosis and volvulus, requiring ileostomy and laciniae debridement. Pathological analysis of all the surgical samples taken from different abdominal sites highlighted changes in the visceral adipose tissue consisting in nodular aggregates of small adipocytes dispersed in a myxoid matrix surrounding blood vessels within abundant fibrosis. The morphologic features resemble those observed in primordial fetal fat and are comparable to those observed in cancer associated cachexia. The diffuse myxoid degeneration of visceral adipose tissue may play a role in the pathogenesis of rectal prolapse in patients with anorexia nervosa. Besides starvation, the mechanism sustaining myxoid degeneration of the adipose tissue is not entirely clear. Whenever possible improving nutritional and clinical conditions should be ideal before any surgical approach.

Microkeratome-assisted superficial anterior lamellar keratoplasty for anterior stromal corneal opacities after penetrating keratoplasty.

PURPOSE: To describe the surgical technique and report the outcomes of patients treated with microkeratome-assisted superficial anterior lamellar keratoplasty for anterior stromal corneal opacities developing after penetrating keratoplasty (PK). METHODS: All patients with post-penetrating keratoplasty anterior stromal opacities treated with microkeratome-assisted superficial anterior lamellar keratoplasty between July 2005 and June 2007 were reviewed. A 130-µm superficial keratectomy was performed, followed by the placement of an appropriately sized donor graft, which was secured with overlay sutures. Refraction, corneal topography, and uncorrected and best-corrected visual acuities (UCVA, BCVA, respectively) were noted at each examination. RESULTS: Nine eyes of 8 consecutive patients were identified. Causes of anterior stromal opacities included dystrophy recurrence (n = 3), post-photorefractive keratectomy haze (n = 2), and scarring after stromal melt (n = 4). BCVA improved in all 9 eyes at final follow-up, and 7 of 9 eyes achieved ≥20/40 within the first month. Average follow-up period was 28 ± 3.9 months. Refractive astigmatism also improved by an average of 0.7 diopters. CONCLUSIONS: Superficial anterior lamellar keratoplasty is a viable and effective alternative to repeat PK in treating anterior stromal scars. It avoids open-globe surgery and exposure to endothelial rejection associated with repeat PK, and visual rehabilitation is considerably quicker.

Intraocular lens exchange 1 week after descemet stripping automated endothelial keratoplasty.

PURPOSE: To report a case of intraocular lens (IOL) exchange performed 1 week after Descemet stripping automated endothelial keratoplasty (DSAEK). METHODS: Describe the clinical findings and outcome of IOL exchange in an 80-year-old man with pseudophakic bullous keratopathy and retained Artisan (Ophtec BV, Groningen, the Netherlands) iris-fixated IOL after uneventful DSAEK. RESULTS: The patient presented on the third postoperative day after DSAEK with disenclavation of the nasal iris claw and inferior dislocation of the IOL. The dislocated IOL was removed and exchanged with an angle-supported open-loop anterior chamber lens 7 days after the initial DSAEK procedure. CONCLUSIONS: Despite the relatively traumatic intraocular manipulation, the donor tissue was unaffected in its position and clarity. This case demonstrates that successful DSAEK surgery is compatible with additional surgical procedures, even if they are performed in the early postoperative period.

Solitary eyelid Kaposi sarcoma in an HIV-negative patient.

PURPOSE: To describe a case of localized Kaposi sarcoma (KS) of the eyelid in an HIV-seronegative patient. METHODS: An 80-year-old man developed an ulcerated nodular tumor-like mass that grew rapidly on his left upper eyelid. There were no similar lesions elsewhere. The eyelid lesion was completely excised and histopathologically examined. Serological analyses and molecular biologic techniques, including polymerase chain reaction, were used. RESULTS: Laboratory examinations were within normal limits, and serology for HIV was negative. Histological sections revealed a vascular proliferation composed predominantly of small slit-like blood vessels and epithelioid spindle cells, supporting the diagnosis of KS. Polymerase chain reaction was positive for human herpesvirus 8. During a 2-year follow-up, no recurrences, development of new lesions, or HIV seroconversions were observed. CONCLUSION: This is a classic KS involving only the eyelid in an HIV-negative patient. Location in the eyelid is a possible, albeit rare, initial solitary manifestation of KS in elderly HIV-negative patients. Surgery is both safe and effective.