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Liposarcoma is a malignant soft tissue tumor with several subtypes, the most common of which is well-differentiated liposarcoma (WDL) or atypical lipomatous tumor (ALT). WDL/ALTs are further divided into three histological subtypes, including lipoma-like, sclerosing, and inflammatory. While the majority of these tumors are predominantly fatty, the sclerosing variant demonstrates diverse histologic and radiographic characteristics, including variable amounts of fibrosis and fat. Because of this histological variability and relative rarity, the sclerosing WDL/ALT can present diagnostic dilemmas. We present two cases of sclerosing WDL/ALT, both of which demonstrated high degrees of fibrosis and a paucity of fat, mimicking desmoid fibromatosis and other fibrotic soft tissue tumors. Thus, it is important for radiologists to be aware of the subtypes of liposarcoma and their unique characteristics, and to consider sclerosing WDL/ALT in cases of fibrotic soft tissue tumors. [ABSTRACT FROM AUTHOR]

Background: Mass-forming intrahepatic cholangiocarcinoma (mICC) is the most frequent type of ICC. In contrast-enhanced computed tomography, mICC is visualized as a hypodense lesion with distal dilatation of intrahepatic bile ducts. The presented case illustrates the unusual manifestation of mICC in a 71-year-old male patient, where despite the extensive tumor mass and the hilar infiltration, the dilatation of intrahepatic bile ducts and cholestasis were not noted. Methods: A literature review on PubMed was performed. Primarily, 547 records were identified, and the titles and abstracts were systematically searched. Regarding the inclusion and exclusion criteria, 31 papers describing the non-cancerous liver lesions mimicking ICC were included in the further analysis. Results: In 41.9% of the analyzed non-cancerous lesions, the obstruction of the bile ducts was not noted, similar to our patient. A significant cholestasis has been found in 30.03% of analyzed patients. The invasion of the liver hilum was noted in one-third of the patients. Conclusions: Atypical radiological features in lesions suspected of ICC, such as the absence of intrahepatic bile-duct dilation, are common in benign lesions. In the case of radiologically atypical lesions suspected of ICC, the diagnostic imaging needs to be correlated with clinical data, and the diagnosis should be confirmed with a pathological examination.

Background: Mass-forming intrahepatic cholangiocarcinoma (mICC) is the most frequent type of ICC. In contrast-enhanced computed tomography, mICC is visualized as a hypodense lesion with distal dilatation of intrahepatic bile ducts. The presented case illustrates the unusual manifestation of mICC in a 71-year-old male patient, where despite the extensive tumor mass and the hilar infiltration, the dilatation of intrahepatic bile ducts and cholestasis were not noted. Methods: A literature review on PubMed was performed. Primarily, 547 records were identified, and the titles and abstracts were systematically searched. Regarding the inclusion and exclusion criteria, 31 papers describing the non-cancerous liver lesions mimicking ICC were included in the further analysis. Results: In 41.9% of the analyzed non-cancerous lesions, the obstruction of the bile ducts was not noted, similar to our patient. A significant cholestasis has been found in 30.03% of analyzed patients. The invasion of the liver hilum was noted in one-third of the patients. Conclusions: Atypical radiological features in lesions suspected of ICC, such as the absence of intrahepatic bile-duct dilation, are common in benign lesions. In the case of radiologically atypical lesions suspected of ICC, the diagnostic imaging needs to be correlated with clinical data, and the diagnosis should be confirmed with a pathological examination. [ABSTRACT FROM AUTHOR]

Intramedullary meningiomas are an exceptionally rare subtype of spinal tumors, accounting for only 5% of primary spinal neoplasms. Given their scarcity and unique characteristics, understanding optimal management approaches is crucial for improved clinical decision-making. This systematic review aims to consolidate existing literature and present a detailed case illustration to enhance understanding of this uncommon spinal tumor entity. A systematic search adhering to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was conducted. Eligible studies included case reports, case series, cohort studies, reviews, and meta-analyses. Data extraction and synthesis focused on demographic characteristics, tumor location, clinical presentation, imaging findings, surgical interventions, histopathological features, and outcomes. A total of 15 high-quality scientific articles were included in the systematic review, providing insights into various aspects of intramedullary meningiomas. Demographic analysis revealed a broad age distribution with an equal gender distribution among affected patients. Common clinical presentations included difficulty walking, sensory disturbances, spastic paraparesis, and urinary incontinence. Neuroimaging findings demonstrated heterogeneous signal intensity variations on T1- and T2-weighted images, with variable enhancement patterns on gadolinium-enhanced images. Surgical interventions, predominantly total resection, resulted in favorable postoperative outcomes in most cases. Intramedullary meningiomas pose diagnostic and therapeutic challenges due to their rarity and unique characteristics. Tailored surgical approaches, incorporating techniques such as intraoperative neurophysiological monitoring and fluorescence-aided resection, are crucial for minimizing neurological deficits and optimizing patient outcomes. Despite their infrequency, recognizing intramedullary meningiomas in the differential diagnosis of spinal tumors is essential for prompt diagnosis and timely intervention, ultimately improving patient prognosis. [ABSTRACT FROM AUTHOR]

The tall cell, columnar, and diffuse sclerosing subtypes are aggressive histologic subtypes of papillary thyroid cancer (PTC) with increasing incidence, yet there is a wide variation in reporting. We aimed to identify and compare factors associated with the reporting of these aggressive subtypes (aPTC) to classic PTC (cPTC) and secondarily identify differences in outcomes. The National Cancer Database was utilized to identify cPTC and aPTC from 2004 to 2017. Patient and facility demographics and clinicopathologic variables were analyzed. Independent predictors of aPTC reporting were identified and a survival analysis was performed. The majority of aPTC (67%) were reported by academic facilities. Compared to academic facilities, all other facility types were 1.4-2.0 times less likely to report aPTC (P < 0.05). Regional variation in reporting was noted, with more cases reported in the Middle Atlantic, despite there being more total facilities in the South Atlantic and East North Central regions. Compared to the Middle Atlantic, all other regions were 1.4-5 times less likely to report aPTC (P < 0.001). Patient characteristics including race and income were not associated with aPTC reporting. Compared to cPTC, aPTC had higher rates of aggressive features and worse 5-y overall survival (90.5% versus 94.5%, log rank P < 0.001). Aggressive subtypes of PTC are associated with worse outcomes. Academic and other facilities in the Middle Atlantic were more likely to report aPTC. This suggests the need for further evaluation of environmental or geographic factors versus a need for increased awareness and more accurate diagnosis of these subtypes. [ABSTRACT FROM AUTHOR]

Primary sclerosing cholangitis (PSC) is a cholestatic disease characterized by chronic progressive bile duct inflammation and has a low incidence rate and poor prognosis in China. There is still no drug therapy that can change the course of PSC, and liver transplantation is the only effective treatment for PSC, with a 5-year survival rate of 85% after transplantation. Drug therapy for PSC is facing great challenges based on the current status of PSC. At present, drugs for the treatment of PSC are in the stage of clinical trials and have shown certain application prospect, among which ursodeoxycholic acid is the most widely studied and commonly used drug. In addition, there are many emerging drugs in the pipeline. This article summarizes the latest advances in drug therapy for PSC. [ABSTRACT FROM AUTHOR]

Immunoglobulin G4-related disease is an immune-mediated condition comprised of a number of various disorders sharing unique pathologic, serologic, and clinical features. Diagnosis of immunoglobulin G4-related sclerosing mastitis is challenging as the clinical and imaging findings mimic breast malignancies or other types of inflammatory mastitis. Herein, we describe a case of a female patient with a painless palpable mass in her right breast. An excisional core biopsy led to the rare diagnosis of immunoglobulin G4-related sclerosing mastitis, and the patient received steroid treatment for a month. To date, the patient has remained disease-free without any recurrence. As immunoglobulin G4-related sclerosing mastitis is a very rare disease, further studies are needed to reach conclusions about the pathogenesis, diagnosis, and treatment of this entity. [ABSTRACT FROM AUTHOR]

Introduction: Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease that is characterized by an inflammatory and fibrotic process affecting bile ducts which eventually develops into liver cirrhosis and liver failure. The aim of this study was to investigate serum IgG subclass distribution in patients with PSC and its possible association with PSC outcomes. Patients and methods: We performed a retrospective analysis of 181 patients who had been diagnosed with PSC between January 1970 and December 2015 and followed at our outpatient clinic. Their demographic, immunological, and clinical characteristics were recorded and analyzed. Results: This study included 181 patients with PSC (120 males, 61 females). There was no association between IgGs and the development of autoimmune hepatitis, cirrhosis, cholangiocarcinoma, liver transplantation, inflammatory bowel disease, and colectomy. Patients with elevated IgG4 had statistically significant higher rates of cholangitis (p = 0.02) and endoscopic retrograde cholangiopancreatography (ERCP) (p = 0.009). High IgG4 values were observed in nine patients who underwent ERCP. In these nine patients, on average, IgG4 was evaluated 5 years after ERCP (min 3 days, max 11 years). Subanalysis considering only IgG4 values evaluated before ERCP showed no significant difference but remains significant if we consider IgG4 values after ERCP. Conclusion: Elevated IgG4 in our study showed a possible association with higher rates of cholangitis and ERCP among patients with primary sclerosing cholangitis. It seems that IgGs may be a useful tool for the prediction of outcomes in patients with PSC. A prospective study is necessary, especially to study the trends of IgGs values during disease as well as the role of possible seroconversion. [ABSTRACT FROM AUTHOR]

Autoimmune liver diseases (ALD) are a group of chronic inflammatory liver diseases mediated by autoimmune response and can progress to liver fibrosis, liver cirrhosis, and even liver failure. Early diagnosis, early treatment, and dynamic follow-up of liver fibrosis in ALD may help to improve the prognosis of the disease and even reverse early-stage liver cirrhosis. Due to the limitations and potential risks of liver biopsy, the search for noninvasive techniques has become a research hotspot in the field of liver fibrosis. This article reviews the recent research advances in serum markers and imaging techniques for liver fibrosis in ALD and analyzes the advantages and disadvantages of each detection method and their development trends. [ABSTRACT FROM AUTHOR]

Objective To investigate the value of serum complement C3 level in determining the stage of liver fibrosis in primary biliary cholangitis (PBC). Methods Clinical data were collected from 108 patients with PBC who attended Tianjin Second People’s Hospital and underwent liver biopsy from January 2012 to October 2022. The degree of liver fibrosis (S0-4) was assessed according to the Scheuer scoring system, with ≥S2 defined as significant liver fibrosis, ≥S3 defined as progressive liver fibrosis, and S4 defined as liver cirrhosis. The independent samples t-test was used for comparison of normally distributed continuous data between two groups, and a one-way analysis of variance was used for comparison between multiple groups； the Mann-Whitney U test was used for comparison of non-normally distributed continuous data between two groups, and the Kruskal-Wallis H test was used for comparison between multiple groups； the chi-square test or the Fisher’s exact test was used for comparison of categorical data between groups. The area under the ROC curve (AUC) was used to evaluate the efficacy of complement C3 in the diagnosis of liver fibrosis in patients with PBC. The Spearman correlation analysis was used to investigate the correlation between complement C3 and liver fibrosis stage. Results Among the 108 patients with PBC, there were 87 (80.6%) female patients and 102 patients (94.4%) with positive autoantibody. As for the stage of liver fibrosis, there were 5 patients (4.6%) in S0 stage, 41 (38.0%) in S1 stage, 23 (21.3%) in S2 stage, 25 (23.1%) in S3 stage, and 14 (13.0%) in S4 stage. There was a significant difference in the level of complement C3 between the patients with different liver fibrosis stages (H=42.891, P<0.001). The level of complement C3 gradually decreased with the aggravation of liver fibrosis, with a negative correlation between them (r=−0.565, P<0.001). Liver stiffness measurement (LSM), aspartate aminotransferase/alanine aminotransferase ratio, aspartate aminotransferase-to-platelet ratio index, and fibrosis-4 were negatively correlated with complement C3, with a correlation coefficient of −0.439 (P<0.001), −0.323 (P=0.001), −0.206 (P= 0.033), and −0.291 (P=0.002), respectively. The multivariate logistic regression analysis showed that complement C3 level was an independent predictive factor for significant liver fibrosis, progressive liver fibrosis, and liver cirrhosis, while LSM was an independent predictive factor for significant liver fibrosis and progressive liver fibrosis. The ROC curve analysis showed that complement C3 had an AUC of 0.731, 0.832, and 0.968, respectively, in the diagnosis of significant liver fibrosis, progressive liver fibrosis, and liver cirrhosis, with a corresponding cut-off value of 1.445, 1.235, and 1.005, respectively, and complement C3 combined with LSM had an AUC of 0.811, 0.941, and 0.976, respectively, in the diagnosis of significant liver fibrosis, progressive liver fibrosis, and liver cirrhosis. There was a significant difference in AUC between complement C3 combined with LSM and complement C3 alone in the diagnosis of significant liver fibrosis (Z=2.604, P=0.009), and there was also a significant difference in AUC between complement C3 combined with LSM and complement C3 alone in the diagnosis of progressive liver fibrosis (Z=3.033, P=0.002)； there was no significant difference in AUC between complement C3 combined with LSM and complement C3 alone in the diagnosis of liver cirrhosis (Z=1.050, P= 0.294), while There was a significant difference in AUC between complement C3 combined with LSM and LSM alone in the diagnosis of liver cirrhosis (Z=2.326, P=0.020).Conclusion Serum complement C3 level has a certain clinical value in assessing the degree of liver fibrosis in patients with PBC, and complement C3 combined with LSM can further improve the efficacy of complement C3 or LSM in the diagnosis of liver fibrosis in PBC. [ABSTRACT FROM AUTHOR]

Background/Aims: Primary sclerosing cholangitis (PSC) represents the most common hepatobiliary extraintestinal manifestation of inflammatory bowel disease (IBD), including ulcerative colitis (UC) and Crohn's disease (CD). Limited data exist on PSC in patients with IBD from India. We aimed to assess the prevalence and disease spectrum of PSC in Indian patients with IBD. Methods: Database of IBD patients at 5 tertiary care IBD centers in India were analyzed retrospectively. Data were extracted and the prevalence of PSC-IBD was calculated. Results: Forty-eight patients out of 12,216 patients with IBD (9,231 UC, 2,939 CD, and 46 IBD unclassified) were identified to have PSC, resulting in a prevalence of 0.39%. The UC to CD ratio was 7:1. Male sex and pancolitis (UC) or colonic CD were more commonly associated with PSC-IBD. The diagnosis of IBD preceded the diagnosis of PSC in most of the patients. Majority of the patients were symptomatic for liver disease at diagnosis. Eight patients (16.66%) developed cirrhosis, 5 patients (10.41%), all UC, developed malignancies (3 colorectal cancer [6.25%] and 2 cholangiocarcinoma [4.16%]), and 3 patients died (2 decompensated liver disease [4.16%] and 1 cholangiocarcinoma [2.08%]) on follow-up. None of the patients mandated surgical therapy for IBD. Conclusions: Concomitant PSC in patients with IBD is uncommon in India and is associated with lower rates of development of malignancies. [ABSTRACT FROM AUTHOR]

Background and Aims: The perianal fistula is a pathological tract between two epithelial surfaces of the anorectal canal and perianal skin. The most common treatment method is fistulotomy which leads to complications, such as incontinency. The present is study aimeds to compare the results of fistulotomy surgery and sclerotherapy in the management of managing the perianal fistula. Materials and Methods: This Randomized controlled clinical study was performed on 24 patients with anorectal fistula. The procedure performed for group 1 was surgical fistulotomy, and group 2 underwent sclerosant agent injection. Recovery time, recurrence rate, post-operative pain, and degree of incontinency were evaluated. The statistical analysis was performed in SPSS software (Version 18), and a p-value less than 0.05 was considered statistically significant. Results: There were 5 (41.7%) men and 7 (58.3%) women in the sclerosing group, as well as 7 (58.3%) men and 5 (41.7%) women in the fistulotomy group. There was no significant relationship between the two groups (P=0.68). The recurrence rate was 3 cases (42.9%) in sclerosing group and 4 cases (57.1%) in the fistulotomy group, which was not statistically significant (P=0.65). The fecal continency score after sclerotherapy was greater than that in the fistulotomy group (P

Abstract Background Erdheim-Chester disease (ECD) is a rare histiocytic disorder recently recognized as a neoplasm due to the discovery of activating MAPK pathway mutations. Hepatic involvement by ECD is extremely rare. Case presentation We describe a case of a 64-year-old male who presented with pruritis, weight loss, and cholestatic liver function tests. Magnetic resonance imaging of the abdomen showed beaded appearance of the intrahepatic biliary tree. A liver biopsy was suggestive of primary or secondary sclerosing cholangitis. Computerized tomography (CT) of the abdomen showed perinephric and periaortic soft tissue stranding suggestive of ECD. 18F-fluorodeoxyglucose positron emission/computerized tomography scan showed a mediastinal hilar mass which turned out to be follicular lymphoma. Histopathology of molluscum-like skin lesions showed CD68 + , Factor XIIIa + , and CD1a-foamy histiocytes with multiple giant cells suggestive of ECD. The patient developed recurrent episodes of ascending cholangitis and his hyperbilirubinemia continued to worsen despite stenting of a common hepatic duct stricture found on endoscopic retrograde cholangiopancreatography. Conclusions The absence of associated inflammatory bowel disease and anti-neutrophil cytoplasmic antibody, as well as the rapidity of disease progression, makes us consider the possibility of hepatic involvement by ECD or an overlap syndrome. We want to highlight that negative histopathology should not delay the diagnosis of ECD as effective and potentially lifesaving therapies with BRAF or MEK pathway inhibitors are now available for these patients.

Autoimmune liver disease is an important immune-mediated pathologic entity involving the liver and intrahepatic bile duct, including autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis. Although it is necessary to ascertain its presence in acute or chronic liver disease without common causes, it is not easy to diagnose this disease straightforwardly because of its rarity. Recently, the incidence and prevalence of autoimmune hepatitis and primary biliary cholangitis have increased in several regions. In contrast, there is limited data dealing with the trend of the epidemiology of primary sclerosing cholangitis worldwide. Physicians should consider the epidemiologic characteristics of autoimmune liver disease because early diagnosis and proper treatment might prevent the progression of advanced liver disease. In addition, more sophisticated epidemiologic studies will be needed to elucidate the trend of these rare diseases nationwide.

Sehnenbeschwerden sind im sportorthopädischen Alltag einer der häufigsten Konsultationsgründe. Vermehrte sportliche Betätigung in der Freizeit, höhere Anforderungen im Leistungssport und neue Trendsportarten führten zu einer Zunahme der Inzidenz von Überlastungsschäden, insbesondere Tendinopathien und Sehnenrupturen. In den vergangenen Jahren haben sich jedoch auch das Verständnis der Pathogenese sowie die Diagnostik und Behandlungsmethoden enorm weiterentwickelt. Unter anderem stehen zahlreiche wirkungsvolle Injektionstherapien mit zunehmend guter Evidenz aus hochwertigen klinischen Studien zur Verfügung. Es ist unbestritten, dass Tendinopathien selten primär eine Entzündung zu Grunde liegt. Vielmehr führt ein multifaktorielles Geschehen zur Degeneration des Sehnengewebes. Die Therapie beruht somit auf der Analyse der auslösenden und unterhaltenden Faktoren, welche dann im Einzelnen adressiert werden müssen, um einen langfristigen Behandlungserfolg zu gewährleisten. Injektionstherapie spielen unterstützend eine immer wichtigere Rolle insbesondere vor dem Hintergrund des oft langwierigen Verlaufs und der besseren Verfügbarkeit, wirkungsvoller, kortison-freier Substanzen. In diesem Artikel werden die unterschieddlichen Präparate und Methoden vorgestellt und hinsichtlich Evidenzlage, Indikationen, Wirkungen und Nebenwirkungsprofilen analysiert. The number of tendon injuries has risen significantly in recent years. Increased leisure time sporting activity, higher demands in competitive sports as well as new trend sports led to an high incidence of overuse injuries in sports. In parallel, the understanding of the pathogenesis of tendinopathies improved and the diagnostic and treatment modalities expanded. It has become evident that tendinopathies are not primarily based on inflammation processes. Treatment needs to be individualized and is based on the analysis of the triggering and maintaining factors, that need to be addressed in order to ensure long-term success. Injection therapy is playing a rising important role. The various injection methods as well at the injectable substances are evaluated in this article with regard to indication and effect. [ABSTRACT FROM AUTHOR]

ABSTRACT BACKGROUND: The prevalence of primary sclerosing cholangitis (PSC) in the general population has not yet been clearly established. The management of PSC should focus on delaying the progression of the disease and restraining its complications. The only curative therapy for the disease remains liver transplantation (LT). PSC is currently the fifth most common indication for LT and corresponds to 5% of all LT indications in adults. AIMS: Our objective is to evaluate the indications and outcomes of PSC patients undergoing LT in three liver transplantation centers in southern Brazil – Hospital Santa Isabel in Blumenau, Santa Catarina state, and Hospital das Clínicas and Hospital Nossa Senhora das Graças, in Curitiba, Parana state). METHODS: This is a longitudinal observational study of patients with PSC who underwent LT in three major Brazilian medical centers. Electronic medical records and study protocols of all patients subjected to LT from January 2011 to December 2021 were retrospectively reviewed. RESULTS: Of the 1,362 transplants performed in the three medical centers, 37 were due to PSC. Recurrence of PSC occurred in three patients (8.1%) in 3.0±2.4 years (range, 1–4 years). The 1-year and 5-year survival rates after the first LT were 83.8 and 80.6%, respectively. The 1-year and 5-year graft survival rates were, respectively, 83.8 and 74.8%. CONCLUSIONS: Our experience with LT in patients with PSC demonstrated good patient and graft survival results. Most deaths were due to common factors in patients undergoing LT.

Background and Aims: Immunoglobulin G4‐related sclerosing cholangitis (IgG4‐SC) is considered a biliary manifestation of IgG4‐related diseases. However, there has been a controversy on the clinical outcomes according to the location of the involved bile duct. We therefore compared the clinical outcomes and long‐term prognosis of IgG4‐SC with proximal bile duct involvement (proximal IgG4‐SC) and IgG4‐SC with distal bile duct involvement (distal IgG4‐SC). Methods: We reviewed the data of patients with IgG4‐SC that were prospectively collected at 10 tertiary centers between March 2002 and October 2020. Clinical manifestations, outcomes, association with autoimmune pancreatitis (AIP), steroid‐responsiveness, and relapse of IgG4‐SC were evaluated. Results: A total of 148 patients (proximal IgG4‐SC, n = 59; distal IgG4‐SC, n = 89) were analyzed. The median age was 65 years (IQR, 56.25–71), and 86% were male. The two groups were similar in terms of jaundice at initial presentation (51% vs 65%; P = 0.082) and presence of elevated serum IgG4 (66% vs 70%; P = 0.649). The two groups showed significant differences in terms of steroid‐responsiveness (91% vs 100%; P = 0.008), association with AIP (75% vs 99%; P = 0.001), and occurrence of liver cirrhosis (9% vs 1%; P = 0.034). During a median follow‐up of 64 months (IQR, 21.9–84.7), the cumulative relapse‐free survival was significantly different between the two groups (67% vs 79% at 5 years; P = 0.035). Conclusions: Relapse of IgG4‐SC frequently occurred during follow‐up. Proximal IgG4‐SC and distal IgG4‐SC had different long‐term outcomes in terms of steroid‐responsiveness, occurrence of liver cirrhosis, and recurrence. It may be advantageous to determine the therapeutic and follow‐up strategies according to the location of bile duct involvement. [ABSTRACT FROM AUTHOR]

Abstract Background Despite emerging reports of secondary sclerosing cholangitis (SSC) in critically ill COVID-19 patients little is known about its imaging findings. It presents as delayed progressive cholestatic liver injury with risk of progression to cirrhosis. Diagnosis cannot be made based on clinical presentation and laboratory markers alone. Magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography (MRCP) can aid in the diagnosis. The aim of this study was to describe MRI/MRCP imaging features of COVID-19-associated SSC. Results Seventeen patients (mean age 60.5 years, 15 male) who underwent MRI/MRCP were included. All had been admitted to intensive care unit (ICU) (median duration of ICU stay 10 weeks, range, 2–28 weeks) and developed acute respiratory distress syndrome requiring mechanical ventilation. On imaging, all patients had intrahepatic bile duct strictures and 10 (58.8%) had associated upstream dilatation. Intrahepatic bile duct beading was seen in 14 cases (82.3%). Only one patient (5.9%) had extrahepatic bile duct stricturing. Patchy arterial phase hyperenhancement and high signal on T2- and diffusion-weighted images were seen in 7 cases (53.8%) and 9 cases (52.9%), respectively. Biliary casts were seen in 2 cases (11.8%). Periportal lymphadenopathy and vascular complications were not seen. Conclusion On MRI/MRCP, COVID-19-associated SSC presents with multiple intrahepatic bile duct strictures with or without upstream dilatation and intrahepatic bile duct beading. Surrounding hepatic parenchymal changes including alterations in enhancement and T2 signal are common. The extrahepatic biliary tree was typically spared and periportal lymphadenopathy was missing in all patients.

Background: Erdheim-Chester disease (ECD) is a rare histiocytic disorder recently recognized as a neoplasm due to the discovery of activating MAPK pathway mutations. Hepatic involvement by ECD is extremely rare. Case presentation: We describe a case of a 64-year-old male who presented with pruritis, weight loss, and cholestatic liver function tests. Magnetic resonance imaging of the abdomen showed beaded appearance of the intrahepatic biliary tree. A liver biopsy was suggestive of primary or secondary sclerosing cholangitis. Computerized tomography (CT) of the abdomen showed perinephric and periaortic soft tissue stranding suggestive of ECD. 18F-fluorodeoxyglucose positron emission/computerized tomography scan showed a mediastinal hilar mass which turned out to be follicular lymphoma. Histopathology of molluscum-like skin lesions showed CD68 + , Factor XIIIa + , and CD1a-foamy histiocytes with multiple giant cells suggestive of ECD. The patient developed recurrent episodes of ascending cholangitis and his hyperbilirubinemia continued to worsen despite stenting of a common hepatic duct stricture found on endoscopic retrograde cholangiopancreatography. Conclusions: The absence of associated inflammatory bowel disease and anti-neutrophil cytoplasmic antibody, as well as the rapidity of disease progression, makes us consider the possibility of hepatic involvement by ECD or an overlap syndrome. We want to highlight that negative histopathology should not delay the diagnosis of ECD as effective and potentially lifesaving therapies with BRAF or MEK pathway inhibitors are now available for these patients. [ABSTRACT FROM AUTHOR]

Background and Aims: The perianal fistula is a pathological tract between two epithelial surfaces of the anorectal canal and perianal skin. The most common treatment method is fistulotomy which leads to complications, such as incontinency. The present is study aimeds to compare the results of fistulotomy surgery and sclerotherapy in the management of managing the perianal fistula. Materials and Methods: This Randomized controlled clinical study was performed on 24 patients with anorectal fistula. The procedure performed for group 1 was surgical fistulotomy, and group 2 underwent sclerosant agent injection. Recovery time, recurrence rate, post-operative pain, and degree of incontinency were evaluated. The statistical analysis was performed in SPSS software (Version 18), and a p-value less than 0.05 was considered statistically significant. Results: There were 5 (41.7%) men and 7 (58.3%) women in the sclerosing group, as well as 7 (58.3%) men and 5 (41.7%) women in the fistulotomy group. There was no significant relationship between the two groups (P=0.68). The recurrence rate was 3 cases (42.9%) in sclerosing group and 4 cases (57.1%) in the fistulotomy group, which was not statistically significant (P=0.65). The fecal continency score after sclerotherapy was greater than that in the fistulotomy group (P<0.05), and the length of hospitalization, postsurgery discomfort, and the time it took for the fistula to heal were all considerably shorter in the sclerosing group. Conclusion: We concluded that injectable sclerotherapy is effective, safe, simple, acceptable and affordable in the treatment of perianal fistula. [ABSTRACT FROM AUTHOR]

Meconium peritonitis has been associated with cystic fibrosis, congenital infections, and bowel disease. A growing number of reports have also associated it with maternal liver disease. This report describes the case of a preterm newborn with meconium peritonitis born from a woman with liver disease and presents a review of the literature on the subject. A male preterm newborn weighing 2595g was admitted to the Neonatal Intermediate Care Unit due to an obstetric ultrasound scan performed at 31 weeks and 3 days of gestation showing calcifications of various sizes throughout his abdomen. His mother had autoimmune hepatitis and sclerosing cholangitis. Postnatal abdominal x-ray images showed calcifications in the liver and right paracolic gutter and abdomen ultrasound scans showed large peritoneal calcifications in the hepatic and splenic capsules and in the pouch of Morrison, in addition to calcifications in the right scrotum.

Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease, which is associated with an increased risk of cholangiocarcinoma (CCA). Novel markers, to complement or replace CA19-9, are urgently needed for the screening of PSC-associated biliary neoplasia. Previous studies have suggested that serum trypsinogen-2 and human chorionic gonadotropin β-subunit (hCGβ) may serve as such markers. Using highly specific in-house immunoassays, we studied trypsin(ogen)-2 and -3, SPINK1 and hCGβ in bile samples of 214 patients, referred for endoscopic retrograde cholangiography. We found that biliary trypsinogen-2 was decreased (p = 0.027) and hCGβ was elevated (p < 0.001) in PSC patients who were diagnosed 1.6 years (median, range 0.1–8.8 years) later with CCA or in whom biliary dysplasia was observed at least twice in brush cytology (n = 11) as compared to PSC patients without CCA or repeated dysplasia (n = 171). The other studied markers did not show significant differences between these groups. Our results warrant further evaluation of hCGβ as a predictive marker for PSC-associated biliary neoplasia.

Congenital ichthyosis is a genodermatosis characterized by abnormal epidermal differentiation. The neonatal period is critical for patients with ichthyosis because of the risk for significant comorbidities and associated mortality, with most complications resulting from impaired barrier function. Early recognition can significantly alter the clinical course of this rare disease. Here we present a neonate with ichthyosis, leukocyte vacuoles, alopecia, and sclerosing cholangitis syndrome (ILVASC), a rare inherited disease, to highlight how an interdisciplinary approach led to prompt assessment, confirmation of a genetic diagnosis and management of potential complications. [ABSTRACT FROM AUTHOR]

Hemangiomas are the most common noncystic benign hepatic tumors and are usually incidentally discovered during routine radiological examinations. The diagnosis of hepatic hemangiomas with a typical presentation is generally easy with plain and cross-sectional imaging; however, it can be complicated when hemangiomas undergo histological changes such as fibrosis. Sclerosed hepatic hemangioma (SHH) is the extreme presentation of this fibrotic process. These atypical lesions can be misdiagnosed as primary hepatic malignancies or metastasis. Their diagnosis is established by histological examination. We report the case of a patient with an SHH, which was misdiagnosed as an intrahepatic cholangiocarcinoma. This article's aim is to draw attention to this infrequent pathology and underline the features of this benign tumor that could suggest its diagnosis prior to surgery to avoid unnecessary hepatic resections.

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal uterine tumor and the histological variant, sclerosing PEComa is exceedingly rare. Sclerosing PEComas preferentially occur in the retroperitoneum and occurrence in the uterine corpus is seldom seen. These tumors pose a diagnostic challenge and need distinction from morphological mimickers such as epithelioid smooth muscle tumors, endometrial stromal sarcoma, and metastatic carcinoma. Accurate diagnosis can be established coupling histomorphology with immunostaining. The distinction from other entities is of prime importance considering the therapeutic and prognostic implications. Herein, we describe a case of uterine sclerosing variant of PEComa with diagnostic difficulties and key to diagnose this entity.

Non-viral liver diseases mainly include nonalcoholic fatty liver disease, alcoholic liver disease, autoimmune liver disease, and cholestatic liver disease, and the prevalence rate of non-viral liver diseases tends to increase in recent years. Takeda G protein-coupled receptor-5（TGR5） belongs to the G protein-coupled receptor superfamily and is activated by primary and secondary bile acids. TGR5 plays an important regulatory role in bile acid homeostasis, basal metabolism, energy balance, and alleviation of inflammatory response and is a potential therapeutic target for many diseases. An increasing number of evidence has shown that TGR5 exerts a protective effect on the liver by improving bile acid and glycolipid metabolism in liver, alleviating liver inflammation, and reducing liver steatosis. This article reviews the recent advances in the basic research on TGR5 in the field of non-viral liver diseases, so as to facilitate the development of the research on TGR5. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: The incidence of abnormal liver function, mainly aspartate aminotransferase and alanine aminotransferase elevations, in patients with COVID-19 is not uncommon, but persistent liver damage after the acute phase of the disease is uncommon and has been recently recognized as a new entity named post-COVID-19 cholangiopathy. Case Report: We report a clinical case with progressive cholestatic disease following severe COVID-19. AST and ALT peaked at hospital admission and while its serum concentration went down, bilirubin and cholestatic liver enzymes started to increase, reaching the maximum at day 122. Magnetic resonance imaging (MRI) revealed a diffuse irregularity of intra- and extrahepatic bile ducts, with multiple focal strictures alternating with mild focal dilations of the biliary tree, suggesting a sclerosing cholangiopathy. A transjugular liver biopsy showed a prominent bile ductular reaction, cholangiocyte injury, inflammatory infiltrate rich in neutrophils, biliary infarctions, marked cholestasis, and portal fibrosis, suggesting the diagnosis of post-Covid-19 secondary sclerosing cholangitis. The patient evolved with a continuous deterioration of liver functions, but liver transplantation was not performed due to his poor clinical condition. Conclusions: Post-COVID-19 SSC is a severe disease with no effective clinical treatment and has liver transplantation as the only treatment for a few selected patients. [ABSTRACT FROM AUTHOR]

Background: Despite emerging reports of secondary sclerosing cholangitis (SSC) in critically ill COVID-19 patients little is known about its imaging findings. It presents as delayed progressive cholestatic liver injury with risk of progression to cirrhosis. Diagnosis cannot be made based on clinical presentation and laboratory markers alone. Magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography (MRCP) can aid in the diagnosis. The aim of this study was to describe MRI/MRCP imaging features of COVID-19-associated SSC. Results: Seventeen patients (mean age 60.5 years, 15 male) who underwent MRI/MRCP were included. All had been admitted to intensive care unit (ICU) (median duration of ICU stay 10 weeks, range, 2–28 weeks) and developed acute respiratory distress syndrome requiring mechanical ventilation. On imaging, all patients had intrahepatic bile duct strictures and 10 (58.8%) had associated upstream dilatation. Intrahepatic bile duct beading was seen in 14 cases (82.3%). Only one patient (5.9%) had extrahepatic bile duct stricturing. Patchy arterial phase hyperenhancement and high signal on T2- and diffusion-weighted images were seen in 7 cases (53.8%) and 9 cases (52.9%), respectively. Biliary casts were seen in 2 cases (11.8%). Periportal lymphadenopathy and vascular complications were not seen. Conclusion: On MRI/MRCP, COVID-19-associated SSC presents with multiple intrahepatic bile duct strictures with or without upstream dilatation and intrahepatic bile duct beading. Surrounding hepatic parenchymal changes including alterations in enhancement and T2 signal are common. The extrahepatic biliary tree was typically spared and periportal lymphadenopathy was missing in all patients. [ABSTRACT FROM AUTHOR]

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with no medical treatment proven to improve survival and postpone liver transplantation. Previous studies have shown the effectiveness of fibrates in primary biliary cholangitis. The current study prospectively evaluated the effect of fenofibrate on PSC patients. We administered 200 mg of fenofibrate to PSC patients in the intervention arm and a placebo in the control arm once per day for 6 months and evaluated liver biochemistries (alkaline phosphatase, aspartate aminotransferase, alanine aminotransferase, bilirubin, and albumin) and the Mayo Risk Score at the start and end of the study. The primary endpoint was defined as a reduction greater than 50% or normalization of ALP levels. Secondary endpoints were an improvement in the Mayo Risk Score and serum bilirubin levels. Thirty patients were included (19 female, 11 male, 40.2 ± 9.2 years old), all under treatment with Ursodeoxycholic acid prior to this study. ALP and ALT levels significantly decreased in the fenofibrate group, by 64.7% (mean difference = 557, p = 0.004, 95% CI = 208.72, 905.27) and 52.78%, (p = 0.006), respectively. The primary endpoint was achieved in 66.7% of patients (10 in 15) in the fenofibrate group versus 20% of patients (3 in 15) in the placebo group (p = 0.009). Other endpoints were not met. As studies have demonstrated lower levels of ALP may improve outcomes for PSC, our study resulted in significantly lower levels of ALP in the fenofibrate group, which could translate into better disease prognosis in PSC. [ABSTRACT FROM AUTHOR]

Background & Aims: Primary sclerosing cholangitis (PSC) and IgG4‐related sclerosing cholangitis (IgG4‐SC) are chronic fibro‐inflammatory immune‐mediated hepatobiliary conditions that are challenging to distinguish in a clinical setting. Accurate non‐invasive biomarkers for discriminating PSC and IgG4‐SC are important to ensure a correct diagnosis, prompt therapy and adequate cancer surveillance. Methods: We performed nuclear magnetic resonance (NMR)‐based metabolomic profiling using serum samples collected prospectively from patients with PSC (n = 100), IgG4‐SC (n = 23) and healthy controls (HC; n = 16). Results: Multivariate analysis of the serum metabolome discriminated PSC from IgG4‐SC with greater accuracy (AUC 0.95 [95%CI 0.90–0.98]) than IgG4 titre (AUC 0.87 [95%CI 0.79–0.94]). When inflammatory bowel disease (IBD) was excluded as a comorbid condition (IgG4‐SC n = 20, PSC n = 22), the diagnostic AUC increased to 1.0, suggesting that the metabolome differences identified are not a result of the increased prevalence of IBD in PSC relative to IgG4‐SC patients. Serum lactate (p <.0001), glucose (p <.01) and glutamine (p <.01) metabolites were increased in IgG4‐related disease (IgG4‐RD) and IgG4‐SC individuals compared to PSC, whereas mobile choline (p <.05), 3‐hydroxybutyric acid (p <.01) and ‐CH3 lipoprotein resonances (p <.01) were decreased. Conclusions: Taken together, serum metabolomic profiling has the potential to be incorporated as a diagnostic criterion, independent of IgG4 titre, to improve the diagnosis of IgG4‐RD and help distinguish IgG4‐SC from PSC. [ABSTRACT FROM AUTHOR]

An inflammatory pseudotumor is a benign disease characterized by tumor-like lesions consisting of inflammatory cells including plasma cells and fibrous tissue. Recently, some inflammatory pseudotumor cases proved to be a form of Immunoglobulin G4-related disease (IgG4-RD). This novel clinical entity, recognized as a fibroinflammatory condition, is characterized by lymphoplasmacytic infiltration with a predominance of IgG4-positive plasma cells, storiform fibrosis, and often elevated serum IgG4 concentrations. We report a case of IgG4-RD in the form of an inflammatory pseudotumor in the liver with combined sclerosing cholangitis. We recommend that for diagnosing IgG4-RD accurately, it is important to obtain adequate tissue samples and follow-up the lesion in clinical practice. [ABSTRACT FROM AUTHOR]

The elucidation of differences between adult and pediatric-onset primary sclerosing cholangitis (PSC) may inform clinical decision making, and whether results of adult PSC clinical trials can be extrapolated to pediatric subjects. A single-center retrospective analysis of PSC subjects diagnosed during the epoch 2000–13 was conducted. Demographic, clinical, and laboratory data were compared between PSC subjects diagnosed between 0–18 (pediatric) and 19+ (adult) years of age. An adverse outcome was defined as PSC-related death, liver transplant, or malignancy. Survival without any of these was defined as event-free survival. Analyses of 28 pediatric-diagnosed and 59 adult-diagnosed subjects revealed that incidence of early portal hypertension (PHT; P = 0.2), laboratory parameters of liver disease severity, and fibrosis grade at diagnosis were comparable between adult and pediatric PSC subjects. Adult-diagnosed PSC subjects had higher incidences of adverse outcomes compared to pediatric-diagnosed PSC subjects (P = 0.02). The age group 0–18 years (n = 30) had significantly better event-free survival compared to the age group more than 40 years (n = 25; P = 0.03). The prevalence of PHT in adult PSC subjects was 2.6 that of pediatric PSC subjects. PHT adversely affected outcomes in both adult (P < 0.001) and pediatric (P = 0.01) subjects. Adult PSC subjects were more likely to develop biliary complications (BCs; P = 0.001), ascites (P = 0.004), and variceal bleed (P = 0.03). Adult PSC subjects were more likely to have extra-hepatic co-morbidities (P < 0.001). Adult subjects had a longer follow-up duration compared to pediatric subjects (P = 0.06). Despite having a comparable clinical, laboratory, and histologic biomarkers of liver disease severity at the time of diagnosis, adult PSC subjects had a worse outcome compared to pediatric PSC subjects. Possible reasons for this finding include higher incidence of PHT, BCs, extra-hepatic co-morbidities, and longer duration of follow-up. [ABSTRACT FROM AUTHOR]

A 15-year-old boy presented to outpatient dermatology clinic for evaluation of a lesion on the hand. Originally small and asymptomatic, the lesion had rapidly enlarged in the six months prior to evaluation. A shave biopsy was performed and histopathologic evaluation demonstrated a well-circumscribed nodular proliferation of dense, bland, epithelioid to spindle cells on a fibrillary background. Tumor cells were diffusely epithelial membrane antigen positive; SI00 and Melan-A were negative. These findings are consistent with a diagnosis of sclerosing perineurioma. This case illustrates the presentation of sclerosing perineurioma in a pediatric patient and we review the pertinent pathologic and immunohistochemical findings necessary for diagnosis. It is imperative to distinguish this entity from other soft tissue tumors on the hand, both benign and malignant, to avoid overly aggressive surgical intervention. [ABSTRACT FROM AUTHOR]

This report describes the cytological diagnosis of sclerosing adenosis along with a brief literature review, with emphasis on overcoming diagnostic challenges and pitfalls. [ABSTRACT FROM AUTHOR]

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal uterine tumor and the histological variant, sclerosing PEComa is exceedingly rare. Sclerosing PEComas preferentially occur in the retroperitoneum and occurrence in the uterine corpus is seldom seen. These tumors pose a diagnostic challenge and need distinction from morphological mimickers such as epithelioid smooth muscle tumors, endometrial stromal sarcoma, and metastatic carcinoma. Accurate diagnosis can be established coupling histomorphology with immunostaining. The distinction from other entities is of prime importance considering the therapeutic and prognostic implications. Herein, we describe a case of uterine sclerosing variant of PEComa with diagnostic difficulties and key to diagnose this entity. [ABSTRACT FROM AUTHOR]

Abstract Background Hepatic sclerosing hemangiomas (HSH) are rare benign vascular tumors that mimic radiologically hepatic malignancies. HSH is characterized by extensive fibrosis and hyalinosis as a result of degeneration and thrombosis. Pre-operative diagnosis is very difficult and most of the cases undergo surgical resections. Case presentation A 65 years old, hepatitis C virus-infected female presented by an accidentally discovered hepatic focal lesion. Magnetic resonance imaging (MRI) showed a T2 hyperintense mass displaying peripheral enhancement in porto-venous phase. The patient had a simultaneous typical hepatic hemangioma and multiple vertebral bodies’ hemangiomata. Tissue-guided biopsy revealed a densely sclerotic stroma containing vascular spaces with occasional obliteration of the lumen. Immunohistochemical staining was performed for Pan CK, CD34, SMA, VEGF, and c-Kit which confirmed the vascular nature of neoplasm and the involuting phase of hemangioma development. The patient was reassured and recommended for a regular radiological follow-up to reassess the lesion size. Conclusion The definitive preoperative diagnosis of HSH is still problematic. However, the awareness of surgeons by this entity, peripheral enhancement in porto-venous phase on dynamic MRI, the presence of simultaneous typical hepatic hemangioma in the absence of cancer history could raise suspicion for HSH. Liver biopsy is still the gold standard in approaching the accurate diagnosis.

Sclerosing mucoepidermoid carcinoma (SMC) is described as a "sclerosing variant" of mucoepidermoid carcinoma, and it is characterized by dense fibrosis and sclerosis of the stroma. SMC with eosinophilia (SMCE) is another and more rare subtype characterized by eosinophilia in addition to the sclerotic stroma common to SMC. However, unlike SMC, SMCE is not listed in the current 4th edition of WHO classification. Here, we describe three cases: one SMC in the parotid gland, one SMCE in the submandibular gland and one SMCE in the minor salivary gland of the oral cavity. The patients included a 71-year-old Japanese male, a 74-year-old Japanese female, and an 81-year-old Japanese female. They each complained of mass formation and underwent surgical resection. Histologically, the tumors mainly consisted of squamous cells with scarce keratinization that formed irregular large and small nests along with cystic structures containing mucous cells against the background of sclerotic stroma. One oral SMCE showed fine nesting and trabecular invasion. The two SMCEs included dense aggregates of eosinophils as well as more prominent lymphoid infiltration. Fluorescence in situ hybridization for MAML2 confirmed split signals in SMC, but not in SMCE. [ABSTRACT FROM AUTHOR]

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Background: Head and neck paraganglioma is a rare neoplasm of the paraganglia. It accounts for <1% of all head and neck tumors. It usually has benign clinical course; however, malignant paraganglioma can only be diagnosed by showing metastatic disease. We undertook a retrospective study to assess the clinical significance of regional lymph nodes metastases in head and neck paragangliomas. Design: From 1993 to 2016, primary head and neck paragangliomas are identified. The patient clinical and histopathologic materials were reviewed. Results: Sixty-five specimens from 62 patients (3 patients with more than 1 specimens) with head and neck paragangliomas were recorded (49 female and 13 males) with mean age of 54 (24-78 years). The locations of the tumors were as follows: carotid body: 30, glomus tympanicum: 11, glomus jugulare: 14, parapharyngeal space: 3, and 1 case each of larynx, skull base, paraglottic area, infratemporal fossa, mastoid, cerebellopontine (CP) angle, and pyriform sinus. On histopathology, we found 5 cases of sclerosing variant. Thirty-two (52%) of the 62 patients had regional lymph node biopsy. Four (12%) of the 32 show metastatic paraganglioma (3 females and 1 male with mean age = 35). Two of the 5 cases of sclerosing variant had positive lymph nodes. No evidence of local recurrence or distant metastasis in the patients with positive lymph nodes with a 6 to 11 years follow-up. One of the 28 patients with negative lymph nodes developed metastatic disease to lumbar spine in 5 years. Conclusion: Metastatic paraganglioma to regional lymph nodes may have indolent clinical behavior, with disease-free survival of up to 11 years. The incidence of metastatic disease in lymph nodes was 4 (12%) of 32. Forty percent (2/5) of the cases with sclerosing variant of paraganglioma had lymph node metastases indicating that this tumor may have a more aggressive histological behavior. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: Immunoglobulin (Ig) G4-related disease is a rare disease of unknown pathophysiology, which can affect multiple organs leading to tissue fibrosis and organ failure. The present case report describes a patient with systemic IgG4-related disease (IgG4-RD) that occurred over a 1-year period and affected multiple organs at different times. Imaging studies, interventional procedures, changes in laboratory parameters, and histopathology demonstrate the novel and known aspects of this disease before and during prednisolone monotherapy and in combination with azathioprine. Case Report: A 64-year-old man presented with weight loss and painless jaundice, which was highly suspicious for cholangiocarcinoma. A thorough medical history together with laboratory tests, imaging procedures, and endoscopic interventions confirmed that surgery was not needed and led to the final diagnosis of histologically-confirmed, IgG4-related sclerosing cholangitis and autoimmune pancreatitis type 1. Other typical organ manifestations of systemic IgG4-RD were diagnosed through a thorough medical review, which led to immunohistochemical reevaluation of past surgical specimens. Besides the IgG4-related organ manifestations, which can include periorbital xanthelasmas, our patient developed a pulmonary adenocarcinoma 6 years after the initial clinical onset of IgG4-RD. After immunosuppressive treatment with prednisolone alone and subsequently in combination with azathioprine, the patient's IgG4-RD resolved. Conclusions: Interdisciplinary collaboration is required to diagnose IgG4-RD that involves multiple organs. Patient medical history remains crucial for diagnosis and attention should be paid to avoiding unnecessary surgery. Tumors (lung adenocarcinomas) and xanthelasmas can develop because of IgG4-RD. Glucocorticoids and additional azathioprine may be advisable for maintenance treatment. [ABSTRACT FROM AUTHOR]

Objective: We aimed to summarize the clinical and pathological features of sclerosing angiomatoid nodular transformation (SANT) in spleen among five cases.Methods: Five cases (male: 3; female: 2; mean age: 47.6 years) with SANT confirmed by pathological analysis between July 2010 and November 2019 in our hospital were included in this study. The clinical, imaging, and pathological data were analyzed retrospectively.Results: Three patients presented with mild abdominal pain or discomfort, while the other two were symptom free. Two patients received ultrasonography (US), and all patients underwent a computerized tomography (CT) scan in our hospital. The typical “spoke wheel” pattern was seen in two cases, and central calcification was detected in one case on the CT scans. All patients indicated peripheral enhancement around the SANT lesion during the arterial phase. Open or laparoscopic splenectomy was performed for treatment. No patient showed recurrence in the follow-up. The pathological characteristics of our cases were in line with those of previous literatures.Conclusions: Peripheral enhancement around the SANT lesion during the arterial phase should be taken into consideration for the diagnosis of SANT as an imaging sign on CT scans. Special attention should be paid to the splenic integrality during the laparoscopic approach, due to the probability of malignancy and the fragility of the spleen.

Primary biliary cholangitis( PBC) is a multifactorial disease caused by the combined effect of genetic susceptibility and environmental triggers. Among the known risk factors for PBC,environmental factors mainly cause the onset of the disease by disrupting mitochondrial immune tolerance. With the deepening of research,especially the application of genome-wide association study technology,some dangerous genes have been found,such as human leukocyte antigen gene IL and X chromosome monomer. This article reviews the research advances in high-risk factors for PBC. [ABSTRACT FROM AUTHOR]

BACKGROUND: The Triplex method is a three-step treatment approach to reducing the appearance of small and medium-sized varicose veins and phlebological imperfections of the lower limbs. OBJECTIVE: This study evaluated the effectiveness, duration of results, adverse events, patient satisfaction, and improvements in quality of life in patients with small and medium-sized varicose veins who were treated with the Triplex method. METHODS: We conducted a six-year follow-up study of 4,000 patients from the years 2012 to 2018. Patients with chronic venous insufficiency of the lower limbs were included. Patients with active or previous phlebitis or phlebostatic ulcers were excluded. An average of three sessions was performed on each patient in the Triplex group. Follow-up assessments were performed 1, 3, 6, and 12 months after the completion of the treatment, and they included a morphofunctional study, histological examination, and photographic documentation. The first step of the treatment approach utilized an injectable shrinkage solution consisting of sodium salicylate, physiological solution with 10% glycerol, and lidocaine, which was injected into the varices to trigger a reduction of the vessel lumen. For patients in whom the targeted vessels were not sufficienty narrowed by the shrinkage solution, the second step - ultrasound-guided foam sclerotherapy, or scleromousse - was utilized, which consisted of either lauromacrogol 1% or sodium tetradecyl sulfate 1%, with one group of patients (n=50) receiving the former and the other group of patients (n=50) receiving the latter. All patients were administered Step 3 of the treatment approach, which usually included varicose treatment with low concentrations of sodium tetradecyl sulfate (STS) 0.2 to 0.50% or lauromacrogol 0.25%, with compression additionally prescribed in some patients. RESULTS: Over a follow-up period of six years, in patients who underwent full Triplex treatment, we observed 1) a greater duration of the narrowing of the vessel's caliber, with disappearance of the varices; 2) reduced utilization of the foam solution at the reflux point due to the narrowing of the gauge; and 3) no relevant hemodynamic effects emerged in patients with recanalization. In patients who underwent Steps 1 and 2 of the treatment approach, reflux was not hemodynamically significant. No significant differences were observed in the lauromacrogol group of patients compared to the sodium tetradecyl sulfate group. CONCLUSION: Compared to patients in whom only scleromousse was performed at the reflux site, the narrowing of the varicose wall that is achieved using the Triplex method is associated with longer lasting results in the treatment of small and medium-sized varicose veins, with physiological recovery of superficial venous circulation. [ABSTRACT FROM AUTHOR]

Inflammatory bowel disease (IBD) is a series of non-specific chronic inflammatory diseases, including ulcerative colitis and Crohn’s disease. Besides gastrointestinal symptoms, IBD patients often have extraintestinal symptoms which may involve various systems such as the skeleton and muscle, skin, eyes, liver and gallbladder, pancreas, nerves, urogenital system, lungs, heart, and blood. Biliary diseases are extraintestinal manifestations of IBD and mainly include primary sclerosing cholangitis, IgG4-associated sclerosing cholangitis, primary biliary cirrhosis, and cholelithiasis. Biliary diseases may manifest as transient abnormal liver function in asymptomatic patients and even life-threatening liver failure, and different biliary diseases may have different treatment methods and prognoses and thus require careful differential diagnosis. This article reviews biliary diseases in IBD patients, in order to help clinicians get familiar with the clinical manifestations and diagnosis and treatment strategies for biliary diseases in IBD patients. [ABSTRACT FROM AUTHOR]

Autoimmune liver diseases mainly include primary biliary cholangitis(PBC), autoimmune hepatitis(AIH), and primary sclerosing cholangitis. Simultaneous or successive occurrence of the features of any two of the above diseases is called overlap syndrome, among which PBC-AIH overlap syndrome is the most common type. Overlap syndrome can progress rapidly to liver cirrhosis and liver failure without timely treatment. This article summarizes the research advances in overlap syndrome of autoimmune liver disease in recent years. [ABSTRACT FROM AUTHOR]