OBJECTIVE: To explore the feasibility of pretreatment nonenhanced magnetic resonance imaging (MRI) in predicting insufficient biochemical response to ursodeoxycholic acid (UDCA) in patients with primary biliary cholangitis (PBC).
METHODS: From January 2009 to April 2022, consecutive PBC patients who were treated with UDCA and underwent nonenhanced MRI within 30 days before treatment were retrospectively enrolled. All MR images were independently evaluated by two blinded radiologists. Uni- and multivariable logistic regression analyses were performed to develop a predictive model for 12-month insufficient biochemical response. Model performances were evaluated by computing the area under the receiver operating characteristic curve (AUC), sensitivity, and specificity.
RESULTS: A total of 74 patients (50.6 ± 11.9 years; 62 females) were included. Three pretreatment MRI features, including hepatomegaly (odds ratio [OR]: 4.580; p = 0.011), periportal hyperintensity on T2-weighted imaging (T2WI) (OR: 4.795, p = 0.008), and narrowing of the bile ducts (OR: 3.491; p = 0.027) were associated with 12-month insufficient biochemical response in the multivariable analysis. A predictive model based on the above indicators had an AUC of 0.781, sensitivity of 85.4%, and specificity of 61.5% for predicting insufficient biochemical response.
CONCLUSIONS: A noninvasive model based on three pretreatment MRI features could accurately predict 12-month insufficient biochemical response to UDCA in patients with PBC. Early identification of PBC patients at increased risk for insufficient response can facilitate the timely initiation of additional treatment.
CONCLUSIONS: A noninvasive predictive model constructed by incorporating three pretreatment MRI features may help identify patients with primary biliary cholangitis at high risk of insufficient biochemical response to ursodeoxycholic acid and facilitate the timely initiation of additional treatment.
CONCLUSIONS: • Noninvasive imaging features based on nonenhanced pretreatment MRI may predict an insufficient biochemical response to UDCA in PBC patients. • A combined model based on three MRI features (hepatomegaly, periportal hyperintensity on T2-weighted imaging, and narrowing of the bile ducts) further improved the predictive efficacy for an insufficient biochemical response to UDCA in PBC patients, with high sensitivity and specificity. • The nomogram of the combined model showed good calibration and predictive efficacy for an insufficient biochemical response to UDCA in PBC patients. In particular, the calibration curve visualised the clinical applicability of the prediction model.

Premature senescence occurs in adult hepatobiliary diseases and worsens the prognosis through deleterious liver remodeling and hepatic dysfunction. Senescence might also arises in biliary atresia (BA), the first cause of pediatric liver transplantation. Since alternatives to transplantation are needed, our aim was to investigate premature senescence in BA and to assess senotherapies in a preclinical model of biliary cirrhosis.
BA liver tissues were prospectively obtained at hepatoportoenterostomy (n=5) and liver transplantation (n=30) and compared to controls (n=10). Senescence was investigated through spatial whole transcriptome analysis, SA-β-gal activity, p16 and p21 expression, γ-H2AX and senescence-associated secretory phenotype (SASP). Human allogenic liver-derived progenitor cells (HALPC) or dasatinib and quercetin (D+Q) were administrated to two-month-old Wistar rats after bile duct ligation (BDL).
Advanced premature senescence was evidenced in BA livers from early stage and continued to progress until liver transplantation. Senescence and SASP were predominant in cholangiocytes, but also present in surrounding hepatocytes. HALPC but not D+Q reduced the early marker of senescence p21 in BDL rats and improved biliary injury (serum γGT and Sox9 expression) and hepatocytes mass loss (Hnf4a).
BA livers displayed advanced cellular senescence at diagnosis that continued to progress until liver transplantation. HALPC reduced early senescence and improved liver disease in a preclinical model of BA, providing encouraging preliminary results regarding the use of senotherapies in pediatric biliary cirrhosis.

UNASSIGNED: Neonatal sclerosing cholangitis (NSC) is a rare and severe autosomal recessive inherited liver disease with mutations in DCDC2, commonly requiring liver transplantation (LT) for decompensated biliary cirrhosis in childhood.
UNASSIGNED: The information of four Chinese patients with NSC caused by mutations in DCDC2 from Children\'s Hospital of Fudan University were gathered. The four patients\' clinicopathological and molecular features were summarized by clinical data, liver biopsy, immunohistochemical, and molecular genetic analysis.
UNASSIGNED: All patients presented with jaundice, hepatosplenomegaly, hyperbilirubinemia and bile embolism, and high serum γ-glutamyl transferase activity (GGT). Liver biopsies revealed varying degrees of bile duct hyperplasia, portal-tract inflammation, and/or fibrosis. Whole-exome sequencing (WES) found novel heterozygous variants of c.1024-1G > T /p.? and c.544G > A /p. Gly182Arg in the DCDC2.
UNASSIGNED: This study expands the genetic spectrum of DCDC2 in NSC.

Liver disease affects one-third of patients with cystic fibrosis (CF) and it is one of the major causes of morbidity and mortality in these patients. Historically considered a disease of childhood, its impact is now seen more often in adulthood. The heterogeneous pattern of CF liver disease and its rapid progression to cirrhosis remain a diagnostic challenge and new questions pertaining to the nature of liver involvement have recently been raised. Non-invasive measures to stratify the severity of liver involvement are increasingly used to predict clinical outcomes. A single treatment, ursodeoxycholic acid, has been used to slow progression of liver disease while recent advances in the field of CF treatments are promising. Management of portal hypertension remains challenging but outcomes after liver transplantation are encouraging. While many questions remain unanswered, a growing number of CF patients reach adulthood and will require care for CF liver disease.

BACKGROUND: Worldwide, liver disease is a leading cause of morbidity and mortality. Its pattern can be updated by periodic data collection and analysis.
OBJECTIVE: To update the existing histopathological pattern of liver diseases at the University of Benin Teaching Hospital, Benin City, Edo state, Nigeria.
METHODS: This was a descriptive cross-sectional retrospective study of all cases of liver biopsies diagnosed histologically between 1st of January, 2012 to 31st of December, 2019. Data was obtained from the surgical pathology register and the age, sex and pathological diagnoses of the lesions analysed.
RESULTS: Liver biopsies were reported for a total of 68 patients during the 8-year study period. The ages of the patients ranged from 2 months to 75 years with a mean age and standard deviation of 28.88 ± 23.21 years. The 3 most common histopathological diagnoses were infectious liver disease (38.23%), malignant neoplastic lesions (26.47%) and cholestatic liver disease (16.17%), with the peak age of infectious liver disease (30-39years) preceding that of malignant liver disease (60-69years) by 3 decades.
CONCLUSIONS: This study shows that chronic hepatitis B infection was the commonest histologic finding of liver biopsies at the University of Benin Teaching Hospital. Screening, vaccination and prompt treatment of hepatitis B infection are recommended to reduce the burden of liver disease. Liver biopsies have been updated to include biliary cirrhosis, biliary atresia, cystic liver diseases and glycogen storage disease thus expanding the scope of histopathological diagnosis that could be made on liver tissue biopsies in this environment.
UNASSIGNED: À l’échelle mondiale, les maladies du foie sont l’une des principales causes de morbidité et de mortalité. Son modèle peut être mis à jour par la collecte et l’analyse périodiques de données.
UNASSIGNED: Mettre à jour le modèle histopathologique existant des maladies du foie à l’hôpital universitaire de l’Université du Bénin, à Benin City, dans l’État d’Edo, au Nigéria.
UNASSIGNED: Il s’agissait d’une étude rétrospective transversale descriptive de tous les cas de biopsies hépatiques diagnostiquées histologiquement entre le 1er janvier 2012 et le 31 décembre 2019. Les données ont été obtenues à partir du registre de pathologie chirurgicale et de l’âge, du sexe et des diagnostics pathologiques. des lésions analysées.
UNASSIGNED: Des biopsies hépatiques ont été rapportées pour un total de 68 patients au cours de la période d’étude de 8 ans. L’âge des patients variait de 2 mois à 75 ans avec un âge moyen et un écart type de 28,88 ± 23,21 ans. Les 3 diagnostics histopathologiques les plus fréquents étaient une maladie hépatique infectieuse (38,23 %), des lésions néoplasiques malignes (26,47 %) et une maladie hépatique cholestatique (16,17 %), l’âge maximal de la maladie hépatique infectieuse (30-39 ans) précédant celui de la maladie hépatique maligne. (60-69 ans) par 3 décennies.
CONCLUSIONS: Cette étude montre que l’infection chronique par l’hépatite B était la constatation histologique la plus fréquente des biopsies hépatiques au CHU de l’Université du Bénin. Le dépistage, la vaccination et le traitement rapide de l’hépatite B sont recommandés pour réduire le fardeau des maladies du foie. Les biopsies hépatiques ont été mises à jour pour inclure la cirrhose biliaire, l’atrésie biliaire, les maladies kystiques du foie et la maladie du stockage du glycogène, élargissant ainsi la portée du diagnostic histopathologique qui pourrait être effectué sur les biopsies des tissus hépatiques dans cet environnement.
UNASSIGNED: Biopsie hépatique, hépatite chronique, carcinome hépatocellulaire, cholangiocarcinome, atrésie biliaire, cirrhose biliaire, maladie du stockage du glycogène.

BACKGROUND: Cholangiocarcinoma and secondary biliary cirrhosis can develop after liver resection for hepatolithiasis and are causes of hepatolithiasis-related death. We determined potential risk factors for hepatolithiasis-related death and subsequent cholangiocarcinoma, including precancerous lesions such as biliary intraepithelial neoplasia (BilIN) and intraductal papillary neoplasm of the bile duct, in patients undergoing liver resection for hepatolithiasis.
METHODS: The study cohort included 62 patients who underwent liver resection for hepatolithiasis without concomitant cholangiocarcinoma and had surgical specimens available for pathological examination. Univariate and multivariate analyses were conducted to examine risk factors associated with subsequent cholangiocarcinoma after hepatolithiasis and hepatolithiasis-related death. In 28 patients with BilIN lesions, the specimens were immunohistochemically stained for γ-H2AX and S100P.
RESULTS: In the study cohort, the causes of death were subsequent cholangiocarcinoma, biliary cirrhosis, and other diseases in 5, 3, and 7 patients, respectively. Liver atrophy, precancerous lesions, postoperative repeated cholangitis, and jaundice for ≥1 week during the follow-up period were risk factors for hepatolithiasis-related death. Multivariate analysis showed that liver atrophy and precancerous lesions were independent risk factors for hepatolithiasis-related death. Liver atrophy or precancerous lesions were also risk factors for subsequent cholangiocarcinoma by univariate analysis. The positive expression of γ-H2AX and S100P was observed in 18 and 14 of the 28 BilIN lesions, respectively.
CONCLUSIONS: Liver atrophy and precancerous lesions with malignant transformation were risk factors not only for subsequent cholangiocarcinoma but also hepatolithiasis-related death after liver resection for hepatolithiasis, indicating that long-term follow-up is necessary even after liver resection in patients harboring these risk factors.
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暂无摘要。

BACKGROUND: Langerhans cell histiocytosis (LCH) is an abnormal accumulation of Langerhans cells in various organs that sometimes induces organ dysfunction. LCH can affect the liver, resulting in sclerosing cholangitis and biliary cirrhosis. However, liver and bile duct involvement is usually observed in the disseminated form of LCH. We herein report a rare case of LCH localized only in the extrahepatic bile duct that resulted in severe liver cirrhosis.
METHODS: A 3-year-old boy with elevated liver enzymes, obstructive jaundice, and dilation of the common bile duct was referred to our institution. Contrast-enhanced computed tomography showed atrophy of the right hepatic lobe, relative hypertrophy of the left hepatic lobe, choledocholiths, and biliary debris extensively with biliary duct dilation. Magnetic resonance cholangiopancreatography revealed dilation of the intrahepatic and extrahepatic bile ducts and multiple choleliths in the gallbladder and common bile duct. Laparoscopic cholecystectomy, intraoperative cholangiography, liver biopsy, and gastrointestinal fiberscopy were performed. A liver specimen showed severe biliary cirrhosis due to sclerosing cholangitis. The patient then underwent living-donor liver transplantation because of severe liver cirrhosis 3 months after the first surgery. The common bile duct was not suitable for duct-to-duct anastomosis and was resected because of severe inflammation. Histologic sections of the common bile duct showed histiocytic cell proliferation. Immunohistochemistry revealed histiocytoses that were positive for Langerin, S-100 protein, and CD1a. However, no histiocytic cell proliferation was noted in the liver tissue. The definitive diagnosis was LCH localized to the extrahepatic bile duct. LCH in the extrahepatic bile duct seemed to cause sclerosing cholangitis. The patient was discharged uneventfully 2 months after living-donor liver transplantation.
CONCLUSIONS: LCH localized to the extrahepatic bile duct is extremely rare; however, LCH can still affect the extrahepatic bile ducts on occasion. LCH should be considered as a differential diagnosis if pediatric patients show the presence of sclerosing cholangitis.

UNASSIGNED: Abdominal complications are often the first indications for cystic fibrosis (CF), a multiorgan disease. A broad range of abdominal manifestations are associated with the disease, including gastrointestinal abnormalities (such as meconium ileus in newborns and distal intestinal obstruction syndrome in older children) and hepatobiliary alterations (e.g., cholelithiasis, microgallbladder, hepatosteatosis, biliary cirrhosis). A characteristic finding is pancreatic involvement, which leads to exocrine and over the course of time to endocrine insufficiency.
UNASSIGNED: Ultrasonography is the preferred and often sole modality for a precise diagnosis of abdominal CF manifestations. However, all imaging modalities can be used, depending on the pathology: X‑ray, fluoroscopic examinations, computed tomography, magnetic resonance imaging (also with application of magnetic resonance cholangiopancreatography).
UNASSIGNED: Scoring systems are useful for standardized diagnostics. Sonographic findings, described using a scoring system, correlate with clinical symptoms, such as pancreatic lipomatosis with abdominal pain (p = 0.018), flatulence (p = 0.006), and gastroesophageal reflux (p = 0.006).
UNASSIGNED: A standardized approach with structured reporting is important due to the numerous abdominal CF manifestations. To enable precise follow-up analyses, scoring systems based on sonographic findings are excellent.

OBJECTIVE: To evaluate magnetic resonance imaging (MRI) features of the liver in primary biliary cholangitis (PBC).
METHODS: We conducted a multicenter retrospective review on 283 patients with PBC who underwent an MRI between 2007 and 2018. Patients with overlap syndromes were excluded. MRI studies were independently reviewed by two abdominal radiologists for liver morphology, signal intensity, postcontrast enhancement, and decompensation. Liver and spleen volumes and normalized liver apparent diffusion coefficient (nlADC) were also calculated. MRI features were correlated with fibrosis stage among a subset of patients who had a liver biopsy within 6 months (n = 72).
RESULTS: The study population was comprised of 283 patients (89% females) and a mean ± SD age of 59.4 ± 11.8 years. Lymphadenopathy (78.1%), periportal hyperintensity (36.7%), and periportal halo sign (27.6%) were the most common features. A positive correlation was found between fibrosis stage and spleen size (r = 0.457, p < 0.001), spleen volume (r = 0.557, p < 0.001) and portal vein diameter (r = 0.287, p = 0.013), and a negative correlation with nlADC (r = - 0.332, p = 0.011). Fibrosis stage also correlated with the presence of surface nodularity (p < 0.001), periportal halo sign (p = 0.04), collaterals (p = 0.033), and splenomegaly (p = 0.002). No significant differences in nlADC values were found in different fibrosis stages. Spleen size and volume were significantly higher in patients with ascites and collaterals (< 0.001). The periportal halo sign was present only in patients with significant fibrosis. None of the MRI features significantly correlated with inflammation grade.
CONCLUSIONS: In PBC, presence of periportal halo sign correlates with significant fibrosis. Heterogeneous T2W intensity, heterogeneous postcontrast enhancement, collaterals, spleen size, and spleen volume correlate with fibrosis stage and may be useful for predicting advanced fibrosis.
CONCLUSIONS: • The presence of periportal halo sign is indicative for significant fibrosis in primary biliary cholangitis. • Liver parenchymal heterogeneous T2 signal intensity, heterogeneous postcontrast enhancement, collaterals, spleen size, and spleen volume correlate with fibrosis stages in PBC and may be useful for predicting advanced fibrosis.