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.

BACKGROUND: A biliary inflammatory myofibroblastic tumor (IMT) is a rare type of mesenchymoma that, although it has a broad age spectrum, usually occurs in adults. Diagnosis is difficult because biliary IMTs often exhibit nonspecific clinical symptoms and imaging features, resulting in delayed or inappropriate treatment. Although most IMTs are benign, some show malignant properties such as infiltration, recurrence, and metastasis.
METHODS: Here, we retrospectively describe a 10-month-old infant who was admitted to our hospital due to stubborn jaundice. The patient responded poorly to routine medical treatment and his clinical manifestations and laboratory tests lacked specificity, so we turned to repeated ultrasound scans and other imaging examinations. As both hepatosplenic ultrasonography and diffusion-weighted magnetic resonance imaging demonstrated a space-occupying lesion, an exploratory laparotomy was performed. The final diagnosis made over two mo after the disease onset was infant biliary cirrhosis caused by a biliary IMT, which partially infiltrated into the liver. This infant is the youngest case of biliary IMTs that has been reported till now. The patient underwent an incomplete resection of the mass and Kasai Portoenterostomy. However, because of cirrhosis, he also received a paternal liver transplant. Since some IMTs show malignant properties, we proceeded with a three-year of follow-up; however, no recurrence or metastasis has been noted.
CONCLUSIONS: Neoplastic disease such as IMTs should be considered when routine medical treatment of obstructive jaundice is not successful. Observation of dynamic imaging changes is helpful for diagnosis. Periodic follow-up is necessary for IMTs.

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: Despite being a benign disease, hepatolithiasis has a poor prognosis because of its intractable nature and frequent recurrence. Nonsurgical treatment is associated with high incidences of residual and recurrent stones. Consequently, surgery via hepatic lobectomy or segmental hepatectomy has become the main treatment modality. Clinical management and resolution of complicated hepatolithiasis with bilateral or diffuse intrahepatic stones remain very difficult and challenging. Repeated cholangitis and calculous obstruction may result in secondary biliary cirrhosis, a limiting factor in the treatment of hepatolithiasis.
METHODS: A 53-year-old woman with a 5-year history of intermittent abdominal pain and fever was admitted to the hepatopancreatobiliary surgery department following worsening symptoms over a 3-d period. Blood tests revealed elevated transaminases, alkaline phosphatase, γ-glutamyl transpeptidase, and total bilirubin, as well as anemia. Magnetic resonance cholangiopancreatography showed dilatation of the intrahepatic, left and right hepatic, common hepatic, and common bile ducts, and multiple short T2 signals in the intrahepatic and common bile ducts. Abdominal computed tomography showed splenomegaly and splenic varices. The diagnosis was bilateral hepatolithiasis and choledocholithiasis with cholangitis. Surgical treatment included hepatectomy of segments II and III, cholangioplasty, left hepaticolithotomy, second biliary duct exploration, choledocholithotomy, T-tube drainage, and accretion lysis. Surgical and pathological findings confirmed secondary biliary cirrhosis. Liver-protective therapy and anti-infectives were administered. The patient developed liver and respiratory failure, severe abdominal infection, and septicemia. Eventually, her family elected to discontinue treatment.
CONCLUSIONS: Liver transplantation, rather than hepatectomy, might be a treatment option for complicated bilateral hepatolithiasis with secondary liver cirrhosis.

Background: Ciliopathies are rare diseases causing renal and extrarenal manifestations. Here, we report the case of a ciliopathy induced by a homozygous pathogenic variant in the TTC21B gene. Case Description: A 47-year-old patient started hemodialysis for chronic kidney disease (CKD) of unknown origin. She presented with early onset of hypertension, pre-eclampsia, myopia and cirrhosis. Renal biopsy showed mild interstitial fibrosis, tubular atrophy, and moderate arteriosclerosis while liver pathology demonstrates grade B biliary cirrhosis. Family history revealed several cases of early-onset severe hypertension and one case of end-stage renal disease (ESRD) needing kidney transplantation at twenty years of age. Clinical exome sequencing showed homozygosis for the pathogenic variant c.626C>T (p.Pro209Leu) in the TTC21B gene. The patient underwent combined liver-renal transplantation with an excellent renal and hepatic graft outcome. Conclusions: TTC21B gene mutations can lead heterogeneous to clinical manifestations and represent an underappreciated cause of ESRD. The paradigm in diagnosis of CKD of early onset and/or of unknown origin is changing and genetic counseling should be performed in all patients and families that meet those criteria. Renal or combined liver-renal transplantation represents the best option for patients suffering from those diseases in terms of prognosis and quality of life.

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.

Hydatid disease is an ongoing issue in endemic areas. Hydatid cysts can be seen in any organ but, liver is one of the most common involved organs. Cystobiliary communication as an overwhelming complication of hepatic hydatid cysts can contribute to the obstructive jaundice, cholangitis, sepsis and even biliary cirrhosis if left untreated. The patient we are trying to present is a 61-year-old farmer who presented with obstructive jaundice, multiple common bile duct stones and biliary cirrhosis attributed to a long-lasting untreated hepatic hydatid cyst. Portal hypertension is introduced to be an uncommon presentation of hydatid cyst. Extrinsic compression of the porta hepatis and obstruction of inferior vena cava are amongst major causes of hydatidosis leading up to portal hypertension as reported in the literature. Portal hypertension in the presented case is proposed to emerge from long-lasting cystobiliary communication ending in biliary cirrhosis.

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.
.