Hepatic Magnetic Resonance Imaging Research Articles

Converging Pathways between Metabolic Dysfunction-Associated Steatotic Liver Disease (MASLD) and Diabetes in Children.

In the past thirty years, childhood obesity rates have risen significantly worldwide, affecting over 340 million children in affluent nations. This surge is intricately tied to metabolic disorders, notably insulin resistance, type 2 diabetes mellitus (T2DM), and the continually evolving spectrum of metabolic-associated (dysfunction) steatotic liver disease (MASLD). This review underscores the alarming escalation of childhood obesity and delves comprehensively into the evolving and dynamic changes of nomenclature surrounding diverse conditions of hepatic steatosis, from the initial recognition of non-alcoholic fatty liver disease (NAFLD) to the progressive evolution into MASLD. Moreover, it emphasizes the crucial role of pediatric endocrinologists in thoroughly and accurately investigating MASLD onset in children with T2DM, where each condition influences and exacerbates the progression of the other. This review critically highlights the inadequacies of current screening strategies and diagnosis, stressing the need for a paradigm shift. A proposed solution involves the integration of hepatic magnetic resonance imaging assessment into the diagnostic arsenal for children showing insufficient glycemic control and weight loss post-T2DM diagnosis, thereby complementing conventional liver enzyme testing. This holistic approach aims to significantly enhance diagnostic precision, fostering improved outcomes in this vulnerable high-risk pediatric population.

Reducing transient severe motion artifacts of gadoxetate disodium-enhanced MRI by oxygen inhalation: effective for pleural effusion but not ascites.

To evaluate the efficacy of low-flow oxygen inhalation in mitigating transient severe motion (TSM) artifacts associated with gadoxetate disodium-enhanced hepatic magnetic resonance imaging (MRI). Patients undergoing gadoxetate disodium-enhanced MRI were included. During the examination, the experimental group received oxygen at 2L/min via nasal cannula, while the control group did not. Images and TSM scores were evaluated and compared across precontrast, arterial, venous, and hepatobiliary phases. Subgroup analyses were conducted based on the presence of pleural effusion or ascites. A total of 325 patients were included. The motion scores were highest in the arterial phase and lowest in the hepatobiliary phase in both groups, but were significantly lower in the experimental group (p < 0.05). The incidence of TSM was significantly lower in the experimental group (3.29%) compared to the control group (13.29%, p = 0.01). While pleural effusion was associated with reduced image quality in both groups (p < 0.05), the image quality in the pleural effusion category was higher in the experimental group than in the control group. Oxygen inhalation showed limited efficacy in mitigating TSM related to ascites. Low-flow oxygen inhalation can effectively reduce the occurrence of gadoxetate disodium-related TSM. Pleural effusion may impair respiratory function and contribute to TSM, which can be alleviated by oxygen supplementation. However, Oxygen inhalation is less effective under the condition of ascites.

Facile Synthesis of Rigid Binuclear Manganese Complexes for Magnetic Resonance Angiography and SLC39A14-Mediated Hepatic Imaging.

Manganese(II)-based contrast agents (MBCAs) are potential candidates for gadolinium-free enhanced magnetic resonance imaging (MRI). In this work, a rigid binuclear MBCA (Mn2-PhDTA2) with a zero-length linker was developed via facile synthetic routes, while the other dimer (Mn2-TPA-PhDTA2) with a longer rigid linker was also synthesized via more complex steps. Although the molecular weight of Mn2-PhDTA2 is lower than that of Mn2-TPA-PhDTA2, their T1 relaxivities are similar, being increased by over 71% compared to the mononuclear Mn-PhDTA. In the presence of serum albumin, the relaxivity of Mn2-PhDTA2 was slightly lower than that of Mn2-TPA-PhDTA2, possibly due to the lower affinity constant. The transmetalation reaction with copper(II) ions confirmed that Mn2-PhDTA2 has an ideal kinetic inertness with a dissociation half-life of approximately 10.4 h under physiological conditions. In the variable-temperature 17O NMR study, both Mn-PhDTA and Mn2-PhDTA2 demonstrated a similar estimated q close to 1, indicating the formation of monohydrated complexes with each manganese(II) ion. In addition, Mn2-PhDTA2 demonstrated a superior contrast enhancement to Mn-PhDTA in in vivo vascular and hepatic MRI and can be rapidly cleared through a dual hepatic and renal excretion pattern. The hepatic uptake mechanism of Mn2-PhDTA2 mediated by SLC39A14 was validated in cellular uptake studies.

An Uncommon Presentation of Hepatocellular Carcinoma

Hepatocellular carcinoma (HCC) is the most frequent type of primary liver cancer, with an important characteristic being the development on a background of cirrhosis. HCC in non-cirrhotic liver is rare. We report a case of an HCC on a non-cirrhotic liver in a 66-year-old patient who was admitted with a 3 months history of right hypochondriac pain and weight loss. He had a medical history of hypertension, type 2 diabetes, obesity, and no alcohol abuse. Abdominal computed tomography (CT) and hepatic magnetic resonance imaging (MRI) showed a 115x100x110mm mass in the segment V and VIII, hypodense with heterogeneous and early arterial enhancement, and clearing in portal and late phase. He underwent a liver biopsy that confirmed an HCC without underlying cirrhosis. Surgical treatment was not an option since our patient presented multiple risk factors of recurrence. The patient underwent two sessions of transarterial chemoembolization (TACE) but the follow-up showed signs of progression.

Ultrasound Attenuation Imaging vs MRI-PDFF, Echogenicity and Liver Function for Assessing Degree of Steatosis in NAFLD and Non-NAFLD Patients.

Nonalcoholic fatty liver disease (NAFLD) is a primary cause of parenchymal liver disease globally. There are currently several methods available to test the degree of steatosis in NAFLD patients, but all have drawbacks that limit their use.The objective of this study is to determine if a new technique, ultrasound (US) attenuation imaging (ATI), correlates with magnetic resonance proton density fat fraction imaging and hepatic echogenicity as seen on gray scale US imaging.Fifty-four patients were recruited at the University of Washington Medical Center from individuals who had already been scheduled for hepatic US or magnetic resonance imaging (MRI). All participants then underwent both hepatic MRI proton density fat fraction and US. Ultrasound images were then evaluated using ATI with 2 observers who individually determined relative grayscale echogenicity.Analysis showed positive correlation between ATI- and MRI-determined fat percentage in the case group (Spearman correlation: 0.50; P = 0.015). Furthermore, participants with NAFLD tended to have a higher ATI than controls (median: 0.70 vs 0.54 dB/cm/MHz; P < 0.001).This study demonstrates that US ATI combined with grayscale imaging is an effective way of assessing the degree of steatosis in patients with moderate to severe NAFLD.

Machine learning-based radiomics analysis of preoperative functional liver reserve with MRI and CT image

ObjectiveThe indocyanine green retention rate at 15 min (ICG-R15) is a useful tool to evaluate the functional liver reserve before hepatectomy for liver cancer. Taking ICG-R15 as criteria, we investigated the ability of a machine learning (ML)-based radiomics model produced by Gd-EOB-DTPA-enhanced hepatic magnetic resonance imaging (MRI) or contrast-enhanced computed tomography (CT) image in evaluating functional liver reserve of hepatocellular carcinoma (HCC) patients.MethodsA total of 190 HCC patients with CT, among whom 112 also with MR, were retrospectively enrolled and randomly classified into a training dataset (CT: n = 133, MR: n = 78) and a test dataset (CT: n = 57, MR: n = 34). Then, radiomics features from Gd-EOB-DTPA MRI and CT images were extracted. The features associated with the ICG-R15 classification were selected. Five ML classifiers were used for the ML-model investigation. The accuracy (ACC) and the area under curve (AUC) of receiver operating characteristic (ROC) with 95% confidence intervals (CI) were utilized for ML-model performance evaluation.ResultsA total of 107 different radiomics features were extracted from MRI and CT, respectively. The features related to ICG-R15 which was classified into 10%, 20% and 30% were selected. In MRI groups, classifier XGBoost performed best with its AUC = 0.917 and ACC = 0.882 when the threshold was set as ICG-R15 = 10%. When ICG-R15 = 20%, classifier Random Forest performed best with AUC = 0.979 and ACC = 0.882. When ICG-R15 = 30%, classifier XGBoost performed best with AUC = 0.961 and ACC = 0.941. For CT groups, the classifier XGBoost performed best when ICG-R15 = 10% with AUC = 0.822 and ACC = 0.842. When ICG-R15 = 20%, classifier SVM performed best with AUC = 0.860 and ACC = 0.842. When ICG-R15 = 30%, classifier XGBoost performed best with AUC = 0.938 and ACC = 0.965.ConclusionsBoth the MRI- and CT-based machine learning models are proved to be valuable noninvasive methods for functional liver reserve evaluation.

Effect of beta-blockers on multiple haemodynamics in cirrhosis: A cross-over study by MR-imaging and hepatic vein catheterization.

Non-selective beta-blockers (NSBB) are widely used in the treatment of patients with cirrhosis. Only about 50% respond with a sufficient reduction in their hepatic venous pressure gradient (HVPG) and NSBB may induce detrimental cardiac and renal effects in the presence of severe decompensation. We aimed to assess the effects of NSBB on haemodynamics using magnetic resonance imaging (MRI) and to assess if these haemodynamic changes were related to the disease severity and HVPG response. A prospective cross-over study of 39 patients with cirrhosis. Patients underwent hepatic vein catheterization and MRI with assessments of HVPG, cardiac function, systemic and splanchnic haemodynamics before and after propranolol infusion. Propranolol induced significant decreases in cardiac output (-12%) and blood flow of all vascular compartments, with the largest reductions seen in the azygos venous (-28%), portal venous (-21%), splenic (-19%) and superior mesenteric artery (-16%) blood flow. Renal artery blood flow fell by -5% in the total cohort, with a more pronounced reduction in patients without ascites than in those with ascites (-8% vs. -3%, p = .01). Twenty-four patients were NSBB responders. Their changes in HVPG after NSBB were not significantly associated with other haemodynamic changes. The changes in cardiac, systemic and splanchnic haemodynamics did not differ between NSBB responders and non-responders. The effects of acute NSBB blockade on renal flow seem to depend on the severity of the hyperdynamic state, with the largest reduction in renal blood flow in compensated patients compared to decompensated patients with cirrhosis. However, future studies are needed to assess the effects of NSBB on haemodynamics and renal blood flow in patients with diuretic-resistant ascites.

Hepatocellular adenoma subtyping by qualitative MRI features and machine learning algorithm of integrated qualitative and quantitative features: a proof-of-concept study

Aim To evaluate hepatocellular adenoma (HCA) subtyping using qualitative magnetic resonance imaging (MRI) features and feasibility of differentiating HCA subtypes using machine learning (ML) of qualitative and quantitative MRI features with histopathology as the reference standard. Materials And Methods This retrospective study included 39 histopathologically subtyped HCAs (13 hepatocyte nuclear factor (HNF)-1-alpha mutated [HHCA], 11 inflammatory [IHCA], one beta-catenin-mutated [BHCA], and 14 unclassified [UHCA]) in 36 patients. HCA subtyping by two blinded radiologists using the proposed schema of qualitative MRI features and using the random forest algorithm was compared against histopathology. For quantitative features, 1,409 radiomic features were extracted after segmentation and reduced to 10 principle components. Support vector machine and logistic regression was applied to assess HCA subtyping. Results Qualitative MRI features with proposed flow chart yielded diagnostic accuracies of 87%, 82%, and 74% for HHCA, IHCA, and UHCA respectively. The ML algorithm based on qualitative MRI features showed AUCs (area under the receiver operating characteristic curve [ROC] curve) of 0.846, 0.642, and 0.766 for diagnosing HHCA, IHCA, and UHCA, respectively. Quantitative radiomic features from portal venous and hepatic venous phase MRI demonstrated AUCs of 0.83 and 0.82, with a sensitivity of 72% and a specificity of 85% in predicting HHCA subtype. Conclusions The proposed schema of integrated qualitative MRI features with ML algorithm provided high accuracy for HCA subtyping while quantitative radiomic features provide value for diagnosis of HHCA. The key qualitative MRI features for differentiating HCA subtypes were concordant between the radiologists and the ML algorithm. These approaches appear promising to better inform clinical management for patients with HCA.

Hemosiderosis in chronic dialysis patients: Monitoring the response to deferasirox by quantitative hepatic magnetic resonance imaging.

Hemosiderosis of chronic dialysis has always been a frequent phenomenon in dialysis; formerly related to blood transfusions before the advent of Erythropoiesis Stimulating Agents (ESA), it is currently in connection with the use of massive doses of injectable iron, to ensure the full therapeutic efficacy of ESA. Few studies have looked at the therapeutic aspect of iron chelators in the dialysis population. We followed 31 dialysis patients treated for secondary hemosiderosis with deferasirox (DFX) at the dose 10mg/kg/day, by hepatic MRI from September 2017 to September 2021, in order to evaluate the efficacy of iron chelators on the reduction of liver iron concentration (LIC). The diagnosis of hemosiderosis was carried for a value of the LIC > 50 μmol/g of dry liver. Chelation resulted in a significant reduction in liver iron burden as measured by liver MRI: (201.4 ± 179.9 vs. 122.6 ± 154.3μmol/g liver) (p=0.000) and in mean ferritin level: (2058.8 ± 2004.9 vs. 644.2 ± 456.6ng/mL) (p=0.002). A gain of 1.1g/dL in mean hemoglobin level: (10.5 ± 1.6 vs. 11.6 ± 2.0g/dL) (p=0.006). A significant increase in mean albumin level: (43 ± 5.5 to 46.2 ± 6.1g/L) (p=0.04). The therapeutic response was clearly influenced by the cause of overload, longer in polytransfused patients (p=0.023) and the degree of overload assessed by MRI (p=0.003) and ferritin level (p=0.04). DFX, prescribed at a dose of 10mg/kg/day, resulted in a significant reduction in hepatic iron burden as measured by liver MRI and ferritin. The therapeutic response was clearly influenced by blood transfusions and the degree of iron overload.

The value of contrast-enhanced portal vein imaging at the hepatobiliary phase obtained with gadobenate dimeglumine for predicting decompensation and transplant-free survival in chronic liver disease.

To investigate the value of contrast-enhanced portal vein imaging at the hepatobiliary phase obtained with gadobenate dimeglumine for predicting clinical outcomes in patients with chronic liver disease (CLD). Three hundred and fourteen CLD patients who underwent gadobenate dimeglumine-enhanced hepatic magnetic resonance imaging were stratified into three groups: nonadvanced CLD (n = 116), compensated advanced CLD (n = 120), and decompensated advanced CLD (n = 78) groups. The liver-to-portal vein contrast ratio (LPC) and liver-spleen contrast ratio (LSC) at the hepatobiliary phase were measured. The value of LPC for predicting hepatic decompensation and transplant-free survival was assessed using Cox regression analysis and Kaplan-Meier analysis. The diagnostic performance of LPC was significantly better than LSC in evaluating the severity of CLD. During a median follow-up period of 53.0months, the LPC was a significant predictor for hepatic decompensation (p < 0.001) in patients with compensated advanced CLD. The predictive performance of LPC was higher than that of the model for end-stage liver disease score (p = 0.006). With the optimal cut-off value, patients with LPC ≤ 0.98 had a higher cumulative incidence of hepatic decompensation than patients with LPC > 0.98 (p < 0.001). The LPC was also a significant predictive factor for transplant-free survival in patients with compensated advanced CLD (p = 0.007) and those with decompensated advanced CLD (p = 0.002). Contrast-enhanced portal vein imaging at the hepatobiliary phase obtained with gadobenate dimeglumine is a valuable imaging biomarker for predicting hepatic decompensation and transplant-free survival in CLD patients. • The liver-to-portal vein contrast ratio (LPC) significantly outperformed liver-spleen contrast ratio in evaluating the severity of chronic liver disease. • The LPC was a significant predictor for hepatic decompensation in patients with compensated advanced chronic liver disease. • The LPC was a significant predictor for transplant-free survival in patients with compensated and those with decompensated advanced chronic liver disease.

Unusual Neurological Manifestation of Langerhans Cell Histiocytosis in an Adult.

Langerhans Cell Histiocytosis is a rare disorder characterized by an abnormal proliferation of Langerhans cells in one or multiple organs. It rarely presents with a central vestibular involvement. We report a case of a multisystem Langerhans Cell Histiocytosis with mucosal, hepatic, and neurological involvement, presenting with dizziness and balance disorders. A 39-year-old woman with a histopathologically confirmed mucosal palatal Langerhans Cell Histiocytosis presented with a history of dizziness for a year. Vestibular examination revealed a saccadic eyes pursuit, an up beating spontaneous nystagmus, a bilateral gaze nystagmus and a prolonged positional nystagmus, in the supine roll test. Pure tone audiometry showed a slight left sensorineural hearing loss at the 8000Hz frequency. Computed tomography (CT) scan showed a bilateral maxillary sinus fullness and a peripheral osteosclerosis of the surrounding bony walls. Hepatic magnetic resonance imaging (MRI) showed a typical hepatic involvement with a hepatomegaly with countless cysts. Temporal bone CT scan and MRI were normal. Cerebral MRI showed an hyperintense nodular signal at T2 FLAIR weighted images lateral to the right pons, at the level of the left middle cerebellar peduncle and at the left mesencephalon. Balance disorders can rarely present a sign of a degenerative neurological cerebellar involvement. Such a rare manifestation can present in different neurological disorders such as Langerhans' cell Histiocytosis.

Clinical application of deep learning and radiomics in hepatic disease imaging: a systematic scoping review.

Artificial intelligence (AI) has begun to play a pivotal role in hepatic imaging. This systematic scoping review summarizes the latest progress of AI in evaluating hepatic diseases based on computed tomography (CT) and magnetic resonance (MR) imaging. We searched PubMed and Web of Science for publications, using terms related to deep learning, radiomics, imaging methods (CT or MR), and the liver. Two reviewers independently selected articles and extracted data from each eligible article. The Quality Assessment of Diagnostic Accuracy Studies-AI (QUADAS-AI) tool was used to assess the risk of bias and concerns regarding applicability. The screening identified 45 high-quality publications from 235 candidates, including 8 on diffuse liver diseases and 37 on focal liver lesions. Nine studies used deep learning and 36 studies used radiomics. All 45 studies were rated as low risk of bias in patient selection and workflow, but 36 (80%) were rated as high risk of bias in the index test because they lacked external validation. In terms of concerns regarding applicability, all 45 studies were rated as low concerns. These studies demonstrated that deep learning and radiomics can evaluate liver fibrosis, cirrhosis, portal hypertension, and a series of complications caused by cirrhosis, predict the prognosis of malignant hepatic tumors, and differentiate focal hepatic lesions. The latest studies have shown that deep learning and radiomics based on hepatic CT and MR imaging have potential application value in the diagnosis, treatment evaluation, and prognosis prediction of common liver diseases. The AI methods may become useful tools to support clinical decision-making in the future. Deep learning and radiomics have shown their potential in the diagnosis, treatment evaluation, and prognosis prediction of a series of common diffuse liver diseases and focal liver lesions.

A rare genetic variant in the manganese transporter SLC30A10 and elevated liver enzymes in the general population.

A genetic variant in the manganese transporter SLC30A10 (rs188273166, p.Thr95Ile) was associated with increased plasma alanine transaminase (ALT) in a recent genome-wide association study in the UK Biobank (UKB). The aims of the present study were to test the association of rs188273166 with ALT in an independent cohort, and to begin to assess the clinical, hepatic, and biochemical phenotypes associated with the variant. We included n = 334,886 white participants from UKB, including 14,462 with hepatic magnetic resonance imaging (MRI), and n = 113,612 individuals from the Copenhagen City Heart Study and the Copenhagen General Population Study combined. Genotyping SLC30A10 p.Thr95Ile identified 816 heterozygotes in the UKB and 111 heterozygotes in the Copenhagen cohort. Compared to noncarriers, heterozygotes had 4 and 5U/L higher levels of ALT in the UKB and Copenhagen cohort, respectively, and 3U/L higher plasma aspartate transaminase and gamma-glutamyl transferase in the UKB. Heterozygotes also had higher corrected T1 on liver MRI, a marker of hepatic inflammation (p = 4 × 10-7), but no change in MRI-quantified steatosis (p = 0.57). Plasma manganese was within the normal range in nine heterozygotes that provided new blood samples. SLC30A10 p.Thr95Ile heterozygotes had an eightfold increased risk of biliary tract cancer in UKB (p = 4 × 10-7), but this association was not replicated in the Copenhagen cohort. SLC30A10 p.Thr95Ile was associated with elevated liver enzymes in two large general population cohorts, and with MRI-quantified hepatic inflammation. A rare genetic variant (p.Thr95Ile) in the manganese transporter SLC30A10 is associated with elevated plasma alanine transaminase (ALT) and higher corrected T1 on liver MRI, markers of liver inflammation. These data support that the variant may increase the risk of liver disease.

GH/IGF-1 axis in a large cohort of ß-thalassemia major adult patients: a cross-sectional study

PurposeGH deficit (GHD) could represent an endocrine issue in ß-Thalassemia Major (ßTM) patients. GH/IGF-1 axis has not been extensively explored in ßTM adults, so far. We aim to assess GHD and IGF-1 deficiency prevalence in ßTM adult population, focusing on the relationship with liver disease.MethodsCross-sectional multi-centre study conducted on 81 adult ßTM patients (44 males, mean age 41 ± 8 years) on transfusion and chelation therapy. GHD was investigated by GHRH + arginine test. IGF-1 levels, routine biochemical exams, Fibroscan, Hepatic Magnetic Resonance Imaging (MRI) and pituitary MRI were collected.ResultsEighteen patients were affected by GHD and 63 were not (nGHD) according to GHRH + arginine test, while basal GH levels did not differ. GHD was associated with a higher BMI and a worse lipid profile (p < 0.05). No significant differences were observed regarding liver function between the two groups. Pituitary MRI scan was normal except for one case of empty sella. The 94.4% and 93.6% of GHD and nGHD, respectively, presented lower IGF-1 levels than the reference range, and mean IGF-1 SDS was significantly lower in GHD patients.ConclusionGHD is frequent in adult ßTM patients and is associated with higher BMI and worse lipid profile. nGHD patients present lower IGF-1 levels as well. There was no relationship between IGF-1 levels and liver disease. Further, multicentric studies with larger cohorts and standardized diagnostic protocols are needed.

Use of blood oxygen level-dependent magnetic resonance imaging to detect acute cellular rejection post-liver transplantation.

Acute cellular rejection (ACR) is a major immune occurrence post-liver transplant that can cause abnormal liver function. Blood oxygen level-dependent (BOLD) magnetic resonance imaging (MRI) can be used to evaluate liver disease, but it has not been utilized in the diagnosis of ACR post-liver transplant. Therefore, the purpose of this study is to evaluate the diagnostic performance of BOLD MRI and to monitor treatment response in recipients with ACR. This prospective study was approved by the local institutional review board. Fifty-five recipients with highly suspected ACR were enrolled in this study. Each patient underwent hepatic BOLD MRI, blood biochemistry, and biopsy before treatment. Of 55 patients, 19 recipients with ACR received a follow-up MRI after treatment. After obtaining the R2* maps, five regions-of-interest were placed on liver parenchyma to estimate the mean R2* values for statistical analysis. Receiver operating characteristic curve (ROC) analysis was performed to assess the diagnostic performance of R2* values in detecting patients with ACR. The histopathologic results showed that 27 recipients had ACR (14 mild, 11 moderate, and 2 severe) and their hepatic R2* values were significantly lower than those of patients without ACR. ROC analysis revealed that the sensitivity and specificity of the R2* values for detection of ACR were 82.1% and 89.9%, respectively. Moreover, the R2* values and liver function in patients with ACR significantly increased after immunosuppressive treatment. The non-invasive BOLD MRI technique may be useful for assessment of hepatic ACR and monitoring of treatment response after immunosuppressive therapy. • Patients with acute cellular rejection post-liver transplant exhibited significantly decreased R2* values in liver parenchyma. • R2* values and liver function were significantly increased after immunosuppressive therapy. • R2* values were constructive indicators in detecting acute cellular rejection due to their high sensitivity and specificity.

Presentation of Congenital Portosystemic Shunts in Children.

Background: Congenital portosystemic shunts (CPSS) are rare vascular anomalies resulting in communications between the portal venous system and the systemic venous circulation, affecting an estimated 30,000 to 50,000 live births. CPSS can present at any age as a multi-system disease of variable severity mimicking both common and rare pediatric conditions. Case presentations: Case A: A vascular malformation was identified in the liver of a 10-year-old girl with tall stature, advanced somatic maturation, insulin resistance with hyperinsulinemia, hyperandrogenemia and transient hematuria. Work-up also suggested elevated pulmonary pressures. Case B: A young girl with trisomy 8 mosaicism with a history of neonatal hypoglycemia, transient neonatal cholestasis and tall stature presented newly increased aminotransferase levels at 6 years of age. Case C: A 3-year-old boy with speech delay, tall stature and abdominal pain underwent abdominal ultrasound (US) showing multiple liver nodules, diagnosed as liver hemangiomas by hepatic magnetic resonance imaging (MRI). Management and outcome: After identification of a venous malformation on liver Doppler US, all three patients were referred to a specialized liver center for further work-up within 12 to 18 months from diagnosis. Angio-computed tomography (CT) scan confirmed the presence of either an intrahepatic or extrahepatic CPSS with multiples liver nodules. All three had a hyperintense signal in the globus pallidus on T1 weighted cerebral MRI. Right heart catheterization confirmed pulmonary hypertension in cases A and C. Shunts were closed either using an endovascular or surgical approach. Liver nodules were either surgically removed if there was a risk of malignant degeneration or closely monitored by serial imaging when benign. Conclusion: These cases illustrate most of the common chief complaints and manifestations of CPSS. Liver Doppler US is the key to diagnosis. Considering portosystemic shunts in the diagnostic work-up of a patient with unexplained endocrine, liver, gastro-intestinal, cardiovascular, hematological, renal or neurocognitive disorder is important as prompt referral to a specialized center may significantly impact patient outcome.

Investigation of Changing Imaging Contrast Using Compressed Sensing in Gd-EOB-DTPA Hepatocyte Phase

In recent years, gadolinium ethoxybenzyl diethylenetriamine pantaacetic acid (Gd-EOB-DTPA) has been commonly used in magnetic resonance imaging (MRI) for liver contrast studies. The purpose of this study was to investigate the effect of Hyper SENSE factor on contrast in the Gd-EOB-DTPA hepatocyte phase. The subjects were 21 patients [16 males and 5 females, aged 50-94 years (mean age 68±12years)] who underwent hepatic contrast-enhanced MRI with Gd-EOB-DTPA from March 2021 to December 2021. The contrast ratios (CRs) of liver/spleen and liver/tumor were measured when the Hyper SENSE factor was changed to 1.0, 1.4 and 1.8. As a result, there was no statistically significant difference in signal intensity and CR with the change in the Hyper SENSE factor. In this study, the acquisition time can be shortened without decreasing the CR; therefore, it was suggested to improve MR images to avoid motion artifact.

Hepatic Adenomatosis in a Young Asymptomatic but High-risk Patient: An Incidental Diagnosis with Potentially Severe Clinical Implications.

Hepatic adenomatosis (HA) is a very rare condition and defined as the presence of 10 or more adenomas in an otherwise normal liver. HA has an incidence of 10–24% in patient with hepatic adenoma and it is more common in women. Most patients with HA are asymptomatic with a normal liver function test and half of cases are detected incidentally on imaging. Although HA is considered a benign disease, some patients may develop potentially fatal complications, such as hypovolaemic shock due to rupture of the liver lesion or malignant transformation to hepatocellular carcinoma.We report the case of a 29-year-old woman who presented to the emergency room after a car accident. Whole-body computed tomography revealed multiple focal hepatic hypervascular lesions in the right lobe of the liver together with a fatty liver. Subsequent hepatic magnetic resonance imaging suggested the diagnosis of HA with a suspicion of focal nodular hyperplasia (FNH). The patient refused to undergo liver biopsy, so we instituted a 3-month surveillance program, which included clinical assessment, liver function tests, tumour marker assessment and blood tests as well as sonographic evaluation for follow-up of the liver lesions.LEARNING POINTSHepatic adenomatosis is an extremely rare disease which predominantly affects women and is associated with hormone consumption, liver steatosis, glycogen storage disease, obesity, metabolic syndrome, abnormalities of hepatic vasculature and maturity onset diabetes of the young (MODY).The two main differential diagnoses include focal nodular hyperplasia (FNH) and well-differentiated hepatocellular carcinoma. Histological confirmation is required when MRI findings are inconclusive or when a malignancy is suspected.In men, resection of adenomas is mandatory due to the high risk of malignant transformation. In woman, a conservative approach with contraceptive discontinuation and biannual follow-up with MRI and alpha-fetoprotein is recommended; resection is needed in case of positive immunohistochemical staining for β-catenin on biopsy, symptomatic adenomas, adenoma increasing in size, and when malignancy cannot be ruled out.

A magnetic resonance imaging modality for non-invasively distinguishing progression of liver fibrosis by visualizing hepatic platelet-derived growth factor receptor-beta expression in mice.

Activated hepatic stellate cells (HSCs) are the most critical cells responsible for liver fibrosis, and platelet-derived growth factor (PDGF) is the most prominent mitogen for HSCs in fibrogenesis. This study aimed to explore the potential of gadolinium (Gd)-labeled cyclic peptides (pPB) targeting PDGF receptor-β (PDGFR-β) as a magnetic resonance imaging (MRI) radiotracer to identify the progression of liver fibrosis by imaging hepatic PDGFR-β expression. Mice treated with carbon tetrachloride (CCl4 ) were used to mimic hepatic fibrosis in vivo. The binding activity of FITC-labeled pPB to PDGFR-β was assessed in cultured human HSCs (HSC-LX2). MRI was performed to visualize hepatic PDGFR-β expression in mice with different degrees of liver fibrosis after Gd-labeled pPB was injected. Hepatic PDGFR-β expression level was correlated with the severity of liver fibrosis, and the majority of cells expressing PDGFR-β were found to be activated HSCs in fibrotic livers. Culture-activated human HSCs expressed abundant PDGFR-β, and FITC-labeled pPB could bind to these cells in a concentration-dependent and time-dependent manner. With Gd-labeled pPB as a tracer, an MRI modality demonstrated that the relative hepatic T1-weighted MRI signal value progressively increased with the severity of hepatic fibrosis and reduced with remission. Hepatic PDGFR-β expression reflects the progression of hepatic fibrosis, and MRI using Gd-labeled pPB as a tracer exhibits potential for distinguishing liver fibrosis staging in mice.

Retinoic acid-conjugated chitosan/manganese porphyrin ionic-complex nanoparticles for improved T1 contrast MR imaging of hepatic fibrosis.

Noninvasive and precise diagnosis of hepatic fibrosis is very important for the preventive therapeutic regimen of hepatic cirrhosis and cancer. In this study, we fabricated T1 contrast Mn-porphyrin (MnTPPS4 )/retinoic acid-chitosan ionic-complex nanoparticles (MRC NPs). The functional properties of MRC NPs were evaluated via transmission electron microscopy (TEM) imaging, release study, cytotoxicity assay, hepatocyte-specific uptake assay, and magnetic resonance (MR) imaging study. TEM images confirmed the typical structure of an ionic-complex NPs with around 100-200 nm of diameter. MnTPPS4 is released from MRC NPs for up to 24 hr in controlled pattern which implies that more reliable and convenient hepatic MR imaging is possible using of MRC NPs in clinical practice. Hepatocytes uptake assay proved retinoic acid-specific targeting of MRC NPs. The same results were observed in animal pharmacokinetic studies. In vitro MR phantom study, MRC NPs showed an increased T1 relaxivity (r1 =6.772 mM-1 s-1 ) in comparison with 3.242 mM-1 s-1 of MnTPPS4 . The result was confirmed again in vivo MR imaging studies. Taken together, MRC NPs displayed a potential for noninvasive diagnostic T1 MR imaging of hepatic fibrosis with improved target specificity and prolonged MR imaging time window.