Wilson disease (WD) primarily presents with hepatic and neurological symptoms. While hepatic symptoms typically precede the neurological manifestations, copper accumulates in the brain already in this patient group and leads to subclinical brain MRI abnormalities including T2 hyperintensities and atrophy. This study aimed to assess brain morphological changes in mild hepatic WD. WD patients without a history of neurologic symptoms and decompensated cirrhosis and control participants underwent brain MRI at 3T scanner including high-resolution T1-weighted images. A volumetric evaluation was conducted on the following brain regions: nucleus accumbens, caudate, pallidum, putamen, thalamus, amygdala, hippocampus, midbrain, pons, cerebellar gray matter, white matter (WM), and superior peduncle, using Freesurfer v7 software. Whole-brain analyses using voxel- and surface-based morphometry were performed using SPM12. Statistical comparisons utilized a general linear model adjusted for total intracranial volume, age, and sex. Twenty-six WD patients with mild hepatic form (30 ± 9 years [mean age ± SD]); 11 women; mean treatment duration 13 ± 12 (range 0-42) years and 28 healthy controls (33 ± 9 years; 15 women) were evaluated. Volumetric analysis revealed a significantly smaller pons volume and a trend for smaller midbrain and cerebellar WM in WD patients compared to controls. Whole-brain analysis revealed regions of reduced volume in the pons, cerebellar, and lobar WM in the WD group. No significant differences in gray matter density or cortical thickness were found. Myelin or WM in general seems vulnerable to low-level copper toxicity, with WM volume loss showing promise as a marker for assessing brain involvement in early WD stages.
- MeSH
- White Matter pathology diagnostic imaging MeSH
- Adult MeSH
- Hepatolenticular Degeneration * pathology diagnostic imaging MeSH
- Liver pathology diagnostic imaging MeSH
- Middle Aged MeSH
- Humans MeSH
- Magnetic Resonance Imaging * MeSH
- Young Adult MeSH
- Brain * pathology diagnostic imaging MeSH
- Gray Matter pathology diagnostic imaging MeSH
- Case-Control Studies MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Young Adult MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Research Support, Non-U.S. Gov't MeSH
Machine learning identifies liver fat fraction (FF) measured by 1H MR spectroscopy, insulinemia, and elastography as robust, non-invasive biomarkers for diagnosing steatohepatitis in liver transplant patients, validated through decision tree analysis. Compared to the general population (~5.8% prevalence), MASH is significantly more common in liver transplant recipients (~30%-50%). In patients with FF > 5.3%, the positive predictive value for MASH ranged up to 97%, more than twice the value observed in the general population.
- MeSH
- Adult MeSH
- Elasticity Imaging Techniques MeSH
- Liver * pathology diagnostic imaging MeSH
- Middle Aged MeSH
- Humans MeSH
- Machine Learning * MeSH
- Liver Transplantation * MeSH
- Fatty Liver * diagnosis diagnostic imaging MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
Alveolární echinokokóza (AE) je vzácné, ale závažné parazitární onemocnění jater, které často napodobuje maligní ložiska. V této kazuistice prezentujeme případ 76letého pacienta, u něhož byla AE diagnostikována náhodně při vyšetření pro renální insuficienci. Ultrazvukové vyšetření odhalilo mnohočetná hyperechogenní ložiska v játrech. Následné CT a MR jater zobrazily mnohočetná ložiska nepravidelných okrajů bez typické vaskularizace, největší o velikosti 71 × 38 mm. Laboratorní výsledky ukázaly zvýšené jaterní enzymy, CRP a renální insuficienci. Core cut biopsie jater potvrdila přítomnost pseudocystických struktur. Vzorky byly následně zaslány do Národní referenční laboratoře pro tkáňové helmintózy, která diagnózu AE potvrdila. Sérologické vyšetření bylo provedeno až po bioptickém vyšetření a potvrdilo přítomnost protilátek proti Echinococcus multilocularis. Pacient byl odeslán do infekční ambulance, kde byla zahájena léčba albendazolem v dávce 800 mg denně. Po rehydrataci a úpravě léčby došlo ke stabilizaci renálních funkcí a při kontrolních zobrazovacích vyšetřeních byl nález stacionární. Tento případ zdůrazňuje nutnost zařazení AE do diferenciální diagnostiky ložiskových procesů jater, zejména u pacientů bez onkologické anamnézy.
Alveolar echinococcosis (AE) is a rare but serious parasitic liver disease that often mimics malignant lesions. We present the case of a 76-year-old patient in whom AE was incidentally diagnosed during examination for renal insufficiency. Abdominal ultrasound revealed multiple hyperechogenic liver lesions. Subsequent MRI showed multiple irregularly bordered lesions without typical vascularization, the largest measuring 71 × 38 mm. Laboratory tests revealed elevated liver enzymes, CRP, and renal insufficiency. A core-cut liver biopsy confirmed the presence of pseudocystic structures. Samples were sent to the National Reference Laboratory for Tissue Helminthiases, which confirmed the diagnosis of AE. Serological testing was performed after the biopsy confirming the presence of antibodies against Echinococcus multilocularis. The patient was referred to an infectious disease clinic, where treatment with albendazole at a dose of 800 mg daily was initiated. After rehydration and adjustment of therapy, renal function stabilized, and follow-up imaging showed stable findings. This case highlights the need to include AE in the differential diagnosis of hepatic lesions, particularly in patients without an oncological history.
- MeSH
- Albendazole pharmacology therapeutic use MeSH
- Biopsy MeSH
- Diagnostic Imaging methods MeSH
- Echinococcosis, Hepatic * diagnosis drug therapy MeSH
- Liver diagnostic imaging pathology MeSH
- Humans MeSH
- Aged MeSH
- Check Tag
- Humans MeSH
- Male MeSH
- Aged MeSH
- Publication type
- Case Reports MeSH
Alterations in tricarboxylic acid (TCA) cycle metabolism are associated with hepatic metabolic disorders. Elevated hepatic acetate concentrations, often attributed to high caloric intake, are recognized as a pivotal factor in the etiology of obesity and metabolic syndrome. Therefore, the assessment of acetate breakdown and TCA cycle activity plays a central role in understanding the impact of diet-induced alterations on liver metabolism. Magnetic resonance-based deuterium metabolic imaging (DMI) could help to unravel the underlying mechanisms involved in disease development and progression, however, the application of conventional deuterated glucose does not lead to substantial enrichment in hepatic glutamine and glutamate. This study aimed to demonstrate the feasibility of DMI for tracking deuterated acetate breakdown via the TCA cycle in lean and diet-induced fatty liver (FL) rats using 3D DMI after an intraperitoneal infusion of sodium acetate-d3 at 9.4T. Localized and nonlocalized liver spectra acquired at 10 time points post-injection over a 130-min study revealed similar intrahepatic acetate uptake in both animal groups (AUCFL = 717.9 ± 131.1 mM▯min-1, AUClean = 605.1 ± 119.9 mM▯min-1, p = 0.62). Metabolic breakdown could be observed in both groups with an emerging glutamine/glutamate (Glx) peak as a downstream metabolic product (AUCFL = 113.6 ± 23.8 mM▯min-1, AUClean = 136.7 ± 41.7 mM▯min-1, p = 0.68). This study showed the viability of DMI for tracking substrate flux through the TCA cycle, underscoring its methodological potential for imaging metabolic processes in the body.
- MeSH
- Acetates metabolism MeSH
- Metabolic Flux Analysis MeSH
- Citric Acid Cycle * MeSH
- Deuterium * MeSH
- Liver * metabolism diagnostic imaging MeSH
- Rats MeSH
- Magnetic Resonance Imaging MeSH
- Rats, Sprague-Dawley MeSH
- Rats, Wistar MeSH
- Animals MeSH
- Check Tag
- Rats MeSH
- Male MeSH
- Animals MeSH
- Publication type
- Journal Article MeSH
PURPOSE: This prospective pilot study aims to evaluate the capabilities of novel quantitative ultrasound (QUS) methods based on attenuation (Att.PLUS) and sound speed (SSp.PLUS) for detecting liver fat. PATIENTS AND METHODS: The study included 56 individuals with biopsy-proven steatosis (percutaneous liver biopsy) ranging from 0 % to 90 % of hepatocytes containing intracellular lipid vacuoles. Histopathology was considered reference standard. Abdominal QUS examinations were conducted using Att.PLUS and SSp.PLUS techniques on the Aixplorer MACH 30 system. Comparative assessments were made using the results of liver biopsy and magnetic resonance spectroscopy (MRS) together with magnetic resonance imaging proton density fat fraction (MRI-PDFF). MR examinations were performed on the Siemens VIDA 3 T system. RESULTS: ROC analysis was conducted for two groups: (a) patients without steatosis (S0) versus those with steatosis (S1 + S2 + S3) yielded AUC values of 0.79 for Att.PLUS and 0.78 for SSp.PLUS, in contrast to an AUC > 0.95 for MRS and MRI-PDFF; and (b) patients without or with mild steatosis (S0 + S1) versus those with severe steatosis (S2 + S3), yielded AUC values of 0.93 for Att.PLUS and 0.89 for SSp.PLUS, in contrast to an AUC > 0.99 for MRS and MRI-PDFF. However, MR methods were superior in detecting liver fat content in obese patients and post-liver transplantation individuals. CONCLUSION: Both QUS parameters (Att.PLUS and SSp.PLUS) appear equivalent at differentiating S0 vs. (S1 + S2 + S3) patients, but the Att.PLUS parameter may be more effective at identifying advanced steatosis (S2 + S3). MR techniques outperformed QUS methods, making them more suitable for clinical studies.
- MeSH
- Biopsy MeSH
- Adult MeSH
- Liver diagnostic imaging pathology MeSH
- Middle Aged MeSH
- Humans MeSH
- Magnetic Resonance Spectroscopy methods MeSH
- Magnetic Resonance Imaging * methods MeSH
- Pilot Projects MeSH
- Prospective Studies MeSH
- Reproducibility of Results MeSH
- Aged MeSH
- Sensitivity and Specificity MeSH
- Ultrasonography * methods MeSH
- Fatty Liver * diagnostic imaging pathology MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Comparative Study MeSH
BACKGROUND AND AIMS: Prolonged fasting, which leads to the mobilization of fat from adipose tissue, can result in the development of hepatosteatosis. However, it is not yet known whether the accumulation of fat in the liver after fasting can be affected by concurrent obesity. Therefore, this study aimed to assess how excessive adiposity influences changes in liver fat content induced by fasting and subsequent refeeding. METHODS AND RESULTS: Ten lean women and eleven women with obesity (age: 36.4 ± 7.9 and 34.5 ± 7.9 years, BMI: 21.4 ± 1.7 and 34.5 ± 4.8 kg/m2) underwent a 60-h fasting period followed by 2 days of isocaloric high-carbohydrate refeeding. Magnetic resonance spectroscopy (MRS) examinations of liver were conducted at baseline, after 48 h of fasting, and at the end of refeeding period. Hepatic fat content (HFC) increased in lean women after fasting, whereas no statistically significant change in HFC was observed in women with obesity. Additionally, fasting led to significant reductions in liver volume in both groups, likely attributable to glycogen depletion, with subsequent restoration upon refeeding. Notably, changes in hepatic fat volume (HFV) rather than HFC inversely correlated with baseline liver fat content and HOMA-IR. CONCLUSION: We demonstrated that prolonged fasting results in accumulation of fat in the liver in lean subjects only and that this accumulation is inversely related to baseline fat content and insulin resistance. Moreover, the study underscored the importance of evaluating hepatic fat volume rather than hepatic fat content in studies that involve considerable changes in hepatic lean volume.
- MeSH
- Adiposity * MeSH
- Time Factors MeSH
- Dietary Carbohydrates administration & dosage MeSH
- Adult MeSH
- Liver * metabolism diagnostic imaging MeSH
- Middle Aged MeSH
- Humans MeSH
- Obesity * physiopathology therapy metabolism MeSH
- Fasting * MeSH
- Diet, Carbohydrate Loading adverse effects MeSH
- Case-Control Studies MeSH
- Fatty Liver diagnostic imaging MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Comparative Study MeSH
Background and Objectives: Liver injury is a rare complication of cardiopulmonary resuscitation. Correct and early diagnosis and treatment are essential. The clinical signs of injury may be masked by the cardiac arrest. We present a single-centre retrospective observational study of traumatic liver injury after cardiopulmonary resuscitation. Materials and Methods: A retrospective analysis of the patients treated for liver injury after cardiopulmonary resuscitation was conducted. Demographic data, the cause of resuscitation, the duration of restoration of spontaneous circulation (ROSC), and the surgical approach were analysed. Results: We have treated nine patients with severe liver injury after cardiopulmonary resuscitation. The diagnosis was made on the basis of cardiopulmonary instability, a fall in the erythrocyte count in eight cases, and was confirmed by CT or ultrasound examination. The last one was diagnosed accidentally on MR. Surgery, in cases of unstable patients, was followed immediately after a diagnosis. We combined liver sutures and intra-abdominal packing with a planned second-look surgery. Five of the nine patients survived. Conclusions: Liver injury after cardiopulmonary resuscitation is rare and is associated with high mortality. The recurrence of cardiopulmonary instability and/or a low or falling red blood cell count are the main signs of this injury. Bedside ultrasound and CT scans are the most important methods to confirm the diagnosis. The rule of surgical repair is the same as in all liver injuries, regardless of aetiology. The key factors for survival include early diagnosis, together with the length of restoration of spontaneous circulation (ROSC).
- MeSH
- Adult MeSH
- Liver * injuries diagnostic imaging MeSH
- Cardiopulmonary Resuscitation * adverse effects MeSH
- Middle Aged MeSH
- Humans MeSH
- Retrospective Studies MeSH
- Aged MeSH
- Heart Arrest etiology MeSH
- Check Tag
- Adult MeSH
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Observational Study MeSH
BACKGROUND: Early identification of those with NAFLD activity score ≥ 4 and significant fibrosis (≥F2) or at-risk metabolic dysfunction-associated steatohepatitis (MASH) is a priority as these patients are at increased risk for disease progression and may benefit from therapies. We developed and validated a highly specific metabolomics-driven score to identify at-risk MASH. METHODS: We included derivation (n = 790) and validation (n = 565) cohorts from international tertiary centers. Patients underwent laboratory assessment and liver biopsy for metabolic dysfunction-associated steatotic liver disease. Based on 12 lipids, body mass index, aspartate aminotransferase, and alanine aminotransferase, the MASEF score was developed to identify at-risk MASH and compared to the FibroScan-AST (FAST) score. We further compared the performance of a FIB-4 + MASEF algorithm to that of FIB-4 + liver stiffness measurements (LSM) by vibration-controlled transient elastography (VCTE). RESULTS: The diagnostic performance of the MASEF score showed an area under the receiver-operating characteristic curve, sensitivity, specificity, and positive and negative predictive values of 0.76 (95% CI 0.72-0.79), 0.69, 0.74, 0.53, and 0.85 in the derivation cohort, and 0.79 (95% CI 0.75-0.83), 0.78, 0.65, 0.48, and 0.88 in the validation cohort, while FibroScan-AST performance in the validation cohort was 0.74 (95% CI 0.68-0.79; p = 0.064), 0.58, 0.79, 0.67, and 0.73, respectively. FIB-4+MASEF showed similar overall performance compared with FIB-4 + LSM by VCTE ( p = 0.69) to identify at-risk MASH. CONCLUSION: MASEF is a promising diagnostic tool for the assessment of at-risk MASH. It could be used alternatively to LSM by VCTE in the algorithm that is currently recommended by several guidance publications.
- MeSH
- Biopsy adverse effects MeSH
- Elasticity Imaging Techniques * MeSH
- Fibrosis MeSH
- Liver Cirrhosis pathology MeSH
- Liver diagnostic imaging pathology MeSH
- Humans MeSH
- Non-alcoholic Fatty Liver Disease * pathology MeSH
- Predictive Value of Tests MeSH
- Check Tag
- Humans MeSH
- Publication type
- Journal Article MeSH
Neonatální infekce virem Herpes simplex (HSV) jsou vzácná, ale potenciálně smrtelná onemocnění. K přenosu infekce dochází nejčastěji během porodu, vzácněji postnatálně. Nejobávanější formou je diseminovaná infekce s multiorgánovým postižením, která je zatížena vysokou morbiditou a mortalitou. V tomto kazuistickém sdělení prezentujeme případ neočekávaného úmrtí novorozence, u kterého pitva odhalila diseminovanou infekci virem Herpes simplex 1 (HSV1).
Neonatal Herpes simplex virus (HSV) infections are rare but potentially fatal diseases. HSV infection is usually acquired when a newborn comes into contact with viable virus during intrapartum transit through infected birth canal. However, some cases are transmitted postnatally. Disseminated HSV infection with multiorgan involvement is the most feared form of the disease and is burdened with high morbidity and mortality. In this case report, we present a case of unexpected death of a neonate in whom the autopsy revealed disseminated Herpes simplex virus 1 (HSV1) infection.
- MeSH
- Liver diagnostic imaging pathology MeSH
- Humans MeSH
- Herpesvirus 1, Human * pathogenicity MeSH
- Multiple Organ Failure diagnosis etiology pathology MeSH
- Death, Sudden etiology pathology MeSH
- Infant, Newborn MeSH
- Perinatal Death * etiology MeSH
- Autopsy methods MeSH
- Lung diagnostic imaging pathology MeSH
- Polymerase Chain Reaction methods MeSH
- Check Tag
- Humans MeSH
- Infant, Newborn MeSH
- Publication type
- Case Reports MeSH
- MeSH
- Liver Diseases, Alcoholic * diagnostic imaging MeSH
- Liver diagnostic imaging MeSH
- Humans MeSH
- Magnetic Resonance Imaging MeSH
- Magnetic Fields MeSH
- Disease Models, Animal MeSH
- Mice MeSH
- Animals MeSH
- Check Tag
- Humans MeSH
- Mice MeSH
- Animals MeSH
- Publication type
- Comment MeSH
- Editorial MeSH