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Query: UMLS:C0239946 (liver fibrosis)
 8,268 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report an 81-year-old woman who presented with motor disturbance in her right hand which was followed by parkinsonism, dementia, and supranuclear gaze palsy. She was well until her age of 73 (1989) when she had an onset of difficulty in using her right hand; she did not have weakness. She also developed small step gait. These symptoms had progressively become worse. She was admitted to our hospital in July of 1992 when she was 75 years old. On admission, she was alert and oriented, but she showed some difficulty in recent memory. She did not have aphasia or ideomotor apraxia, but she showed limb-kinetic apraxia in her right hand, ideational apraxia, dressing apraxia, constructional apraxia, tactile agnosia, and left-right disorientation. Alien-hand syndrome was observed in her right hand. Ocular movement was within normal limit for her age. She had oro-lingual dyskinesia. Otherwise, cranial nerves were intact. She walked in small-steps. She had rigidity and fine myoclonic movements in her right upper extremity. Deep reflexes were within normal limits and symmetric. Superficial and deep sensations were intact. Laboratory findings were unremarkable. She was discharged on August 15, 1992 for outpatient follow-up. Her motor and mental symptoms were progressive. By October of 1992, she developed supranuclear vertical gaze palsy, marked rigidity in the neck, and astereognosis. By June 1993, she became unable to walk without support. MRI taken in May of 1994 revealed atrophy of insular cortices, temporal lobe tips and parietal lobes more on the left side; the third ventricle was slightly dilated. She was admitted to another hospital on June 30, 1994. She had become a bed-ridden state with marked dementia and dysphagia. She developed fever on November 5, 1996 and expired on December 16 of the same year. She was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had corticobasal degeneration. Other diagnoses entertained included progressive supranuclear palsy, pallidonigroluysian atrophy, diffuse Lewy body disease, and Pick's disease. But the most of the participants agreed with the chief discussant's diagnosis. Post-mortem examination revealed aspiration pneumonia in the lungs and liver fibrosis apparently due to viral hepatitis. In the central nervous system, frontal and parietal lobes were atrophic more on the left side. Atrophy was accentuated in the superior frontal gyri, precentral and postcentral gyri, and superior and inferior parietal lobuli. Neuronal loss and astrocytosis were seen in these regions with scattered ballooned neurons. The substantia nigra showed marked neuronal loss and gliosis; neuronal loss was also seen in the pars reticulata. The outer and inner segments of globus pallidus and the periacqueductal gray matter showed gliosis, however, no apparent neuronal loss was seen. Putamen, subthalamic nucleus, and the dentate nucleus were preserved. Pathologic changes were consistent with the diagnosis of corticobasal degeneration. It was interesting to note that anti-tau immunostaining and Gallyas staining revealed neuropil threads and astrocytic plaques in the cortical areas, and intracytoplasmic inclusion bodies in the cortical neurons; these inclusions were not stained by Bodian stain. Tuft-shaped astrocytes which may be seen in progressive supranuclear palsy were not observed in this patient. Although corticobasal degeneration and progressive supranuclear palsy share some neurological features in common, this patient showed typical pathologic changes of corticobasal degeneration.
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PMID:[An 81-year-old woman with progressive motor disturbance, extrapyramidal features, dementia, and oculomotor palsy]. 956 8

Cirrhosis is a progressive, diffuse process of liver fibrosis that is characterized by architectural distortion and the development of a spectrum of nodules ranging from benign regenerative nodules to premalignant dysplastic nodules to overtly malignant hepatocellular carcinoma. The purpose of this essay is to demonstrate the ex vivo MR and pathology findings of these nodules as well as other masses that can be seen in the cirrhotic liver. The optimal conditions under which ex vivo imaging can be performed allow greater spatial resolution than that achieved with in vivo imaging, without artifacts that may degrade image quality. Clearly, contrast-enhanced MRI is essential for both the diagnosis and the characterization of focal lesions in the cirrhotic liver. However, the use of ex vivo imaging precludes the evaluation of these important in vivo pulse sequences.
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PMID:Focal lesions in the cirrhotic liver: high resolution ex vivo MRI with pathologic correlation. 1075 77

Sinusoidal capillarization induces microcirculatory changes in liver cirrhosis and fibrosis. The purpose of this study was to assess whether contrast-enhanced MRI can be used to demonstrate the effects of sinusoidal capillarization in liver fibrosis. Dynamic MRI after injection of a low-molecular-weight contrast agent of 0.56 kDa (Gd-DOTA), and two high-molecular-weight contrast agents of 6.47 kDa and 52 kDa (P792 and P717) was performed in rabbits with liver fibrosis induced by cholesterol and diethylstilbestrol. The hepatic distribution volume accessible to the high-molecular-weight agents decreased in the rabbits with liver fibrosis (P792: 7.8% +/- 1.7% vs. 10.1% +/- 1.8% in normal rabbits, P =.038; P717: 6.2% +/- 2.1% vs. 9.7% +/- 1.6% in normal rabbits, P =.007), whereas the hepatic mean transit time (MTT) of the low-molecular-weight agent was increased (15.9 +/- 8.0 s vs. 8.8 +/- 2.6 s in normal rabbits, P =.015). In rabbits with liver fibrosis, the clearance of indocyanine green (ICG) was correlated with the volume accessible to the high-molecular-weight agents (P792: r = 0.810, P =.015; P717: r = 0.857, P =.007). The collagen content of the liver was inversely correlated with the distribution volume of P717 (r = -.833, P =.010) and with the ICG clearance (r = -.810, P =.015). It was concluded that the microcirculatory changes induced by sinusoidal capillarization in liver fibrosis can be demonstrated noninvasively with MRI.
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PMID:Capillarization of the sinusoids in liver fibrosis: noninvasive assessment with contrast-enhanced MRI in the rabbit. 1265 40

The noninvasive evaluation of liver fibrosis is a major clinical goal in liver diseases. Our aim was to identify MRI parameters to quantify liver fibrosis in vivo in an animal model of liver fibrosis with slight inflammation. We evaluated serum hyaluronate, liver hydroxyproline, area of liver fibrosis (image analysis), and 1.5-T MRI in 10 sham rats and 24 bile duct ligated rats with different stages of liver fibrosis. Liver signal intensity (SI)/muscle SI ratio and liver relaxation times (rT) were measured on T1 and T2 weighted sequences at different echo (TE) or recovery (RT) times of MRI. Among the 66 MRI parameters tested, the highest correlation with the area of fibrosis was observed for rT2 (r=0.78, P < 0.01). The area of liver fibrosis was independently predicted by five MRI variables (adjusted R (2)=0.78, with R (2)=0.64 for rT2 and rT1). Diagnostic accuracy for liver fibrosis was 100% using two variables: liver/muscle SI ratio on T2 at 30-ms TE and liver/muscle SI ratio on T1 at 50-ms RT. We conclude that in this animal model, fibrosis could be diagnosed with an accuracy of 100% using two MRI parameters. The quantification of liver fibrosis was very accurate either with only one MRI parameter (r=0.78 for rT2) or with five parameters (r=0.90) in this cholestatic model.
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PMID:Diagnosis and measurement of liver fibrosis by MRI in bile duct ligated rats. 1742 35

Liver fibrosis and cirrhosis are predisposing factors for the development of hepatocellular carcinoma (HCC). Hemosiderosis has also been described to trigger carcinogenesis. A significant iron overload, as found in hereditary hemochromatosis (HHC), is a risk factor for HCC and may also promote the symptoms of porphyria cutanea tarda (PCT). A 68-year old male patient presented to our clinic with a suspected HCC, elevated alpha-fetoprotein but normal liver function tests. He reported a 25 year-old history of vitiligo upon exposure to sunlight. The patient underwent an extended left hemihepatectomy, and the recovery was uneventful, with the exception of a persistent hyperbilirubinemia. Perfusion problems and extrahepatic cholestasis were ruled out by CT-scan with angiography and MR-cholangiopancreatography. However, MRI showed an iron overload. Histology confirmed the HCC (pT3, pN0, G3, R0) and revealed a portal fibrosis and hemosiderosis. Based on the skin lesions we suspected a PCT that was confirmed by laboratory tests showing elevated porphyrin, uroporphyrin, coproporphyrin and porphobilinogen. Concurrently, molecular diagnostics revealed homozygosity for the C282Y mutation within the hemochromatosis HFE gene. After phlebotomy and normalization of liver function tests the patient was discharged. This is the first case ever showing the unusual combination of HCC in a fibrotic liver with HHC and PCT. This diagnosis not only warrants oncological follow-up but also symptomatic therapy to normalize iron metabolism and thereby improve liver function and alleviate the symptoms of HHC and PCT. Thus progression of fibrosis may be prevented and liver regeneration supported.
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PMID:An unhappy triad: hemochromatosis, porphyria cutanea tarda and hepatocellular carcinoma-a case report. 1746 5

Chronic liver disease is a world-wide problem that causes progressive hepatic fibrosis as a hallmark of progressive injury. At present, the gold standard for diagnosing hepatic fibrosis is liver biopsy, which is an invasive method with many limitations, including questionable accuracy and risks of complications. MR elastography (MRE), a phase-contrast MRI technique for quantitatively assessing the mechanical properties of soft tissues, is a potential noninvasive diagnostic method to assess hepatic fibrosis. In this work, MRE was evaluated as a quantitative method to assess the in vivo mechanical properties of the liver tissues in a knockout animal model of liver fibrosis. This work demonstrates that the shear stiffness of liver tissue increases systematically with the extent of hepatic fibrosis, as measured by histology. A linear correlation between liver stiffness and fibrosis extent was well-defined in this animal model. An additional finding of the study was that fat infiltration, commonly present in chronic liver disease, does not significantly correlate with liver stiffness at each fibrosis stage and thus does not appear to interfere with the ability of MRE to assess fibrosis extent. In conclusion, MRE has the potential not only for assessing liver stiffness, but also for monitoring potential therapies for hepatic fibrosis.
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PMID:Quantitative assessment of hepatic fibrosis in an animal model with magnetic resonance elastography. 1765 77

Hepcidin inhibits intestinal absorption of iron through internalisation of ferroportin. Its discovery helps to better understand the genetic iron overloads. The insulin resistance-hepatic iron overload (IR-HIO)--also coined as the dysmetabolic iron overload syndrome--is a common cause or iron overload. This article is a review about genetic iron overloads and IR-HIO. Type 1 haemochromatosis C282Y +/+ accounts for 95% of the haemochromatosis. Hepatic fibrosis may develop if serum ferritin is higher than 1000 microg/l but can be partially reversible with phlebotomies. Juvenile haemochromatosis (type 2) and type 3 haemochromatosis (mutation of the transferrin receptor 2) are very uncommon. Several mutations of the ferroportin gene can cause usually mild iron overload of autosomal dominant inheritance. Aceruleoplasminemia is an uncommon disorder involving cerebral iron overload. The causes and consequences of the IR-HIO are unknown. Treatment of IR-HIO is focused on metabolic syndrome and phlebotomies are questionable because the overload is moderate and intestinal absorption of iron seems to be low. MRI (or other non invasive methods) is needed to truly assess iron overload because serum ferritin overestimates it in metabolic syndrome. Several points have to be elucidated: how HFE interferes with hepcidin in type 1 haemochromatosis; the causes of variability of iron overload; the benefits of populations screening; the advantage of phlebotomies in IR-HIO; the use of new oral iron chelators.
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PMID:[Genetic iron overloads and hepatic insulin-resistance iron overload syndrome: an update]. 1858 23

The prognosis and management of liver disease greatly depends on the amount of liver fibrosis. Non-alcoholic fatty liver disease (NAFLD), ranging from simple steatosis to non-alcoholic steatohepatitis (NASH), is emerging as a major cause of liver disease in Western countries because of the increasing prevalence of obesity and type 2 diabetes. A key issue in patients with NAFLD is the differentiation of NASH from simple steatosis. It is particularly important to identify NASH patients as they are at greatest risk of developing complications such as cirrhosis, liver failure and hepatocellular carcinoma. The limitations of liver biopsy (invasive procedure, sampling errors, interobserver variability and non-dynamic fibrosis evaluation) have stimulated the search for non-invasive approaches for the assessment of steatosis and liver fibrosis in patients with NAFLD. A variety of methods, including serum markers, imaging techniques such as ultrasound, CT, MRI and measurement of liver stiffness by transient elastography, have been proposed for the non-invasive assessment of steatosis and hepatic fibrosis. This review discusses the advantages and limitations of these different methods in clinical practice.
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PMID:Non-invasive diagnosis of steatosis and fibrosis. 1919 29

MRI has the potential of providing a noninvasive assessment of liver pathology. This work introduces a portal pressure gradient (PPG) model derived from fluid mechanics, where the PPG is proportional to the average velocity and inversely proportional to the vessel area in the upper part of portal vein. Using a phase-contrast spoiled gradient echo sequence, the PPG model was verified in a phantom study and was tested in an animal study using 35 rats with various degrees of hepatic fibrosis induced by carbon tetrachloride (CCl(4)). Histological examination was conducted to determine the severity of hepatic fibrosis. The fibrosis score monotonically increased with the duration of CCl(4) treatment. The PPG was highly correlated with nonzero fibrosis scores (r(2) = 0.90, P < 0.05). There was a significant difference between control and cirrhosis groups (P < 0.0006, alpha < 0.0018). The difference between control and fibrosis (noncirrhosis) groups (P < 0.002, alpha < 0.006) was also significant. Without the administration of any contrast agent, the MRI-PPG approach shows promise as a noninvasive means of evaluating liver fibrosis.
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PMID:Evaluation of hepatic fibrosis with portal pressure gradient in rats. 1925 77

Incidental liver nodules are more and more frequently encountered because of increasing sensitivity of recent imaging techniques. The identification of biliary cyst or hemangioma is usually easy. In other cases, the etiological diagnosis relies on careful radiological analysis of the pattern of the arterial phase enhancement following contrast medium injection. When there is no early arterial enhancement, a liver biopsy is usually indicated to establish the diagnosis. A strong arterial contrast enhancement pattern is indicative of hepatocellular tumor, benign or malignant. In this situation, it is crucial to establish if there is underlying liver fibrosis. In case of cirrhosis, the diagnosis of hepatocellular carcinoma is the most probable. If the non tumorous liver is normal, focal nodular hyperplasia and hepatocellular adenoma should be differentiated. The distinction between these two tumors is important because only hepatic adenoma carries a significant risk of complications (bleeding or hepatocellular carcinoma) leading to surgical resection of lesions that do not regress after steroid withdrawal. Contrast enhanced MRI and contrast ultrasound are most useful tools for the diagnosis of nodular regenerative hyperplasia but liver biopsy can be necessary in atypical forms. In recent years, the understanding of molecular mechanisms associated with adenoma occurrence allowed for the proposal of a new classification already of practical interest in the management of patients.
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PMID:[Incidental ultrasound finding of a 3 cm liver nodule in a 45-year-old woman taking hormonal substitution after hysterectomy performed for cervix carcinoma]. 1976 17


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