1/34. Wilson's disease coexisting with viral hepatitis type C: a case report with histological and ultrastructural studies of the liver.Histopathological and ultrastructural findings in the liver of a female patient who suffered from Wilson's disease (WD) and viral hepatitis type C (HCV) are reported. light and electron microscopy examinations demonstrated a variety of morphological alterations--many of them frequently seen in livers of patients with WD and others that can be found in cases presenting HCV infection. The influence of coexistence of these two diseases on morphological changes is discussed.- - - - - - - - - - ranking = 1keywords = hepatitis (Clic here for more details about this article) |
2/34. Fulminant Wilsonian hepatitis unmasked by disease progression: report of a case and review of the literature.Among various hepatic manifestations of Wilson's disease, fulminant hepatic failure is the most uncommon entity and requires a detailed clinicopathological analysis for correct diagnosis. Left unrecognized and without proper therapy, in time the disease rapidly progresses to death. We describe a 24-year-old woman who died within five weeks of the onset of Wilson's disease, which presented with a dramatic course. Discriminating features of the disease are discussed with regard to the literature.- - - - - - - - - - ranking = 0.8keywords = hepatitis (Clic here for more details about this article) |
3/34. Development of cytochrome P450 2D6-specific LKM-autoantibodies following liver transplantation for Wilson's disease -- possible association with a steroid-resistant transplant rejection episode.BACKGROUND/AIMS: Antibodies to cytochrome P450 2D6, also known as LKM1-autoantibodies, are characteristic for a subgroup of patients with autoimmune hepatitis, but can also occasionally be found in hepatitis c. We observed the occurrence of LKM1-autoantibodies 4 months after liver transplantation for Wilson's disease, in close association with a steroid-resistant rejection episode, in the absence of evidence for autoimmune hepatitis or hepatitis c. methods: Sera from several time points prior to and following transplantation were tested for LKM-reactivity by immunofluorescence, ELISA and Western blotting. Antigen specificity was confirmed by Western blotting analysis on different cytochrome P450 isoenzymes. The absence of viral hepatitis C and hepatitis G virus infection was confirmed by polymerase chain reaction. The serum of the organ donor was also tested. RESULTS: All the sera prior to transplantation and up to 4 months after transplantation were LKM-negative by all assay systems used. In the course of a steroid-resistant rejection episode at this time, the patient developed LKM antibodies at high titre (70% in inhibition ELISA) and has remained positive since (now more than 4 years). Reactivity was exclusively to the cytochrome isoenzyme 2D6. hepatitis c infection never occurred, but hepatitis G was transiently present many years prior to transplantation. The donor serum was negative for all autoantibodies and for hepatitis c and G virus infection. DISCUSSION: We here describe a patient developing LKM1-autoantibodies without evidence of autoimmune or viral hepatitis. The close temporal association with a transplant rejection episode suggests immunological mechanisms of rejection together with hepatocellular injury as a pathogenetic mechanism.- - - - - - - - - - ranking = 1.8keywords = hepatitis (Clic here for more details about this article) |
4/34. February 2000: dementia with motor dysfunction in a patient with liver disease.Acquired (non-Wilsonian) hepatocerebral degeneration (AHCD) is an irreversible neurological condition characterized by dementia, dysarthria, and motor disturbances. It has been described in patients with severe liver disease of many causes, and notably in patients with surgically or spontaneously created porto-systemic shunts. We report a case of AHCD in a patient with end-stage liver disease due to alcohol abuse and hepatitis c. In addition, this patient showed pathologic evidence of the less commonly reported "shunt myelopathy" in the absence of a surgically created porto-systemic shunt. The myelopathy was associated with a dramatic vacuolation involving especially the deep motor cortex. Electron microscopy suggested that the vacuolation was due mainly to disruption of abnormal astrocytes.- - - - - - - - - - ranking = 0.2keywords = hepatitis (Clic here for more details about this article) |
5/34. Wilson's disease with superimposed autoimmune features: report of two cases and review.We describe two females, 15 and 23 years old, respectively, who presented with classical features of Wilson's disease (WD) and several features of autoimmune hepatitis (AIH). The first patient was initially diagnosed as AIH and treated with prednisolone which caused clinical improvement, with an increase of serum albumin from 22 to 30 g/L, and a decrease of aspartate aminotransferase from 103 to 47 U/L. Subsequent diagnosis of WD and introduction of penicillamine gave excellent improvement and complete normalization of liver function tests. The second patient, at first also diagnosed as having AIH, was treated with steroids and azathioprine with initial improvement, but subsequent deterioration. The diagnosis of WD was made 2 years after initial diagnosis of AIH, as the patient reached end-stage liver disease and required a transplant. Therefore, d-penicillamine treatment was not attempted. We conclude that, in patients with AIH, a thorough screening for WD is necessary, particularly when the response to steroid therapy is poor. Conversely, in patients suffering from WD with superimposed features of AIH, a combination of steroids and penicillamine may be of benefit.- - - - - - - - - - ranking = 0.2keywords = hepatitis (Clic here for more details about this article) |
6/34. Reappearance of HBsAg with compartmentalized different HBV strains in allograft versus PBMC of the recipient.A woman who was positive for anti-hepatitis B surface antigen (anti-HBs) and anti-hepatitis B core antigen (anti-HBc) received an orthotopic liver transplantation from an anti-HBc-seropositive donor in November 1985. Reappearance of hepatitis B surface antigen (HBsAg) was noted 5 months after the transplantation, but it was not associated with significant liver inflammation. Ten years after the transplantation, results of serum hepatitis b virus (HBV) dna study, by nested polymerase chain reaction, were negative. However, HBV dna was detected in the transplanted liver tissues and peripheral blood mononuclear cells. Different strains were identified in these two organs. An adw strain was found in the transplanted liver, whereas an adr strain with long segment deletions in the core gene was found in the peripheral blood mononuclear cells. Covalently closed circular HBV dna was not detected in any of the tissues examined. Occult HBV infection in the donor as well as the recipient is common in HBV endemic areas. The recipient in this case had reappearance of hepatitis B surface antigen (HBsAg) in the serum after transplantation. Nevertheless, 10 years later, two different strains of HBV were identified in different organs, without cross infection. The present case demonstrates that HBsAg reappearance was not associated with reactivation of the virus and liver inflammation. This type of HBsAg reappearance did not appear to produce a significant hazard to the transplanted liver.- - - - - - - - - - ranking = 1keywords = hepatitis (Clic here for more details about this article) |
7/34. Clinical and neuroradiological improvement in chronic acquired hepatocerebral degeneration after branched-chain amino acid therapy.We report two patients with chronic acquired hepatocerebral degeneration (CAHD) who showed neurological and radiological improvement after the administration of branched-chain amino acids (BAA). The first patient with chronic hepatitis c presented with progressive parkinsonism for 7 months, whereas the second patient with liver cirrhosis presented with progressive ataxia for 15 months. T1-weighted magnetic resonance imaging (MRI) showed symmetric high intensity signals in the lenticular nuclei in both patients. In the first patient, single photon emission computed tomography (SPECT) disclosed a marked decrease in cerebral blood flow in the parieto-occipital regions. In the second patient, T2-weighted MRI demonstrated symmetric high intensity signals in the deep cerebral and cerebellar white matter. After the administration of BAA, their neurological signs and radiological abnormalities markedly improved in both patients. CAHD might be a reversible and treatable disorder where aromatic amino acids are deeply involved in its pathogenesis.- - - - - - - - - - ranking = 0.2keywords = hepatitis (Clic here for more details about this article) |
8/34. Liver support--a task for nephrologists? Extracorporeal treatment of a patient with fulminant Wilson crisis.BACKGROUND: patients with Wilson's disease may present with cirrhosis, acute hepatitis or fulminant hepatic failure. Without urgent orthotopic liver transplantation, a fulminant Wilson crisis has a mortality of 100%. We report on an 18-year-old female patient with fulminant hepatic failure due to Wilson crisis. methods: The molecular adsorbent recirculating system (mars) was used to eliminate albumin-bound toxins and to bridge waiting until an organ became available. RESULTS: A total of 18 mars sessions and 4 plasma exchange sessions were performed. bilirubin levels and hepatic encephalopathy improved under mars therapy. A total of 75 mg copper was removed until serum copper levels were within the normal range. copper elimination was measured in 15 mars treatments, which removed a total of 12.9 mg copper. Four plasma exchange sessions, with a total exchange of 11 liters of plasma, removed 12 mg copper. Urinary copper elimination with penicillamine was 50 mg. CONCLUSION: mars was an effective method to stabilize a patient with Wilson crisis, contributed to copper elimination and gained time for liver transplantation. The risk of high-urgency transplantation could be avoided. Liver support was easy in the hands of nephrologists familiar with extracorporeal therapy.- - - - - - - - - - ranking = 0.2keywords = hepatitis (Clic here for more details about this article) |
9/34. Prominent cerebellar symptoms with unusual magnetic resonance imaging findings in acquired hepatocerebral degeneration.BACKGROUND: Cerebellar lesions revealed by abnormal signals on magnetic resonance images are extremely rare in acquired hepatocerebral degeneration (AHCD). OBJECTIVE: To report a case of AHCD with prominent cerebellar findings both clinically and radiologically. DESIGN AND SETTING: Case report and tertiary-care hospital. PATIENT: A 46-year-old man complained of progressive speech difficulties of 5 months' duration. Two years earlier, he had been diagnosed as having cirrhosis of the liver caused by alcoholism and hepatitis b virus infection. RESULTS: The patient had progressive ataxic dysarthria and limb and gait ataxia as manifestations of AHCD. magnetic resonance imaging of the brain revealed distinctive symmetrical T2 high-signal intensities in the bilateral cerebellar hemispheres and brachium pontis, which were consistent with his neurologic deficits. Simultaneously, high T1 signals in the bilateral pallidum and ventral midbrain were noted, which are typical manifestations of AHCD. Follow-up magnetic resonance imaging 3 months later showed the same cerebellar signs and abnormal signals. CONCLUSIONS: The cerebellar cortex and middle cerebellar peduncle are considered highly vulnerable structures to metabolic insults in liver disease. Findings from our patient suggest that dominant cerebellar deficits with compatible T2 high-signal lesions are another type of clinical manifestation in AHCD.- - - - - - - - - - ranking = 0.2keywords = hepatitis (Clic here for more details about this article) |
10/34. Rare presentation of Wilson's disease: a case report.A female presented with pain in left flank, detected to have bilateral renal calculi with deranged liver functions. On investigation found her to have Wilson's disease with hypercalciuria and incomplete distal renal tubular acidosis. Patient was started on penicillamine following which her hepatitis improved but hypercalciuria persisted after 10 weeks of follow up. The rarity of such presentation and literature review for the same is discussed.- - - - - - - - - - ranking = 0.2keywords = hepatitis (Clic here for more details about this article) |
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