1/43. A case of giant cell hepatitis recurring after liver transplantation and treated with ribavirin.A patient who underwent orthotopic liver transplantation for giant cell hepatitis with cirrhosis and in whom giant cell hepatitis recurred twice after orthotopic liver transplantation is reported. He was treated with ribavirin with an excellent result. The literature on this subject is reviewed. This observation clearly confirms the efficacy of ribavirin for the treatment of giant cell hepatitis, thus providing evidence for its viral origin.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/43. Postinfantile giant cell hepatitis complicating ulcerative colitis: a case report and review of the literature.Giant cell hepatitis is common in the neonatal period. When present in adults, it is known as postinfantile giant cell hepatitis (PGCH). PGCH can arise in the context of viral, drug-related, and autoimmune disorders but, in many other cases, its etiology remains unclear. We report a case of PGCH occurring in the setting of autoimmune hepatitis and ulcerative colitis. This case highlights the close association between PGCH and autoimmune disorders and the need to recognize it as a hepatic complication of inflammatory bowel disease.- - - - - - - - - - ranking = 0.71428571428571keywords = giant (Clic here for more details about this article) |
3/43. Recurrent, progressive giant cell hepatitis in two consecutive liver allografts in a middle-aged woman.In this report, we present a 41-year-old woman who developed rapidly progressive "giant cell hepatitis" that lead to end-stage liver disease. She underwent a successful liver transplantation in 1989. However, the giant cell hepatitis recurred in the allograft, resulting in cirrhosis within 4 years. She underwent a second liver transplantation in 1993. After 2 years of a relatively stable course, she again developed cirrhosis and was awaiting liver transplantation at the time of this report. The histopathologic features in the two allografts were identical to her original disease. Despite extensive investigations, no etiology for her liver disease could be found.- - - - - - - - - - ranking = 0.85714285714286keywords = giant (Clic here for more details about this article) |
4/43. Post-infantile giant cell hepatitis associated with autoimmune hepatitis and polyarteritis nodosa.We report the case of a patient with corticosteroid-responsive giant cell hepatitis associated with typical manifestations and changes of polyarteritis nodosa from the kidney and central nervous system. Initially, the patient presented with transient right hemiparesis, followed by spontaneous remission without any abnormalities on computed tomography scan, magnetic resonance imaging and cerebrospinal fluid examination. A few months later he was admitted to our clinic because of icterus, peripheral oedema and abdominal distension. He was found to have clinical signs of active cirrhosis. Serological tests for hepatitis b, C and hiv virus were negative. serum ceruloplasmin. a1-AT and ferritin levels were within normal limits. Antinuclear antibodies were positive (1: 160). Liver biopsy showed micronodular cirrhosis with many eosinophils in the portal tracts and giant hepatocytes with multiple nucleoli in the lobule. Fulfilling the diagnostic criteria for autoimmune hepatitis, he was started on treatment with prednisolone and azathioprine, resulting in both clinical and biochemical responses. Four years later he presented with severe pain at the right costovertebral angle. ultrasonography revealed a haematoma at the right kidney, and selective angiography of the abdominal aorta, renal arteries and hepatic artery documented microaneurysms in both kidney and liver arteries. Because of severe haemorrhage, right nephrectomy was performed. histology of kidney specimen showed characteristic lesions of polyarteritis nodosa. Several months later, while on treatment with prednisolone and cyclophosphamide, the patient experienced a fatal episode of brain haemorrhage. An association between autoimmune hepatitis, polyarteritis nodosa and postinfantile giant cell hepatitis has not been reported previously.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
5/43. Idiopathic neonatal giant cell hepatitis presenting with acute hepatic failure on postnatal day one.We report a term male infant presenting on postnatal day 1 with fulminant hepatic failure. Described congenital infection, metabolic disorders, and cardiovascular etiologies of acute neonatal liver failure were assessed and eliminated. A liver biopsy on postnatal day 10 showed neonatal giant cell hepatitis (NGCH) with an unusual degree of fibrosis for this early postnatal age. NGCH is a clinical diagnosis of cholestatic disorders of unknown etiology in the newborn, and, to our knowledge, has not been previously associated with immediate neonatal hepatic failure. The giant cell transformation is a common response to a variety of insults and only rarely occurs beyond the neonatal period. Most cases present with cholestatic jaundice and varying degrees of coagulopathy, and, many, as in this case, show progressive resolution.- - - - - - - - - - ranking = 0.85714285714286keywords = giant (Clic here for more details about this article) |
6/43. Depletion of mitochondrial dna in the liver of an infant with neonatal giant cell hepatitis.A boy presented with lactic acidosis, hepatomegaly, hypoglycemia, generalised icterus, and muscle hypotonia in the first weeks of life. At the age of 2 months, neonatal giant cell hepatitis was diagnosed by light microscopy. Electron microscopy of the liver revealed an accumulation of abnormal mitochondria and steatosis. Skeletal muscle was normal on both light and electron microscopy. At the age of 5 months, the patient died of liver failure. Biochemical studies of the respiratory chain enzymes in muscle showed that cytochrome-c oxidase (complex IV) and succinate-cytochrome-c oxidoreductase (complex II III) activities were (just) below the control range. When related to citrate synthase activity, however, complex IV and complex II III activities were normal. Complex I activity was within the control range. The content of mitochondrial dna (mtDNA) was severely reduced in the liver (17% to 18% of control values). Ultracytochemistry and immunocytochemistry of cytochrome-c oxidase demonstrated a mosaic pattern of normal and defective liver cells. In defective cells, a reduced amount of the mtDNA-encoded subunits II-III and the nuclear dna-encoded subunits Vab was found. cells of the biliary system were spared. immunohistochemistry of mtDNA replication factors revealed normal expression of dna polymerase gamma. The mitochondrial single-stranded binding protein (mtSSB) was absent in some abnormal hepatocytes, whereas the mitochondrial transcription factor A (mtTFA) was deficient in all abnormal hepatocytes. In conclusion, depletion of mtDNA may present as giant cell hepatitis. mtTFA and to a lesser degree mtSSB are reduced in mtDNA depletion of the liver and may, therefore, be of pathogenetic importance. The primary defect, however, is still unknown.- - - - - - - - - - ranking = 0.85714285714286keywords = giant (Clic here for more details about this article) |
7/43. Mutations in the sterol 27-hydroxylase gene (CYP27A) cause hepatitis of infancy as well as cerebrotendinous xanthomatosis.Follow-up investigations were undertaken on a previously reported patient who had severe familial giant cell hepatitis in infancy associated with substantially increased urinary excretion of bile alcohol glucuronides. By the age of 11 years, he had developed a profile of cholanoids in plasma and urine that closely resembled the pattern seen in cerebrotendinous xanthomatosis (CTX). Sequencing of the sterol 27-hydroxylase gene (CYP27A) showed that he was homozygous for a deletion (525/526delG) that causes a frameshift and a premature stop codon. This genotype has previously been described in an adult female with classical symptoms of CTX (tendon xanthomata, cataracts and deteriorating cognitive function). A review of past medical histories of a group of patients with CTX revealed that prolonged neonatal cholestatic jaundice was common. The family histories also revealed fetal and neonatal deaths among siblings of patients with CTX. We conclude that defective activity of cholesterol 27-hydroxylase can lead to neonatal cholestatic jaundice ('hepatitis of infancy'), which may be self-limiting. After a latent period, however, progressive accumulation of cholesterol and cholestanol can lead to the xanthomata, neurodegeneration, cataracts and atherosclerosis that are typical of CTX.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
8/43. A fatal case of postinfantile giant cell hepatitis in a patient with chronic lymphocytic leukaemia.Postinfantile giant cell hepatitis has been associated with various aetiologies, including drug taking, autoimmune diseases and viral infections. We present a fatal case of giant cell hepatitis in a patient with chronic lymphocytic leukaemia. No liver biopsy was available ante-mortem. The patient was treated with corticosteroids and aciclovir for suspected autoimmune hepatitis and reactivation of Epstein-Barr virus in the context of his haematological malignancy. Post-mortem liver biopsy showed severe giant cell hepatitis while the study of liver tissue by electron microscopy revealed paramyxo-like viral particles in the cytoplasm of the affected hepatocytes similar to those observed in previous reports of giant cell hepatitis. This case illustrates that the diagnosis of the underlying cause of giant cell hepatitis may be complicated because a heterogeneous group of different aetiologies needs to be investigated. The identification of the causative agent is essential before commencing any kind of therapy. A few sporadic case reports of paramyxo-like virus related, postinfantile giant cell hepatitis have shown that ribavirin was quite effective treatment but further clinical evaluation is needed.- - - - - - - - - - ranking = 1.4285714285714keywords = giant (Clic here for more details about this article) |
9/43. Mutations in SRD5B1 (AKR1D1), the gene encoding delta(4)-3-oxosteroid 5beta-reductase, in hepatitis and liver failure in infancy.BACKGROUND: A substantial group of patients with cholestatic liver disease in infancy excrete, as the major urinary bile acids, the glycine and taurine conjugates of 7alpha-hydroxy-3-oxo-4-cholenoic acid and 7alpha,12alpha-dihydroxy-3-oxo-4-cholenoic acid. It has been proposed that some (but not all) of these have mutations in the gene encoding delta(4)-3-oxosteroid 5beta-reductase (SRD5B1; AKR1D1, OMIM 604741). AIMS: Our aim was to identify mutations in the SRD5B1 gene in patients in whom chenodeoxycholic acid and cholic acid were absent or present at low concentrations in plasma and urine, as these seemed strong candidates for genetic 5beta-reductase deficiency. patients and subjects: We studied three patients with neonatal onset cholestatic liver disease and normal gamma-glutamyl transpeptidase in whom 3-oxo-delta(4) bile acids were the major bile acids in urine and plasma and saturated bile acids were at low concentration or undetectable. Any base changes detected in SRD5B1 were sought in the parents and siblings and in 50 ethnically matched control subjects. methods: dna was extracted from blood and the nine exons of SRD5B1 were amplified and sequenced. Restriction enzymes were used to screen the dna of parents, siblings, and controls. RESULTS: Mutations in the SRD5B1 gene were identified in all three children. Patient MS was homozygous for a missense mutation (662 C>T) causing a Pro198Leu amino acid substitution; patient BH was homozygous for a single base deletion (511 delT) causing a frame shift and a premature stop codon in exon 5; and patient RM was homozygous for a missense mutation (385 C>T) causing a Leu106Phe amino acid substitution. All had liver biopsies showing a giant cell hepatitis; in two, prominent extramedullary haemopoiesis was noted. MS was cured by treatment with chenodeoxycholic acid and cholic acid; BH showed initial improvement but then deteriorated and required liver transplantation; RM had advanced liver disease when treatment was started and also progressed to liver failure. CONCLUSIONS: Analysis of blood samples for SRD5B1 mutations can be used to diagnose genetic 5beta-reductase deficiency and distinguish these patients from those who have another cause of 3-oxo-delta(4) bile aciduria, for example, severe liver damage. patients with genetic 5beta-reductase deficiency may respond well to treatment with chenodeoxycholic acid and cholic acid if liver disease is not too advanced.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
10/43. Fate of infants with neonatal hepatitis: pediatric surgeons' dilemma.Thirty-five cases of neonatal hepatitis (20 males and 15 females) were reviewed, 3 of whom were lost during the follow-up, leaving 32 patients for review. There were 10 late deaths and 22 patients survived, 18 of whom with a normal bilirubin level and 4 with a bilirubin level of greater than 1.0 mg/dL. In the 18, jaundice disappeared between the ages of 4 and 7 months. The current lifestyles of the patients include 4 adults aged 19 to 21 who are either working or at university, while the other 18 children are all making good progress at school. Except for moderate growth retardation in 3 children, all are growing well. In all 10 patients who died, liver failure persisted until the time of death. Three died of other causes and 7 died of neonatal hepatitis itself between 4 months and 7 years of age. Four patients ran a fulminating course resulting in death between the ages of 4 and 12 months. All 7 had growth and developmental retardation. A histological examination showed that in those who died, there was significantly more periportal fibrosis, inflammation in the periportal area, and diffuse giant cell transformation. These results indicate that some infants with neonatal hepatitis have a poor prognosis and, therefore, the identification of such a condition requires a careful, long-term follow-up.- - - - - - - - - - ranking = 0.14285714285714keywords = giant (Clic here for more details about this article) |
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