1/96. 3-hydroxy-3-methylglutaric aciduria presenting with Reye like syndrome: report of one case.We report the case of a patient with 3-hydroxy-3-methylglutaric aciduria who presented with a repeat attack of Reye like syndrome clinically. vomiting and somnolence, generalized tonic and clonic convulsions with hepatomegaly, hyperammonemia, liver function impairment, and mild metabolic acidosis were the presenting signs. 3-hydroxyisovaleric, 3-methylglutaric, 3-methylglutaconic and 3-hydroxy-3-methylglutaric acids were detected in the urine by gas chromatography-mass spectrometry. 3-methylglutarylcarnitine was also identified in the urine by fast atom bombardment and tandem mass spectrometry. Therefore, the possibility of metabolic disease should be considered in neonates and infants with repeat attacks of Reye like syndrome and a history of similarly affected siblings.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
2/96. Influenza infection in patients before and after liver transplantation.infection with influenza virus poses specific problems in pediatric and adult liver transplant recipients, both before and after liver transplantation. These include a higher rate of pulmonary and extrapulmonary complications, development of rejection with graft dysfunction, prolonged shedding of influenza virus, and increased drug-resistance. Hepatic decompensation may occur during influenza infection in patients with cirrhosis. Current prophylaxis includes yearly vaccination with trivalent inactivated vaccine. Appropriate diagnosis and prompt treatment of any upper respiratory infections are indicated in these patients. In this review, we describe a case of influenza viral pneumonia in an adult liver transplant recipient, review basic and clinical aspects of influenza infection in this patient population, and discuss current modes of prevention and treatment in detail.- - - - - - - - - - ranking = 7keywords = liver (Clic here for more details about this article) |
3/96. Hereditary fructose intolerance presenting as Reye's-like syndrome: report of one case.Hereditary fructose intolerance (HFI) is an autosomal recessive disease caused by catalytic deficiency of aldolase B (fructose-1, 6-bisphosphate aldolase). Herein we report on a case of hereditary fructose intolerance with initial presentation of episodic unconsciousness, seizure, hypoglycemia, hepatomegaly, and abnormal liver function since the patient was 11 months old. She was diagnosed as Reye's-like syndrome according to a liver biopsy done at 20 months of age. As she grew up, cold sweating, abdominal pain or gastrointestinal discomfort shortly after the intake of fruits was noted and she developed an aversion to fruits, vegetables and sweet-tasting foods. At 9 years of age, a fructose tolerance test signified a positive result that induced hypoglycemia, transient hypophosphatemia, hyperuricaemia, elevation of serum magnesium, and accumulation of lactic acid. Appropriate dietary management and precautions were recommended. The patient has been symptom-free and exhibited normal growth and development when followed up to 12 years of age.- - - - - - - - - - ranking = 2keywords = liver (Clic here for more details about this article) |
4/96. Reye's syndrome in an adult: a case report.Reye's syndrome (RS), a condition characterized by encephalopathy and fatty liver, is associated with aspirin use and carries a poor prognosis. The majority of patients with RS are children and adolescents. We report a case of RS in an adult.- - - - - - - - - - ranking = 91.271519803314keywords = fatty liver, fatty, liver (Clic here for more details about this article) |
5/96. Metabolic cause of Reye-like syndrome.The most frequent metabolic cause of Reye-like syndrome is medium chain acyl-CoA dehydrogenase (MCAD) deficiency. The authors describe a gypsy boy who was repeatedly hospitalised due to symptoms of Reye-like syndrome (serious hypoglycemia, loss of consciousness, seizures, increased values of aminotransferases, decreased values of free carnitine). The diagnosis of MCAD deficiency was established by analysis of plasmatic acylcarnitines by use of tandem mass spectrometry. dna analysis proved the most common K329E (G985) mutation in gene for MCAD deficiency in homozygous state. The authors have emphasised the advantage of tandem mass spectrometry in the diagnosis of disorders of fatty acid beta-oxidation. This highly sophisticated method can detect most of these disorders from dry blood spots disregarding the symptoms and type of mutation.- - - - - - - - - - ranking = 12.381278640048keywords = fatty (Clic here for more details about this article) |
6/96. brain ultrastructure in Reye's syndrome.Cerebral biopsies were obtained for electron microscopy 48 and 72 hours after the onset of encephalopathy from a child with severe Reye's syndrome. Gravely ill at the time of craniectomy to relieve cerebral hypertension, the child survived and recovered good brain function; therefore, the biopsy findings appear to reflect the organelle pathology of the brain at a severe yet reversible stage in the disease process. The cardinal ultrastructural changes in the brain in Reye's syndrome are astrocyte swelling and partial deglycogenation, myelin bleb formation and universal injury of neuron mitochondria. The mitochondrial injury consists of matrix disruption with moderate but not massive swelling. dilatation of rough endoplasmic reticulum and nuclear changes occurred only in neurons with severely altered mitochondria. The organelle pathology of the brain in this case did not resemble the organelle pathology of the brain in human "hepatic encephalopathy" or in experimental ammonia intoxication in primates. The mitochondrial ultrastructure of the cerebral neurons resembled the unique mitochondrial ultrastructural changes seen in the liver parenchyma in Reye's syndrome.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
7/96. Acute liver disease and encephalopathy mimicking reye syndrome. A report of three cases.Three patients are described whose clinical presentation suggested reye syndrome, and in whom the initial laboratory investigations supported the diagnosis. The subsequent clinical and biochemical evolution of the illness differed from that of reye syndrome. The liver biopsy of each patient revealed changes in centrilobular hepatocytes rather than the diffuse small droplet fatty change characteristic of reye syndrome. In each of them normal liver functions were regained. The findings in these patients suggest that a firm diagnosis of reye syndrome cannot be made without histologic examination of the liver.- - - - - - - - - - ranking = 19.381278640048keywords = fatty, liver (Clic here for more details about this article) |
8/96. Acute liver failure and encephalopathy (Reye's syndrome?) during salicylate therapy.A case of hepatotoxicity and encephalopathy (Reye's syndrome?) associated with salicylate therapy is presented and the aetiology of this syndrome is discussed. Hepatotoxicity developed with salicylate serum concentrations not exceeding therapeutic serum levels. The importance of controlling serum salicylate concentration and transaminase activity particularly during the first fourteen days of therapy is emphasized.- - - - - - - - - - ranking = 4keywords = liver (Clic here for more details about this article) |
9/96. Reye's syndrome in an adult.Reye's syndrome (fatty infiltration of the liver with encephalopathy) is an uncommon disease of children and has not previously been noted in adult patients. We present a 25-year-old male who survived this syndrome after presenting a stuporous state. Etiologic and pathophysiologic mechanisms are considered.- - - - - - - - - - ranking = 13.381278640048keywords = fatty, liver (Clic here for more details about this article) |
10/96. An aflatoxin in the liver of a patient with Reye-Johnson syndrome.Liver specimens from 8 patients with Reye-Johnson syndrome, 2 patients with acute encephalopathy, and 10 patients without liver disease were analyzed for aflatoxins. An aflatoxin was obtained from the liver of one patient, a 15-year-old girl who had clinical, laboratory, and pathologic features of Reye-Johnson syndrome. Thin-layer chromatography, fluorescence emission, infrared spectroscopy, and derivatization studies characterized the aflatoxin as related structurally to but not identical with aflatoxin b1. This report is added to previous ones from thailand, new zealand, and czechoslovakia in which the association of aflatoxins and fatty liver has been found in Reye-Johnson syndrome.- - - - - - - - - - ranking = 97.271519803314keywords = fatty liver, fatty, liver (Clic here for more details about this article) |
| | Next -> |