Cases reported "Liver Failure"

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1/483. Tyrosinemia type 1 should be suspected in infants with severe coagulopathy even in the absence of other signs of liver failure.

    Tyrosinemia type l is an inherited metabolic disorder attributable to deficiency of fumarylacetoacetate hydrolase, a terminal enzyme in the degradation pathway of tyrosine. Affected individuals may present with any of a number of signs and symptoms, including failure to thrive, fever, vomiting, diarrhea, hepatomegaly, ascites, jaundice, renal fanconi syndrome, or conditions such as rickets and hepatocellular carcinoma.1 If untreated, the patient may die of acute liver failure before the second year of life, or from chronic liver failure or hepatocellular carcinoma before the end of the second decade of life.2 Although overt liver failure with coagulopathy may be part of the presentation of tyrosinemia, a significant coagulopathy in the absence of overt signs of liver disease has not been emphasized as a clue to the diagnosis of this condition. We report two tyrosinemic infants who presented with severe coagulopathies and no other signs of liver failure to stress this diagnostic point.
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2/483. liver transplantation for endstage hepatitis c cirrhosis in a patient with primary hypogammaglobulinaemia.

    liver transplantation was performed in a patient with primary hypogammaglobulinaemia, chronic hepatitis c and hepatic failure. The immediate posttransplant period was uncomplicated. Owing to a stricture of the choledochojejunostomy the patient was reoperated with construction of a hepaticojejunostomy 11 months posttransplant. The patient remained hepatitis c virus (HCV) rna-positive, with high and increasing levels of HCV. Liver biopsies demonstrated the recurrence of HCV. 14 months after the transplantation the patient developed severe diarrhoea caused by cryptosporidium parvum. The infection did not respond to available therapeutic measures. He deteriorated with development of liver failure and died 18 months after the transplantation. The present case report illustrates the difficulties associated with organ transplantation in patients with primary hypogammaglobulinaemia.
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ranking = 944.44756202003
keywords = hepatitis, chronic hepatitis, b
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3/483. "The silent killer": chronic acetaminophen toxicity in a toddler.

    We report a case fatality from chronic acetaminophen (APAP) toxicity in an 18-month-old toddler, born 14 weeks premature, who had been receiving less than the standard toxic threshold of the pediatric suspension of APAP for 4 days prior to presentation. Furthermore, he had been on prolonged total parenteral nutrition (TPN) as an infant. We hypothesize that TPN-induced hepatic changes may have diminished the patient's hepatic reserve, making him more susceptible to APAP toxicity. We propose that different "therapeutic" APAP dosing may be needed for those with underlying risk factors for hepatotoxicity.
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ranking = 0.29411764705882
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4/483. Favorable nutritional outcome after isolated liver transplantation for liver failure in a child with short bowel syndrome.

    A patient with short gut syndrome presented with end-stage liver disease. She underwent an isolated liver transplantation at the age of 3.5 years. parenteral nutrition was discontinued 1.5 years after surgery. At 7 years of age, the patient has maintained normal nutritional indices and growth while on a normal oral diet. This observation suggests that liver transplantation alone can be a valuable alternative to the combined small bowel/liver transplantation in short bowel syndrome when intestinal adaptation is expected and if the ileocecal valve is present and that improvement of gut function can occur after successful liver transplantation.
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ranking = 0.82352941176471
keywords = b
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5/483. Falsely low calcium measurements after high volume plasma exchange in a patient with liver failure.

    A 63-year-old male with lactic acidosis secondary to fialuridine-induced liver failure underwent seven plasma exchanges while awaiting orthotopic liver transplantation. Following plasma exchange, total serum calcium concentrations measured by conventional clinical chemistry methods were significantly lower than the elemental calciums determined by atomic absorption spectroscopy (P = 0.004). The difference in calcium measured by atomic absorption and by conventional methods correlated with serum citrate concentration (R = 0.77) Following the first exchange, the serum lactic acid concentration decreased from 10.2 to 4.4 mmol/L. These results suggest that plasma exchange may aid in the removal of metabolic products such as lactic acid in patients with liver failure. However, the accumulation of unmetabolized citrate may also result in falsely low total calcium measurements in some patients who undergo plasma exchange.
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ranking = 0.47058823529412
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6/483. Severe hepatotoxicity associated with bromfenac sodium.

    Subacute hepatitis and liver failure occurred in a 40-yr-old woman following a 1-month course of treatment with the nonsteroidal anti-inflammatory drug bromfenac. Serologies for hepatitis a, B, and C were negative, as were antinuclear antibodies and ceruloplasmin. A transjugular liver biopsy demonstrated submassive hepatic necrosis. The clinical course was complicated by encephalopathy, fluid retention, and spontaneous bacterial peritonitis, prompting consideration for liver transplantation. With supportive measures, jaundice and fluid retention resolved over a 3-month period. We conclude that prolonged use of bromfenac was the etiological agent in this case, and that this drug can cause severe hepatotoxicity resulting in liver failure.
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ranking = 299.87148513356
keywords = hepatitis, b
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7/483. Low-dose cytarabine-induced hepatic and renal dysfunction in a patient with myelodysplastic syndrome.

    We report a 49-year-old female patient with secondary myelodysplastic syndrome who developed liver dysfunction and acute renal failure caused by low-dose cytosine arabinoside (Ara-C) therapy. Treatment of low-dose Ara-C has widely been used for acute myelogeous leukemia and myelodysplastic syndrome, and it is thought to be a low toxicity except for myelosuppression. The patient complained of a transient adverse reaction in the second and third course of therapy. This rare case indicates that careful observation should be carried out during low-dose Ara-C therapy in view of allergic reactions.
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ranking = 0.58823529411765
keywords = b
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8/483. carbamazepine-induced hepatorenal failure in a child.

    carbamazepine is used to manage generalized tonic-clonic, partial, and mixed partial and generalized seizure disorders in children. It is frequently associated with neurologic adverse events, stevens-johnson syndrome, and hematologic side effects. Hepatic and renal adverse effects are less common but can cause death. A 6-year-old boy receiving carbamazepine for partial seizure disorder developed hepatorenal failure. With discontinuation of the drug, his liver and kidney function returned to normal within 2 weeks.
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ranking = 0.47058823529412
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9/483. Late manifestation of Indian childhood cirrhosis in a 3-year-old German girl.

    We report on a 3.8-year-old German girl who presented with signs of subacute liver failure based on a 4-month history. Investigations done before admission excluded infectious, metabolic and immunological diseases. Laboratory findings were increased values of aminotransferases, hyperbilirubinaemia, hyperammonaemia and deteriorated plasmatic coagulation. Caeruloplasmin and serum copper concentrations were normal; however, urinary copper excretion was elevated (80 microg/l). Liver biopsy showed a micronodular liver cirrhosis and an extremely high liver copper concentration (1400 microg/g dry weight). Epidemiological investigations revealed an elevated copper concentration (8645 microg/l, normal: <2000) and a low pH value (6.3) of the drinking water supplied by copper pipes. The girl had been exposed to copper-contaminated drinking water since the age of 2 years. CONCLUSION: Laboratory, histopathological findings and a proven chronic copper intoxication lead to the diagnosis of Indian childhood cirrhosis in a German girl. Whereas this disease is mostly described in patients with increased copper intake in infancy, our patient developed toxic liver cirrhosis with relatively late copper exposure. Indian childhood cirrhosis should be considered in the differential diagnosis of early childhood liver cirrhosis.
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ranking = 0.76470588235294
keywords = b
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10/483. Long-term extracorporeal bilirubin elimination: A case report on cascade resin plasmaperfusion.

    Acute hepatic failure develops as a disease entity of rather diverse origin. With disease progression, toxic bilirubin levels may cause severe complications which include AV-nodal blockage, cardiac arrhythmia, impaired consciousness, generalized seizures, and status epilepticus. Treatment choices to prevent clinical deterioration comprise of costly and limited available orthotopic liver transplantation, utilization of extracorporeal bioartificial liver support devices and haemoperfusion/plasmaperfusion treatment with activated charcoal/anion exchange filters. Here, we present a patient with acute drug-induced cholestatic hepatitis. Excessively elevated bilirubin levels were accompanied by cardiac and cerebral complications. Extracorporeal resin perfusion treatment (Plasorba, BR-350) was successfully performed over a 50-day period without activation of the coagulation system or side effects. bilirubin levels were lowered to a minimum of 225 micromol/l, with concurrent clinical improvement. In conclusion, extracorporeal anion exchange plasmaperfusion may be a viable long-term treatment for hyperbilirubinaemic side effects in overt cholestatic hepatitis.
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ranking = 300.51854395709
keywords = hepatitis, b
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