1/98. 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.- - - - - - - - - - ranking = 1keywords = duct (Clic here for more details about this article) |
2/98. Severe multisystemic hypersensitivity reaction to carbamazepine including dyserythropoietic anemia.OBJECTIVE: To report a case of multisystemic hypersensitivity reaction to carbamazepine. CASE SUMMARY: An 81-year-old white man was admitted to our hospital because of fever, morbilliform pruritic rash, and jaundice. Fifty days before admission he had taken carbamazepine 200 mg p.o. tid because of seizures. During the first few days following admission, a maculopapular rash progressed to generalized erythroderma with subsequent extensive skin exfoliation. After discontinuing carbamazepine the fever disappeared within 72 hours and hepatic function tests returned to normal within four days. Moreover, after admission the hemoglobin values gradually fell to 6.7 g/100 mL. A bone marrow aspirate showed hypercellularity with marked dyserythropoietic abnormalities, and the bone marrow biopsy showed large and diffused infiltration due to the presence of a low-grade small lymphocytic lymphoma. No specific therapy for the lymphoma was undertaken. The biochemical follow-up showed a total improvement of hemoglobin values. Eight months after drug discontinuation, the patient was asymptomatic; peripheral blood cell count and hemoglobin concentrations were persistently normal. DISCUSSION: To the best of our knowledge, this is the first published case report implicating carbamazepine as the cause of anemia associated with bone marrow hypercellularity and dyserythropoietic changes, instead of hypocellularity and reduction of erythroid precursors. An interesting point raised by our observation is the possible relation between carbamazepine intake and actual lymphoproliferative disease. The development of non-Hodgkin's lymphoma following carbamazepine treatment has been reported, with regression after the drug was discontinued. However, in our case, a bone marrow biopsy repeated eight months after drug discontinuation confirmed the diagnosis of low-grade lymphoma. CONCLUSIONS: This case report describes a severe multisystemic reaction, characterized by generalized erythroderma; and renal, hepatic, and bone marrow failure in a patient who started carbamazepine therapy 50 days beforehand.- - - - - - - - - - ranking = 1keywords = duct (Clic here for more details about this article) |
3/98. Erythropoietic protoporphyria with fatal liver failure.A 33-year-old woman with a history of photosensitivity, persistent abdominal pain, and liver dysfunction was admitted to our department because of abdominal pain and progression of liver dysfunction. On admission, levels of protoporphyrin and coproporphyrin within erythrocytes were markedly increased. Autofluorescent erythrocytes were also detected, leading to a diagnosis of erythropoietic protoporphyria. A liver biopsy specimen revealed cirrhosis with dark brown granules filling hepatocytes, bile canaliculi, and bile ductules. Transfusion of washed erythrocytes, hemodialysis, and administration of cholestyramine and beta-carotene transiently improved levels of porphyrins and liver function. The patient died of rupture of esophageal varices followed by multiple organ failure. However, the treatments were believed to have extended survival.- - - - - - - - - - ranking = 40.509306285245keywords = bile duct, bile, duct (Clic here for more details about this article) |
4/98. Hepatopathy in two infants with short-bowel syndrome and cytomegalovirus infection.In children with short-bowel syndrome and the need for long-term parenteral nutrition, hepatic dysfunction is a multifactorial phenomenon that has not been completely understood. Alterations in gut motility lead to intraluminal stasis which is thought to be a major etiologic factor for bacterial overgrowth and subsequent cholestasis, especially when the ileocecal valve is absent. We report on two infants with short-bowel syndrome caused by gastroschisis and intestinal atresia. The intestinal lengths after resection were 18 and 55 cm. Long-term parenteral nutrition (PN) was obligatory due to intestinal shortness in the first patient and dilatation of the preatretic bowel segment with ineffective peristalsis in the second patient. Despite multiple trials of enteral nutrition and medical therapy for gut decontamination and stimulation of bowel motility, hepatopathy developed in both patients in a similar period of time and to about the same degree. At the age of 4 and 6 weeks, respectively, increasing bilirubin values were measured. Deterioration of liver function and thrombocytopenia at the age of 3 to 4 months led to the diagnosis of acute cytomegalovirus (CMV) infection. Treatment with ganciclovir followed. Both patients died of acute liver failure at the age of 7 and 9 months, respectively. Additional hepatic injury secondary to CMV infection might have contributed to the rapid deterioration of liver disease. Screening for further hepatotoxic factors, especially infectious etiologies, is therefore recommended in children with short-bowel syndrome. liver transplantation should be considered early in cases of progressive hepatic dysfunction.- - - - - - - - - - ranking = 2688.290399712keywords = cholestasis (Clic here for more details about this article) |
5/98. Subacute hepatic failure associated with a new antidiabetic agent, troglitazone: a case report with autopsy examination.An autopsy case of fatal subacute hepatic failure after administration of troglitazone is described. The liver dysfunction developed about five months after the patient, a sixty-three-year-old woman, had been initially treated with troglitazone. The patient developed hepatic failure and died despite various hepatic auxiliary treatments such as plasmapheresis. autopsy findings revealed focal liver cell necrosis, cholestasis and steatosis with infiltration of lymphocytes and neutrophils and lack of regenerative activity. The causative mechanism of liver dysfunction may be metabolite aberration, as a result of accumulation of hepatotoxic metabolite(s), in a category of idiosyncratic liver injury. It is proposed to monitor liver function strictly and periodically for the diabetic patients prescribed troglitazone.- - - - - - - - - - ranking = 2688.290399712keywords = cholestasis (Clic here for more details about this article) |
6/98. liver transplantation in a patient with acute liver failure due to sickle cell intrahepatic cholestasis.BACKGROUND: Sickle cell intrahepatic cholestasis is a potentially catastrophic complication of sickle cell anemia Once acute liver failure develops, transplantation is the only option. We describe a patient with sickle cell intrahepatic cholestasis who underwent liver transplantation. methods: Data were obtained from the chart. Serial hemoglobin S levels were monitored, and measures were taken to maintain hemoglobin S <20% to prevent sickle cell crisis. RESULTS: Although the allograft functioned well initially, the patient developed veno-occlusive disease and required repeat transplantation at 5 months after transplant. Histologic examination of the explant revealed occlusion of the terminal hepatic venules due to fibrosis and packed red cells. Repeat transplant was complicated by thrombosis of the intrahepatic portion of the hepatic artery, and sepsis. The patient died of sepsis after a third transplant. CONCLUSION: liver transplantation for sickle cell disease involving the liver may carry a high risk of graft loss due to vascular problems. Repeat transplantation may not be feasible if disease recurs.- - - - - - - - - - ranking = 16129.742398272keywords = cholestasis (Clic here for more details about this article) |
7/98. Neonatal repair of Ebstein's anomaly: indications, surgical technique, and medium-term follow-up.BACKGROUND: Ebstein's anomaly in the severely symptomatic neonate is usually fatal. Because the mortality for various surgical interventions has been prohibitively high, the indications for operation in these critically ill neonates are unclear. methods: We reviewed our results with biventricular repair of three consecutive severely symptomatic neonates (2.8 to 3.2 kg) at our institution since 1994. Each had associated complex cardiac pathology, including multiple muscular ventricular septal defects (n = 1), pulmonary stenosis with functional pulmonary atresia (n = 1), and anatomic pulmonary atresia (n = 1). Preoperatively, all infants had severe tricuspid regurgitation, Great Ormond Street Ebstein echocardiogram scores greater than 1.3:1 (grade 3 or 4) and cardiothoracic ratio greater than 0.85. Two patients were severely cyanotic. Hepatic and renal insufficiency with diffuse coagulopathy was present preoperatively in two patients. Surgical repair consisted of (1) reconstruction of a competent monocuspid tricuspid valve, (2) right ventriculorrhaphy, (3) subtotal closure of atrial septal defect (ASD), (4) aggressive reduction atrioplasty, and (5) repair of all associated cardiac defects. RESULTS: There were no early or late deaths. All patients are currently asymptomatic, without medications, and in sinus rhythm. At 5-year follow-up, trivial tricuspid regurgitation is present in 1 and mild regurgitation in 2 patients. On the basis of these results and review of the current literature, we propose new indications for surgical repair in the neonate with Ebstein's anomaly. CONCLUSIONS: Biventricular repair of Ebstein's anomaly in the critically ill neonate is feasible and medium-term durability of the repair is excellent. Therefore, conventional management of these patients should be revised and early surgical repair encouraged.- - - - - - - - - - ranking = 1keywords = duct (Clic here for more details about this article) |
8/98. Cutaneous porphyria in a neonate with tyrosinaemia type 1.A term infant born to consanguineous parents presented at birth with hypoglycaemia, thrombocytopenia, coagulopathy and hyperbilirubinaemia associated with polycythaemia due to delayed cord clamping. Despite phototherapy and correction of polycythaemia by partial exchange transfusion, coagulopathy, hypoglycaemia and conjugated hyperbilirubinaemia persisted, suggesting hepatic failure. Metabolic work-up led to the diagnosis of tyrosinaemia type 1 on day 4. Two--(2-nitro-4-trifluoromethylbenzoyl)--1,3 cyclohexanedione (NTBC) treatment, started on day 5, resulted in progressive clinical improvement and unambiguous biochemical response. Severe skin purpuric lesions occurred in areas exposed to phototherapy. These resolved slowly after its discontinuation. urine analysis sampled just before and 6 days after starting NTBC treatment showed high levels of type 1 coproporphyrin isomers. Such findings do not seem directly related to tyrosinaemia type 1 where succinylacetone inhibits delta-aminolevulinic acid (delta-ALA) dehydratase and where the accumulation of delta-ALA results in neurotoxicity without photosensitivity. CONCLUSION: We describe a cutaneous form of porphyria in a neonate presenting with severe liver failure due to tyrosinaemia type 1. This porphyria is tentatively attributed to a secondary accumulation of coproporphyrins due to cholestasis, as reported in the bronze baby syndrome and recently described in neonates with purpuric phototherapy-induced eruption, rather than to a primary defect of porphyrin metabolism. The hypothesis of a direct effect of tyrosinaemia type 1 on porphyrin excretion is also discussed.- - - - - - - - - - ranking = 2688.290399712keywords = cholestasis (Clic here for more details about this article) |
9/98. Liver injury due to tetrabamate (Atrium): an analysis of 11 cases.BACKGROUND: Tetrabamate (Atriums), widely used in the treatment of tremor and ethanol-withdrawal symptoms, has been incriminated as a potential cause of reversible acute hepatitis. OBJECTIVE: We report here on 11 patients who experienced tetrabamate-related liver injury, in order to evaluate their clinical, histopathological and evolutive features. patients AND methods: Between 1987 and 1998, 34 cases of tetrabamate-associated acute hepatitis were spontaneously reported to the regional pharmacovigilance center of Lyon. Eleven cases were considered probably to be drug-induced. RESULTS: There were three males and eight females aged 31-82 years (mean, 57 years). The duration of treatment ranged from 33 to 206 days, and indication for treatment was depressive disorders, tremor or prevention of alcohol withdrawal symptoms. Clinical symptoms were asthenia (n = 9), jaundice (n = 3) and/or diffuse rash (n = 3). The pattern of liver injury was cytolytic (n = 10) or cholestatic (n = 1). Three patients presented biological features of hepatic failure. A percutaneous liver biopsy was performed in six patients. Histological examination of the liver specimen showed a large spectrum of lesions: massive hepatocellular necrosis (n = 1), centrilobular and nonconfluent hepatocyte necrosis (n = 2), intracellular cholestasis (n = 3), and granulomatous hepatitis (n = 1). Tetrabamate was discontinued in all patients. In seven patients, a complete recovery was observed 3 weeks to 3 months after drug withdrawal. Two patients, despite a rapid improvement of liver function tests, died from unrelated causes. The remaining two patients died from irreversible hepatic failure. CONCLUSIONS: Our data strongly suggest that tetrabamate may induce acute liver injury, which may eventually result in life-threatening liver failure.- - - - - - - - - - ranking = 2688.290399712keywords = cholestasis (Clic here for more details about this article) |
10/98. Cholestatic syndromes in renal transplant recipients with HCV infection.We present two distinct types of cholestatic syndrome identified in eight renal transplant (RTx) patients with HCV infection. Four patients developed fibrosing cholestatic hepatitis (FCH) and four, vanishing bile duct syndrome (VBDS). All patients with FCH were anti-HCV (-) at the time of Tx and developed a cholestatic profile 1-4 months post-Tx, with high HCV-rna levels. Immunosuppressive therapy was drastically reduced. Two patients died of sepsis and liver failure 16 and 18 months post-Tx, and the other two showed marked improvement and seroconverted to anti-HCV. Regarding the patients with VBDS, three were anti-HCV (-) and one was anti-HCV ( )/HBsAg ( ) at the time of RTx. Two patients became anti-HCV ( ) 1 year, and one patient, 3 years post-Tx. Two patients developed progressive VBDS and died of liver failure 2 and 3 years after onset, and two showed marked improvement after withdrawal of immunosuppression. In two of the patients, the progression of the disease coincided with elevation in serum HCV rna levels. We concluded that a progressive cholestatic syndrome acquiring features of FCH or VBDS may develop in HCV-infected RTx patients. The association with high viral load implicated the virus in the pathogenesis. Drastic reduction of immunosuppression may favourably affect the outcome.- - - - - - - - - - ranking = 34.378365374863keywords = bile duct, bile, duct (Clic here for more details about this article) |
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