Liver transplantation for acute Budd-Chiari syndrome in identical twin sisters with Factor V leiden mutation.

BACKGROUND: Budd-Chiari syndrome (BCS) is uncommon in the children. The cause of BCS comprises several diseases leading to thrombophilia. Activated protein C resistance as a result of a single gene mutation in factor V, the so called factor V Leiden (FVL), is the most common cause of thrombophilia. METHODS: We report a simultaneous occurrence of BCS in identical twin sisters of 13 years of age with heterozygous FVL mutation. RESULTS: One sister presented with acute BCS leading to fulminant hepatic failure. She underwent liver transplantation with subsequent normalization of activated protein C resistance. The other twin sister, who was diagnosed with extensive thromboses of the inferior vena cava, portal vein, and hepatic veins, was successfully managed by aggressive chemical and mechanical thrombolysis followed by therapeutic anticoagulation. Genomic DNA studies confirmed heterozygosity of FVL mutation in the sisters' father and older brother. CONCLUSIONS: The exact cause of the BCS in children should be thoroughly and rapidly investigated, and, if necessary, immediate family members should also be tested for genetic defects in factor V or concomitant thrombophilia.

Using a dopamine type 1A receptor agonist in high-risk patients to ameliorate contrast-associated nephropathy.

OBJECTIVE: The objective of our study was to evaluate the effects of fenoldopam mesylate, a dopamine type 1A receptor agonist and a potent renal vasodilator that markedly increases renal blood flow, on kidney function of patients who were receiving iodinated contrast material for an interventional procedure and thought to be at high risk of contrast-associated nephropathy. MATERIALS AND METHODS: We retrospectively reviewed the records of all patients who received fenoldopam mesylate to determine the acute and, when possible, the longer term effects on kidney function. RESULTS: Twenty-nine cases were reviewed. The average serum creatinine value before contrast administration was 2.55 mg/dL (range, 1.3-5.8 mg/dL) [corrected]. Twenty-four hours after contrast administration, serum creatinine was measured in 28 of the 29 patients. The serum creatinine values had decreased in 16 of the 28 patients by an average of 0.55 mg/dL [corrected]. In nine patients, the serum creatinine value had not changed. Two of the three increases in the serum creatinine value appear to have been caused primarily by problems that did not involve the contrast material. CONCLUSION: The use of fenoldopam mesylate at appropriate doses offers patients at high risk for contrast-associated nephropathy a chance to avoid this complication. To learn the extent and true nature of the effect of fenoldopam mesylate in this patient population requires a rigorous scientific trial, which is currently underway.