Liver transplantation for neurologic Wilson's disease: reflections on two cases within a mexican cohort.

BACKGROUND: Liver transplantation (LT) in Wilson s disease (WD) is mostly indicated when progressing liver disease or acute liver failure occurs. In patients with neurological manifestations, this procedure has not gained wide acceptance based on previous reports of dismal prognosis. OBJECTIVE: To describe a Mexican cohort of pa- tients with WD with special focus on LT in patients with deteriorating neurological manifestations. MATERIAL AND METHODS: Patients with confirmed WD and their first-degree relatives were evaluated at the hepatology clinic of a tertiary referral hospital. Attention was placed on therapy and outcome. RESULTS: Eleven patients were followed for a period of up to 80 months (7 probands and 4 affected family members), 10 patients having hepatic manifestations and 4 having neuro psychia- tric phenomena. Pharmacologic treatment was uniform in most patients, and LT was indicated in 2 cases because of deteriorating neurological status. These patients had total remission of their neurological manifestations with marked improvement on imaging studies. CONCLUSIONS: Follow-up and pharmacologic treatment was flawed by several adverse conditions present in our population. Patients with progressing neurological disease had a favorable outcome after LT, a similar response to the one reported by several authors. In conclusion, LT should be strongly considered for the treatment of these patients.

Utility of the MAYO End-Stage Liver Disease score, King's College Criteria, and a new in-hospital mortality score in the prognosis of in-hospital mortality in acute liver failure.

INTRODUCTION: Several prognostic scores attempt to aid in the selection of patients with acute liver failure (ALF) to be treated either medically or by liver transplantation; however, their lack of fulfillment does not predict spontaneous survival in ALF and refined prognostic criteria are needed to improve such selection. Our aim was to evaluate and compare a new ALF in-hospital mortality prediction score versus King's College Criteria (KCC) and model for End-Stage Disease (MELD) score. METHODS: First-time ALF-diagnosed individuals admitted to our institution (n = 58) were grouped according their final outcome as "alive" or "death," and those significantly different variables between groups entered into a logistic regression and lineal regression models. An ALF in-hospital mortality score (ALFIHMS) was produced and its sensitivity, specificity, and area under receiver operator characteristics were compared with those of KCC and MELD scores. RESULTS: Since no significant differences (P = .81) in mortality rates between fulminant and subfulminant hepatic failure were found, no further analysis according to ALF's classification was performed. After obtaining and comparing ALFIHMS with KCC and MELD, we found that ALFIHMS prediction accuracy is higher than that of KCC and MELD score and that an ALFIHMS cutoff point >15 points is associated with an in-hospital mortality probability >50%. CONCLUSIONS: ALFIHMS has higher prognostic accuracy than KCC and MELD scores in ALF.