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.

[Seropositivity for Chlamydia pneumoniae in patients with primary biliary cirrhosis]. / Seropositividad para Chlamydia pneumoniae en pacientes con cirrosis biliar primaria.

INTRODUCTION: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by inflammatory injury and bile duct destruction. Recent studies suggest that Chlamydia pneumoniae could be associated with the development of PBC. The aim of this study was to determine the seroprevalence of C. pneumoniae in a cohort of patients with PBC. PATIENTS AND METHODS: The presence of IgG antibodies against C. pneumoniae was investigated in 46 patients with PBC and in 105 subjects without cirrhosis. RESULTS: Twenty-one patients (46%) with PBC had antibodies against C. pneumoniae compared with 74 subjects (71%) in the control group (OR = 0.6; 95% CI, 0.3-1.2; p = NS). Subanalysis of the PBC group showed that patients with C. pneumoniae antibodies had a higher frequency of advanced Child-Pugh stages (24% A, 52% B and 24% C vs 64% A, 32% B and 4% C; p = 0.01), a higher score on the Mayo Clinic Prognostic Index (7.8 +/- 2.1 vs 5.6 +/- 1.2; p = 0.004), a higher frequency of ascites (29% vs 4%; OR = 9.6; 95% CI, 1-87; p = 0.02), higher total bilirubin levels (4.5 +/- 2.5 mg/dl vs 2.4 +/- 4.3 mg/dl, p = 0.001) and lower serum albumin levels (2.6 +/- 0.9 g/dl vs 3.3 +/- 0.6 g/dl, p = 0.02). CONCLUSION: No association was found between C. pneumoniae infection and PBC in this study. An association was found between the severity of PBC and C. pneumoniae, which may suggest a deleterious effect of C. pneumoniae infection or a predisposition in advanced stages of PBC to acquire infection with this microorganism.

Seropositividad para Chlamydia pneumoniae en pacientes con cirrosis biliar primaria / Seropositivity for Chlamydia pneumoniae in patients with primary biliary cirrhosis

Introducción: La cirrosis biliar primaria (CBP) es una enfermedad hepática colestásica crónica, que se caracteriza por lesión inflamatoria y destrucción de conductos biliares. Estudios recientes indican que Chlamydia pneumoniae podría estar asociada al desarrollo de CBP. El objetivo de este estudio ha sido determinar la seroprevalencia de C. pneumoniae en una cohorte de pacientes con CBP. Pacientes y métodos: Se buscaron anticuerpos antiinmunoglobulina G contra C. pneumoniae en 46 pacientes con CBP y 105 sujetos sin cirrosis hepática. Resultados: Entre los pacientes con CBP, 21 (46%) tuvieron anticuerpos, en comparación con 74 (71%) del grupo control, con una odds ratio de 0,6 (intervalo de confizanza del 95%, 0,3-1,2; p no significativa). El subanálisis del grupo con CBP mostró que los pacientes seropositivos presentaron mayor frecuencia de estadios avanzados de Child-Pugh (el 24% A, el 52% B y el 24% C, frente a un 64% A, un 32% B y un 4% C; p = 0,01), mayor puntuación en la escala pronóstica de la Clínica Mayo (7,8 ± 2,1 frente a 5,6 ± 1,2; p = 0,004), mayor frecuencia de ascitis (un 29 frente a un 4%; odds ratio de 9,6; intervalo de confianza del 95%, 1,1-87; p = 0,02), concentraciones mayores de bilirrubina total (4,5 ± 2,5 frente a 2,4 ± 4,3 mg/dl; p = 0,001) y valores menores de albúmina (2,6 ± 0,9 frente a 3,3 ± 0,6 g/dl; p = 0,02). Conclusión: No fue posible establecer una asociación entre C. pneumoniae y CBP. Encontramos una relación entre la gravedad de la CBP y la seropositividad para C. pneumoniae, lo que podría apuntar a un efecto perjudicial de esta infección o bien a una predisposición en etapas avanzadas para adquirir infecciones por C. pneumoniae

Introduction: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by inflammatory injury and bile duct destruction. Recent studies suggest that Chlamydia pneumoniae could be associated with the development of PBC. The aim of this study was to determine the seroprevalence of C. pneumoniae in a cohort of patients with PBC. Patients and Methods: The presence of IgG antibodies against C. pneumoniae was investigated in 46 patients with PBC and in 105 subjects without cirrhosis. Results: Twenty-one patients (46%) with PBC had antibodies against C. pneumoniae compared with 74 subjects (71%) in the control group (OR = 0.6; 95% CI, 0.3-1.2; p = NS). Subanalysis of the PBC group showed that patients with C. pneumoniae antibodies had a higher frequency of advanced Child-Pugh stages (24% A, 52% B and 24% C vs 64% A, 32% B and 4% C; p = 0.01), a higher score on the Mayo Clinic Prognostic Index (7.8 ± 2.1 vs 5.6 ± 1.2; p = 0.004), a higher frequency of ascites (29% vs 4%; OR = 9.6; 95% CI, 1-87; p = 0.02), higher total bilirubin levels (4.5 ± 2.5 mg/dl vs 2.4 ± 4.3 mg/dl, p = 0.001) and lower serum albumin levels (2.6 ± 0.9 g/dl vs 3.3 ± 0.6 g/dl, p = 0.02). Conclusion: No association was found between C. pneumoniae infection and PBC in this study. An association was found between the severity of PBC and C. pneumoniae, which may suggest a deleterious effect of C. pneumoniae infection or a predisposition in advanced stages of PBC to acquire infection with this microorganism