RESUMEN
BACKGROUND: The prognosis of Wilson's disease (WD) has changed radically since the introduction of medical therapy with chelators and zinc. However, there is an unmet need for methods to evaluate the long-term treatment response and the liver disease progression in order to identify treatment failures. The galactose elimination capacity test (GEC) is a physiological measure of the total metabolic capacity of the liver, and a strong predictor of long- and short-term mortality in patients with liver cirrhosis. Our aim was to investigate if the GEC test is useful for evaluation of treatment response and prediction of treatment failures in WD patients. METHODS: We included all patients with WD in Denmark from 1992 through 2017 and retrieved data on GEC along with data on transplantation and death. RESULTS: In total, 37 patients had completed one or more GEC tests. Of these, 31 were alive (three transplanted) and six were dead (two transplanted). A total of 24 patients had completed more than one GEC test. All 18 alive, nontransplanted patients showed improvement in GEC values following onset of treatment, except one patient, who was clinically confirmed with treatment failure. All six patients who underwent liver transplantation or died had a prior decline in their GEC. The difference in GEC development between patients alive and not transplanted and patients dead or transplanted was significant (p < .001). Index GEC values could not predict transplantation or death (p = .26). CONCLUSION: The GEC test is a promising tool for monitoring treatment response and identifying treatment failures in patients with WD.
Asunto(s)
Degeneración Hepatolenticular , Galactosa/metabolismo , Degeneración Hepatolenticular/diagnóstico , Humanos , Cirrosis Hepática , Pruebas de Función HepáticaRESUMEN
Atrial fibrillation (AF) is the most common arrhythmia and increases with age. This rising prevalence of AF is contributing to an increasing public health and economic burden. The 2018 Healthcare Cost and Utilization Project National Inpatient Sample dataset was used. All patients ≥15 years with a principal discharge diagnosis of AF were included. The patient population was divided into an "older" cohort (aged ≥65 years) and a "younger" (aged <65 years). Desired outcomes included hospital length of stay, discharge disposition, hospital charges, and in-hospital mortality. A generalized linear mixed model was used to calculate hospitalization rates for the "younger" and "older" groups. We identified 896,328 AF hospitalizations. Younger patients (18.1%) were more likely to be male (65.5% vs 49.9%), to smoke (21.6% vs 6.1%), and to use alcohol (9.7% vs 2.1%). Older patients were more likely to have heart failure (49.6% vs 43.9%) and hypertension (84.6% vs 76.1%). Hospitalization rates increased with increasing age groups. Older patients had higher in-hospital mortality (4.6% vs 2.9%) and were more likely to be discharged to another facility (31.6% vs 13.2%). AF hospitalization rates vary between hospitals across the United States. Hospital divisions with greater than expected admissions for AF, when compared with the national mean, were driven by higher "older" patient hospitalizations. In conclusion, older patients account for most AF hospitalizations. Older patients have higher AF morbidity and mortality. Hospitalization rates for AF increase with increasing increments of age.
Asunto(s)
Fibrilación Atrial , Humanos , Masculino , Femenino , Fibrilación Atrial/epidemiología , Fibrilación Atrial/terapia , Hospitalización , Alta del Paciente , Hospitales , Pacientes InternosRESUMEN
BACKGROUND: Hepatic macrophage (Kupffer cell) hyperplasia is often described in Wilson's disease (WD). In many liver diseases, Kupffer cell activation is related to disease severity, liver function, and fibrosis but the importance in WD is unknown. Kupffer cell activation can be assessed by the P-concentration of soluble (s)CD163, metabolic liver function by the galactose elimination capacity (GEC), and fibrosis by Fibroscan. We investigated the associations between sCD163, selected inflammatory cytokines, GEC, and liver fibrosis in Danish WD patients. METHODS: In a cross-sectional design, we studied 29 stable and well-treated patients (male/female15/14) with a median age of 35 years (IQR 24-50). P-sCD163 and cytokines were measured by ELISA. The GEC was measured by intra-venous galactose loading. RESULTS: The median P-sCD163 value at 2.96 mg/L (1.97-3.93) was high in the normal range (0.7-3.9) and seven patients (24%) had a value above the upper normal value. sCD163 correlated with TNF-α, IL-6 and IL-8 (rho> 0.50, p < 0.005). A higher sCD163 value was closely associated with a lower GEC (rho = - 0.51, p = 0.02). sCD163 was not related to the liver fibrosis indices. CONCLUSIONS: Stable WD patients showed various degrees of Kupffer cell activation which was accompanied by loss of metabolic liver function. Neither activation nor liver function was related to liver fibrosis. The findings suggest that in WD inflammatory Kupffer cell activation may be involved in the loss of liver function over time. sCD163 may serve as a non-invasive biomarker of loss of liver function in WD, which the degree of fibrosis evidently may not. This study is registered at clinical trials with name: "sCD163 and sMR in Wilsons Disease - Associations With Disease Severity and Fibrosis", NCT02702765. Date of registration: 26.02.16. Date of enrolment of the first participant to the trial: 17.03.16. ULR: https://clinicaltrials.gov/ct2/show/NCT02702765 .
Asunto(s)
Degeneración Hepatolenticular/metabolismo , Degeneración Hepatolenticular/patología , Activación de Macrófagos/fisiología , Antígenos CD/metabolismo , Antígenos de Diferenciación Mielomonocítica/metabolismo , Biomarcadores/metabolismo , Estudios Transversales , Humanos , Interleucina-6/metabolismo , Interleucina-8/metabolismo , Hígado/metabolismo , Hígado/patología , Cirrosis Hepática/metabolismo , Cirrosis Hepática/patología , Receptores de Superficie Celular/metabolismo , Factor de Necrosis Tumoral alfa/metabolismoRESUMEN
Non-alcoholic fatty liver disease (NAFLD) is characterized by liver fat accumulation and non-alcoholic steatohepatitis (NASH) with inflammation and fibrosis, which may lead to cirrhosis also in childhood. NAFLD/NASH in children are related to obesity and the metabolic syndrome, and incidence and prevalence are expected to increase. Children having liver steatosis and elevated liver enzymes are most often asymptomatic, and a liver biopsy is necessary for correct diagnosis and staging. The treatment should focus on lifestyle changes, as pharmacological therapy needs further evaluation.