Acute liver failure (ALF) due to Wilson disease (WD) is invariably fatal without emergency liver transplantation. Therefore, rapid diagnosis of WD should aid prompt transplant listing. To identify the best method for diagnosis of ALF due to WD (ALF-WD), data and serum were collected from 140 ALF patients (16 with WD), 29 with other chronic liver diseases and 17 with treated chronic WD. Ceruloplasmin (Cp) was measured by both oxidase activity and nephelometry and serum copper levels by atomic absorption spectroscopy. In patients with ALF, a serum Cp <20 mg/dL by the oxidase method provided a diagnostic sensitivity of 21% and specificity of 84% while, by nephelometry, a sensitivity of 56% and specificity of 63%. Serum copper levels exceeded 200 g/dL in all ALF-WD patients measured (13/16), but were also elevated in non-WD ALF. An alkaline phosphatase (AP) to total bilirubin (TB) ratio <4 yielded a sensitivity of 94%, specificity of 96%, and a likelihood ratio of 23 for diagnosing fulminant WD. In addition, an AST:ALT ratio > 2.2 yielded a sensitivity of 94%, a specificity of 86%, and a likelihood ratio of 7 for diagnosing fulminant WD. Combining the tests provided a diagnostic sensitivity and specificity of 100%. In conclusion, conventional WD testing utilizing serum ceruloplasmin and/or serum copper levels are less sensitive and specific in identifying patients with ALF-WD than other available tests. More readily available laboratory tests including alkaline phosphatase, bilirubin and serum aminotransferases by contrast provides the most rapid and accurate method for diagnosis of ALF due to WD.

OBJECTIVES Hyperamylasemia (HA) is often reported in patients with acute liver failure (ALF). Direct toxic effects of acetaminophen on the pancreas have been postulated, but the occurrence of HA in other etiologies raises the question of whether multiorgan failure is part of the pathogenesis of HA in this setting. Our main aim was to describe and analyze the incidence, clinical characteristics, and outcomes of HA in ALF of different etiologies. METHODS Patients enrolled in the Acute Liver Failure Study Group registry with an admission amylase value available were included. For the purpose of this analysis, HA was defined as ≥3× upper limits of normal. Patients were classified as having acetaminophen (APAP)- or non-APAP–induced ALF, and by amylase group: normal (< 115), mildly elevated (115–345), or HA (>345). Significant variables identified by univariate analysis were added to a multiple linear regression model. The primary outcome was overall survival. RESULTS In total, 622 eligible patients were identified in the database, including 287 (46%) with APAP-induced ALF; 76 (12%) patients met the criteria for HA. Among patients with HA, 7 (9%) had documented clinical pancreatitis. The incidence of HA was similar among APAP (13%) and non-APAP (12%) patients. Although HA was associated with renal failure and greater Model for End-stage Liver Disease scores for both groups, HA was not an independent predictor of mortality in multivariate analysis. CONCLUSIONS Although not an independent predictor of mortality, HA in ALF was present in all etiologies and was associated with diminished overall survival. HA appeared to be related to renal dysfunction in both groups and multiorgan failure in non-APAP ALF.

Background:Red cell exchanges (RCEs) lead to improvement in tissue oxygenation and reduction in inflammatory markers in sickle cell disease (SCD) patients who present with acute chest syndrome (ACS). The aim of this study is to evaluate the effects of automated-RCE (auto-RCE) on oxygen saturation (SpO2) on-air, blood counts, the time to correct the parameters and length of hospitalization after the exchange in SCD patients presenting with ACS.Subjects and Methods:This was 2 years study involving five SCD patients; the time for SpO2 on air to increase to ≥95% and chest symptoms to resolve, postprocedure, as well as the length of in-patient hospitalization was recorded. All data were entered into Statistical Package for Social Sciences Version 20.0 (SPSS Inc., Chicago, IL, USA) computer software for analyses.Results:The study involved 4 (80%) hemoglobin (Hb) SS and 1 (20%) HbSC patients. The median time of SpO2 recovery was 24 h, ranging from 6 to 96 h. About 60% (3/5) of patients achieved optimal SpO2 within 24 h post-RCE, while discharge from intensive care unit was 24 h after auto-RCE in one patient. The Hb concentration was significantly higher, while the total white cell and absolute neutrophil counts were significantly lower at the time of resolution of symptoms, compared to before auto-RCE (P < 0.05). The average post auto-red cell transfusion symptoms duration was 105.6 (24–240) h while mean inpatient stay was 244.8 (144–456) h.Conclusion:Auto-RCE could reverse hypoxia in ACS within 24 h.

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