Recurrent liver failure in a 25-year-old female.

Acute liver failure (ALF) is defined as severe and sudden liver dysfunction leading to coagulopathy and encephalopathy in a previously healthy person without preexisting liver disease. Almost half of adult cases of ALF are due to acetaminophen toxicity, with 21% labeled indeterminate or other. We present a patient with a second episode of ALF, both episodes being initiated by catabolic stress. Elevated aminotransferases, jaundice, an elevated international normalized ratio, and confusion were typical of idiopathic ALF, and a low serum ceruloplasmin level initially led to a misdiagnosis of acute Wilson disease. Citrullinemia type I, a urea cycle defect caused by a deficiency of argininosuccinate synthetase, was diagnosed on the basis of plasma amino acids and was confirmed by molecular testing. Urea cycle defects are not generally considered causes of ALF in adults and are described rarely in children beyond the neonatal period. Our case illustrates the importance of screening patients with idiopathic ALF for a metabolic disorder. A prompt diagnosis and timely treatment enabled her to recover fully without the need for liver transplantation.

Analysis of risk factors predictive of early mortality and urgent ERCP in acute cholangitis.

BACKGROUND: Multifactor prognostic scoring systems have been developed for acute pancreatitis to identify those patients with a potentially poor prognosis. A similar system for patients with acute cholangitis is still lacking. GOALS: To identify common clinical, biochemical, and etiologic variables that can be used to predict mortality and the need for early endoscopic retrograde cholangiopancreatography (ERCP) in patients with acute cholangitis. STUDY: A retrospective study of 108 patients with acute cholangitis was performed at a single center. Univariate analysis and logistic regression were used to identify variables that were significantly associated and predictive of mortality and need for early ERCP. RESULTS: Univariate analysis identified 18 variables significantly associated with mortality and 15 variables that predicted the need for early ERCP. Through logistic regression total bilirubin (P<0.01), partial prothrombin time (P<0.01), and presence of a liver abscess (P<0.01) were found to be significant in predicting mortality. Alanine aminotransferase (P<0.01) and white blood cell count (P<0.01) were determined to be predictive of a need for early ERCP. The scoring systems for predicting mortality (93.9%, 80.7%) and early ERCP (98%, 91%) were both highly sensitive and specific, respectively. CONCLUSIONS: Acute cholangitis is a disease that presents with varying severity. We report a scoring system that can be used to identify patients at high risk of early mortality and those that may benefit from earlier ERCP.

Safety and efficacy of aspartame-based liquid versus sucrose-based liquids used for dilution in oral sodium phosphate solutions for colonoscopy preparations.

The aim of this study was to investigate whether an oral sodium phosphate solution (OSPS) mixed with aspartame-based clear liquids as the diluent would yield improved colon cleansing results compared to an OSPS mixed with sucrose-based liquids as the diluent. Fifty-one patients undergoing colonoscopy were prospectively randomized into two groups to receive different OSPS colonoscopy preparations, with sucrose-based or aspartame-based liquids used as diluents. The primary end point was the quality of the colonoscopy preparation and secondary end points were serum electrolytes before and after preparations. No significant difference in colonoscopy preparation quality was seen between the two OSPS diluent groups (Mantel-Haenzel chi (2) = 0.795, P = 0.484). There were no significant differences in mean electrolyte shifts of sodium, potassium, blood urea nitrogen (BUN), creatinine (Cr), or BUN/Cr ratios between the two groups. There was a statistically significant increase in serum phosphorous in the aspartame-based group compared to the sucrose-based diluent group (P = 0.021). In conclusion, there was no clinically detectable difference in colonoscopy preparation quality between the two OSPS diluent groups. This study suggests that passive fluid transport by aquaporins may well be the major mediator of fluid shifts in the study subjects. This result suggests the potential importance of aquaporins and minimizes the importance of sodium glucose cotransporter SGLT1 in fluid and electrolyte transport in the human gastrointestinal tract. Aspartame or its constituent amino acids may enhance phosphate absorption across the human small intestine.