The aim of this study was to assess the factors, including surgical portosystemic shunts, which affect survival in adults with Budd-Chiari syndrome. Multivariate retrospective analysis was performed using characteristics recorded at the time of diagnosis in 120 patients admitted from 1970 to 1992, of whom 82 were treated with surgical portosystemic shunts and 38 received only medical therapy. The 1-, 5-, and 10-year survival rates were 77 ؎ 4%, 64 ؎ 5%, and 57 ؎ 6%, respectively. Survival was significantly better in the subgroup of patients diagnosed after versus before 1985. In both subgroups, and in patients with, as well as in patients without surgical shunts, 4 factors were found to be inversely and independently related to survival: age, response of ascites to diuretics, Pugh score, and serum creatinine. In patients diagnosed since 1985, an index combining these 4 factors allowed to differentiate patients with a good outcome (5-year survival 95%) from those with a poor outcome (5-year survival 62%; P F .05). There was no statistically significant and independent influence of surgical portosystemic shunts on survival. In conclusion, age, severity of liver failure, and presence of refractory ascites are the main prognostic factors in Budd-Chiari syndrome. Increased survival in recent years is consistent with improved management of hypercoagulable states as well as improved general care. It is uncertain whether surgical portosystemic shunting favorably modifies survival. Therefore, we recommend that surgical shunting should be restricted to management of refractory ascites or variceal bleeding in patients with otherwise good prognostic factors. (HEPATOLOGY 1999;30:84-89.)
venous lesions have been recognized. The first is thrombosis In contrast with the well-recognized membranous obfollowed by diffuse fibrous obliteration. This lesion affects struction of the inferior vena cava, short-length hepatic the hepatic veins more commonly than the inferior vena cava, vein stenoses are not well-recognized causes of hepatic and, when systematically investigated, an associated hypervenous outflow block. The aim of this study was to ascercoagulable state is usually identified. [2][3][4] The second type of tain the prevalence, causes, manifestations, and outvenous lesion is represented by a short-length stenosis, often come of short-length hepatic vein stenoses. We described as a web or a membrane, located at the terminal performed a retrospective study of patients with shortpart of a vein. This lesion usually affects the inferior vena length hepatic vein stenosis among 86 patients with hecava and is commonly encountered in the absence of an obvipatic venous outflow block who were seen between 1970 ous thrombogenic condition. 5,6 Short-length inferior vena caand 1992. There were 25 patients with short-length heval stenosis has long been thought to be of congenital origin, 5 patic vein stenosis. A thrombogenic condition was idenbut recent evidence suggested that it can be the sequela of a tified in 14 patients (56%). The lesions of the accompaprevious caval thrombosis.6 nying hepatic veins in these patients were variable Short-length hepatic vein stenoses has received little atten-(short-length stenoses, thromboses, or nonspecific tion. The purpose of this report is to describe the prevalence, changes) and similar to that seen in patients without causes, manifestations, and outcome of hepatic venous outshort-length hepatic vein stenosis. In 3 necropsied cases, flow block caused by short-length hepatic vein stenoses. the venous lesions were suggestive of fibrous sequela of prior thromboses. In patients with short-length hepatic PATIENTS AND METHODS vein stenosis, splenomegaly (28% vs. 55%, P õ .05) and hypersplenism were significantly less common; serum From January 1970 to December 1992, 147 patients with hepatictransaminase (P õ .001) and creatinine levels (P õ .02) venous outflow block were admitted to the Liver Units or Surgery were lower, prothrombin was higher (P õ .001), and 5-Departments at Hôpital Beaujon, Hôpital Louis Mourier, and Hôpital de la Salpêtrière. The diagnoses of hepatic venous outflow block were year survival was significantly better (Kaplan-Meier esmade when obstructions of the hepatic veins or inferior vena cava timates: 80% vs. 50%, P õ .05). In patients with hepatic were shown by imaging investigations or when centrilobular distenvenous outflow block, short-length hepatic vein stenosis sion was seen at liver biopsy in the absence of cardiac failure or is a common lesion that appears to be the sequela of pericarditis. 7 Imaging studies of the hepatic veins were reviewed.localized thrombosis. Long-term anticoagulation and Thirty-six cases (24.5%) were excluded because ...
In preliminary observations, significant amounts of free cysteine, a neurotoxic amino acid, were noted in the urine of asphyxiated or septic‐shocked neonates. The present study was conducted to determine whether free urinary cysteine was elevated in these critically ill neonates compared with a control group, and to assess the clinical significance of this generation. Free cysteine was measured in the urine of newborn infants with perinatal asphyxia (n= 16) or neonatal sepsis (n= 14) and the urine of a control group (n= 10) by ion‐exchange chromatography. Relationships between cysteine levels and the clinical severity, sulfite supply and neurological outcome of the patients were then studied. Urinary cysteine was 27.6 (15‐49) mmol mol−1 creatinine for the patients but was not detectable in the control group. Cysteine levels were correlated with the severity of neonatal septic shock but not with the grade of perinatal asphyxia and did not have a specific influence on the neurological outcome of these patients. The correlation between cysteine level and the severity of neonatal septic shock was indirect and probably linked to higher sulfite administration in this population. Conclusion: The mean daily supply of sulfites is high in critically ill neonates, mainly originating from dopamine and generating significant amounts of cysteine. Although a worsening effect attributable to cysteine on the neurological outcome of the patients could not be demonstrated, the appropriateness of cryptic administration of sulfites by way of drug excipients is called into question.
The mean daily supply of sulfites is high in critically ill neonates, mainly originating from dopamine and generating significant amounts of cysteine. Although a worsening effect attributable to cysteine on the neurological outcome of the patients could not be demonstrated, the appropriateness of cryptic administration of sulfites by way of drug excipients is called into question.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.