11/32. hepatorenal syndrome: resolution of ascites by continuous renal replacement therapy in an alcoholic coinfected with hepatitis b, C, and human immunodeficiency viruses.A 39-yr-old male with hepatorenal syndrome type 1 and refractory ascites was treated with continuous renal replacement therapy (CRRT) resulting in clinical improvement. He was positive for antibodies to hepatitis b, C, and human immunodeficiency viruses, and had a history of chronic alcohol and iv drug abuse. The patient had 4 hospital admissions during a 12-wk period. He first presented with advanced liver disease including pedal edema and a serum ammonia level of 56 micromol/L (reference range: 11 - 35 micromol/L). In subsequent admissions, he had asterixis, nausea, vomiting, jaundice, and worsening pedal edema. On his 4th admission, there was lethargy, tense ascites, decreased urinary output, bilateral edema of the lower extremities and scrotum, serum creatinine of 6.2 mg/dl (reference range: 0.6 - 1.5 mg/dl), and weight gain of 16 kg during the prior 8 wk. During the first 3 hospitalizations, he was treated with lactulose with slight improvement. On the 4th admission, he was started on low-dose dopamine (3 microg/kg/min) and 25% salt-poor albumin without clinical improvement. A pulmonary artery catheter was placed and hemofiltration by CRRT was performed for 5 days, with removal of 26.7 L of fluid and a net reduction of 11 kg of body weight. serum creatinine decreased to 4.2 mg/dl during CRRT and was 2.2 mg/dl at hospital discharge 2 weeks later. His PaO(2) improved from 66 to 78 mmHg and his systemic vascular resistance increased from 571 to 799 dyne.sec/cm(5). CRRT was effective in relieving severe fluid retention and producing marked clinical improvement. We suggest that CRRT should be considered for the treatment of refractory ascites including that caused by hepatorenal syndrome.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
12/32. Acute fatty liver of pregnancy associated with preeclampsia: management of hepatic failure with postpartum liver transplantation.Acute fatty liver of pregnancy is a potentially fatal disorder. We report a patient complicated by preeclampsia, coagulopathy, encephalopathy, and hepatorenal syndrome successfully managed by postpartum hepatic transplantation.- - - - - - - - - - ranking = 0.16666666666667keywords = syndrome (Clic here for more details about this article) |
13/32. Continuous arteriovenous hemofiltration in the patient with hepatorenal syndrome. A case study.CAVH has gained increasing medical acceptance, as favorable outcomes in the critically ill have proven its worth. Unfortunately, CAVH is often used as a "last ditch" effort for the patient with multisystem organ failure and septicemia. Performing the procedure in such a setting sometimes seems little more than an exercise in futility. However, as more patients such as the patient described in this case study are shown to benefit from CAVH, the nursing role needs to be examined both in performing the procedure and in caring for the critically ill patients. The nursing literature has kept abreast of the procedural aspects of CAVH. Now, critical care nurses must move on to the patient-oriented approach, share case studies, and work together to formulate nursing diagnoses, develop specialized care plans, and identify areas for nursing research.- - - - - - - - - - ranking = 0.66666666666667keywords = syndrome (Clic here for more details about this article) |
14/32. Infantile refsum disease: an inherited peroxisomal disorder. Comparison with zellweger syndrome and neonatal adrenoleukodystrophy.Three patients affected by infantile refsum disease are described with mental retardation, minor facial dysmorphia, chorioretinopathy, sensorineural hearing deficit, hepatomegaly, failure to thrive and hypocholesterolaemia. Initially, only an accumulation of phytanic acid was thought to be present. More recent findings showed a biochemical profile very similar to that found in classical zellweger syndrome or neonatal adrenoleukodystrophy. Morphologically typical peroxisomes were absent in the liver. All three disorders are associated with multiple peroxisomal dysfunction. Because of these similarities pertinent clinical data of our three patients are compared with those of reported patients diagnosed as having infantile refsum disease, neonatal adrenoleukodystrophy or zellweger syndrome who survived for several years. attention is drawn to the difference in severity of clinical features, ranging from infantile Refsum's disease to neonatal adrenoleukodystrophy and, finally, to zellweger syndrome.- - - - - - - - - - ranking = 1.1666666666667keywords = syndrome (Clic here for more details about this article) |
15/32. maternal mortality due to the hepatorenal syndrome of pre-eclampsia. A case report.A maternal death in the 23rd wk of pregnancy is described. The patient was gravida 8, with 6 previous abortions and one growth-retarded term infant. The clinical symptoms and biochemical findings strongly suggest that the mode of death was septic shock, with the hepatorenal syndrome due to pre-eclampsia as the underlying cause.- - - - - - - - - - ranking = 0.83333333333333keywords = syndrome (Clic here for more details about this article) |
16/32. Multiple peroxisomal enzymatic deficiency disorders. A comparative biochemical and morphologic study of Zellweger cerebrohepatorenal syndrome and neonatal adrenoleukodystrophy.Biologic, morphologic, and biochemical investigations performed in 2 patients demonstrate multiple peroxisomal deficiencies in the cerebrohepatorenal syndrome of Zellweger (CHRS) and neonatal adrenoleukodystrophy (NALD). Very long chain fatty acids, abnormal bile acids, including bile acid precursors (di- and trihydroxycoprostanoic acids), and C29-dicarboxylic acid accumulated in plasma in both patients. Generalized hyperaminoaciduria was also present. peroxisomes could not be detected in CHRS liver and kidney; however, in the NALD patient, small and sparse cytoplasmic bodies resembling altered peroxisomes were found in hepatocytes. Hepatocellular and Kupffer cell lysosomes were engorged with ferritin and contained clefts and trilaminar structures believed to represent very long chain fatty acids. Enzymatic deficiencies reflected the peroxisomal defects. Hepatic glycolate oxidase and palmitoyl-CoA oxidase activities were deficient. No particle-bound catalase was found in cultured fibroblasts, and ether glycerolipid (plasmalogen) biosynthesis was markedly reduced. Administration of phenobarbital and clofibrate, an agent that induces peroxisomal proliferation and enzymatic activities, to the NALD patient did not bring about any changes in plasma metabolites, liver peroxisome population, or oxidizing activities.- - - - - - - - - - ranking = 0.83333333333333keywords = syndrome (Clic here for more details about this article) |
17/32. Computerized tomography and ultrasound in the diagnosis of cerebro-hepato-renal syndrome of Zellweger.Renal cortical cysts were demonstrated by computerized tomography and ultrasound in a patient with cerebro-hepato-renal syndrome who did not have patellar calcifications.- - - - - - - - - - ranking = 0.83333333333333keywords = syndrome (Clic here for more details about this article) |
18/32. The reversal of the hepatorenal syndrome in four pediatric patients following successful orthotopic liver transplantation.Four pediatric patients are presented in whom profound renal failure (hepatorenal syndrome) developed in association with severe end-stage liver disease. All four patients had successful orthotopic liver transplantation. Special emphasis is given to the preoperative and postoperative renal function in the patients, and the criteria used to establish the diagnosis of the hepatorenal syndrome are discussed. In the initial work on liver transplantation and reversal of the hepatorenal syndrome, two of the three patients recovered renal function but died in the perioperative period. The four patients presented in this report have not only had reversal of the hepatorenal syndrome after successful orthotopic liver transplantation but have also survived long term. The four patients have been followed up for periods ranging from 18 months to 4.5 years. Three of the four patients have maintained near normal renal function, whereas the fourth patient (who had a left nephrectomy for obstruction and sepsis) has had a significant decline in renal function.- - - - - - - - - - ranking = 1.3333333333333keywords = syndrome (Clic here for more details about this article) |
19/32. An unusual case of ectodermal dysplasia.The syndrome was characterized by striking hypoplasia of nails, malformations of hands and feet, curly hair, small lower teeth and seizures. There were no similarly affected relatives. Death occurred at 31 months with the patient apparently in status epilepticus with terminal hepatorenal syndrome.- - - - - - - - - - ranking = 0.33333333333333keywords = syndrome (Clic here for more details about this article) |
20/32. Improvement of hepatorenal syndrome by transjugular intrahepatic portosystemic shunt.hepatorenal syndrome (HRS) is a functional renal failure occurring in advanced liver cirrhosis with ascites. It is due to renal cortical vasoconstriction resulting from complex hemodynamic disturbances related to cirrhosis and portal hypertension. There is no consistently effective therapy except for liver transplantation. We report a case of severe HRS in a patient with advanced liver cirrhosis and portal hypertension. Three sessions of hemodialysis were performed because of severe renal failure (serum urea 83 mg/dl, serum creatinine 6 mg/dl). Creation of an intrahepatic portosystemic shunt reduced the portocaval gradient from 18 to 7 mm Hg. Spectacular improvement of the renal function was observed soon after the procedure, with spontaneous recovery of diuresis and a return of serum urea and creatinine to baseline values. The patient unfortunately died 2 months later from adult respiratory distress syndrome post emergency surgery for a massive bleed related to a duodenal ulcer. Throughout this episode, the renal function remained stable. The postmortem examination showed histologically normal kidneys. We conclude that the intrahepatic portosystemic shunt can improve renal function in cirrhotic patients with HRS; it could be used in patients awaiting liver transplantation to reverse preoperative renal failure.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
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