11/45. The clinical course of patients with type 1 hepatorenal syndrome maintained on hemodialysis. GOAL: Report the natural coarse of hepatorenal syndrome in 4 patients who were maintained on chronic hemodialysis. BACKGROUND: The diagnosis of hepatorenal syndrome carries a grave prognosis with a mortality rate >90% and a median survival time of <2 weeks without orthotopic liver transplantation. STUDY: We report the clinical course of 4 patients with hepatorenal syndrome who underwent long-term (greater than 3 weeks) hemodialysis in an attempt to bridge them to orthotopic liver transplantation. The etiologies of cirrhosis were: chronic hepatitis c infection (n = 2), alcoholic liver disease (n = 1), and primary sclerosing cholangitis (n = 1). RESULTS: Mean survival time on hemodialysis was 236 days (range: 31 to 460 days). All patients survived their initial hospitalization and were discharged from the hospital. However, only one patient received orthotopic liver transplantation. Mean number of hospital admissions was 11 (range: 4 to 18) while receiving hemodialysis at an average rate of 2.2 (range: 1.1 to 5) admissions/patient month. Mean number of days spent in hospital while on hemodialysis support was 85 days (range: 15 to 199 days) at an average rate of 11.2 (range: 8.3 to 15) hospital days/patient month. An average of 33% (range: 26% to 48%) of the days of the prolonged survival on hemodialysis was spent in hospital. CONCLUSION: Although our 4 patients with hepatorenal syndrome demonstrated long-term survival with hemodialysis, their prolonged survival was at the cost of a very heavy burden of morbidity and in-patient stay. The advisability of maintenance hemodialysis in patients with hepatorenal syndrome should be judged on an individual basis. ( info) |
12/45. Reconsidering hepatorenal syndrome. Throw in the towel? Not so fast! For many years, hepatorenal syndrome was considered a uniformly and rapidly fatal complication of end-stage liver disease. Although the syndrome still carries a poor long-term prognosis, increased understanding of its hemodynamic derangements has led to new pharmacologic treatments that significantly improve short-term outcomes. In this article, Drs Tong, Hurley, and Hayashi discuss a case of remarkable reversal of hepatorenal syndrome with use of oral midodrine hydrochloride, subcutaneous octreotide acetate, and intravenous albumin. The authors highlight the great progress that has been made in this field and review new therapeutic options that are on the market or under study. It is important for physicians who are caring for patients with hepatorenal syndrome to know about and consider the available treatments before an approach of "supportive care only" is taken. ( info) |
| No more than 11 cases of carcinosarcoma of the liver have been reported in the past 40 years that fulfill the definition of hepatocellular carcinoma combined with differentiated sarcomatous elements. Most cases consist of hepatocellular carcinoma with 1 to 2 heterologous elements. We report a case of a 51-year-old woman with liver carcinosarcoma consisting of 3 carcinomatous components and 4 sarcomatous components. Hepatocellular carcinoma, fibrolamellar type, was accompanied by neuroendocrine carcinoma (neuron-specific enolase and synaptophysin positive) and adenocarcinoma (cytokeratin 7 and 20 positive). The sarcomatous elements consisted of poorly differentiated spindle cell neoplasm (vimentin positive), leiomyosarcoma (smooth muscle actin positive), rhabdomyosarcoma (desmin positive), and osteosarcoma. To our knowledge, this is the first case of liver carcinosarcoma with this many differentiated heterologous features. There are differing views on the pathogenesis of this tumor. Findings in this case support the view that metaplasia of carcinomatous cells gives rise to the sarcomatous elements. ( info) |
14/45. Artificial renal and liver support in a severe hepatorenal syndrome of childhood. This case report describes a special management approach in a child aged 4.5 years with a severe form of hepatorenal syndrome in which the final diagnosis was familial hemophagocytic lymphohistiocytosis (FHL). The patient presented with grade IV hepatic coma and acute renal failure (ARF). While the diagnosis was difficult at the time of admission, as well as during acute treatment, artificial liver support was established for elimination of bilirubin and other metabolic by-products by using charcoal column plasma perfusion (CCPP) and bilirubin-adsorbing resin column plasma perfusion (BRCPP). serum levels of bilirubin showed a notable decrease after each of the four treatment sessions. Additional artificial renal function replacement by continuous arteriovenous hemofiltration and hemodialysis (CAVH, CAVHD, respectively) had a marked lowering effect on urea and creatinine serum concentrations. Both artificial liver and renal support contributed to the general clinical improvement and survival of the patient. Further experience in these therapies will be needed to establish better prognosis in such fatal or acute similar conditions. ( info) |
15/45. Tc-99m DTPA and I-131 hippurate renography. Findings in hepatorenal syndrome. Tc-99m DTPA and I-131 OIH renography were performed simultaneously in a patient with hepatorenal syndrome. blood flow was delayed and diminished bilaterally; there was Tc-99m DTPA and I-131 OIH retention in the parenchyma with no evidence of tracer retention in the collecting systems. The I-131 OIH renogram curve demonstrated a steadily rising pattern, whereas the Tc-99m DTPA curve demonstrated an initial vascular peak and was subsequently flat. There was no appreciable response to furosemide. These findings are not specific for hepatorenal syndrome, and the diagnosis is based on the characteristic clinical setting and the exclusion of other causes of renal failure. A brief literature review and a discussion of differential diagnosis are included. ( info) |
| 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. ( info) |
17/45. 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. ( info) |
18/45. Case study: end stage liver failure patient managed with CAVH pretransplant. The patient met 4 of the 5 expected outcomes. Effective therapy with CAVH was maintained in the ICU setting and in the operating room. The catheters functioned throughout the entire treatment without signs or symptoms of infection, bleeding, or thrombosis. She received hyperalimentation, filtration replacement fluid, and adequate fluid boluses to maintain volume status, nutritional requirements and vital signs. Cardiac vasopressor medications were required for a short period. The one problem that did occur was related to the anticoagulation therapy. The PTT was checked every 4 hours and the heparin drip was titrated to keep the PTT at 50 to 60 seconds. At one point, the PTT was 92 seconds but immediate measures were undertaken to reverse the condition. In the situation where a patient has an underlying coagulopathy and a potential source of bleeding, the nursing recommendation is that hourly bedside ACTs with every 4 hour PTTs should be performed as a routine part of the care. CRRT has been used successfully in critically ill unstable patients with multiorgan involvement. These therapies provide an avenue for collaboration between nursing professionals in nephrology and critical care. The focus of CRRT can now progress from the technical aspects of the procedures to patient centered nursing issues. ( info) |
19/45. Hyperlactatemia and increasing metabolic acidosis in hepatorenal failure treated by hemofiltration. We report a case of increasing hyperlactatemia in the course of repeated treatment by machine hemofiltration (MHF) using a lactate-buffered replacement solution. The hyperlactatemia was associated with a reduction in mean arterial pressure, and in the majority of treatments a metabolic acidosis developed. Hyperlactatemia due to exogenous lactate may not be as benign as previously discussed. ( info) |
| 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. ( info) |