Primary sclerosing cholangitis, chronic ulcerative colitis, and bile duct dysplasia--Case presentation.
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Bile Duct Diseases↗
Biomedical subjects
K V Narayanan Menon
Publications and source records attributed to K V Narayanan Menon.
Factor XII deficiency acquired by orthotopic liver transplantation: case report and review of the literature.
Transmission of congenital clotting factor deficiencies after orthotopic liver transplantation is rare. There are published reports of liver donor-to-recipient transmission of protein C deficiency with dysfibrinogenemia, protein S, factor VII and factor XI deficiencies. We report a case of transmission of factor XII deficiency with liver transplantation in a patient with Budd-Chiari syndrome. There was a persistent elevation of the activated partial thromboplastin time (aPTT), but no evidence of bleeding while the patient was maintained on warfarin. The presence of a persistently abnormal aPTT may raise suspicion for the presence of a clotting factor deficiency; however, deficiencies of other clotting factors may not be readily apparent on routine blood tests performed in a donor. Being aware of the possibilities of transmission of these inherited deficiencies of coagulation factors will aid in their early detection and management in the transplant donor and recipient.
Diagnosis, Differential↗
MELD accurately predicts mortality in patients with alcoholic hepatitis.
Assessing severity of disease in patients with alcoholic hepatitis (AH) is useful for predicting mortality, guiding treatment decisions, and stratifying patients for therapeutic trials. The traditional disease-specific prognostic model used for this purpose is the Maddrey discriminant function (DF). The model for end-stage liver disease (MELD) is a more recently developed scoring system that has been validated as an independent predictor of patient survival in candidates for liver transplantation. The aim of the present study was to examine the ability of MELD to predict mortality in patients with AH. A retrospective cohort study of 73 patients diagnosed with AH between 1995 and 2001 was performed at the Mayo Clinic in Rochester, Minnesota. MELD was the only independent predictor of mortality in patients with AH. MELD was comparable to DF in predicting 30-day mortality (c-statistic and 95% CI: 0.83 [0.71-0.96] and 0.74 [0.62-0.87] for MELD and DF, respectively, not significant) and 90-day mortality (c-statistic and 95% CI: 0.86 [0.77-0.96] and 0.83 [0.74-0.92] for MELD and DF, respectively, not significant). A MELD score of 21 had a sensitivity of 75% and a specificity of 75% in predicting 90-day mortality in AH. In conclusion, MELD is useful for predicting 30-day and 90-day mortality in patients with AH and maintains some practical and statistical advantages over DF in predicting mortality rate in these patients. MELD is a useful clinical tool for gauging mortality and guiding treatment decisions in patients with AH, particularly those complicated by ascites and/or encephalopathy.
Adult↗
Living donor liver transplantation using a right lobe graft in an adult with situs inversus.
Situs inversus totalis is a rare anatomic variant in which there is a complete mirror image of the thoracic and abdominal viscera. The common association of situs inversus and biliary atresia has led to a variety of modifications of surgical techniques utilizing both living donor and deceased donor liver grafts, with mixed results in the pediatric liver transplant population. The use of a living donor liver graft in an adult with situs inversus has not yet been described. However, living donor liver transplantation (LDLT) has produced excellent results in the adult population, particularly in the cholestatic population, which may be disadvantaged by the model for end-stage liver disease system. This is the first report of a successful living donor right liver graft in an adult with end-stage liver disease secondary to primary sclerosing cholangitis and situs inversus totalis.
Anastomosis, Surgical↗
Analysis of factors that predict alcohol relapse following liver transplantation.
Alcoholic liver disease has become a major indication for liver transplantation in the United States. Factors that predict alcohol relapse after liver transplantation are poorly defined. The aim of our study was to identify predictors of alcohol relapse in patients undergoing liver transplantation for alcoholic liver disease. One hundred and eleven patients undergoing liver transplantation for alcoholic liver disease between 1985 and 1999 were identified from our database. Patients were selected for liver transplantation if their risk of relapse was felt to be low by the transplant team. A chart review was conducted to determine if relapse had occurred, the presence or absence of factors that were thought to predict relapse, and survival. Demographic and psychosocial variables were analyzed using univariate and multivariate logistic regression to identify independent predictors of relapse. The median duration of abstinence before liver transplantation was 15 months (range: 1-120). Hepatitis C virus was present in 64% of patients. A family history of alcoholism in a first-degree relative was identified in 38%, and 78% received treatment for alcoholism before liver transplantation. The mean duration of follow-up was 44.1 +/- 3.7 months. There were 29 deaths (26%) overall. Seventeen patients (15%) returned to alcohol use. On multivariate analysis a family history of alcoholism was found to be an independent predictor of alcohol relapse (P=.03). Further prospective studies are needed to examine this association in greater detail to provide targeted treatment for alcoholism both before and after liver transplantation.
Adult↗
A pilot study of the safety and tolerability of etanercept in patients with alcoholic hepatitis.
BACKGROUND: Alcoholic hepatitis is a cause of major morbidity and mortality, and effective therapeutic regimens to treat this condition are lacking. Both experimental and clinical evidence indicates that tumor necrosis factor alpha (TNF), and the downstream cytokine interleukin-6 (IL-6), correlate with disease severity and may contribute to the pathogenesis and clinical sequelae of alcoholic hepatitis, thereby implicating a possible role for inhibition of TNF in the treatment of alcoholic hepatitis. OBJECTIVE: The aim of the current study was to assess the safety and tolerability of a p75-soluble TNF receptor:FC fusion protein (etanercept), an agent that binds and neutralizes soluble TNF in patients with alcoholic hepatitis in the form of an open-label pilot trial. METHODS: Etanercept administration was targeted for 2 wk duration in 13 patients with moderate or severe alcoholic hepatitis as assessed by a discriminant function value greater than 15 and/or the presence of spontaneous hepatic encephalopathy. RESULTS CONCLUSIONS: On an intention-to-treat basis, the 30-day survival rate of patients receiving etanercept was 92% (12/13). Adverse events that were encountered included infection, hepatorenal decompensation, and GI bleeding, which required premature discontinuation of etanercept in 23% of patients (3/13). This is the first study to examine TNF inhibition with etanercept in patients with alcoholic hepatitis and the results of this study support the rationale for larger controlled studies to further assess safety and efficacy.
Adult↗
MELD and other factors associated with survival after liver transplantation.
Allocation of cadaveric livers for transplantation in the United States is now based on the severity of illness as determined by the model for end-stage liver disease (MELD) score, a function of bilirubin, creatinine and international normalized ratio (INR). The aim of our study was to determine the association of various pre-transplant risk factors, including the MELD score, on patient survival after orthotopic liver transplantation (OLT). The medical records of 499 consecutive patients (233 female, 266 males, mean age 50.9 +/- 10.6 years) undergoing cadaveric OLT at our institution between June 1990 and February 1998 were reviewed. In the 407 patients alive at the latest contact, follow-up was 4.7 years, with a minimum of 20 months (maximum of 9.4 years). Variables considered for analysis included MELD score, age, pre-transplant renal dysfunction requiring dialysis, Child-Pugh classification, underlying liver disease, diabetes mellitus, and heart disease (ischemic/valvular/other). There were 92 deaths during follow-up. In univariate analysis, the MELD score, renal failure requiring hemodialysis pre-OLT, age > 42 years, and underlying etiology of liver disease were significantly associated with death during long-term follow-up. In multivariate models, age, underlying etiology of liver disease and renal failure requiring hemodialysis were independent predictors of death after OLT.
Female↗
Safety and efficacy of estrogen therapy in preventing bone loss in primary biliary cirrhosis.
OBJECTIVE: Estrogen therapy has been found to be useful in the treatment of postmenopausal osteoporosis. However, concern about its potential for worsening cholestasis has limited its use in postmenopausal women with primary biliary cirrhosis. The aim of the present study was to determine the safety as well as the efficacy of estrogen replacement therapy with respect to bone mass in postmenopausal women with primary biliary cirrhosis. METHODS: Bone mineral density of the lumbar spine (measured using dual energy x-ray absorptiometry) of 46 unselected postmenopausal women with primary biliary cirrhosis receiving estrogen replacement therapy was compared with 46 age-matched women with primary biliary cirrhosis not receiving estrogen replacement therapy, and with the expected normal bone mineral density adjusted for age and ethnic group. RESULTS: The mean duration of follow-up was 4.8 +/- 0.4 yr. Treatment with estrogens resulted in a significantly lower rate of bone loss (0.002 g/cm(2)/yr +/- 0.028 vs 0.009 g/cm(2)/yr +/- 0.020, p = 0.05). Worsening cholestasis attributable to estrogen replacement therapy did not occur in any patient. One patient (2%) discontinued therapy because of side effects. CONCLUSIONS: Estrogen replacement therapy in postmenopausal patients with primary biliary cirrhosis is safe and may be effective in decreasing the rate of bone loss.
Adult↗
Sustained remission after corticosteroid therapy for type 1 autoimmune hepatitis: a retrospective analysis.
Autoimmune hepatitis commonly relapses after corticosteroid therapy, and long-term management strategies have been proposed based on the premise that repeated relapses after drug withdrawal are inevitable. Our goal was to determine the frequency that remission can be sustained after its induction and termination of therapy. A total of 107 patients who had entered remission on conventional regimens were assessed for sustained remission after initial treatment and after relapse and re-treatment. Re-treatment strategies included conventional regimens and long-term maintenance schedules. Twenty-two patients (21%) achieved a sustained remission after initial treatment, and 24 of 85 patients who relapsed and were re-treated (28%) had a similar outcome. The probability of a sustained remission was 47% after 10 years of follow-up. Patients who sustained remission after initial therapy were distinguished only by a lower serum gamma-globulin level at entry. Conventional re-treatment schedules after relapse were able to induce a sustained remission more commonly then long-term maintenance schedules (59% vs. 12%, P =.00002). In conclusion, patients who respond to initial corticosteroid therapy can achieve a sustained remission after treatment withdrawal or after relapse and re-treatment. All patients are candidates for this outcome, and withdrawal of medication, even during maintenance schedules, is necessary to assess its likelihood.
Adolescent↗
Unsuspected primary hepatic angiosarcoma associated with portal vein thrombosis complicating cirrhosis.
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Aged↗
Non-A to E hepatitis.
PURPOSE OF THE REVIEW: The list of possible hepatotropic viruses continues to grow with the discovery of the GB virus-C, the TT virus and the SEN virus. There is emerging data on the biology of these newly discovered :In spite of continuing research into the pathogenicity of the GB virus-C and the TT virus, definite evidence linking them to acute or chronic liver disease is lacking. The SEN virus was reported in 2000, and although there seems to be an association between virus and transfusion-related hepatitis, more data are awaited before definite conclusions can be drawn. The effect of GB virus-C, the TT virus and the SEN virus co-infection on other viral and non-viral hepatitides has also been studied in some detail. Again, there is no definite evidence so far that these viruses modify other liver diseases. SUMMARY: At the present time, diagnostic testing for these viruses does not seem to be warranted outside of clinical studies. The discovery of these viruses, however, paves the way for further research into novel viral agents that infect humans, other among hosts.
Hepatitis Viruses↗
Ultrasound-assisted percutaneous liver biopsy performed by a physician assistant.
OBJECTIVE: Percutaneous liver biopsy is an essential diagnostic tool utilized in the management of patients with liver disease. This procedure is generally performed by a physician and has a small but well-defined complication rate. We report on the complication rate and efficiency of ultrasound-assisted percutaneous liver biopsy performed by an experienced physician assistant. METHODS: One thousand eighty-six consecutive outpatient liver biopsies (847 hepatic allografts and 239 native livers) were performed at a single center by a physician assistant between June, 1996 and June, 2000. Patients with hepatic mass lesions, unusual hepatic anatomy, and uncorrectable coagulopathy (international normalized ratio > 1.7, platelet count < 50 x 10(9)/L) were excluded. Bedside ultrasonography was used to determine the optimal site for the liver biopsy. Liver biopsies were performed with a 15-gauge Jamshidi aspiration biopsy needle. Patients were observed for 3 h after biopsy, followed by dismissal with subsequent contact in 24 h to assess outcome and complications. RESULTS: Adequate tissue was obtained in 1084 cases (99.8%), with a mean tissue length of 3.2 cm. After the procedure, narcotic analgesia was necessary in 116 (10%) of the patients undergoing liver biopsies. The overall complication rate requiring hospitalization was 0.6%. Major complications requiring intervention occurred in four patients (0.4%). There were no deaths resulting from liver biopsies. CONCLUSION: We conclude that outpatient percutaneous liver biopsy can be safely and effectively performed by a trained physician assistant.
Biopsy↗
Etiology and natural history of primary sclerosing cholangitis.
The etiology of primary sclerosing cholangitis remains unknown. Bacteria, toxins, viral infections, and immunological and genetic factors have all been proposed as etiological agents. Portal bacteremia, toxins absorbed from the diseased colon in inflammatory bowel disease, and cytomegalovirus and reovirus infections have been implicated by various investigators but there is little evidence to support these hypotheses. The close association between primary sclerosing cholangitis and various human leukocyte antigen haplotypes is now well established and lends support to the theory that immunologic and genetic mechanisms may be involved in its pathogenesis. Patients with primary sclerosing cholangitis may have elevated levels of circulating immune complexes, immunoglobulins, and non-organ specific autoantibodies. The association between ulcerative colitis and primary sclerosing cholangitis remains unexplained and both groups of patients have a high prevalence of antibodies to the perinuclear cytoplasmic antigen. The long-term prognosis in primary sclerosing cholangitis is tempered by the development of cholangiocarcinoma in 6%-30% of patients when followed over long periods of time. Detecting cholangiocarcinoma early in a patient with primary sclerosing cholangitis is one of the most frustrating problems faced by a clinician while caring for these patients. The long-term outlook for patients with primary sclerosing cholangitis and cholangiocarcinoma remains dismal, whatever the treatment modality employed. However, the development of a multivariate statistical survival model from long-term survival data from the Mayo Clinic and other centers has been a major step in identifying individual primary sclerosing cholangitis patients at low, moderate, and high risk of dying. Such models have been useful for stratifying patients in therapeutic trials, for in patient counseling, and in patient selection and timing of liver transplantation.
Cholangitis, Sclerosing↗