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PubMed2006

Microscopic polyangiitis associated with primary biliary cirrhosis: a causal or casual association?

The association between microscopic polyangiitis (MPA) and primary biliary cirrhosis (PBC) has seldom been reported. We describe a patient with PBC and MPA who presented with polyarthritis and pulmonary nodules followed by pauci-immune crescentic glomerulonephritis and liver dysfunction. Detection of p-ANCA, antimyeloperoxidase, and antimitochondrial antibodies along with liver and renal histopathology allowed a diagnosis of MPA and PBC. We also discuss 2 other cases that could be unrecognized associations of both diseases. Further reports are necessary to clarify if the coexistence between PBC and MPA is causal or casual.

Autoimmune Diseases↗
PubMed1991

[Radionuclide cavography before and after percutaneous transluminal angioplasty in Budd-Chiari web: case report].

A case of the Budd-Chiari syndrome due to a web of the hepatic inferior vena cava (IVC) is reported. A 54-year-old male with mild liver dysfunction was suspected with IVC obstruction from the screening CT which revealed liver cirrhosis with marked caudate lobe enlargement and dilatation of azygous and hemiazygous vein. Subsequent radionuclide cavography with 99mTc-HSA clearly demonstrated IVC obstruction, but failed to clarify the site or type of the obstruction. Finally contrast cavography diagnosed a web of the hepatic IVC, which was treated by percutaneous transluminal angioplasty (PTA). During two-year follow-up after PTA none of the radionuclide cavographies showed reocclusion of the IVC and as a result contrast cavography was avoided. Radionuclide cavography, therefore, was a useful method for evaluating IVC obstruction before and after PTA for the Budd-Chiari web.

Angioplasty, Balloon, Coronary↗
PubMed1985

[A case of surgical correction of the inferior vena cava obstruction with Budd-Chiari syndrome].

A 37-year-old man is presented with Budd-Chiari syndrome associated with a membranous obstruction of the inferior vena cava. Clinical symptoms were ascites, leg edema and dilatation of the superficial abdominal veins. Aggressive surgery was carried out for this case. Midsternal incision and right subcostal oblique incision were made, and hepatic segment of the inferior vena cava was reconstructed using pericardial patch after endovenectomy and partial cardiopulmonary bypass was used without temporary interruption of liver circulation. Blood flow from the right hepatic vein was restored. Postoperative course was not eventful and this patient remains free of any evidence of ascites and liver dysfunction after operation. This procedure may be useful particularly for protecting the liver function which is damaged by hemostasis.

Adult↗
PubMed1997

[Study of mechanisms of pancreatic fibrosis and structural changes in liver cirrhotic patients].

Pancreatic intralobular fibrosis in liver cirrhotic patients was studied immunohistochemically using alpha-smooth muscle actin (alpha SMA). Similarly micronodular arrangements of acinar cells and hypertrophy of Langerhans' islands in liver cirrhotic patients were studied using proliferating cell nuclear antigen (PCNA). In addition, pancreata in liver cirrhotic patients were studied from apoptotic side. We investigated the histopathologic autopsy findings of 103 patients: 15 patients with alcoholic liver cirrhosis, 49 patients with non-alcoholic liver cirrhosis and, as controls, 39 patients without a history of alcohol abuse or liver dysfunction. alpha SMA positive area ratio and PCNA positive cell ratio in liver cirrhotic patients were higher than in the controls. In contrast, apoptotic cell ratio in liver cirrhotic patients was lower than in the controls. alpha SMA positive area ratio in patients with pancreatic intralobular fibrosis was higher than in patients without pancreatic intralobular fibrosis. PCNA positive cell ratio in patients with micronodular arrangements of the acinar cells was higher than in patients without micronodular arrangements of the acinar cells. We conclude that pancreata in liver cirrhotic patients are predominant of proliferating changes.

Adult↗
PubMed1998

Post-liver transplantation myocardial dysfunction.

Patients with end-stage liver disease usually show a hyperdynamic circulatory state. It has previously been reported that patients who develop myocardial depression in the early post-liver transplantation period are more prone to organ failure and death. We reviewed the records of 754 adult patients undergoing liver transplantation at our institution and identified 7 patients who initially showed hyperdynamic circulation, but then developed reversible dilated cardiomyopathy in the early posttransplantation period. All identifiable causes of cardiac dysfunction, such as myocardial ischemia, thyroid dysfunction, and electrolyte imbalances, were excluded. Left ventricular ejection fraction decreased from a preoperative median baseline of 60% to 20% (P = .02), with four-chamber dilatation on echocardiogram. All these patients required supportive care, including mechanical ventilation, afterload reduction, inotropic support, and monitoring in the intensive care unit. Cardiac function subsequently improved in all patients, with ejection fraction increasing to a median of 50%. All patients were discharged from the hospital. At a median follow-up of 15 months, there was no recurrence of heart failure. The increased peripheral resistance seen after successful liver transplantation may be an important causative factor.

Adolescent↗
PubMedDate not supplied

Flutamide-associated acute liver failure.

The nonsteroidal antiandrogenic drug flutamide [4'-nitro-3'-(trifluoromethyl)isobutyranilide] is a safe and generally well-tolerated drug used for the treatment of prostate cancer. We describe the case of a 74-year-old male who developed life-threatening acute liver failure during flutamide therapy. Other causes of acute liver failure were appropriately ruled out and there was no evidence of active prostate cancer or liver metastases. The use of the Naranjo probability scale indicated a highly probable relationship between the development of acute liver failure and flutamide therapy. Severe liver dysfunction has been rarely documented in patients treated with flutamide, even though cases of fulminant liver failure have been described. A few cases have been reported also among patients with hirsutism being treated with flutamide. The mechanisms responsible for the occurrence of hepatotoxicity during treatment with flutamide are unknown. Mitochondrial dysfunction seems to be implicated. The potential of flutamide to act as a potent hepatotoxin should be borne in mind when treatment with this drug is being planned.

Acute Kidney Injury↗
PubMed1977

Postoperative treatment of patients after liver resection for trauma: a follow-up study.

In the last ten years, 89 hepatic resections were performed for trauma. Thirty-three patients survived and were followed up for one month to seven years: 15 patients had right lobectomy, nine left lobectomy, and nine left lateral segmentectomy. Complications were primarily pulmonary. All patients had transient derangement of liver function tests, but only three patients had liver dysfunction. Long-term follow-up showed no ill effects from the liver resection. Important postoperative treatment includes (1) adequate dependent drainage, (2) maintenance of blood volume, (3) intravenous albumin and glucose, (4) adequate nutritional support, and (5) selective use of intravenous glucagon.

Adolescent↗
PubMed2003

Clinical and diagnostic characteristics of complex III deficiency due to mutations in the BCS1L gene.

We investigated two siblings of a Spanish family presenting with congenital lactic acidosis. They had severe failure to thrive, liver dysfunction, and renal tubulopathy. An isolated biochemical complex III deficiency was detected in liver. A search for mutations in the human bc1 synthesis like (BCS1L) gene was undertaken. Direct sequencing revealed a missense mutation R45C and a nonsense mutation R56X, both located in exon 1 of BCS1L. The missense mutation in combination with a loss of function of the second allele is responsible for the isolated complex III deficiency in this family.

ATPases Associated with Diverse Cellular Activitie↗
PubMed1994

Estimation of remnant liver function before hepatectomy by means of technetium-99m-diethylenetriamine-pentaacetic acid galactosyl human albumin.

To improve the results of hepatectomy in cirrhotic patients, the likely reserve function of the liver was evaluated before surgery. Asialoglycoprotein receptor (ASGP-R) is a hepatic cell surface receptor specific for galactose-terminated glycoproteins. Technetium-99m-diethylene triamine pentaacetic acid-galactosyl human serum albumin (99mTc-GSA) is a newly developed analog ligand to ASPG-R. The probable functional reserve of the remnant liver after hepatectomy was estimated preoperatively as the hepatic binding protein (HBP) concentration specific for ASGP-R on the hepatocellular membrane of the remnant liver. This estimate was based on the effective liver volume rate, obtained by the uptake of 99mTc-GSA. In all, 3 normal volunteers, 3 patients with chronic hepatitis (CH), 9 patients with liver cirrhosis (LC), 2 patients with hepatic cystadenoma, 3 patients with hepatocellular carcinoma (HCC) associated with CH, and 21 HCC patients with LC were studied. The mean value +/- SD obtained for HBP in normal volunteers (three cases) and in patients with mild (four cases), moderate (two cases), and severe liver damage (five cases) were 0.74 +/- 0.03 microM, 0.43 +/- 0.042 microM, 0.31 +/- 0.05 microM, and 0.20 +/- 0.05 microM, respectively. Most of the cases in which the preoperative HBP of the remnant liver was above 0.22 microM had a good postoperative course irrespective of the type of hepatectomy. On the other hand, in subjects with a remnant liver HBP of between 0.22 and 0.11 microM, postoperative severe liver dysfunction occurred in about 50% of cases. In all cases with a remnant liver HBP below 0.1 microM, the prognosis was very poor, indicating that hepatectomy should be avoided. The HBP concentration detected by the 99mTc-GSA study is a very sensitive indicator of changes in the hepatic functional reserve, and the HBP value for the functional reserve of the remnant liver is extremely useful for estimating the liver function before and after hepatectomy.

Adult↗
PubMedDate not supplied

Renal vein to portal vein collaterals in three cases of renal cell carcinoma extending into the inferior vena cava: consequences for chemoembolization.

Three patients with renal vein and inferior vena cava (IVC) tumor thrombus from left renal cell carcinoma (RCC) showed Lipiodol deposits in the liver following selective SMANCS (styrene maleic acid neocarzinostatin)/Lipiodol embolization of the renal tumors. In 2 of the 3 patients, renal-portal communications were demonstrated during selective renal arteriography. In 1 of these patients, considerable liver dysfunction occurred after the second renal chemoembolization and Lipiodol deposits persisted in the liver for about 1 month. We conclude that these anastomoses need to be considered prior to embolization therapy of patients with RCC and tumor thrombus in the renal vein and IVC. Sclerosing substances or larger particles may be better embolic agents in such patients.

Aged↗
PubMed2006

Liver involvement in Langerhans cell histiocytosis.

Liver involvement in Langerhans cell histiocytosis (LCH) typically presents with hepatomegaly and other signs of liver dysfunction. We present an 11-month-old child having only minimally elevated liver enzymes as an indication of liver involvement. Using sonography as the initial diagnostic tool followed by MRI, LCH of the liver was revealed. A review of sonographic, CT, MRI and MR cholangiopancreatography findings in liver LCH is presented. We recommend that physicians consider sonography and MRI screening for liver involvement in patients with newly diagnosed LCH, as periportal involvement may be present with little or no liver function abnormality present, as in this patient.

Diagnosis, Differential↗
PubMed2006

The role of monocyte chemoattractant protein-1 in biliary atresia.

BACKGROUND: The aim of this study was to explain the role of monocyte chemoattractant protein-1 (MCP-1) in biliary atresia (BA). METHODS: Concentrations of serum MCP-1 and collagen type IV were measured in 38 patients with BA by using commercially available kits. MCP-1 was also assessed in liver biopsy specimens by using immunohistochemistry. Subjects were classified into groups. Group 1 comprised BA patients with normal liver function (n = 13), group II comprised BA patients with moderate liver dysfunction (n = 18), group III comprised BA patients older than 20 years awaiting liver transplantation (n = 7), and the control group comprised age-matched patients without evidence of liver disease (n = 23). RESULTS: Serum MCP-1 levels were significantly increased in group II compared with group I (P < .0001) and the control group (P < .0001). Serum MCP-1 levels in group III were lower than in the control group (P < .0001). There was a significant linear correlation between serum MCP-1 levels and type IV collagen levels in group II. Group II subjects with portal hypertension (PH) had higher MCP-1 levels than those without PH (P = .0009). Biopsy specimens showed MCP-1 was expressed mainly on biliary epithelial cells, vascular endothelial cells, and hepatocytes in group II. CONCLUSIONS: These findings suggest that MCP-1 probably plays a significant role in the development of progressive liver fibrosis in BA.

Adolescent↗
PubMed2004

Liver transplantation from maastricht category 2 non-heart-beating donors: a source to increase the donor pool?

INTRODUCTION: The demand for liver transplantation has increasingly exceeded the supply of cadaver donor organs. Non-heart-beating donors (NHBDs) may be an alternative to increase the cadaver donor pool. The outcome of 20 liver transplants from Maastricht category 2 NHBD was compared with that of 40 liver transplants from heart-beating donors (HBDs). After unsuccessful cardiopulmonary resuscitation (CPR), cardiopulmonary support with simultaneous application of chest and abdominal compression (CPS; n = 6) or cardiopulmonary bypass (CPB; n = 14) was used to maintain the donors. RESULTS: At a minimum follow-up of 2 years, actuarial patient and graft survival rates with livers from Maastricht category 2 NHBD were 80% and 55%, respectively. Transplantation of organs from these donors was associated with a significantly higher incidence of primary nonfunction, biliary complications, and more severe initial liver dysfunction compared with organs from HBDs. The graft survival rates was 83% for livers from NHBDs preserved with CPS and 42% in those maintained with CPB.

Adolescent↗
PubMed1996

Fulminant hepatic failure in pregnant women: acute fatty liver or acute viral hepatitis?

BACKGROUND: Hepatitis E virus, which is endemic in our region, can cause severe liver dysfunction in pregnant women and this can be clinically confused with acute fatty liver of pregnancy. METHODS: We studied the clinical and laboratory data as well as the maternal and fetal outcomes of 12 pregnant women presenting with fulminant hepatic failure in order to determine the etiology of the disease. The clinical diagnoses were subsequently correlated with serologic assays for acute HEV infection. All patients were severely ill with deep jaundice, grade 3-4 encephalopathy and abnormal prothrombin times. RESULTS: A clinical diagnosis of acute viral hepatitis was made in nine patients and of acute fatty liver in the other three cases. IgM and IgG antibodies confirmed acute viral hepatitis E in six of the nine patients while one had acute hepatitis A infection. HEV IgM and IgG antibodies were, however, also positive in two of the three patients thought to have acute fatty liver. Maternal and fetal mortality were 16.6% and 50%, respectively. CONCLUSIONS: We conclude that hepatitis E is the usual cause of acute liver failure in our pregnant women and that clinical and laboratory features do not permit accurate distinction between acute HEV infection and acute fatty liver of pregnancy. The prognosis in patients with acute HEV infection is much better than in other groups with severe liver failure (mortality 16% vs 68%).

Acute Disease↗
PubMed1997

Nutritional and prognostic significance of insulin-like growth factor 1 in patients with liver cirrhosis.

Most of the traditional parameters for nutrition assessment have important limitations in patients with chronic liver disease. Insulin-like growth factor 1 (IGF-1) has been found to be regulated by nutrition and proposed as a nutritional marker. Its nutritional significance in patients with liver cirrhosis, however, has not been investigated. Serum IGF-1 as well as traditional anthropometric, visceral, and immunologic parameters were evaluated in 64 hospitalized cirrhotics, followed up clinically for 2 y. IGF-1Z-score averaged -2.16 +/- 1.08 and inversely correlated with Child-Pugh score (P < 0.01), the most reliable composite score reflecting the severity of liver disease. IGF-1Z-score was not different in patients with or without signs of energy malnutrition, as defined by values of midarm muscle circumference (MAMC) and/or triceps skinfold (TSF) < 5th percentile. Moreover, IGF-1Z-score did not correlate with MAMC or TSF. Despite its correlation with all visceral proteins, the reduction of IGF-1 was much greater and more frequent than that of visceral proteins. Patients with IGF-1Z-score < median values (-2.5) showed lower long-term survival rates compared with patients with IGF-1Z-score > -2.5 (P < 0.01). These data indicate that serum IGF-1 is not related to energy malnutrition in cirrhotic patients, while it appears to be a good predictor of survival and an early marker of liver dysfunction. Multiple factors, most of which are related to the severity of the liver disease, may contribute to the reduction of IGF-1. This multifactorial pathogenesis probably accounts for its prognostic significance.

Adult↗
PubMed1997

Usefulness of FDG-PET in diagnosing primary lymphoma of the liver.

This case report shows for the first time the usefulness of positron emission tomography (PET) with 2-[18F]-fluoro-2-deoxy-D-glucose (FDG) in the diagnosis of primary non Hodgkin's lymphoma of the liver. Results of FDG-PET, which in contrast to other imaging techniques offers the advantage of screening the whole body, demonstrated a high glycolytic activity of a solitary mass in the liver with central necrosis (loss of glycolytic activity), but no spread of lymphoma to the body. These results were confirmed by ultrasound, computed tomography, magnetic resonance imaging and were biopsy proven. From our findings we conclude that in patients with liver masses with high uptake of FDG, lack of liver dysfunction and absence of signs indicating other malignancies, a primary lymphoma of the liver should be considered as a possible diagnosis.

Female↗
PubMed1993

Vitamin E and liver damage in MZ heterozygous infants with alpha 1-antitrypsin deficiency.

Low levels of alpha 1-antitrypsin can predispose affected infants to develop a wide spectrum of liver diseases. Heterozygous PiMZ carriers can be affected by a subclinical liver involvement in the first six months of life. The liver damage appears to be mediated by the activity of toxic oxygen waste products originating from partially unchecked proteases which can cause enough damage to impair hepatic function significantly. In the present study it was found that the antioxidant properties of vitamin E were able to reduce the frequency of liver dysfunction in PiMZ carriers at two but not at five months of age. Liver damage is highly related to low levels of alpha-tocopherol in the plasma. These findings show that oxidative free radicals can promote liver damage in inadequately protected young infants, such as those affected by alpha 1-antitrypsin deficiency. The protective role of vitamin E in relation to the developmental expression of other antioxidant scavengers is discussed.

Alleles↗
PubMed2003

Fatty liver in type 2 diabetes mellitus: relation to regional adiposity, fatty acids, and insulin resistance.

The current study was undertaken to examine metabolic and body composition correlates of fatty liver in type 2 diabetes mellitus (DM). Eighty-three men and women with type 2 DM [mean body mass index (BMI): 34 +/- 0.5 kg/m2] and without clinical or laboratory evidence of liver dysfunction had body composition assessments of fat mass (FM), visceral adipose tissue (VAT), liver and spleen computed tomography (CT) attenuation (ratio of liver to spleen), muscle CT attenuation, and thigh adiposity; these assessments were also performed in 12 lean and 15 obese nondiabetic volunteers. Insulin sensitivity was measured with a euglycemic insulin infusion (40 mU. m-2. min-1) combined with systemic indirect calorimetry to assess glucose and lipid oxidation, and with infusions of [2H2]glucose for assessment of endogenous glucose production. A majority of those with type 2 DM (63%) met CT criteria for fatty liver, compared with 20% of obese and none of the lean nondiabetic volunteers. Fatty liver was most strongly correlated with VAT (r = -0.57, P < 0.0001) and less strongly but significantly associated with BMI (r = -0.42, P < 0.001) and FM (r = -0.37, P < 0.001), but only weakly associated with subcutaneous adiposity (r = -0.29; P < 0.01). Fatty liver was also correlated with subfascial adiposity of skeletal muscle (r = -0.44; P < 0.01). Volunteers with type 2 DM and fatty liver were substantially more insulin resistant those with type 2 DM but without fatty liver (P < 0.001) and had higher levels of plasma free fatty acids (P < 0.01) and more severe dyslipidemia (P < 0.01), a pattern observed in both genders. Plasma levels of cytokines were increased in relation to fatty liver (r = -0.34; P < 0.01). In summary, fatty liver is relatively common in overweight and obese volunteers with type 2 DM and is an aspect of body composition related to severity of insulin resistance, dyslipidemia, and inflammatory markers.

Adipose Tissue↗