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PubMed1983

A case of juvenile hepatocellular carcinoma associated with liver cirrhosis in a family clustering of HBs antigen carriers and liver diseases.

We describe a case of hepatocellular carcinoma in a 16-year-old boy whose mother, aunts, uncles, and cousins had liver dysfunction associated with HBs-Ag. Postmortem examination in this case revealed a hepatocellular carcinoma with a trabecular and partially pseudoglandular pattern involving the whole left and most of the right lobe, associated with liver cirrhosis of the postnecrotic type. Postmortem examination of the liver revealed numerous HBs-Ag positive hepatocytes demonstrated by Orcein staining in the nontumorous cirrhotic area, but not in the tumorous hepatocytes. Vertical transmission of HBV from his mother to the patient was suspected, and autopsy findings revealed continuous infection of HBV. Hepatocellular carcinoma in a young patient, especially when associated with HBs-Ag positive liver cirrhosis, as described here is rare in the English and Japanese literature.

Adolescent↗
PubMed1993

Diabetes mellitus-associated glycogen storage hepatomegaly: report of a case and review of the Japanese literature.

A huge hepatomegaly was seen in a 30-yr-old female diabetic who was treated with high dose of insulin for her uncontrollable food ingestion. The liver function at the peak of the hepatic enlargement showed a moderate increase of transaminases, alkaline phosphatase, and gamma-glutamyl transpeptidase. The histology of the enlarged liver revealed PAS-positive granules in enlarged hepatocytes, indicating the presence of massive glycogen storage. On admission, she was maintained under a calorie-restricted diet and received approximately 15 to 20 units per day of insulin supplement. At one month after admission, a marked shrinkage of her enlarged liver and restoration of normal liver function were observed concomitantly with the return of fair control of her blood sugar levels. One year later, she had an episode of diabetic ketoacidosis which subsequently was treated with a continuous low-dose infusion of insulin; however, she showed neither hepatomegaly nor liver dysfunction during this episode. There have been 20 cases reported of Japanese diabetics with marked hepatomegaly, in whom the vigorous treatment of diabetic ketoacidosis with insulin seemed to be a trigger of the enlarged liver. This has occurred mostly in patients with insulin-dependent diabetes mellitus. We present a case of non-insulin-dependent diabetes mellitus with glycogen storage hepatomegaly, presumably due to excessive insulin supplements. This suggests that glycogen storage hepatomegaly in diabetics may not be only due to an acute restoration from diabetic ketoacidosis, but may also be due to an overinsulinization in an attempt to maintain a euglycemic condition in spite of excess food intake.

Adult↗
PubMed2006

Morbidity and mortality of patients with invasive group A streptococcal infections admitted to the ICU.

STUDY OBJECTIVES: To describe the clinical features and outcome of patients with invasive group A streptococcal (GAS) infections admitted to the ICU. DESIGN: Prospective, population-based surveillance for invasive GAS infections was conducted in Ontario from January 1992 until June 2002. All 62 patients meeting clinical and/or histopathologic criteria for invasive GAS who were admitted to the ICUs of four university-affiliated hospitals in Toronto, Canada were included. Demographic and clinical information were obtained retrospectively by chart review. ICU morbidity data included the occurrence of organ dysfunction (renal, hepatic, coagulation, ARDS), treatment, and interventions such as hemodialysis and mechanical ventilation. MEASUREMENTS AND RESULTS: ARDS developed in 34%, renal dysfunction developed in 55%, hepatic dysfunction developed in 64%, and coagulopathy developed in 69% of patients. A total of 56% of patients were treated with IV polyspecific IgG (IVIG), 81% were intubated and placed on mechanical ventilation, and 21% required renal replacement therapy. The median durations of ICU and hospital stay were 5.3 days and 15.0 days, respectively. The overall mortality was 40%. Mortality correlated directly with acute physiology and chronic health evaluation II score and the number of dysfunctional organs. Survivors were younger, had lower severity of illness scores, fewer dysfunctional organs, and were less likely to have shock or to receive treatment with vasopressors, mechanical ventilation, or pulmonary artery catheters. There was no association between the use of IVIG, surgical intervention, or clindamycin, and survival. Variables independently associated with mortality on multivariable analysis were the presence of coagulopathy (p = 0.0005) and liver dysfunction (p = 0.0123). CONCLUSIONS: Patients with invasive GAS infection admitted to the ICU have a high mortality rate. In this group of patients, coagulopathy and liver failure were independently associated with mortality. We did not observe any association between the use of IVIG, surgical intervention, or clindamycin, and survival.

APACHE↗
PubMed2002

Rheological properties of blood in patients with chronic liver disease.

We analyzed rheologic parameters, including erythrocyte rigidity (ER), whole blood and plasma viscosity, erythrocyte and platelet count, hemoglobin, hematocrit, mean corpuscular volume (MCV), fibrinogen, erythrocyte sedimentation rate (ESR), cholesterol, triglyceride, high-density lipoprotein (HDL), low-density lipoprotein (LDL), very-low density lipoprotein (VLDL), and gamma globulin levels in 18 patients with chronic liver disease and 20 healthy volunteers. Fifteen patients had cryptogenic cirrhosis while 3 had chronic active hepatitis. ER and MCV was significantly higher in the patient group than the control group while whole blood and plasma viscosities were significantly lower. There were significant correlations between ER and blood and plasma viscosity, ER and MCV, plasma and blood viscosity, HDL and plasma viscosity and a negative correlation between ER and ESR. Our results demonstrate that erythrocytes become more rigid in chronic liver disease. We suggest that erythrocytes with increased rigidity can impair hepatic microvascular circulation and thus contribute to liver dysfunction.

Adult↗
PubMed2006

Serum leptin concentrations in chronic hepatitis.

The objectives of this research were to investigate the leptin levels among Chronic Hepatitis B Virus (HBV), Chronic Hepatitis C Virus (HCV) and non-alcoholic steatosis hepatitis (NASH) diseases of Thai patients compared with controls. Twenty of each HBV, HCV and NASH patients compared with sixty people as the control group from the Outpatient Department at the Hospital for Tropical Diseases, Bangkok, Thailand were investigated. Fasting blood samples were collected for investigation of leptin concentration, liver enzyme function tests and hematological variables. The serum leptin concentration of liver patients was significantly higher than that of control subjects. It might be due to the accumulations of fat cells in liver disease patients. However, there is no relationship between leptin level and other parameters such as BMI, ALT, AST, ALP and hematological variables. Liver enzyme functions levels are much higher in patients groups. White blood cells counts, platelets and hematocrit values are slightly lower in liver disease patients. Therefore, it is concluded that physiological regulation of leptin maintains in relation to body fat, even in chronic viral liver diseases. This finding and the apparent stage suggest the possibility that in the course of chronic viral diseases, serum leptin levels may reflect the extent of liver dysfunction.

Adult↗
PubMed2003

Chronic graft-versus-host disease following varicella-zoster virus infection in allogeneic stem cell transplant recipients.

We describe 2 allogeneic stem cell transplantation patients who developed chronic graft-versus-host disease (GVHD) after dermatomal varicella-zoster virus (VZV) infection. Localized zoster did not respond to oral valaciclovir but did resolve with intravenous aciclovir. However, skin eruptions, eye/oral dryness, and liver dysfunction were observed at the healing stage of localized zoster, suggesting development of GVHD. Intensification of immunosuppressive therapy was required to control GVHD. Quantitative real-time PCR for VZV DNA was used to distinguish liver involvement by chronic GVHD from visceral dissemination of VZV in 1 patient. VZV infection may trigger chronic GVHD after allogeneic stem cell transplantation.

Chickenpox↗
PubMed2006

Liver resective surgery: a multivariate analysis of postoperative outcome and complication.

INTRODUCTION: Notwithstanding technical advances and high experience of liver resection of specialized centers, the rate of complications after surgical resection could be high. In this study, we analyzed causes and foreseeable risk factors linked to postoperative morbidity on the ground of data derived from a single center surgical population. MATERIALS AND METHODS: From September 1989 to March 2005, 134 consecutive patients had liver resection for Hcc and 153 consecutive patients with liver metastasis (derived from either colorectal cancer or noncolorectal cancer) at our department. We performed 22 major hepatectomy, 20 left hepatectomy, 14 trisegmentectomy, 77 bisegmentectomy and/or left lobectomy, 74 segmentectomy, and 80 wedge resection. RESULTS: In-hospital mortality rate was 4.5%, about 7% in Hcc cases and 2.6% in liver metastasis. Morbidity rate was 47.7%, caused by the rising of ascites (10%), temporary impairment liver function (19%), biliary fistula (6%), hepatic abscess (25%), hemoperitoneum (10%), and pleural effusion (30%) sometimes combined each other. Some variables, associated with the technical aspects of surgical procedure, are responsible of the rising of complication as: Pringle maneuver length of more than 20 minutes (p=0.001); the type of liver resection procedure [major hepatectomy (p=0.02), left hepatectomy (p=0.04), trisegmentectomy (p=0.04), bisegmentectomy and/or left lobectomy (p=0.04)]; and the request of an amount of blood transfusion of more than 600 cc (p=0.04). Also, both liver dysfunction, in particular Child A vs B and C (p=0.01), and histopathological grading (p=0.01) are associated with a high rate of postsurgical complication in Hcc cases. CONCLUSION: We make the following recommendations: every liver resection should be planned after intraoperative ultrasonography, anatomical surgical procedure should be preferred instead of wedge resection, and modern devices should be used, like Argon Beam and Ligasure dissector, to reduce the incidence of both intraoperative and postoperative bleeding and biliary leakage.

Adult↗
PubMed2003

Strategy of gene therapy for liver cirrohosis and hepatocellular carcinoma.

BACKGROUND/PURPOSE: Liver cirrhosis and hepatocellular carcinoma (HCC) are major causes of morbidity and mortality worldwide, without effective therapies. Our aim was to establish a novel approach for the diseases utilizing in-vivo gene therapy. METHODS: To achieve effective gene expression in vivo, we employed a well-established transfection method, hemagglutinating virus of Japan (HVJ)-liposome. Liver cirrhosis, characterized by parenchymal collapse, was induced by dimethylnitrosamine (DMN) in rats, leading to accumulation of fibrous tissue. Muscle-directed gene transfer of hepatocyte growth factor (HGF) was performed in this model. As an approach for HCC therapy, suicide gene therapy using ganciclovir (GCV) with transfer of the herpes thymidine kinase (HSVtk) gene was tested. Alpha-fetoprotein (AFP) is highly expressed in many cases of HCC. To achieve specific gene expression of HSVtk in AFP-producing tumors, we employed the HSVtk gene driven by the AFP promoter (AFP-TK1), encapsulated in the HVJ-liposome. APF-producing HUH7 tumors in vivo were established in the livers of severe combined immunodeficiency (SCID) mice, by the injection of HUH7 cells into the portal vein. RESULTS: All cirrhotic rats died of liver dysfunction by 7 weeks after the initial injection of DMN. After repeated transfection with the HGF gene, increased concentrations of HGF in plasma and tyrosine phosphorylation of the c-Met/HGF receptor in the liver were detected, and the established massive hepatic fibrosis had almost totally disappeared and all cirrhotic rats survived. Repeated in-vivo transfection with AFP-TK1, using the HVJ-liposome, followed by GCV treatment, achieved abrogation of tumors in the liver, and improved survival. CONCLUSIONS: Our data indicate that the gene therapy may hold promise for the treatment of patients with liver cirrhosis and HCC.

Animals↗
PubMed1999

Different clinical aspects of debrancher deficiency myopathy.

OBJECTIVE: To characterise the main clinical phenotypes of debrancher deficiency myopathy and to increase awareness for this probably underdiagnosed disorder. METHODS: The diagnosis of debrancher deficiency was established by laboratory tests, EMG, and muscle and liver biopsy. RESULTS: Four patients with debrancher deficiency myopathy were identified in the Tyrol, a federal state of Austria with half a million inhabitants. Clinical appearance was highly variable. The following phenotypes were differentiated: (1) adult onset distal myopathy; (2) subacute myopathy of the respiratory muscles; (3) severe generalised myopathy; and (4) minimal variant myopathy. Exercise intolerance was uncommon. The clinical course was complicated by advanced liver dysfunction in two patients and by severe cardiomyopathy in one. All had raised creatine kinase concentrations (263 to 810 U/l), myogenic and neurogenic features on EMG, and markedly decreased debrancher enzyme activities in muscle or liver biopsy specimens. The findings were substantiated by a review of 79 previously published cases with neuromuscular debrancher deficiency. CONCLUSIONS: This study illustrates the heterogeneity of neuromuscular manifestations in debrancher deficiency. Based on the clinical appearance, age at onset, and course of disease four phenotypes may be defined which differ in prognosis, frequency of complications, and response to therapy.

Age of Onset↗
PubMed2002

Clinical review: splanchnic ischaemia.

Inadequate splanchnic perfusion is associated with increased morbidity and mortality, particularly if liver dysfunction coexists. Heart failure, increased intra-abdominal pressure, haemodialysis and the presence of obstructive sleep apnoea are among the multiple clinical conditions that are associated with impaired splanchnic perfusion in critically ill patients. Total liver blood flow is believed to be relatively protected when gut blood flow decreases, because hepatic arterial flow increases when portal venous flow decreases (the hepatic arterial buffer response [HABR]). However, there is evidence that the HABR is diminished or even abolished during endotoxaemia and when gut blood flow becomes very low. Unfortunately, no drugs are yet available that increase total hepato-splanchnic blood flow selectively and to a clinically relevant extent. The present review discusses old and new concepts of splanchnic vasoregulation from both experimental and clinical viewpoints. Recently published trials in this field are discussed.

Animals↗
PubMed2006

Marked hepatomegaly due to AA type amyloidosis in a case with Castleman's disease.

Hepatic amyloidosis complicated with Castleman's disease is quite rare. A 48-year-old woman was referred to our hospital with general fatigue, low-grade fever, anemia, thrombocythemia, and liver dysfunction. Physical examination revealed anemia and hepatomegaly and abdominal computed tomography showed marked hepatomegaly and right upper abdominal masses. Technetium-99m pyrophosphate (99mTc-PYP) scintigraphy revealed the diffuse abnormal uptake of the enlarged liver, suggesting amyloid deposition. Liver biopsy showed destruction of the liver structure and the massive deposition of AA type amyloid protein. Surgical resection was performed on the abdominal masses. Histological examination of the masses showed Castleman's disease (plasma cell type). After resection, her fever resolved and the liver size gradually decreased to within the normal range. This case shows that surgical resection of the main lesion is effective for hepatomegaly due to AA type amyloidosis associated with Castleman's disease.

Amyloidosis↗
PubMed2001

[Liver transplantation: which indications? which results?].

UNLABELLED: THE ONLY TREATMENT: Liver transplantation (LT) is currently the final treatment for most types of end-stage liver diseases including alcoholic cirrhosis, so far alcoholic cirrhosis has become the first indication for LT in France and other western countries, accounting for 25% of all procedures. However due to ethical issues and also the discrepancy between the theoretical number of candidates and available organs, indications for LT in alcoholic cirrhosis must be rigorously defined. INDICATIONS: On the average, candidates are 50 years old, males in two-thirds of the cases, with pre-terminal liver disease combining advanced-stage liver dysfunction (PT < 40%, bilirubin > 50 mumol/l, albumin < 30 g/l) and intractable ascitis. The gain in survival being best in patients with severe cirrhosis (Child-Pugh grade C), these patients should be given priority when liver function fails to improve despite prolonged abstention. For less advanced diseases (Child-Pugh B or A), LT can be considered after failure of symptomatic medical treatments or in case a small hepatocellular carcinoma develops. CONTRAINDICATIONS: The list includes presence of extrahepatic organ failure, generally related to alcohol-tobacco abuse (cardiomyopathy, pancreatitis, neuropathy, squamous cell carcinoma ...) and precarious psychosocial situations exposing the patient to the risk of recurrent alcoholism and non-compliance after transplantation. Predictive factors of after recidivism after transplantation are preoperative abstinence of less than 6 months duration, denial of alcoholism, lack of familial and occupational support, antisocial behavior and a history of psychiatric disorders or drug abuse. Patients with several of these risk factors cannot reasonably be considered as candidates for LT. Inversely, transplantation should be proposed for patients with no or few risk factors due to the excellent physical and social outcome observed. Generally, a full 6 months of preoperative abstinence is required by most transplantation centers. The aim is to avoid underestimation for liver function recovery and to limit the risk of recurrent alcoholism. RESULTS: Using the above criteria, LT for alcoholic cirrhosis can restore a satisfactory quality of life and provides a 5-year survival to the order of 65%, similar to results obtained in other indications for LT. Recurrent pathological consumption of alcohol occurs in 10 to 15% of the cases, generally with moderate effects on the liver graft. PRACTICAL ATTITUDE: Using the currently accepted selection criteria, less than 5% of the patient with alcoholic cirrhosis actually undergo transplantation. For these patients, LT enables to treat both cirrhosis and alcoholic disease in 80% of the cases. Based on these results, segregating among patients with severe alcoholic cirrhosis considered as reasonable candidates for LT after a complete pluridisciplinary preoperative work-up must be avoided.

Contraindications↗
PubMed2005

[Cytomegalovirus infection accompanied with acute pancreatitis after kidney transplantation].

OBJECTIVE: To explore the prevention and management of human cytomegalovirus (HCMV) infection accompanied with acute pancreatitis after kidney transplantation. METHODS AND RESULTS: A retrospective analysis of 5 patients with acute pancreatitis after kidney transplantation was conducted. The incidence of acute pancreatitis after kidney transplantation was 2.3% (5/217). All the 5 cases were complicated by active HCMV infection, 3 of which had hyperlipemia and 2 had liver dysfunction. Three cases were finally cured while the other 2 died, one of which was due to respiration failure arising from HCMV interstitial pneumonia accompanied with hemorrhagic necrotizing pancreatitis, and the other due to fulminating liver function failure because of active HCMV infection accompanied with hemorrhagic necrotizing pancreatitis. CONCLUSION: Active HCMV infection is the most important factor responsible for acute pancreatitis, and early diagnosis and treatment are crucial to lower mortality rate.

Adult↗
PubMed2006

[Adverse effects of intravenous arsenic trioxide and their prevention].

OBJECTIVE: To study the main side effects of As(2)O(3) and the way of prevention. METHODS: The changes of body weight and various systems of body were observed after treatment with As(2)O(3) injection. The arsenic content in blood, urine and hair was detected with atom absorbed-spectrum analysis. RESULTS: Mild reactions were observed in 57.43% (147/256) of the patients and they could subside after cessation of arsenic treatment or allotherapy. Chronic mild poisoning manifestations including arsenic furuncle, liver dysfunction and peripheral nerve injury were found in 2.73% (7/256) of the patients and they subsided gradually after treatment. Chronic severe poisoning was found in 1.17% of the patients and all of them died of liver failure. As(2)O(3) might cause decrease of peripheral blood WBC in catabatic patients. There was no infection after allopathy without ceasing arsenic medication. The results showed that As(2)O(3) could distribute over and discharge from the plasma without accumulation in blood. The results also demonstrated the main route of excretion for As(2)O(3) is through urine and there is definite accumulation in the hair 50 days after treatment. CONCLUSION: Most of the side effects of As(2)O(3) are mild and recoverable. Allotherapy could be effective to relieve the complications without stopping arsenic medication. A few patients with complicating hepatitis may suffer from chronic poisoning. As(2)O(3) may cause increase of peripheral blood WBC in induced catabatic APL patients. The side effects must be prevented early.

Adolescent↗
PubMed1984

Impaired renal acidification in alcoholic liver disease.

The prevalence of impaired renal acidification in alcoholic liver disease and its relationship to clinical and biochemical features were evaluated during a one-year period in a 60-bed liver unit. No cases of overt renal tubular acidosis (RTA) were found; all of 12 patients with low serum bicarbonate values and normal anion gap proved to have chronic respiratory alkalosis. However, there was a 57% prevalence of incomplete distal RTA in 42 patients who were tested with an acid load. Subjects with RTA had higher serum bilirubin levels (5.3 +/- 6.1 v 2.1 +/- 2.7 mg/dL) and lower prothrombin times (45% +/- 22% v 64% +/- 20%). Urinary pH correlated directly with serum bilirubin levels (r = +.38) and inversely with prothrombin times (r = -.46). The frequency of ascites and encephalopathy did not differ notably between the two groups of patients. No pathogenetic relation was observed with avid sodium retention, decreased excretion of nonreabsorbable anions, and elevated urinary excretion of bile acids. Therefore, we conclude that impaired renal acidification in alcoholic liver disease may be a sign of liver cell failure since it is more frequently observed in patients with a greater degree of liver dysfunction.

Acid-Base Equilibrium↗
PubMed1987

Galactose elimination kinetics in sepsis. Correlations of hepatic blood blow with function.

To study hepatic blood flow with clearance techniques during sepsis, it is essential to work within the limitations of the test being applied. Based on galactose elimination kinetics, this study validates galactose clearance at low concentrations as an estimate of effective hepatic blood flow in a rat peritonitis model of cecal ligation and puncture. Hepatic function as determined by galactose elimination capacity fell 25% at ten hours after induction of peritonitis, which correlated closely with the 20% reduction in effective hepatic blood flow at the same time point despite a normal cardiac output. The pattern of reduced flow and reduced function is consistent with intrahepatic flow redistribution. Inadequate flow at the microvascular level with secondary cellular injury may explain the liver dysfunction observed during sepsis.

Animals↗
PubMed1991

Management of haemangioma of the liver: comparison of results between surgery and observation.

Cavernous haemangioma of the liver was diagnosed in 33 patients using hepatic angiography between 1978 and 1988. Thirteen of these patients underwent surgery. There were no deaths after operation; morbidity included upper gastrointestinal bleeding in two patients and liver dysfunction in one. The follow-up study showed no evidence of recurrence in any of the 13 patients up to 143 months after operation. In the operated patients, a small haemangioma was left in the liver of two for anatomical reasons, but the size of these tumours showed no change over 3 years. The remaining 20 patients had no specific treatment for haemangioma and were followed up for between 18 months and 8 years. Of these 20 patients, 19 had tumours of less than 5 cm in size. As there was no enlargement or rupture of haemangiomas of less than 5 cm, it was felt that these could be observed. Cases having a potential for exposure to trauma, rapid tumour growth, or displaying severe symptoms should be considered for surgical treatment; this can be justified due to low morbidity and no mortality.

Female↗
PubMed2003

Lytic infection of Epstein-Barr virus (EBV) in hemophagocytic syndrome associated with EBV-induced lymphoproliferative disorder.

This report describes lytic infection with Epstein-Barr virus (EBV) in three cases of hemophagocytic syndrome (HPS) presumably associated with EBV-induced lymphoproliferative disorder. All cases were previously healthy females with ages ranging from 52 to 87 years who showed a fulminant clinical course with accompanying fever, liver dysfunction, and disseminated intravascular coagulation. Cases 1 and 2 showed proliferation of atypical T lymphocytes, and case 3 showed proliferation of atypical B lymphocytes. Hemophagocytic histiocytes were observed in the bone marrow, lymph nodes, spleen, and liver. Atypical lymphocytes in all cases showed a positive reaction for both EBV-encoded small RNA (EBER) indicating latent infection with EBV and immediate early mRNAs of the Bam HI fragment of lower stranded frame (BHLF), indicating lytic infection by in situ hybridization. Interestingly, BHLF-positive cells were predominant in all cases. It is possible that reactivation of EBV infection may be involved in triggering the induction of cytokines and abnormal activation of histiocytes.

Aged↗