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Aberrant left hepatic artery arising from the left gastric artery and liver function after radical gastrectomy for gastric cancer.

An aberrant left hepatic artery, arising from the left gastric artery (LHLG), either as an accessory or replacing the left hepatic artery, is occasionally seen in patients with gastric cancer. Resection of LHLG, as part of the gastrectomy procedure, may cause liver dysfunction. The surgical records of a group of 141 patients who had undergone gastrectomy for gastric cancer following preoperative angiography were reviewed. Twenty-eight of the patients had an LHLG preoperatively; in 15 the artery was severed during surgery and in the remainder it was preserved. Postoperative liver function was assessed by measuring changes in lactate dehydrogenase, glutamic oxaloacetic transaminase, and glutamic pyruvic transaminase activities. The greatest changes in liver function were observed in patients in whom the LHLG has been severed; the changes in liver function among patients whose LHLGs were preserved were similar to those in patients who had no LHLG. Notably, the wider the area fed by the LHLG, the greater was the change in liver function. The observed liver dysfunctions were transient, however, and normal function resumed within 7 days after operation.

Adult↗

Transient galactosemia detected by neonatal mass screening.

BACKGROUND: The Paigen method has detected not only persistently galactosemic patients, but also many children with transient galactosemia during the neonatal period. The diagnosis and clinical course of 389 patients with transient galactosemia detected by neonatal mass-screening from 1986 to 1996 in the Hiroshima prefecture were evaluated. METHODS: Enzyme assays for galactose metabolism, measurement of blood galactose levels, erythrocyte galactose-1-phosphate levels, serum total bile acid (TBA) levels and liver function tests were performed at the first visit by patients to our hospital. Liver function and the mental and physical development of patients were evaluated during the follow-up period (approximately 1 year). RESULTS: The diagnoses were classified as follows: 253 patients with unknown cause, 128 heterozygotes and two homozygotes for galactose enzyme deficiency (galactose-1-phosphate uridyltransferase, galactokinase, UDP-galactose 4-epimerase) and six heterozygotes for Duarte variant. Twelve patients showed high serum levels of TBA (> 80 mumol/L), which suggests the presence of portal-systemic shunts during the neonatal period causing galactosemia. Most patients showed normal mental and physical development during infancy. However, of 25 patients with mild to moderate abnormal liver function tests of unknown etiology after the neonatal period, five showed poor weight gain coincident with liver dysfunction. In almost all patients, levels of transferase decreased to the normal range by 1 year of age. CONCLUSION: We found that the prognosis of transient galactosemia was almost always favorable. However, patients should be followed for at least 1 year, because late liver dysfunction, which might cause poor weight gain, occurred in 6% of our patients.

Bile Acids and Salts↗

Intestinal zinc absorption in cirrhotic patients.

We studied intestinal zinc absorption and daily urinary zinc excretion in 13 healthy controls and 27 cirrhotic patients (14 alcoholics, 13 non-alcoholics) with normal serum creatinine levels. Intestinal zinc absorption was determined by the oral zinc tolerance test. Serum levels were significantly lower in cirrhotics, whether alcoholic or non-alcoholic, than in healthy controls (6.2 +/- 2.1 mumol/L vs. 11.6 +/- 2.1 mumol/L; p less than 0.001). Daily urinary zinc excretion was significantly higher in alcoholic cirrhotics (17.6 +/- 9.4 mumol/d vs. 11.4 +/- 4.1 mumol/d; p less than 0.05). Intestinal zinc absorption was significantly reduced in cirrhotics and correlated with the degree of liver dysfunction. During the oral tolerance test, urinary zinc excretion was not significantly higher in cirrhotics than in controls. We conclude that the low serum zinc levels in cirrhotics are of multifactorial origin. Impaired intestinal absorption seems to play a role, particularly in patients with more severe liver dysfunction, but increased urinary excretion may contribute to zinc deficiency in alcoholics.

Administration, Oral↗

[Effect of dibutyryl cyclic AMP in the treatment of hepatocellular carcinoma--intraarterial infusion therapy combined with anticancer agent for hepatocellular carcinoma with portal vein thrombosis].

Dibutyryl cyclic AMP was administered to 7 cases with hepatocellular carcinoma and its tumor thrombosis in portal vein, combined with intraarterial infusion of Mitomycin C or Adriamycin with implanted reservoir. Among these cases, tumor regressed in 5 cases, and therapeutic effect on tumor thrombosis was observed in 4 cases. The median survival time after initial treatment was about 5 months in 5 cases of Vp3, and more than 18 months in 2 cases of Vp2. Reduction of liver dysfunction by cholinesterase and hepaplastin test was found in most cases, and no severe side effects were observed. It is suggested that dibutyryl cyclic AMP has an antitumor effect on hepatocellular carcinoma, especially on its tumor thrombosis in portal vein, and also may assist in recovery from liver dysfunction.

Aged↗

[Type II citrullinemia triggered by acetaminophen].

We report a 19-year-old man with type II citrullinemia triggered by the administration of acetaminophen when he caught a cold. He was admitted to our hospital of impairment of consciousness and abnormal behaviors. On admission he was comatose and laboratory data revealed respiratory alkalosis which was probably induced by hyperammonemia. Liver dysfunction was mild. Plain CT scans showed brain edema, and EEG revealed triphasic waves. Analysis of plasma and urine amino acids showed a significant increase in citrulline and the activity of argininosuccinate synthetase was markedly reduced in a liver biopsy specimen. Histopathology of the liver revealed inflammatory changes which was probably induced by acetaminophen. Branched chain amino acids transfusion was effective for consciousness disturbance, and sodium benzoate was effective for hyperammonemia. We reviewed 28 patients reported in the literature from 1981 to 1992 to evaluate the probable triggering episodes in type II citrullinemia. Four categories of probable trigger were found, which included 1) stress, 2) liver dysfunction, 3) alcohol, and 4) drugs. Avoidance of these triggers, early diagnosis, and treatment seem most important for the good prognosis and the quality of life.

Acetaminophen↗

[Primary biliary cirrhosis with polymyositis successfully treated with prednisolone and ursodeoxycholic acid].

A 65-year-old woman was given a diagnosis of polymyositis in April 1991. She was treated with prednisolone until December 1993, at which time muscle strength had increased and high blood pressure had developed. In May 1994 she was hospitalized for muscle weakness and mild liver dysfunction. Prednisolone was given and the levels of hepatobiliary enzymes decreased. Immunological examination revealed strongly positive results for anti-mitochondria antibody and M-2 antibody, which lead to the diagnosis of primary biliary cirrhosis. administration of ursodeoxycholic acid in addition to prednisolone was followed by normalization of liver function and a decrease in the production of the autoantibodies. Although polymyositis can be complicated by autoimmune diseases, reports of complication by primary biliary cirrhosis are rare, here we report that treatment with the combination of ursodeoxycholic acid and prednisolone was successful in a patient with liver dysfunction and primary biliary cirrhosis.

Aged↗

[Liver transplantation and functions of the graft liver].

Partial liver transplantation from living donors is a new surgical operation on patients in the final stage of liver dysfunction. Among about 90 operations so far done in the world, 34 were performed at the Second Department of Surgery in Kyoto University Hospital (as of June, 1992). Good but limited cooperation between surgeons and clinical laboratories has contributed to saving the lives of as many as 28 patients. Analysis of laboratory data and clinical course of patients indicated that pre-, mid-, and postoperational examinations of blood flow through the graft liver by the use of Doppler echography and the monitoring of the liver capacity to generate ATP by the aid of the arterial ketone body ratio are most important for early detection of dysfunctioning liver grafts. An unusually high incidence of the transient hyperphosphatasemia-like elevation of alkaline phosphatase and a frequent appearance of liver-type arginase in serum during the postoperative stage seemed to indicate some pathological changes of the liver.

Adolescent↗

[Reversible inhibitor of MAO-A(RIMA)].

Monoamine oxidase(MAO) inhibitors had been used in the treatment of depression since 1950s, but there is no MAO inhibitor available clinically in Japan. Liver dysfunction and tyramine's effect are main problems with MAO inhibitors. Reversible inhibitors of MAO-A(RIMA), new and improved MAO inhibitors, were developed and are widely used in Europe. RIMA causes less tyramine's effect and less liver dysfunction than classical MAO inhibitors do. Moclobemide, a RIMA, is under development in Japan(Phase II). An introduction of RIMA in Japan will be useful for the treatment of refractory depression and other anxiety disorders. We discussed pharmacological properties, clinical efficacy and safety of RIMA.

Anxiety Disorders↗

Biliobiliary fistulas manifested by worsening liver function--a case report.

We report a case of cholecystolithiasis with biliobiliary fistulas from gallbladder to hepatic ducts, which were manifested by worsening liver dysfunction. Although it was not diagnosed preoperatively, it was successfully treated by cholecystectomy with closure of fistulas by the gallbladder wall. This case suggests that an internal biliary fistula may be possible, when the gallbladder wall is thickened and shrunken in the case of cholecystolithiasis, accompanied with liver dysfunction despite no dilatation of the common bile duct.

Aged↗

Urinary sulfated bile acids: a new simple urine test for cholestasis in infants and children.

BACKGROUND/PURPOSE: Measurement of urinary sulfated bile acid (USBA) has been reported as a simple urine test that reflects the degree of cholestasis. The authors report the diagnostic value of this new laboratory test in various cholestatic conditions affecting infants and children. METHODS: A urine sample was collected from 4 surgical neonates with parenteral nutrition-induced cholestasis and 48 patients with biliary atresia (BA). USBA was measured by direct enzymatic assay. RESULTS: In 3 patients receiving parenteral nutrition, USBA increased with caloric gains. For one surgical patient, a decrease in calories because of liver dysfunction resulted in a decrease of USBA, closely reflecting the fluctuations of caloric intake. In patients with BA, a significant positive correlation was noted between serum bile acid and USBA (r = 0.85; P <.01). Ten of 14 febrile episodes in 6 patients with liver dysfunction and increased C-reactive protein showed elevated USBA, thus diagnosed as cholangitis. Four febrile episodes caused by viral infection showed no elevation of USBA. CONCLUSIONS: USBA is a simple and sensitive noninvasive test reflecting the degree of cholestasis in infants and children. USBA correlated highly with serum bile acid levels and may be helpful in diagnosis of cholestasis caused by cholangitis without blood sampling.

Bile Acids and Salts↗

Inflammatory liver disease shortens atracurium-induced neuromuscular blockade in rats.

BACKGROUND: and objective Inflammatory liver dysfunction in rats leads to a prolonged vecuronium-induced neuromuscular blockade due to insufficient metabolism. A coexisting resistance against the drug partly counteracts this prolongation. The present study investigates the pharmacodynamics of atracurium whose metabolism does not depend on liver function. METHODS: Male Sprague-Dawley rats (n=14; 290 +/- 30 g) were randomly allocated to either a group in which liver inflammation was induced by intravenous injection of 60 mg kg(-1) heat-killed Corynebacterium parvum or to a control group. On day 5 after injection, liver function was assessed using the aminopyrine breath test. Under propofol anaesthesia, duration of action of atracurium (4.8 mg kg(-1)) was measured by evoked mechanomyography (stimulation of the sciatic nerve; contraction of the gastrocnemius muscle). Nitric oxide concentrations, as variables for the severity of the inflammation, were assessed by measurement of nitrite/nitrate plasma concentrations. RESULTS: In C. parvum-injected rats, nitrite/nitrate plasma concentrations were increased (972 +/- 597 vs. 25 +/- 7 micromol L(-1)), the aminopyrine turnover was depressed (1.7 +/- 0.4% vs. 3.5 +/- 0.5%), and the atracurium-induced neuromuscular blockade was shortened (372 +/- 128 s vs. 1081 +/- 234 s). CONCLUSIONS: A systemic inflammatory response syndrome with liver dysfunction results in decreased sensitivity to atracurium. Further investigations are needed regarding a possible up-regulation of acetylcholine receptors or an increased protein binding of atracurium during sepsis to clarify reasons behind this phenomenon.

Alanine Transaminase↗

Liver function tests and urinary albumin in house painters with previous heavy exposure to organic solvents.

The serum activities or concentrations of aspartate aminotransferase (ASAT), alanine aminotransferase (ALAT), alkaline phosphatase (ALP), albumin, gamma-glutamyl transpeptidase (GGT), bilirubin (BIL), cholic acid (CHOL), chenodeoxycholic acid (CHENO), and transferrin with isoelectric point 5.7, and the urinary excretion of albumin were determined among male current or former house painters (n = 135) and house carpenters (n = 71) who had worked in their trades for at least 10 years before 1970. Workers who showed a value above the 90th percentile among the carpenters in at least one of the tests ASAT, ALAT, GGT, BIL, CHOL, or CHENO were regarded as showing "possible signs of liver dysfunction". Each participant's lifetime solvent exposure was evaluated by interview. The painters were divided into categories with low, intermediate, and heavy cumulative exposure during life (LTSE) or during the most exposed year (MEYSE). All participants stated none or slight recent exposure. The prevalence of possible signs of liver dysfunction increased with solvent exposure category according to LTSE as well as MEYSE with a numerically higher risk estimate in the heavy exposure category for MEYSE than for LTSE. ALP activity increased with exposure category according to both exposure estimates. This increase seemed to be due to an interaction between exposure to solvents and current or previous long term intake of medicines potentially toxic to the liver. None of these results was affected by whether or not the subjects had been exposed to solvents during the year before the investigation. The exposure to solvents was not significantly related to any other outcome variable. It is concluded that long term heavy exposure to solvents may elicit changes in conventional liver function tests indicative of a mild chronic effect on the liver. The findings also suggest that heavy solvent exposure during short time periods is a more likely cause of the findings than lifetime cumulative solvent exposure and that an interaction between solvent exposure and medicines potentially harmful to the liver may be important in the causation of the effects.

Aged↗

Does early treatment with high-dose methylprednisolone alter the course of hepatic regimen-related toxicity?

Hepatic regimen-related toxicity (RRT) is a serious complication of stem cell transplantation. Cytokine activation may be involved in the pathogenesis. Corticosteroids are potent inhibitors of cytokine production, and, therefore could play a role in the treatment of hepatic RRT. Between January 1994 and June 1998, 28 of 782 consecutive transplant patients (3.6%) developed hepatic RRT (20 veno-occlusive disease (VOD) and eight liver dysfunction of uncertain etiology (LDUE) as defined by Seattle criteria), and were treated with high-dose methylprednisolone (MP, 500 mg/m2 i.v. every 12 h for six doses), initiated upon increase in serum total bilirubin to > or =4 mg/dl. Other causes of liver dysfunction were excluded. Response to therapy with high-dose MP was defined as reduction in total bilirubin by 50% within 10 days of initiation of MP. Overall, 17 patients (61%) responded to treatment (12 patients with VOD, five patients with LDUE). The bilirubin in responding patients decreased from a mean of 8.6 mg/dl (range, 4-17.9) at the start of MP to 4.1 mg/dl (range, 0.5-17.9) 10 days later. There were no statistically significant differences between responders and non-responders in the day treatment with high-dose MP was initiated (P = 0.38), total serum bilirubin (P = 0.17) and percent weight gain at the time high-dose MP was started (P = 0.10) or the calculated probability of fatal outcome from VOD (18% for responders, 23% for non-responders; P = 0.30). A lower pre-transplant DLCOc was observed among non-responders (P = 0.04). At 100 days post-transplant, hepatic RRT resolved in all 13 survivors who responded to high-dose MP, and in one non-responding patient. No serious toxicities due to high-dose MP were observed. We conclude that resolution of hepatic RRT occurred in the majority of patients treated with high-dose MP in this study; however, randomized controlled trials are required to determine the efficacy of high-dose MP for treatment of hepatic RRT.

Adult↗

Outflow block secondary to stenosis of the inferior vena cava following living-donor liver transplantation?

Although it is well known that outflow block is caused by stenosis or occlusion of hepatic vein anastomoses following living donor liver transplantation (LDLT), there have been few reports on inferior vena cava (IVC) stenosis following LDLT. In this paper, we report two cases of IVC stenosis and hepatic vein outflow block following right hepatic LDLT in the absence of stenosis of any of the anastomoses. Both patients presented with liver dysfunction, an ascitic fluid volume of approximately 2000 mL, and congestion in their biopsy specimens, and venocavography demonstrated IVC stenosis with gradients of more than 10 mmHg in patients with a dominant inferior right hepatic vein (IRHV) anastomosis. After a Gianturco expandable metallic stent successfully implanted in the IVC, the patient's liver function recovered and the volume of ascitic fluid decreased. The pathogenesis of hepatic vein outflow block secondary to IVC stenosis following LDLT may involve the anastomosis with the IRHV, which is the dominant draining vein of the graft and larger than the RHV, caudal to the IVC stenosis and a significant IVC pressure gradient that results in increased IRHV pressure. In conclusion, it is important to include hepatic vein outflow block in the differential diagnosis when patients who have undergone right hepatic LDLT in which anastomosis of the large IRHV has been performed develop manifestations of liver dysfunction.

Adult↗

Safety and efficacy of holmium: YAG laser lithotripsy in patients with bleeding diatheses.

PURPOSE: We assessed the safety and efficacy of ureteroscopy and holmium:YAG laser lithotripsy for treating upper urinary tract calculi in patients with known and uncorrected bleeding diathesis. MATERIALS AND METHODS: We retrospectively reviewed the charts at 2 tertiary stone centers to identify patients with known bleeding diathesis who were treated with holmium:YAG laser lithotripsy for upper urinary tract calculi. A total of 25 patients (29 upper urinary tract calculi) underwent ureteroscopic holmium laser lithotripsy. Bleeding diathesis involved warfarin administration for various conditions in 17 patients, liver dysfunction in 3, thrombocytopenia in 4 and von Willebrand's disease in 1. The mean international normalized ratio, platelet count and bleeding time were 2.3, 50 x 109/l. and greater than 16 minutes in patients on warfarin and in those with liver dysfunction, thrombocytopenia and von Willebrand's disease, respectively. RESULTS: Overall the stone-free rate was 96% (27 of 28 cases) and 29 of 30 procedures were completed successfully without significant complication. In a patient treated concomitantly with electrohydraulic lithotripsy significant retroperitoneal hemorrhage required blood transfusion. CONCLUSIONS: Upper tract urinary calculi in patients with uncorrected bleeding diathesis can be safely managed by contemporary small caliber ureteroscopes and the holmium laser as the only modality of lithotripsy. Ureteroscopic holmium laser lithotripsy without preoperatively correcting hemostatic parameters limits the risk of thromboembolic complications and costs associated with an extended hospital stay. Avoiding electrohydraulic lithotripsy is crucial for decreasing bleeding complications in this cohort of patients.

Adult↗

The natural history of type B Niemann-Pick disease: results from a 10-year longitudinal study.

OBJECTIVES: Type B Niemann-Pick disease (NPD-B) caused by acid sphingomyelinase deficiency is a rare, autosomal recessive, lysosomal storage disorder with a broad range of disease severity. The objectives of this study were to document the natural history of the disease in a large, clinically heterogeneous patient population that was followed for a period of 10 years and to determine how genotype influences phenotype. METHODS: Twenty-nine patients with NPD-B had serial evaluations at least 9 months apart. Organ volumes, hematologic indices, lipid concentrations, pulmonary function, and hepatic activity were studied, and individual phenotypic severity was compared with genotype. RESULTS: All patients with intact spleens had splenomegaly (mean value: 12.7 multiples of normal [MN]; range: 4.5-27.3 MN), and all but 1 had hepatomegaly (mean volume: 1.91 MN; range: 0.93-3.21 MN). At initial visit, 39% had thrombocytopenia and 3% had leukopenia. At final visit, the percentages increased to 54% and 34%, respectively. Mean annual decreases in platelet count and leukocyte count were 7 x 10(3) and 0.2 x 10(3) per mm3, respectively. The typical atherogenic lipid profile was worse in older patients. A total of 69% of patients had low diffusion capacity for carbon monoxide, and more than one third had low forced expiratory volume in 1 second, forced vital capacity, and forced expiratory volume in 1 second/forced vital capacity at initial visit. All measurements of pulmonary function showed a gradual deterioration over time. Liver dysfunction was characterized by stable elevation of hepatic transaminases and bilirubin. Homozygotes for DeltaR608, P323A, and P330R had milder disease than patients with all other genotypes. CONCLUSIONS: The natural history of NPD-B is characterized by hepatosplenomegaly with progressive hypersplenism, worsening atherogenic lipid profile, gradual deterioration in pulmonary function, and stable liver dysfunction.

Adolescent↗

Transfusion-associated graft-versus-host disease.

We reported two patients showed similar clinical course after blood transfusion. Case 1 was 74-year-old female, received transfusion of 200 ml of whole blood from her son, because of hemorrhagic shock for fematemesis from gastric ulcer. After transfusion, she died of liver dysfunction and severe aplastic anemia. Case 2 was a 73-year-old male, received transfusion of three unites of concentrated red blood cells for anemia following the orthopaedic treatment. High fever and erythema was appeared suddenly after transfusion. He died of liver dysfunction and the autopsy was performed. In case 1 the diagnosis was transfusion-associated graft-versus-host disease (GVHD) and in case 2 drug-induced-aplastic anemia (DIAA) was suspected. Differentiating the diagnosis between GVHD and DIAA is clinically difficult.

Aged↗

Cyclosporine metabolite pattern in blood from patients with acute GVHD after BMT.

The cyclosporine (CYA) metabolite patterns in blood were evaluated in patients with liver dysfunction after allogeneic BMT. Fifty-five BMT patients were included in the study until discharge from hospital. Blood trough levels of CYA and 12 of its metabolites were quantified using HPLC. The patients were assigned to three groups: group I (no acute GVHD, n = 23), group II (acute GVHD of the skin and good liver function, overall acute GVHD: grade I: n = 18, grade II: n = 2) and group III (acute GVHD and liver dysfunction, overall acute GVHD: grade II: n = 2, grade III/IV: n = 8). Analysis of the trough blood concentrations of CYA and its metabolites revealed higher concentrations of metabolite AM19 in group III than in the other groups without reaching statistical significance. During acute GVHD of the liver, the metabolites AM19 (p < 0.01), AM1c9 (p < 0.05) and AM1A (p < 0.05) were significantly elevated compared with patients with normal liver function while CYA and all other metabolites did not differ. The CYA metabolite pattern in patients with acute GVHD and liver involvement was identical with that of liver graft patients during acute graft rejection, while the metabolite patterns of the patients without acute GVHD paralleled that of kidney grafted patients with normal liver function. Acute GVHD of the liver leads to an impaired elimination of CYA with increased blood concentrations of single CYA metabolites.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗