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PubMed2003

Living-related liver transplantation for Alagille syndrome.

Alagille syndrome (AGS) is an autosomal dominant genetic disorder characterized by chronic cholestasis, congenital heart disease, peculiar facies, butterfly-like vertebrae, and posterior embryotoxon. Liver dysfunction is the common presentation of AGS, and liver transplantation may be indicated. This study examines the outcome of living-related liver transplantation (LRLT) for AGS. Twenty patients with AGS (median age 5.0 years, range 0.6-12.9) underwent LRLT at Kyoto University Hospital between June 1990 and February 2002. Five potential donors were excluded because of paucity of intrahepatic bile ducts diagnosed by preoperative liver biopsy and one because of a hepatic vascular anomaly. The overall 5-year patient survival was 80.4%. Three patients died as the result of the following: complications related to surgery, heart failure caused by progressive pulmonary artery stenosis, and a graft with unsuspected bile duct paucity. Liver dysfunction was improved in all successful cases, and catch-up growth occurred in 90% of patients. LRLT is an efficacious treatment modality for AGS if donors are selected by cautious evaluation to rule out unsuspected bile duct paucity.

Alagille Syndrome↗
PubMedDate not supplied

The association of hepatic glycogen depletion with hyperammonemia in cirrhosis.

Hyperammonemia is a well-recognized metabolic abnormality which occurs in cirrhotic patients with advanced liver dysfunction. We recently documented that hyperglucagonemia that occurs as a result of hepatic glycogen depletion may be responsible for this hyperammonemia by promoting gluconeogenesis to provide glucose as a fuel for functioning of several organ systems. Thus, hepatic glycogen depletion may be the initial process responsible for hyperammonemia. Since the glucose rise following intravenous glucagon administration is a reflection of hepatic glycogen breakdown, we studied the effect of glucagon (1 mg) injection on plasma glucose, insulin and ammonia levels after an overnight fast in cirrhotic patients and normal subjects. Glucose rise was significantly stunted, and ammonia rise was significantly greater in patients with advanced liver dysfunction as compared to normal subjects. Furthermore, the smaller the glucose increment, the earlier the ammonia rise. The smallest glucose responses were seen in the patients with the highest basal plasma ammonia levels. Finally, significant negative relationships were noted between the glucose response to glucagon administration (delta glucose) and the degree of liver dysfunction as reflected by Composite Clinical Laboratory Index, as well as basal ammonia and ammonia responses (delta ammonia) on the other. Therefore, this study suggests that hepatic glycogen depletion may be the initial event leading to elevated plasma ammonia concentrations in hepatic cirrhosis.

Adult↗
PubMed1996

Superinfection with hepatitis C virus in hemodialysis patients with hepatitis B surface antigenemia: its prevalence and clinical significance in Taiwan.

A survey of hepatitis B surface antigen (HBSAG) and antibodies against hepatitis C virus (anti-HCV) in 173 patients undergoing hemodialysis in Taiwan revealed that 15 (9%) patients were positive for both HBSAG and anti-HCV, 106 (61%) were positive for anti-HCV alone, and 14 (8%) were positive for HBSAG alone. Most HBSAg positivity was acquired before the onset of hemodialysis. Anti-HCV positivity, however, was mainly acquired via the hemodialysis procedure. Patients with dual markers were younger (43.7 +/- 3.3 years old, p = 0.0274), had the longest period on hemodialysis (6.6 +/- 1.3 years, p < 0.001), and more severe liver dysfunction. When compared with those who were negative for both markers, patients with both HBSAG and anti-HCV had an increased incidence of chronicity (5/15 vs. 2/38; p < 0.05), ultrasonographic cirrhosis (5/15 vs. 1/38; p < 0.05), and clinical decompensation (2/15 vs. 0/38; p < 0.05). Their risk for developing ultrasonographic cirrhosis and clinical decompensation was also greater than that of patients with anti-HCV alone (5/15 vs. 8/106 and 2/15 vs. 2/106; p < 0.05 for both). The presence of HBSAG alone, however, did not increase the incidence of liver dysfunction. The presence of anti-HCV alone was only associated with a greater elevation of serum alanine aminotransferase (44.2 +/- 5.5 vs. 19.1 +/- 2.5 U/l; p < 0.05) and an increased incidence of chronicity (30/106 vs. 2/38; p < 0.05). Our results indicate that a high prevalence of HCV superinfection impose a significant risk on a large population of HBSAG-positive hemodialysis patients in Taiwan. As the coexistence of anti-HCV and HBSAG is associated with more severe liver dysfunction, it is urgent to devise effective methods to prevent HCV circulation in a hemodialysis environment-especially in a hepatitis B virus endemic area such as Taiwan.

Adolescent↗
PubMed2006

Circulating cytokines, chemokines, and stress hormones are increased in patients with organ dysfunction following liver resection.

BACKGROUND: Humoral mediators are potentially involved in the pathogenesis of postoperative complications following surgery. The aim of the present study is to evaluate the postoperative responses of circulating cytokines, chemokines, and stress hormones following liver resection, and their effects on postoperative infectious complications and organ dysfunction. PATIENTS AND METHODS: Perioperative plasma concentrations of interleukin (IL)-6, IL-10, IL-4, IL-8, macrophage chemoattractant protein (MCP)-1, cortisol, macrophage migration inhibitory factor (MIF), and leptin were measured by immunoassays in 128 consecutive patients undergoing liver resection. RESULTS: Forty-three patients had postoperative infection and 11 had infection-related organ dysfunction. Plasma levels of all mediators except for IL-4 increased postoperatively. Postoperative levels of IL-6, IL-10, IL-8, MCP-1, cortisol, and leptin were significantly higher in patients with organ dysfunction than in those without organ dysfunction (P < 0.05). However, postoperative MIF levels were not affected by postoperative infection or organ dysfunction. Plasma levels of IL-6, IL-10, IL-8, and MCP-1 were positively correlated with operation time (P < 0.0001) or blood loss (P < 0.0001), and higher in patients with jaundiced liver (P < 0.05). In univariate logistic regression analyses, elevated IL-6, IL-10, IL-8, and MCP-1, advanced age, large volume of blood loss, long operation time, long hepatic ischemia time, and major liver resection were significantly correlated with postoperative infection (P < 0.05). In multivariate analyses, IL-6 and IL-10 were significant predisposing factors for postoperative infection (P < 0.05), and blood loss and IL-6 for organ dysfunction (P < 0.01). CONCLUSIONS: These results suggest that IL-6, IL-10, IL-8, MCP-1, cortisol, and leptin are released after liver resection in response to surgical stress and correlated with postoperative infection and organ dysfunction, and that of these circulating mediators, IL-6 and IL-10, have a close relationship to the complications.

Adult↗
PubMed1989

Effects of glycyrrhizin (SNMC: stronger Neo-Minophagen C) in hemophilia patients with HIV infection.

Glycyrrhizin (GL) not only has an inhibitory effect on HIV replication but also exhibits interferon-inducing and natural killer (NK)-enhancing effects and improves liver dysfunction. Thus, large doses of GL (200-800 mg/day) were intravenously administered for more than 8 weeks to 9 hemophilia A patients with HIV infection (asymptomatic carrier, AC). Lymphocyte count increased in all 9 cases. OKT4 OKT8 ratio was elevated in 6 out of the 9 cases and OKT4-positive lymphocytes increased in 8 out of the 9 cases; 66.7% and 88.9% improvement, respectively. Changes in NK cell activity and mitogenic responsiveness to PHA, Con A and PWM were not significant. Liver dysfunction, noted in 4 cases, clearly improved. Serum electrolytes, protein, lipids, and renal function were within normal levels and no serious side-effects were observed during treatment. On the other hand, in 3 cases of hemophilia without HIV infection, the number of OKT4 lymphocytes was not significantly altered during treatment. From these results, large dose administration of GL to HIV-positive hemophilia patients (AC) seems to be effective in preventing development of AC into AIDS by raising the number of decreased OKT4 lymphocytes and improving liver dysfunction.

Acquired Immunodeficiency Syndrome↗
PubMedDate not supplied

Clinical vignette in antiretroviral therapy: jaundice.

HIV caregivers face many challenges following initiation of ART. The development of jaundice is uncommon but worrisome. In this case, two distinct and contrasting episodes of jaundice were observed. In the first instance, isolated elevation of the indirect bilirubin without elevation of the alkaline phosphatase was noted. The normal PT and serum aminotransferase levels indicate the absence of intrinsic liver dysfunction. Elevations in the indirect bilirubin may result from either impaired uptake/conjugation or excess production. The latter, usually from acquired hemolysis, may be a complication of an occult NHL. A work-up for this AIDS-related malignancy was not initiated since the caregivers recognized jaundice as a complication of IDV, which inhibits UDP-glucuronyl transferase and produces a Gilbert's-like syndrome. Physicians can expect to encounter this syndrome even more frequently with ATV. Experienced patients given RTV-boosted ATV have experienced elevations of unconjugated hyper-bilirubinemia in up to 45 percent of cases in clinical trials. However, such elevations do not reflect liver dysfunction and symptomatic jaundice requiring dosage reduction that occurred infrequently (7 to 8 percent of study patients). Counseling patients about this syndrome may promote adherence and prevent self-directed interruptions of ATV that compromise efficacy. The second case of jaundice provides a more formidable diagnostic challenge. The triad of LFT abnormalities (mild elevation of aminotransferases, normal PT, and marked cholestatic jaundice) implies an acute process that is mildly toxic to hepatocytes without affecting their synthetic function. The subacute nature of the patient's cholestatic jaundice suggests either intrahepatic infiltrative disease of the liver or extrahepatic obstruction of the biliary tree, most likely due to the patient's relatively modest level of pain and lack of fever. Despite LFT abnormalities occurring 17 months after a switch in his ART, cumulative drug-related toxicities must still be considered. Ritonavir can produce significant elevations in the AST/ALT, especially with pre-existing chronic liver disease as with hepatitis C virus coinfection. The NRTIs can produce hepatic steatosis, a result of mitochondrial toxicity and impaired fatty acid oxidation. However, jaundice and cholestasis are not typical of the latter syndrome. With a negative contrast CT that excludes parenchymal liver disease, investigation of the biliary tree to assess the presence of AIDS-related cholangitis was the next step. Performing a sphincterotomy or stent placement, and obtaining brushings or biopsy specimens to determine the extent of extrahepatic obstruction may help define a pathogen and be life-saving. The negative results of the ERCP justify the final diagnostic step, a liver biopsy to evaluate microscopic infiltrative disease that might not have been detected on contrast abdominal CT. Examples might include granulomatous disease (MAC), fungal etiologies (histoplasmosis), carcinomatosis (lymphoma, hepatoma, cholangiocarcinoma), and microvascular disease (bacillary angiomatosis). The failure to observe granulomatous inflammation in the liver does not exclude MAC infection, as MAC may involve other peri-aortic or mesenteric lymph nodes. This form of IRIS is unlikely given the abdominal CT findings, lack of systemic complaints, and extended persistence of liver aminotransferases. The nonspecific results of the liver biopsy are a common outcome in advanced AIDS patients with elevated alkaline phosphatase levels. Despite not having identified a pathogen, the biopsy establishes chronic liver disease and prompts re-evaluation and change of treatment to NFV. The subsequent normalization of the patient's aminotransferase levels suggests a prior adverse effect of LPV/r in the setting of unexplained, chronic liver disease. Most importantly, this case highlights the importance of HIV caregivers to review ART for safety when noting chronic liver dysfunction. Patients need to be counseled to minimize acetaminophen use, to consume alcohol in moderation, and to avoid behavior with risk for hepatitis C. Finally, all HIV patients should receive appropriate vaccination against hepatitis A and B if serology shows lack of protective immunity.

Acquired Immunodeficiency Syndrome↗
PubMed2026

Comparative safety of lipid-lowering drugs alone or in combination: insights from a systematic review and network meta-analysis.

BACKGROUND AND AIMS: Although the safety profile of lipid-lowering therapies (LLTs) is known, there are no comprehensive comparative assessments. We aimed to compare the risk of muscle-related events, diabetes, liver dysfunction, and cognitive disorders among LLTs through a network meta-analysis. METHODS AND RESULTS: Databases were searched from inception to May 2025. Eligible studies included adult patients, using statins, ezetimibe, PCSK9 monoclonal antibodies (PCSK9mAbs), inclisiran, bempedoic acid, or their combinations as intervention, reporting the information about any of the selected adverse events, a total sample size of &#x2265;200 subjects, and had &#x2265;1 month of intervention. Pooled estimates were assessed by fixed effects model within a frequentist setting. Pooled relative risks (RR) and their 95% confidence interval were estimated. A total of 303,397 subjects from 153 RCTs were included. Bempedoic acid ranked the lowest risk of myalgia (vs PCSK9mAbs, RR 0.80 [0.69, 0.93]). PCSK9mAbs were associated with lower incidence of creatine kinase (CK) elevation, diabetes, and liver dysfunction comparing to statins (statins vs PCSK9mAbs, RR 1.44 [1.14, 1.81], RR 1.13 [1.05, 1.22], and RR 1.38 [1.17, 1.62], respectively). In terms of muscle-related events and cognitive disorders, no significant risk differences were found among treatments and their combinations. CONCLUSIONS: PCSK9mAbs appear to have a more favourable safety profile regarding the risk of CK elevation, diabetes, and liver dysfunction. Bempedoic acid seem to be a better choice for subjects with high risk of myalgia. This information can be valuable when selecting therapy for specific patient subgroups at higher risk of certain adverse events.

Humans↗
PubMed1980

Prevalence of type B and non-A, non-B hepatitis in hemophilia: relationship to chronic liver disease.

To assess the relationship of liver dysfunction and hepatitis markers in hemophilic patients treated with factor VIII or IX concentrates, we studied 103 patients with hemophilia A and B for 6-36 mo. Elevated serum alanine aminotransferase was noted in 79% of the patients, with 51% of the patients showing persistent elevation for longer than 6 mo. Thirteen patients (12%) were HBsAg-positive, with 8 patients showing persistence of HBsAg and abnormal serum alanine aminotransferase for more than 6 mo. Overall, anti-HBs was detected in 77% of patients. Twelve episodes of acute hepatitis were documented in 10 patients during 36 mo. Six episodes were due to hepatitis B virus. The remaining 6 episodes were due to non-A, non-B hepatitis with negative HBsAg and absence of seroconversion to hepatitis B virus, hepatitis A virus, cytomegalovirus, and Epstein-Barr virus. In the six episodes of non-A, non-B hepatitis, the incubation period was less than 10 days in 3 patients and 30 days in 2 patients. In all cases with non-A, non-B hepatitis, the illness was symptomatic, but mild. Serum alanine aminotransferase returned to normal within 4 mo in 2 patients, but in 3 patients serum alanine aminotransferase persisted longer than 6 mo. One patient developed an acute B hepatitis 40 wk after non-A, non-B hepatitis. Thus, infection with the hepatitis B virus still remains prevalent as a cause of acute hepatitis in hemophiliacs receiving commercial factor concentrates, and accounts for chronic liver dysfunction in patients with persistent HBs antigenemia. In addition, acute non-A, non-B hepatitis, appears to be relatively common in hemophiliacs, and non-A, non-B virus may account for many cases of persistent liver dysfunction in these patients.

Acute Disease↗
PubMed1993

Risk factors for primary dysfunction after liver transplantation--a multivariate analysis.

In a retrospective analysis on 323 orthotopic liver transplant procedures performed between July 1984 and October 1991 the incidence of two forms of primary dysfunction (PDF) of the liver: primary nonfunction (PNF), and initial poor function (IPF) were studied. The incidence of PDF was 22% (73/323) with 6% PNF (20/323) and 16% IPF (53/323), while 78% (250/323) had immediate function (IF). Occurrence of both IPF and PNF resulted in a higher graft failure rate (P < 0.001), retransplantation rate (P < 0.001), and patient mortality (P < 0.003) within the first three months after OLTx. Univariate analyses of donor and recipient factors and their influence on PDF demonstrated that longer donor hospitalization (> 3 days), older donor age (> 49 years), extended preservation times (> 18 hr), and fatty changes in the donor liver biopsy, as well as reduced-size livers, younger recipient age, and renal insufficiency prior to OLTx, significantly affected the incidence of IPF and PNF. Multivariate analysis of potential risk factors showed that reduced-size liver (P = 0.0001), fatty changes on donor liver biopsy (P = 0.001), older donor age (P = 0.009), retransplantation (P = 0.01), renal insufficiency (P = 0.02), and prolonged cold ischemia times (P = 0.02) were independently associated with a higher incidence of IPF and PNF. No statistical correlation was found between PDF and etiology of ESLD, nutritional status of the recipient, UNOS status, and Child-Pugh classification in this study. We conclude that PNF and IPF are both separate clinical entities that have a significant effect on outcome after OLTx. Routine donor liver biopsies are recommended to decrease the rate of IPF and PNF. The combination of risk factors shown to be significant for PDF should be avoided--and, if that is not possible, the only variable that can be controlled, the preservation time, should be kept as short as possible.

Adolescent↗
PubMed2002

Longitudinal bone loss in postmenopausal women with primary biliary cirrhosis and well-preserved liver function.

OBJECTIVES/DESIGN: Increased rate of bone loss has been reported in women with primary biliary cirrhosis (PBC) and varying degree of liver dysfunction. Whether bone loss is increased in patients without liver dysfunction is unclear. The aim of this study was to estimate retrospectively the rate of bone loss in postmenopausal women with PBC and well-preserved liver function. SUBJECTS/INTERVENTIONS: Forty-three women with PBC, and classified as Child-Pugh class A, were included. Bone mineral density (BMD) was measured by dual energy X-ray absorptiometry at the lumbar spine and the femoral neck. RESULTS: Median time between measurements of BMD was 26 months (range, 12-48 months). Twenty women were not receiving any bone protective treatment, i.e. hormone replacement therapy (HRT), bisphosphonates or vitamin D/calcium supplementation, whilst 23 women received such treatment. Mean annual bone loss in the former group was 0.38 +/- 2.56% and 0.42 +/- 2.29% at the lumbar spine and the femoral neck, respectively. Women receiving treatment, however, increased their BMD by 1.92 +/- 3.76% and 0.15 +/- 2.75% at the lumbar spine and the femoral neck, respectively. At the lumbar spine the difference with regard to changes in BMD between untreated and treated women was statistically significant (P = 0.02). Women who received HRT (n = 11) increased their BMD at the lumbar spine by 2.95 +/- 3.91%, P = 0.03 when compared with untreated women. CONCLUSION: Bone loss in postmenopausal women with PBC and well-preserved liver function is not increased above normal. Treatment with bone protective treatment, mainly HRT, improves BMD at the lumbar spine.

Adult↗
PubMed1994

Sphincter of Oddi dysfunction following liver transplantation. Screening by bedside manometry and definitive manometric evaluation.

Although sphincter of Oddi dysfunction (SOD) has been extensively studied in the nontransplant setting, the diagnostic criteria after liver transplantation are not well defined and have been based on clinical features without manometric documentation. The purpose of this study was twofold: (1) to determine the manometric patterns associated with SOD following orthotopic liver transplantation (OLT) and (2) to define the usefulness of bedside T-tube manometry as a screening tool for SOD. ERCP with simultaneous manometry of the sphincter of Oddi (SO) was performed in five patients following OLT with choledochocholedochostomy (CDCD) between 1990 and 1992. The diagnosis of SOD was suspected based on persistently elevated liver function tests, distal common bile duct dilatation in the absence of strictures, and an elevated resting bile duct pressure as measured by bedside T-tube manometry. Two different manometric patterns of SOD were observed. The first pattern (N = 4) consisted of elevated SO basal pressures, infrequent simultaneous phasic activity, and an abnormal response to cholecystokinin-octapeptide (CCK-OP). The second pattern (N = 1) consisted of low basal pressures and absent phasic activity. Four patients were successfully treated with papillotomy and stenting, while the fifth patient required conversion to a choledochojejunostomy because of a concomitant anastomotic stricture. The abnormal SO manometric profiles in patients suspected of having SOD after OLT were different from those observed in the nontransplant setting. Bedside T-tube manometry allowed measurement of the resting bile duct pressure and may be a useful screening tool for SOD.

Adult↗
PubMed1998

Renal dysfunction in liver cirrhosis: renal duplex Doppler US vs. scintigraphy for early identification.

A diagnostic tool to detect early renal dysfunction before it becomes irreversible would be useful in cirrhosis. This study was carried out to evaluate the role of Doppler sonography and Tc-99m DTPA renography in the detection of early renal dysfunction in patients with different grades of liver cirrhosis. Renal arteries of 43 patients with cirrhosis and normal renal function tests were compared with 15 age and gender matched normal subjects as a control group using colour Doppler sonography and Tc-99m DTPA scintigraphy. The patients were categorized into three groups, A (14), B (14) and C (15), according to a modified Child's classification that assesses the severity of liver cirrhosis. Doppler results revealed a highly significant increase in both the pulsatility and resistive indices in groups B and C compared with either group A patients or control subjects and in group C compared with group B (P < 0.001) in the main renal arteries as well as in the interlobar and arcuate arteries. Insignificant differences were observed between group A and controls (PI: control 0.96+/-0.08, group A 0.95+/-0.07, group B 1.26+/-0.06, group C 1.48+/-0.06; RI: control 0.57+/-0.02, group A 0.58+/-0.02, group B 0.66+/-0.01, group C 0.72+/-0.02). Abnormal renograms in the form of delayed appearance (34+/-14.6 s), diminished blood flow bilaterally with prolonged secretory (12+/-4.5 min) and excretory phases (> 30 min) and poor response to intravenous frusemide were only observed in group C patients. Radionuclide computed glomerular filtration rate was within the normal range in patients of group A (81+/-9.5 ml/min) and group B (78+/-8.4 ml/min) and reduced only in patients of group C (34+/-14.5 ml/min). Thus Doppler sonography can detect an increase in renal vascular resistance in patients with moderately severe cirrhosis (Child grade B) when renography was normal. We conclude that Doppler sonography can be used for earlier identification of cirrhotic patients with a higher risk of impending renal failure earlier than renography and may also be used to guide therapeutic approaches.

Adult↗
PubMed1990

Intrahepatic cholestasis due to hypersensitivity reaction to procainamide.

Hypersensitivity reactions to procainamide involving liver dysfunction are rare. We describe a patient who developed liver dysfunction after procainamide administration, manifested by fever, jaundice, elevated bilirubin concentration, and alkaline phosphatase concentration. Hepatobiliary scintigraphy demonstrated good hepatic uptake of the radionuclide without movement from hepatic parenchyma. To our knowledge, this is the first reported case of procainamide-induced intrahepatic cholestasis as demonstrated by radionuclide hepatobiliary scintigraphy.

Aged↗
PubMed1993

Effect of fasting on hepatocytes cold stored in University of Wisconsin solution for 24 hours.

Although there have been improvements in liver preservation, liver dysfunction still remains a serious consequence of liver transplantation. This may be related to cold ischemic injury since the incidence of dysfunction increases with longer preservation times. However, even some livers preserved for short periods of time (less than 15 hr) develop liver dysfunction. One possible cause may be the lack of adequate nutritional support, and the donor may be exposed to prolonged periods of hyponutrition. In this study, we have compared the effects of fasting on functions of hepatocytes isolated from the rat. Hepatocytes were cold stored in University of Wisconsin solution for 24 hr and analyzed at the end of preservation as well as at the end of rewarming in Krebs-Henseleit buffer for 120 min. The glycogen content of fed cells was 1.57 mumol/mg protein and this was reduced by 95% in cells from fasted rats. After cold storage and rewarming, hepatocytes from fasted rats lost 84.2 +/- 2.5% of the total cellular lactate dehydrogenase versus only 32.7 +/- 3.8% (P < 0.001) in cells from fed rats. Also, ATP and reduced glutathione content of fasted cells were significantly reduced, free fatty acids were higher (P = 0.0154), and protein synthesis was reduced to 41% of controls (versus only 88% in fed cells), although there were no differences in phospholipid content. When hepatocytes from fasted rats were rewarmed in Krebs-Henseleit buffer containing fructose (10 mM), lactate dehydrogenase release was reduced from 80% to 34.4 +/- 0.2% and ATP content was significantly higher with fructose than without. Hepatocytes from fasted rats, therefore, are more sensitive to cold ischemic injury than cells from fed rats. The increased sensitivity appears related to the lack of glycogen as a source of substrates for metabolism during rewarming. This is supported by the fact that addition of fructose, which is metabolized readily by hepatocytes through glycolysis, suppressed rewarming injury to cells from fasted rats. The nutritional status of the donor, therefore, may play a pivotal role in the results of liver preservation and transplantation. Effective donor nutritional management may reduce the incidence of liver dysfunction after transplantation.

Adenosine↗
PubMed2006

Prolonged use of dexmedetomidine in an infant with respiratory failure following living donor liver transplantation.

We used dexmedetomidine for more than 2 months in a mechanically ventilated infant without serious adverse effects. An infant with liver cirrhosis of unknown cause underwent living donor liver transplantation at the age of 9 months. Long-term mechanical ventilation was required postoperatively, and midazolam with fentanyl had been used to sedate the patient. They required increase to 1.7 mg.kg(-1).h(-1) and 3.5 microg.kg(-1).h(-1), respectively, which were still inadequate. On postoperative day 29, dexmedetomidine was added. The rate of dexmedetomidine infusion was increased gradually to 1.4 microg.kg(-1).h(-1). It was discontinued temporarily to exclude drug-induced liver dysfunction. However, without dexmedetomidine, adequate sedation level was unattainable. Liver dysfunction was likely to be attributed to cytomegalovirus infection and after restarting dexmedetomidine, the respiratory condition improved. He was extubated 10 weeks after the operation. Dexmedetomidine was successfully tapered off over the following 2 weeks with no signs of withdrawal. Dexmedetomidine was a useful sedative for an infant who required mechanical ventilation for a prolonged period of time.

Anesthesia↗
PubMed2002

Incomplete septal cirrhosis associated with Wegener's granulomatosis.

Incomplete septal cirrhosis, which is included in the spectrum of hepatoportal sclerosis, is characterized by parenchymal nodularity, incomplete fibrous septa, clustered or dispersed portal tract remnants, and abnormal spacing of portal tracts and hepatic veins. Hepatoportal sclerosis is known to be associated with collagen vascular diseases. Here, we describe a 73 year-old-female with incomplete septal cirrhosis. At 57 years, she presented with respiratory symptoms, and lung biopsy disclosed active arteritis with granuloma. Perinuclear antineutrophilic cytoplasmic antibody was also positive. Immunosuppressive therapy was done under the diagnosis of Wegener's granulomatosis. At 63 years, liver dysfunction was noted, and laparoscopy revealed uneven surface of the liver and dilatation of the umbilical vein. Liver dysfunction progressed, and she developed encephalopathy and massive ascites. She died of sepsis at 73 years. At autopsy the liver (700 g) was macronodular with several deep depressions. The parenchyma showed fine and diffuse nodularity. Grossly visible portal and hepatic veins were patent. The above-mentioned histologic features characterizing incomplete septal cirrhosis were found. This is the first report of incomplete septal cirrhosis associated with Wegener's granulomatosis implying that vascular and extravascular lesions of Wegener's granulomatosis might have been related to the pathogenesis of incomplete septal cirrhosis.

Arteries↗
PubMedDate not supplied

Intravenous nutrition and hepatic dysfunction.

Abnormalities in biochemical liver function tests in 127 general surgical patients who had a course of intravenous nutrition have been reviewed. Only 26 patients had liver function tests considered to be normal on commencing intravenous nutrition and they were included in this retrospective study. During intravenous nutrition the most sensitive biochemical test of liver dysfunction was gamma-glutamyl transpeptidase--all patients having an elevated gamma-glutamyl transpeptidase level by week 4. Most abnormalities were transient whereas the elevation of alkaline phosphatase was prolonged beyond week 9. Patients with major sepsis were found to have almost double the incidence of abnormal liver function test values compared with patients with no evidence of sepsis. Only patients who were transfused more than 8 units of blood showed a significant rise in bilirubin. Liver function tests in patients who received smaller transfusions showed no difference from patients who did not receive any blood. Patients with below normal anthropometric measurements on commencing intravenous nutrition were more likely to develop abnormalities in aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and gamma-glutamyl transpeptidase.

Alanine Transaminase↗