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Effects of isolated small bowel transplantation on liver dysfunction caused by intestinal failure and long-term total parenteral nutrition.

It has not been fully determined whether isolated small bowel transplantation (ISBTx) can reverse liver dysfunction caused by intestinal failure requiring long-term total parenteral nutrition (TPN). A boy with congenital microvillus inclusion disease presented with vomiting and severe diarrhea since the first day of life and had been managed by TPN since then. He suffered from catheter-related sepsis several times. At 14 yr of age he developed progressive hepatosplenomegaly with thrombocytopenia and coagulopathy. He underwent ISBTx with an ileal graft from his blood-identical grandmother at the age of 16 yr. Oral feeding was started on the 14th day after ISBTx and gradually increased. TPN was completely withdrawn after 5 months. Liver was palpated 5 cm below the costal margin before ISBTx, while it became non-palpable 5 months after ISBTx. Serum liver enzyme levels and prothrombin time normalized in the 5 months following ISBTx. Liver biopsy showed marked steatosis, slight cholestasis, and mild bridging fibrosis before ISBTx. Although histological examination of liver biopsy revealed complete disappearance of steatosis 7 and 11 months after ISBTx, liver fibrosis remained unchanged. This clinical experience has shown that although steatosis and cholestasis are reversible after successful ISBTx and withdrawal of TPN, liver fibrosis may remain unchanged.

Adolescent↗

Microscopic polyangiitis that presented liver dysfunction prior to noted renal manifestations.

In microscopic polyangiitis (MPA), renal manifestations are very common as first symptoms. Here, we report a case of MPA which presented liver dysfunction prior to noted renal manifestations. A 58-year-old woman was hospitalized because of a fever for 8 weeks. A laboratory examination revealed marked elevation of alkaline phosphatase and gamma-glutamyl transpeptidase, while blood urea nitrogen and creatinine levels remained normal. Although apparent renal dysfunction developed in this case soon after hospitalization, physicians should be aware of the variety of clinical manifestations in MPA. Moreover, antineutrophil cytoplasmic autoantibodies were found to be helpful for diagnosing MPA.

Female↗

Does the use of insulin in a patient with liver dysfunction increase water retention in the body, i.e. cause insulin oedema?

A 68-year-old female with mild diabetes mellitus was admitted because of acute liver dysfunction due to autoimmune hepatitis. While 40 mg/day of prednisolone improved hepatic dysfunction dramatically, her diabetic milieu deteriorated seriously. The induced hyperglycaemia could not be controlled sufficiently, despite a high dose of insulin (> 110 units/day), suggesting the existence of insulin insensitivity and hyperinsulinaemia. Soon after introduction of insulin therapy, she developed severe anasarca, including marked peripheral oedema, ascites and pleural effusion. Anasarca eventually subsided within 4 weeks with the use of a diuretic agent. We conjectured that the side effects of insulin, such as anti-natriuresis and increased vascular permeability, might be pronounced in the presence of the hepatic dysfunction that accompanies insulin insensitivity, hyperinsulinaemia and hypoalbuminaemia.

Aged↗

[Successful combined therapy with ATG, cyclosporin and G-CSF for both liver dysfunction and bone marrow failure in hepatitis-associated aplastic anemia].

A 28-year-old man developed cryptogenic hepatitis in January 1999, and treatment with glycyrrhizic acid improved his liver function. From June, however, pancytopenia began to develop gradually. The patient received G-CSF against leukocytopenia (WBC 1,100/microliter, neutrophils 590/microliter) and was transferred to our hospital in August 1999. A diagnosis of hepatitis-associated aplastic anemia was made on the basis of liver dysfunction (AST 156 IU/l, ALT 386 IU/l), hypoplastic bone marrow, and pancytopenia (WBC 4,400/microliter, neutrophils 3,340/microliter under G-CSF administration, Hb 9.8 g/dl, platelets 2.4 x 10(4)/microliter, reticulocytes 4.7 x 10(4)/microliter). Immediately after starting combined therapy with ATG, cyclosporin, and G-CSF, his liver function began to improve and was normalized on day 7. Pancytopenia began to ameliorate on day 9, and blood parameters on day 60 were WBC 4,200/microliter (without G-CSF administration), Hb 12.0 g/dl, platelets 9.0 x 10(4)/microliter, and reticulocytes 4.1 x 10(4)/microliter. Although the prognosis of hepatitis-associated aplastic anemia is generally poor, immunosuppressive therapy was markedly effective for both pancytopenia and hepatic dysfunction in the present case.

Adult↗

VAB-6 chemotherapy causes spurious elevation of alpha-fetoprotein associated with liver dysfunctions.

In order to elucidate the mechanism of elevation of alpha-fetoprotein (AFP) which we often observed during VAB-6 chemotherapy, we analyzed sequential changes of AFP, liver enzymes and bilirubin in 10 patients with evaluable disseminated testicular cancer who were treated with VAB-6 chemotherapy. None of the patients had previous liver disease or hepatic involvement. During the early phase of each course of chemotherapy, AFP showed a temporary elevation associated with reversible increase in liver enzymes and bilirubin. These changes returned to normal before the next course of chemotherapy. In each patient, marked tumor regression occurred as a result of VAB-6 chemotherapy. Nine of the 10 patients remain free of disease after treatment. We conclude that during VAB-6 chemotherapy, a temporary elevation of AFP is common, associated with reversible liver dysfunctions, and that this spurious elevation of the tumor marker, most likely caused by a heavy dose of cisplatin, should not be interpreted as related treatment failure.

Adult↗

The prevalence of gallstones in chronic liver disease is related to degree of liver dysfunction.

BACKGROUND/AIMS: Earlier studies have indicated an elevated risk for gallstone disease in patients with cirrhosis. This study aimed to evaluate the prevalence of gallstones in patients with chronic liver disease (CLD) with respect to sex, etiology, and severity of liver disease. METHODOLOGY: Four hundred and thirteen adults (176 women), mean age 51.2+/-14 years, with CLD, who had undergone liver biopsy during 1978-1993, and from whom sera were available, were investigated retrospectively. The results were compared with a population-based ultrasonography study of 556 healthy men and women, in their 40s and 60s. RESULTS: The prevalence of gallstones in patients with CLD did not differ from that in the control population. An increased frequency was observed in patients with CLD initially classified as cryptogenic, of whom the majority (60%) later were reclassified as chronic hepatitis C. The frequency of gallstones was also high in PiZ-heterozygotes for alpha1-antitrypsin deficiency (5/21, 24%) compared to non-PiZ-carriers (17/389, 4.8%), (p<0.001). In 67 patients with histologic evidence of cirrhosis, 30% (20/67) had gallstones (vs. 15% in the general population, p<0.01). The prevalence of gallstones increased significantly from Child's class A (16%) to C (56.2%). The difference was significant in males (18.2% vs. 62.5%, p=0.033), but not in females. Fifty percent of the patients with gallstones were symptomatic. CONCLUSIONS: Progressive liver dysfunction is a risk factor for gallstones particularly in males. HCV infection and PiZ carriership may further increase biliary lithogenesis.

Adult↗

Generalized eruption with severe liver dysfunction associated with occupational exposure to trichloroethylene.

Generalized dermatitis due to trichloroethylene is rare. A 21-year-old printer developed exfoliative dermatitis with mucous membrane involvement, fever and liver dysfunction after a 2-week occupational exposure to trichloroethylene. Positive patch-test reactions to trichloroethylene and to one of its metabolites, trichloroethanol, were observed. This dermatitis is considered to be mediated by a delayed-type hypersensitivity mechanism.

Adult↗

Pregnancy-associated severe liver dysfunction.

Determining the cause of liver disease in pregnancy can present a difficult challenge for clinicians. Minor elevations in aminotransferases may be a harbinger of life-threatening processes, such as acute fatty liver of pregnancy (AFLP) or hemolysis, elevated liver enzyme levels, low platelet count (HELLP) syndrome. Preeclampsia, HELLP syndrome, and AFLP form a spectrum of disease that ranges from involving mild symptoms to severe life-threatening multiorgan system dysfunction. They have been shown to be the primary causes of severe hepatic dysfunction during pregnancy. This article attempts to define the clinical and diagnostic features, pathophysiology, and treatment options of these diseases.

Fatty Liver↗

[Statistical analysis of liver dysfunction in patients with pulmonary tuberculosis using the data base control system].

A program HEPATOX has been devised for the study of the frequency of hepatic dysfunction signs within the system of data base control FoxPro. The analysis of the data available for 377 tuberculous patients showed a more important role of the laboratory diagnosis compared to clinical symptoms, a predisposing role of prior hepatitides and active inflammation in the onset of hepatic dysfunction in conditions of on-going chemotherapy. The authors present the frequency spectrum for clinical and laboratory findings from tuberculous in patients with liver dysfunctions. The efficacy of computers in phthisiopulmonology is demonstrated.

Adult↗

[Hepatopulmonary syndrome: relationship with liver dysfunction and systemic hemodynamic disorder].

BACKGROUND AND OBJECTIVE: The hepatopulmonary syndrome (HPS) causes an increased alveolar to arterial gradient of oxygen and in advanced phases hypoxemia, as the result of pulmonary vasodilation. In liver cirrhosis, it has been demonstrated the existence of splachnic vasodilation and also in other vascular beds. Our main objectives were to know the hemodynamic status, the renal function and the condition of some humoral systems in patient diagnosed of HPS. PATIENTS AND METHOD: We studied consecutively 32 cirrhotic patients Divided in two groups, a group of 18 cirrhotic patients with normal gaseous exchange (NGE), and another group of 14 cirrhotic patients diagnosed of HPS by contrast-enhanced transthoracic echocardiography and/or lung and brain scintigraphy with 99Tc albumin macroaggregates. They were all in rest in bed, upon alcohol and tobacco abstinence and on a diet of 50 mEq of sodium. Cardiovascular drugs were all withheld during 4 days in order to reach steady state. RESULTS: Patients of the HPS group were characterized by a more advanced index of Child-Pugh and presence of clubbing and vascular spiders. They presented a greater degree of hypoxemia in a sitting position, greater hypocapnia and smaller transference factor values (TLCO). They also showed a hyperkinetic circulatory condition characterized by smaller arterial blood pressure, greater cardiac index, smaller vascular resistances and greater femoral flows, with smaller clearance of creatinine, elimination of urinary sodium, urinary volume/24 h and an increased plasmatic volume, accompanied with a greater activation of the renin-angiotensin-aldosterone axis and a greater urinary elimination of nitrites and nitrates. CONCLUSIONS: The pulmonary vasodilation that explains the HPS is a constitutive part of the systemic vasodilation occurring in liver cirrhosis, and it is related to the degree of liver dysfunction as measured by the classification of Child-Pugh. The greater activation of the renin-aldosterone system and the rise of the plasmatic volume express a highest grade of arterial underfilling caused by an increment in the nitric oxide production.

Female↗

Presence of endotoxemia and its relationship to liver dysfunction in patients with typhoid fever.

Twenty-one patients with typhoid fever were studied to evaluate the presence of endotoxin in peripheral blood and its relationship to the incidence and features of hepatic dysfunction which may occur during this disease. The limulus test for endotoxin was positive in the plasma samples of all patients prior to treatment. Liver dysfunction, as assessed by fasting and postprandial serum bile acid levels and by standard biochemical tests, occurred in 90% of patients. In seven, the injury was purely cholestatic (elevation of postprandial serum bile acid levels, alone); in 12, it was of mixed cholestatic-hepatocellular type (elevation of both serum bile acids and aminotransferase levels). After recovering, the limulus test was negative and liver function tests returned to normal values in all patients. The results demonstrate that endotoxemia is present in patients with typhoid fever. In addition, since endotoxin can impair bile secretion, our results suggest that endotoxin may have a pathogenetic role in the development of liver injury during typhoid fever.

Adolescent↗

Ursodeoxycholic acid treatment of idiopathic thrombocytopenic purpura with liver dysfunction.

Ursodeoxycholic acid (UDCA) is known to reduce immunoglobulin from B cells and cytokine production from T cells. We found that UDCA increased the platelet count in two idiopathic thrombocytopenic purpura (ITP) patients who have liver dysfunction. UDCA was tolerated and did not cause diarrhea in the amounts used. Further investigation is needed to evaluate the effectiveness of UDCA in ITP patients.

Adult↗

Detection of hepatitis G virus from serum and liver of a patient with long-term liver dysfunction after autologous bone marrow transplantation.

Long-term effects after blood or bone marrow transplantation (BMT) are emerging as an important issue, as more patients are included in BMT programmes and as this procedure becomes more successful. Long-term liver dysfunction, mainly due to chronic graft-versus-host disease or hepatitis C virus infection, is a well-known complication. Nevertheless, the diagnosis of liver disease in this patient group is sometimes difficult and, despite adequate studies, it may remain undetected. A novel hepatitis-associated virus, hepatitis G virus (HGV), has recently been identified. The virus belongs to the Flaviviridae family and is known to be parenterally transmitted, although there is no clear evidence to implicate this agent in causing acute or chronic hepatitis. We report a patient who developed mild, but persistent, abnormalities in transaminases for 2 years after an autologous BMT. HGV RNA was detected in both serum and liver. HGV RNA persisted in serum for at least 8 months. No other known hepatitis virus was found. This report provides the first direct evidence of a patient with long-term liver abnormalities after a BMT in whom the only known hepatitis virus isolated was the HGV.

Adult↗

Diagnosis by routine scintigraphy of hepatic reticuloendothelial failure before severe liver dysfunction.

OBJECTIVES: The prognosis of hepatic reticuloendothelial failure is said to be poor. Scanning with the radiocolloid 99mTc phytate is needed for diagnosis; as a rule; only seriously ill patients are so investigated. We use 99mTc phytate for liver scans of almost all inpatients with liver disease. This routine made diagnosis of a mild form or early stage of the disease possible. We evaluated the clinical findings of the five patients we have diagnosed, in an attempt to find why four survived. METHODS: Radiocolloid scans were taken starting 20-30 min after the intravenous injection of 111 MBq of 99mTc phytate. Hepatobiliary images were taken by use of 99mTc pyridoxylidine-5-methyl trytophan, and hepatic receptor images were taken by use of 99mTc-labeled diethylenetriaminepentaacetic acid coupled with galactosyl human serum albumin. RESULTS: The livers were not visible in the radiocolloid scans, so the diagnosis of hepatic reticuloendothelial failure was considered. In the two other imaging examinations, the livers were visible. The cause was identified as heavy alcohol intake in four cases and toluene hepatotoxicity in one case. Histological examinations showed cirrhosis in two patients; the three other patients did not have cirrhosis. All five patients had anemia, and three had infections. One patient died of multiple organ failure, and the four other patients survived. Long-term observation by radiocolloid scanning was possible in one patient in whom radionuclide uptake into the liver rose as hepatic function improved. CONCLUSIONS: This disorder is associated with a temporary decrease in Kupffer cell function and hence is liable to be complicated by infection, which can result in death. If the cause is removed promptly, recovery is likely. There being a mild form of this disease, previously not generally diagnosed, probably accounts for the outcomes being good in all of our patients except the one patient with severe liver dysfunction at the time of diagnosis.

Adult↗

Analysis of human cytomegalovirus-infected peripheral blood mononuclear cells from infants with liver dysfunction by flow cytometry and the polymerase chain reaction.

Perinatal human cytomegalovirus (HCMV) infection often involves the hepatobiliary tract, but infected individuals usually remain asymptomatic. We investigated the role of CD8+ lymphocytes in 13 infants with liver dysfunction associated with perinatal HCMV infection. In three patients more than 40% of CD8+ cells were positive for HCMV immediate early antigen (IEA) and late antigen (LA) by flow cytometry after selection of T lymphocytes subpopulations. In the other 10 infants, 20% to 30% of CD8+ cells were positive for HCMV IEA and LA. HCMV IE DNA was detected in CD8+ cells from one infant, in CD4+ cells from one infant, and in both CD4+ and CD8+ cells from three infants. HCMV infection of CD8+ cells may play an important role in the process of perinatal primary infection.

Antigens, Viral↗

[A case of severe Legionnaires' disease complicated by rhabdomyolysis, acute renal failure, liver dysfunction and encephalopathy].

A 67-year-old male was admitted to our hospital due to a high fever with abnormal shadows on chest X-ray films. On admission, his laboratory data showed hyponatremia, rhabdomyolysis and liver dysfunction. Encephalopathy, acute renal failure and respiratory failure developed, despite fluid management and antimicrobial therapy. His condition worsened rapidly in a few days enough to require mechanical ventilation. Legionnaires' disease was suspected, because pneumonia was found to be associated with multiple organ dysfunction. Intravenous erythromycin and methylprednisolone were administered. The patient's condition was rapidly improved, although he needed hemodialysis for 30 days. Later, indirect fluorescent antibody testing of the patient's serum against Legionella pneumophila was definitely positive (1:1024). We reported the first case of severe Legionnaires' disease in Miyazaki Prefecture, Japan.

Acute Kidney Injury↗