1/318. Phantom steatosis of the liver: report of a case.A patient, referred under a diagnosis of metastatic liver tumors, was found to have multiple areas of focal fatty change (FFC) which, during follow-up, exhibited discordant evolutions. To our knowledge, this phenomenon-regression of a FFC lesion with concurrent appearance or progression of other similar lesions in the same patient, has been reported in only one previous case. FFC can be strongly suggested by clinical, biochemical and radiologic criteria. However, an exact diagnosis can only be made with biopsy. To avoid misdiagnosing a malignancy as FFC and vice versa, biopsy should be performed without hesitation in all patients in whom a change in approach is possible.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
2/318. Successful liver transplantation using an 87 year-old donor.The increasing number of patients listed for liver transplantation and the persistent low number of potential donors have led, in recent years, to the expansion of organ availability, as is the case of liver procurement from elderly donors. So far, results reported in the literature are controversial and only a few reports described satisfactory outcomes of transplantation using donors older than 60 years. In the case presented herein, we report the successful elective liver transplantation of a 65 year-old patient, who received a graft from an 87 year-old donor, whose well-preserved hemodynamic conditions, normal hepatic function, absence of parenchymal alterations, as well as short ischemia time, were the key points for this good outcome. Even very old donors can be suitable for liver transplantation. A careful evaluation of all the possible donor-related risk factors, mostly the presence of steatotic changes at liver biopsy, is always mandatory.- - - - - - - - - - ranking = 1.8keywords = liver (Clic here for more details about this article) |
3/318. Late recurrence and rapid evolution of severe hepatopulmonary syndrome after liver transplantation.recurrence of hepatopulmonary syndrome (HPS) after orthotopic liver transplantation (OLT) in an adult has never been reported. We describe a 23-year-old woman who initially underwent OLT because of debilitating and severe HPS associated with nonalcoholic steatohepatitis (NASH). Although the clinical resolution of HPS was well documented day 117 post-OLT, the reappearance of NASH was documented by liver biopsy. Severe hypoxemia because of recurrent HPS rapidly evolved beginning approximately day 700 post-OLT. Retransplantation was attempted, but the patient died post-OLT of sepsis and/or multiorgan failure.- - - - - - - - - - ranking = 1.2keywords = liver (Clic here for more details about this article) |
4/318. Microvesicular steatosis, hemosiderosis and rapid development of liver cirrhosis in a patient with Pearson's syndrome.BACKGROUND/AIMS: Pearson's marrow-pancreas syndrome consists of refractory sideroblastic anemia with vacuolization of marrow precursors and exocrine pancreas dysfunction. patients with this disease usually have large deletions of the mitochondrial genome. We report a patient with Pearson's syndrome who had predominantly hepatic manifestations such as microvesicular steatosis, hemosiderosis and rapidly developing cirrhosis. methods: Analysis of the mitochondrial and nuclear genomes, determination of enzyme activities and of the hepatic iron content were performed using standard techniques of molecular biology and biochemistry. RESULTS: The patient had typical ringed sideroblasts in a bone marrow smear and a 7436-bp deletion of the mitochondrial genome in all tissues investigated, compatible with Pearson's syndrome. He died within 3 months after birth due to liver failure. Histopathological analysis of the liver revealed complete cirrhosis with signs of chronic cholestasis, microvesicular steatosis and massive hemosiderosis. In addition, the patient was heterozygous for the C282Y and H63D mutations of the hemochromatosis gene. CONCLUSIONS: Pearson's syndrome should be added to the list of neonatal diseases which can cause microvesicular steatosis, hepatic accumulation of iron and liver cirrhosis.- - - - - - - - - - ranking = 1.4keywords = liver (Clic here for more details about this article) |
5/318. Long-chain 3-hydroxyacyl-coa dehydrogenase deficiency: variable expressivity of maternal illness during pregnancy and unusual presentation with infantile cholestasis and hypocalcaemia.patients with long-chain 3-hydroxyacyl-coa dehydrogenase (LCHAD) deficiency present with a Reye-like syndrome, cardiomyopathy, or sudden unexpected death. We describe an unusual presentation in a patient with unsuspected LCHAD deficiency. The proband presented at 2 months of age with an acute infantile hypocalcaemia and vitamin d deficiency associated with occult, unexplained cholestatic liver disease. Sudden, unexpected death occurred at 8 months. Molecular analysis revealed homozygosity for the prevalent LCHAD (1528G > C, E474Q) mutation. The mother had pre-eclampsia during the third trimester of her pregnancy. In a subsequent pregnancy, she developed severe acute fatty liver of pregnancy (AFLP) and intrauterine fetal death at 33 weeks of gestation. In conclusion, infantile hypocalcaemia is an unusual phenotype associated with LCHAD deficiency. The maternal pregnancy history documents that fetal LCHAD deficiency is associated with a spectrum of maternal illnesses ranging from pre-eclampsia to life-threatening AFLP.- - - - - - - - - - ranking = 0.4keywords = liver (Clic here for more details about this article) |
6/318. Cytogenetic studies in a patient with acute granulocytic leukemia of seven and one-half years duration.During a 7 1/2-yr period we monitored a chromosomally aberrant cell line in a woman with acute granulocytic leukemia (AGL) whose disease followed a rather unusual course. Her initial remission induced with 6-mercaptopurine (6-MP) and prednisone was maintained for 52 mo with biweekly doses of methotrexate (MTX) given orally. Because signs of liver dysfunction occurred, maintenance therapy was stopped. After 15 mo without chemotherapy, she suffered her first relapse (5 yr 7 mo after the initial diagnosis). A second remission, again induced with 6-MP and prednisone, was maintained for 1 yr, after which a second relapse occurred. Another remission lasting for only 4 mo was followed by a relapse of the leukemic process which led to her death. Cytogenetic studies of marrow cells and peripheral blood at the time of her initial diagnosis showed abnormal stem lines with characteristic chromosome markers. A small percentage of malignant cells bearing these markers persisted in her marrow during the years of her prolonged remission. At the time of her first relapse, 75% of her marrow cells had the marker karyotype, and at the time of her death (7 1/2 yr after the leukemia was diagnosed) all analyzable marrow metaphases had the characteristic chromosome changes.- - - - - - - - - - ranking = 0.2keywords = liver (Clic here for more details about this article) |
7/318. Acute jaundice in pregnancy: acute fatty liver or acute viral hepatitis?In this case, the difficulty in differential diagnosis between acute viral hepatitis and acute fatty liver of pregnancy was analyzed. These 2 conditions often raise controversal question regarding the decision making on emergency anesthesia for cesarean section to avert complications and optimize management. The dilemma in which an anesthesiologist is put is whether to promise the anesthesia straightaway in the face of a demonstrable acute jaundice in pregnancy to advise a postponement of surgery until a turn for the better. In this embarrassing situation, the authors suggest that a postpronement of surgery is rational to observe the development during which both the mother and the fetus should be closely monitored. Once the necessity of a cesarean section outweighs the benefit of transitional conservative treatment, it should be performed immediately.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
8/318. Hepatic carnitine palmitoyltransferase I deficiency presenting as maternal illness in pregnancy.The spectrum of clinical presentation of fatty acid oxidation defects (FAOD) continues to expand. One FAOD, L-3-hydroxyacyl-coa dehydrogenase (LCHAD) deficiency has been associated with liver disease in pregnancies involving a heterozygous mother carrying an affected fetus. Hepatic carnitine palmitoyltransferase (CPT I) deficiency typically presents as a Reyelike syndrome in children between 8 and 18 mo. of age. We have investigated a family in which the mother developed liver disease consistent with acute fatty liver of pregnancy (AFLP) and hyperemesis gravidarum in her two successive pregnancies. Neither child nor their mother was found to carry the common LCHAD G1528C mutation. Both children were subsequently shown to have absent activity of CPT I. This is the first report of CPT I deficiency presenting as maternal illness in pregnancy.- - - - - - - - - - ranking = 0.6keywords = liver (Clic here for more details about this article) |
9/318. Pseudolesion in segment IV of the liver with focal fatty deposition caused by the parabiliary venous drainage.A case with a pseudolesion associated with focal fatty deposition in segment IV of the liver observed on conventional CT and CT during arterial portography caused by the parabiliary venous drainage is presented. Close observation of the common hepatic angiography was helpful to recognize this unusual vessel as a cause of this pseudolesion. Selective catheterization of the posterior superior pancreaticoduodenal artery and CT during its venous phase confirmed the etiology of the pseudolesion.- - - - - - - - - - ranking = 1keywords = liver (Clic here for more details about this article) |
10/318. Lactic acidosis and hepatic steatosis associated with use of stavudine: report of four cases.BACKGROUND: An association between use of zidovudine and didanosine and a rare but life-threatening syndrome of hepatic steatosis, lactic acidosis, and myopathy has been reported. OBJECTIVE: To describe the syndrome of hepatic steatosis, lactic acidosis, and myopathy in four patients taking stavudine. DESIGN: Case series. SETTING: A community hospital in washington, D.C., and National Institutes of health Clinical Center, Bethesda, maryland. patients: Two men and two women with hiv-1 infection who were taking stavudine presented with lactic acidosis and elevated levels of aminotransferases. All patients required intensive care. MEASUREMENTS: Levels of lactic acid, alanine aminotransferase, aspartate aminotransferase, amylase, and lipase; computed tomography of the abdomen; liver biopsy (two patients); and muscle biopsy (two patients). RESULTS: Histologic findings consistent with mitochondrial injury confirmed the diagnosis of hepatic or muscle abnormality. CONCLUSION: Because hepatic steatosis may be life-threatening, physicians should consider it as a possible cause of elevated hepatic aminotransferase levels among patients taking stavudine.- - - - - - - - - - ranking = 0.2keywords = liver (Clic here for more details about this article) |
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