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Query: UMLS:C0015695 (fatty liver)
 13,941 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients presenting with acute fatty liver of pregnancy were studied. Because of similarities between acute fatty liver of pregnancy and Reye's syndrome, we investigated hepatic ultrastructure, urea-cycle enzyme activities, and plasma amino acids. Initial liver biopsies obtained 12 and 21 days after the onset of illness demonstrated microvesicular fat deposition and mitochondrial ultrastructural changes, including pleomorphism and abundant crystalline inclusions. In both biopsies, activity of the mitochondrial urea-cycle enzyme OTC was markedly below normal limits. Activity of the other mitochondrial urea-cycle enzyme, CPS, was low in one patient. Abnormalities of these enzymes persisted in second biopsies obtained at 9 and 28 weeks, respectively. By 44 weeks all urea-cycle enzyme activities had returned to normal in one patient. However, in the other patient OTC activity was still reduced at 52 weeks, although it had doubled in comparison to previous biopsies. Morphological changes of the mitochondria generally improved in parallel with the urea-cycle enzymes. Plasma amino acids, obtained at the time of the initial biopsies, demonstrated a generalized hypoaminoacidemia with the exception of glutamate. Serial observations in patients with this rare disease indicate that there are similarities with Reye's syndrome, in particular, reduced activity of the mitochondrial urea-cycle enzymes. But there are important differences. (1) Enzymatic and ultrastructural abnormalities of mitochondria persist for a longer period of time than in Reye's syndrome. (2) Mitochondrial ultrastructure is different. (3) Plasma amino acid profiles are different.
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PMID:Abnormalities of hepatic mitochondrial urea-cycle enzyme activities and hepatic ultrastructure in acute fatty liver of pregnancy. 46 76

Liver diseases in pregnancy can occur as a complication of gestation or independently. Acute fatty liver of pregnancy is a rare disease of unknown etiology specifically associated to gestation. The frequency is increased in the first pregnancy, in the last trimester and in multiple pregnancies. Because of the high maternal and perinatal mortality early diagnosis is necessary for prompt termination of pregnancy. This case report is intended to facilitate the differentiation between acute fatty liver of pregnancy and other liver diseases in pregnancy. The differential diagnoses include preeclampsia, HELLP syndrome, intrahepatic cholestasis of pregnancy and acute viral hepatitis.
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PMID:[Acute fatty liver of pregnancy]. 154 19

A case is reported of acute fatty liver of pregnancy with maternal and foetal survival inspite of jaundice, encephalopathy, renal failure, gastro-intestinal haemorrhage and serious coagulation problems. The acute fatty liver is a rare disease of unknown aetiology which occurs during the third trimester of pregnancy. Early diagnosis based on liver biopsy obtained by the transjugular route, immediate delivery of the foetus and intensive care improve an otherwise fatal prognosis.
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PMID:[Acute fatty liver of pregnancy]. 381 47

Six further cases of acute fatty liver of pregnancy are presented and discussed in relation to the world literature on this rare disease of pregnancy, the aetiology of which is still unknown. The importance of vomiting in late pregnancy is emphasized. A common feature seems to be a metabolic stress, possibly related to infection, which tips a susceptible liver into metabolic failure.
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PMID:Acute fatty liver of pregnancy. 688 87

Acute fatty liver of pregnancy is a rare disease which may be letal if diagnosis is missed. The pathogenesis is not completely clear, but there is some evidence that some cases have been associated with a genetic deficiency of fatty acid beta-oxidation. Other predisposing factors include primiparity, multiple pregnancy, male fetal sex and pre-eclampsia. Clinical presentation and laboratory findings are often unspecific. Increasing serum aminotransferases are characteristic in the early stage of the disease. Liver biopsy establishes the diagnosis and typically shows microvesicular, centrilobular fatty changes of hepatocytes. Differential diagnosis includes the HELLP-Syndrome, cholestasis of pregnancy, pre-eclampsia and viral or drug induced hepatitis. Without adequate treatment liver failure with coagulopathy and encephalopathy may develop. Two cases of acute fatty liver in pregnancy in an early stage are presented. Clinical and histopathological findings as well as diagnostic and therapeutic procedures are discussed.
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PMID:[Acute fatty liver in pregnancy: clinical and histopathological course. Case report]. 1188 62

Steatosis of the liver, demonstrating itself as the acute liver failure during the third trimester of pregnancy (Acute Fatty Liver of Pregnancy--AFLP) is a rarely observed liver pathology. Herewith we describe the case of a 19-years old pregnant patient with this rare disease. The authors present the clinical course, dynamics, diagnostics, and outcome of treatment in this unique case. In this patient the ultimate treatment chosen was the liver transplantation. The opinions, concerning liver transplantation in AFLP (expressed in the world literature) are somewhat controversial. This paper presents the first case of AFLP treatment with orthotopic liver transplantation (OLTx) in Poland.
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PMID:Orthotopic liver transplantation for acute liver failure resulting from "acute fatty liver of pregnancy". 1511 33

Pyomyositis is an acute bacterial infection manifesting as pyemic abscess formation in the skeletal muscles. We examined 8 autopsy cases (seven males, one female; age range 21-75 years) of fatal nontropical pyomyositis to better describe individual case characteristics and pathologic features of this rare disease. The pathogen most frequently involved was Staphylococcus aureus. In most cases, there were several abscesses and multiple sites involved. The trunk, shoulder girdle, and thigh muscles were most frequently affected and involvement of multiple sites was a common finding. In 6 cases, a recent trauma had occurred to the anatomic location where the pyemic abscesses were found. Three deceased were known as intravenous drug abusers. Except for the presence of pyomyositis, liver diseases such as cirrhosis in 3 cases, and a fatty liver in 2 cases were the most frequent autopsy findings. Death was due to sepsis in all cases. Because pyomyositis may develop in association with intravenous catheterization in the clinical setting, the question whether pyomyositis was caused by an infected or improperly placed indwelling intravenous catheter may be of forensic importance in the light of alleged medical malpractice. According to our observations, severe underlying illnesses seem not always necessary for fatal outcome of pyomyositis. Because a detailed dissection of superficial as well as deep skeletal muscles during autopsy is a prerequisite for the diagnosis, the disease may be overlooked when this essential step is not performed.
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PMID:Fatal pyomyositis: a report of 8 autopsy cases. 1852 Apr 79

Insulin resistance is a major factor in the pathogenesis of type 2 diabetes and underpins the strong association between obesity and diabetes. Paradoxically, the metabolic consequences of having 'too much' fat (obesity) are remarkably similar to those of having 'too little' fat (lipodystrophy): a finding that has generated considerable interest in a rare disease. In both cases, excess energy accumulates as lipid in ectopic sites such as the liver (fatty liver) and skeletal muscle, where it plays a central role in the pathogenesis of insulin resistance, dyslipidemia and type 2 diabetes. Human lipodystrophies are characterised by a total or partial deficiency of body fat, and may be inherited or acquired in origin. Genetically engineered mice with generalised lipodystrophy manifest many of the features of the human disorder, including hyperphagia, fatty liver, hypertriglyceridaemia, insulin resistance and type 2 diabetes, providing a useful tractable model of the human disorder. Partial lipodystrophy, which causes similar, albeit milder, metabolic problems in humans has been more difficult to mimic in the mouse. This review discusses key translational studies in mice with generalised lipodystrophy, including fat transplantation and the use of recombinant leptin replacement therapy. These studies have been instrumental in advancing our understanding of the underlying molecular pathogenesis of ectopic lipid accumulation and insulin resistance, and have prompted the initiation and subsequent adoption of leptin replacement therapy in human lipodystrophies. This review also considers the possible reasons for the apparent difficulties in generating mouse models of partial lipodystrophy, such as interspecies differences in the distribution of fat depots and the apparent lack of sexual dimorphism in fat mass and distribution in mice compared with the dramatic differences present in adult humans.
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PMID:Mouse models of inherited lipodystrophy. 1989 86

Primary hepatic capillary hemangioma in adults is very rare. Here, we report a case of hepatic capillary hemangioma in a 71-year-old woman. She had abnormal liver function tests, and abdominal sonography revealed a 2-cm nodular lesion and fatty liver. Computed tomography scan revealed a hypervascular tumor. During 2 years' follow-up, the hepatic tumor enlarged to 3 cm in diameter. Serological tests showed no evidence of chronic viral hepatitis or increased level of alpha-fetoprotein. In fear of hepatocellular carcinoma, she received atypical hepatectomy. Microscopically, the tumor turned out to be a capillary hemangioma in a background of steatohepatitis. We searched the literature, and only six similar cases were found. We made a brief review of this rare disease entity and described its clinicopathological features.
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PMID:Adult capillary hemangioma of the liver: case report and literature review. 2180 46

Acute fatty liver of pregnancy is a rare disease that affects women in the third trimester of pregnancy. Although infrequent, the disease can cause maternal mortality. The diagnosis is not always clear until the pregnancy is terminated, and significant complications, such as acute pancreatitis, can occur. Pancreatic involvement typically only occurs in severe cases after the development of hepatic and renal impairment. To date, little knowledge is available regarding how the disease causes pancreatitis. Treatment involves supportive measures and pregnancy interruption. In this report, we describe a case of a previously healthy 26-year-old woman at a gestational age of 27 wk and 6 d who was admitted with severe abdominal pain and vomiting. This case illustrates the clinical and laboratory overlap between acute fatty liver of pregnancy and pancreatitis, highlighting the difficulties in differentiating each disease. Furthermore, the hypothesis for this overlapping is presented, and the therapeutic options are discussed.
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PMID:Acute fatty liver of pregnancy associated with severe acute pancreatitis: A case report. 2506 5


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