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

Magnetic resonance imaging (MRI) and computed tomography were used to diagnose fatty liver of pregnancy in a patient with preeclampsia and thrombotic thrombocytopenia purpura. The clinical course included multiple system failure that necessitated mechanical ventilation, dialysis, and plasmapheresis. The MRI picture is described in this entity for the first time. Both imaging techniques are discussed and compared.
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PMID:Magnetic resonance imaging and computed tomography scan for the diagnosis of acute fatty liver of pregnancy. 162 28

A woman presented in the third trimester of pregnancy with epigastric pain, elevated liver enzymes, and thrombocytopenia. The frozen-section liver biopsy findings were compatible with acute fatty liver of pregnancy. The light and electron microscopic findings were those of preeclampsia. All clinical and laboratory abnormalities resolved before delivery.
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PMID:Elevated liver enzymes and thrombocytopenia in the third trimester of pregnancy: an unusual case report and a review of the literature. 266 87

Thirteen members of a family carrying a gene for pedigree of hypobetalipoproteinemia were analyzed for lipoprotein compositions, apolipoprotein (apo) B levels, and apo B isoforms. Judging from low density lipoprotein (LDL)-cholesterol (Chol) and apo B levels, a 75-year-old proband, a father who died of unknown fever, thrombopenia, and anemia, and his wife were heterozygous for hypobetalipoproteinemia. The proband had ataxic movement of hands and gait disturbance in later life. Three of four living siblings had extremely low levels of LDL-Chol (6 mg/dL) and LDL-apo B (2 mg/dL), and were postulated to have homozygous hypobetalipoproteinemia. Electrophoresis revealed marked deficiency of apo B-100, although trace amounts were noted in LDL. In contrast, apo B-48 was present in chylomicrons obtained after a fatty meal in the two patients with homozygous hypobetalipoproteinemia, indicating a selective deficiency of apo B-100 but not apo B-48. The defect in these patients seemingly is different from abnormal apo B-37 reported recently for a family with hypobetalipoproteinemia. Clinically, acanthocytotic red blood cells (8% to 12%), fatty liver, and low levels of serum lipid-soluble vitamins A and D were noted in homozygotes. One heterozygous sibling had 26 mg/dL LDL-Chol and 5 mg/dL LDL-apo B levels. All seven subjects in the third generation had low levels of Chol (85 to 140 mg/dL), LDL-Chol (40 to 63 mg/dL) and LDL-apo B (10 to 20 mg/dL). They also showed mild acanthocytosis (0.5% to 2%) and a decrease of fat-soluble vitamins in plasma.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Homozygous hypobetalipoproteinemia with spared chylomicron formation. 290 27

We conducted a pilot study to assess the feasibility and efficacy of postdilution hemofiltration (PDHF) in the management of acute hepatic failure. From January 1984 through May 1986, we encountered seven patients with acute hepatic failure and entered these consecutive patients in the study; three had non-A, non-B hepatitis and one each had type B hepatitis, fulminant Wilson's disease (hepatolenticular degeneration), acute allograft (liver) failure, and acute fatty liver of pregnancy. Two of these seven patients were unable to undergo PDHF because of a precarious hemodynamic status. Of the five patients treated with PDHF, four had amelioration of hepatic encephalopathy; in two of these patients, a close temporal relationship was noted between the improvement and the procedure. Four patients had appreciable thrombocytopenia related to PDHF and bleeding complications. Our preliminary results support a possible role for PDHF as a temporary artificial liver support system for patients with acute hepatic failure.
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PMID:Postdilution hemofiltration in the management of acute hepatic failure: a pilot study. 327 72

Young rats were force-fed a lysine + arginine-devoid diet or a complete diet for 3 days, and selected biochemical and morphologic studies were conducted. Rats force-fed the experimental diet in comparison with those force-fed the control diet for 3 days showed decreased body weight gain, hepatomegaly with periportal fatty liver, pancreatic and splenic atrophy, and enhanced 14C-leucine incorporation into hepatic proteins. Differences in the experimental animals were observed in the free amino acid levels of serum (decreased lysine, arginine, and ornithine) and liver (decreased ornithine), in blood chemistries (decreased levels of ammonia N2, uric acid, cholesterol, protein, albumin, alkaline phosphatase, LDH and SGOT) and in hematologic findings (leukocytopenia and thrombocytopenia after a morning feeding). The experimental findings in young rats force-fed the lysine + arginine-devoid diet were compared with those reported to develop in children with lysinuric protein intolerance (LPI), an autosomal recessive defect in diamino acid transport. Children with LPI as described by others reveal a number of similarities as well as a number of differences in comparison to the findings in the experimental animals. The comparison suggests that some of the pathological manifestations of LPI may be related to a deficiency of diamino acids but others must be due to different alterations in this complex human disease.
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PMID:Chemical pathology of diamino acid deficiency: considerations in relation to lysinuric protein intolerance. 393 96

The clinical features, laboratory investigations and histopathology of 12 patients with idiopathic acute fatty liver of pregnancy are presented. Repeated vomiting, starting in the last trimester, was the cardinal symptom. Seven patients had proteinuria, hypertension and peripheral oedema before jaundice appeared. Caesarian section and induction of labour led to a lower than expected maternal mortality (33 X 3 per cent) and foetal mortality (66 X 7 per cent). There was a high incidence of twin and male births. Neutrophilia, thrombocytopenia and normoblasts were a uniform feature and uric acid levels were universally high. These findings may be useful in diagnosis in conjunction with liver function tests. Hepatic histology showed pathognomonic microvesicular fat in swollen hepatocytes with central nuclei and centrilobular distribution. However, a diffuse pattern and the presence of significant inflammation and fibrin deposits led to an initial misdiagnosis in two patients. Histology of fetal livers and five placentae was normal. Seven subsequent normal pregnancies occurred in four patients. Acute fatty liver of pregnancy may be confused with acute hepatitis or toxaemia on both clinical and histological grounds. Accurate diagnosis should lead to improved management and lessen maternal and fetal mortality. This justifies more intensive and urgent investigation of nausea, vomiting and jaundice in the last trimester of pregnancy.
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PMID:Idiopathic acute fatty liver of pregnancy in 12 patients. 715 26

A 35 year old primigravida with a triplet pregnancy developed polyuria and epigastric pain in the 31st week of pregnancy. During that week, emergency cesarean section was performed due to evidence of liver disease and imminent fetal hypoxia. Three girls were delivered who were healthy apart from transient neonatal respiration distress syndrome. Following surgery, the mother developed HELLP syndrome with hemolysis, increased transaminases and thrombocytopenia. She also developed diabetes insipidus with daily urine outputs of up to 7000 ml and poor response to desmopressin. Both the HELLP syndrome and the diabetes insipidus resolved spontaneously within ten days. In pregnant patients with right upper quadrant pain, HELLP syndrome or acute fatty liver of pregnancy should be considered. The association of diabetes insipidus with acute fatty liver of pregnancy is an established, but rare phenomenon. As far as is known, this is the first report of a patient presenting with a combination of HELLP syndrome and diabetes insipidus. Patients with HELLP syndrome have a good prognosis, if the diagnosis is early and the pregnancy terminated at the right time. With close supervision further pregnancies are possible.
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PMID:[Triplet pregnancy with HELLP syndrome and transient diabetes insipidus]. 818 5

Preeclampsia is a multisystem disorder of pregnancy whose clinical presentation is related to the importance and the extent of maternal microvasculature damage. 16 patients with preeclampsia, thrombocytopenia (< 100,000/mm3), elevated liver enzymes and hemolysis are described. Weinstein in 1982 assigned the acronym of Hellp syndrome for this clinical presentation of preeclampsia. This syndrome was seldom recognized during the first years of the study but was diagnosed later on, in 1 pregnancy out of 600 parturitions and in 5% of preeclampsia in 1991. Two out of the patients developed eclamptic seizures. The mode of delivery was as follows: 9 out of 16 had an emergency Caesarean section; the 7 other patients had vaginal delivery after induction of labor. Delivery occurred before 32 weeks of gestation for 6 out of 17 newborns. Outcome was good for 14 of the 17 newborns (one twin pregnancy). Two fetuses died: one in utero and one during interruption of the pregnancy at 24 weeks of gestation. A small for gestational age preterm of 32 weeks died on his second day of life. Early diagnosis of Hellp Syndrome, especially when abdominal pains are present, allows a prompt management of these patients, including delivery, which appeared in our experience, the only way to avoid fetomaternal complications. The thrombotic microangiopathics and acute fatty liver of pregnancy are potential imitations of Hellp syndrome and they must be considered for differential diagnosis. The frequency of Hellp Syndrome varies from one study to another. It can be increased up to 6 fold if referred to the number of pregnancies and up to 3 fold if referred to the number of preeclampsias. These differences can be real but may be due to bias related to differences in practice between centers and also differences in definitions of Hellp Syndrome and preeclampsia.
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PMID:[The Hellp syndrome: a frequent (?) obstetric emergency]. 835 77

Hepatic dysfunction is common in patients who receive intensive chemotherapy and it is important to determine the etiology in order to institute appropriate therapy. The role of laparoscopic liver biopsy in patients with neutropenia, thrombocytopenia, or both was evaluated as a mean of making treatment decisions and as a determinant of clinical outcome. Laparoscopic liver biopsy was performed in 29 subjects who were receiving intensive cytotoxic therapy with or without bone marrow transplantation. One to three direct-vision laparoscopic liver biopsies were performed in each patient using a Tru-cut needle during general anesthesia. Platelet concentrate transfusions were usually given before, during, and immediately after biopsy. Bleeding was controlled with spatula electrocautery. Thirty-two biopsies were obtained in 29 patients. At the time of liver biopsy, white blood cell and platelet counts ranged from 0 to 14,300/microliters (median: 2500/microliters), and 1000 to 47,000/microliters (median: 20,000/microliters), respectively. Bleeding at the liver biopsy site was readily controlled during the procedure without clinical evidence of significant bleeding; no procedure-related complications were noted and no patients required re-exploration. All biopsies were informative and the lesions observed in 32 biopsies revealed graft-versus-host disease (n = 5), hepatic candidiasis (n =1), hepatic veno-occlusive disease (n = 3), cholestasis (n = 19), hemosiderosis (n = 26), toxic injury (n = 8), hepatic steatosis (n = 4), granuloma (n = 1), viral infection (n =1), and malignancy (n = 1). Laparoscopic liver biopsy has been proven to be an effective means of assessing the cause of liver dysfunction in patients who were thrombocytopenic and immunosuppressed. The diagnosis obtained at laparoscopic liver biopsy altered therapy in nine of 29 (31%) patients.
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PMID:Laparoscopic liver biopsy to evaluate hepatic dysfunction in patients with hematologic malignancies: a useful tool to effect changes in management. 872 71

Acute fatty liver of pregnancy is a rare clinical entity unique to pregnancy that occurs during the third trimester. The obstetric team must be familiar with this disease because early diagnosis and prompt delivery have dramatically improved prognosis, which was often fatal for both mother and child. Clinicians must have a high index of suspicion for this condition when a woman has nausea or vomiting, abdominal pain (particularly epigastric), jaundice, polyuria-polydipsia (without diabetes), increased serum transaminase activity or thrombocytopenia in late pregnancy. The disease rarely recurs during a subsequent pregnancy. The cause is unknown, but some cases of acute fatty liver of pregnancy have been associated with a genetic deficiency of fatty acid beta-oxidation. Because of the possibility of this congenital deficiency, infants of affected mothers should undergo close follow-up from birth.
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PMID:Acute fatty liver of pregnancy. 963 7


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