TI: Maternal acute fatty liver of pregnancy associated with fetal trifunctional protein deficiency: molecular characterization of a novel maternal mutant allele.
AU: Isaacs-JD Jr; Sims-HF; Powell-CK; Bennett-MJ; Hale-DE; Treem-WR; Strauss-AW
AD: Department of Obstetrics and Gynecology, Washington University School of Medicine, St.Louis, Missouri, USA.
SO: Pediatr-Res. 1996 Sep; 40(3): 393-8
ISSN: 0031-3998
PY: 1996
LA: ENGLISH
CP: UNITED-STATES
AB: Acute fatty liver of pregnancy (AFLP) is a devastating late gestational complication with many similarities to the inherited disorders of mitochondrial fatty acid oxidation. We report the molecular defects in a woman with AFLP and her infant who subsequently was diagnosed with trifunctional protein (TFP) deficiency. We used single-stranded conformation variance and DNA sequence analyses of the human TFP alpha-subunit gene, which encodes the long chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) activity, to demonstrate a C to T mutation (C1678T) in exon 16 present on one allele in the mother and the affected infant. This creates a premature termination codon (R524Stop) in the LCHAD domain. Using reverse transcriptase-PCR amplification of the alpha-subunit mRNA from cultured fibroblasts, we demonstrated that transcripts containing this R524Stop mutation are present at very low levels, presumably because of rapid mRNA degradation. The affected infant also had the common E474Q mutation (nucleotide G1528C) on the second allele. Thus, he is a compound heterozygote. The father and two normal siblings are heterozygous for this E474Q mutation. This initial delineation of the R524Stop mutation provides evidence of the heterogeneity of genetic defects responsible for TFP deficiency and AFLP. MESH: Acute-Disease; Adult-; Alleles-; Exons-; Fatty-Liver-genetics; Fetal-Proteins-genetics; Multienzyme-Complexes-chemistry; Multienzyme-Complexes-genetics; Mutation-; Nucleic-Acid-Hybridization-methods; Oligonucleotide-Probes; Pedigree-; Peptide-Fragments-genetics; Pregnancy-; 3-Hydroxyacyl-CoA-Dehydrogenases-genetics MESH: *Fatty-Liver-physiopathology; *Fetal-Proteins-deficiency;
*Multienzyme-Complexes-deficiency; *Pregnancy-Complications-physiopathology;
*3-Hydroxyacyl-CoA-Dehydrogenases-deficiency
TG: Case-Report; Female; Human; Support,-U.S.-Gov't,-P.H.S.
PT: JOURNAL-ARTICLE
CN: AM20407AMNIADDK
RN: EC 1.1.1.211; EC 1.1.1.35; 0; 0; 0; 0; 0
NM: long-chain-3-hydroxyacyl-CoA-dehydrogenase; 3-Hydroxyacyl-CoA-Dehydrogenases; fatty-acid-beta-oxidation-multienzyme-complex; Fetal-Proteins; Multienzyme-Complexes; Oligonucleotide-Probes; Peptide-Fragments
AN: 97018658
UD: 9704
TI: Acute fatty liver of pregnancy, hemolysis, elevated liver enzymes, and low plateletssyndrome, and long chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency [see comments]
CM: Comment in: Am J Gastroenterol 1996 Nov;91(11):2262-4
AU: Treem-WR; Shoup-ME; Hale-DE; Bennett-MJ; Rinaldo-P; Millington-DS; Stanley-CA; Riely-CA; Hyams-JS
AD: Division of Pediatric Gastroenterology and Nutrition, Hartford Hospital, University of Connecticut School of Medicine, Farmington, USA.
SO: Am-J-Gastroenterol. 1996 Nov; 91(11): 2293-300
ISSN: 0002-9270
PY: 1996
LA: ENGLISH
CP: UNITED-STATES
AB: BACKGROUND: The similarity of the hepatic pathology in acute fatty liver of pregnancy (AFLP) to that seen in children with inherited disorders of intramitochondrial fatty acid oxidation (FAO) suggests that there may be a genetic basis for some cases of AFLP. OBJECTIVE: The purpose of this study was to examine patients with AFLP and their offspring to determine if there were women with AFLP who were heterozygous for the FAO defect, long chain 3-hydroxyacyl CoA dehydrogenase (LCHAD) deficiency. METHODS: We evaluated 12 women previously diagnosed with AFLP. Provocative fasting studies and skin biopsies for examination of their cultured skin fibroblasts were performed to search for a generalized defect in FAO both in vivo and in vitro. Cultured skin fibroblasts from AFLP patients, their children, and their husbands were also examined specifically for LCHAD activity. RESULTS: Of 12 women with a previous episode of AFLP, eight had reduced LCHAD activity consistent with being heterozygous for LCHAD deficiency. The eight heterozygotes had a total of nine pregnancies complicated by AFLP. In seven of those nine pregnancies, the women developed severe preeclampsia and hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome. Of the nine offspring delivered from these pregnancies, four were confirmed to be affected with homozygous LCHAD deficiency. Three other deceased infants were presumed to be LCHAD-deficient based on clinical findings, postmortem examination, and confirmed heterozygote parents. The remaining two infants delivered after pregnancies complicated by AFLP had LCHAD activity in the heterozygous range and are healthy at 18 and 24 months of age. Consistent with the known autosomal recessive nature of this defect, five tested husbands of LCHAD heterozygous women with a history of AFLP and affected infants also showed reduced LCHAD activity. CONCLUSIONS: These studies indicate that a significant subgroup of women with AFLP are heterozygous for LCHAD deficiency and that careful observation of their offspring for signs of this disorder is warranted. Severe preeclampsia appears to increase the risk of AFLP in LCHAD heterozygous women.
MESH: Acute-Disease; Adult-; Biopsy-; Fatty-Liver-diagnosis; Fibroblasts-enzymology; Fibroblasts-pathology; Heterozygote-; HELLP-Syndrome-diagnosis; Pregnancy-; Pregnancy-Complications-diagnosis; Recurrence-; Risk-Factors; Skin-pathology;3-Hydroxyacyl-CoA-Dehydrogenases-genetics
MESH: *Fatty-Liver-genetics; *HELLP-Syndrome-genetics; *Pregnancy-Complications-etiology; *3-Hydroxyacyl-CoA-Dehydrogenases-deficiency
TG: Female; Human; Support,-Non-U.S.-Gov't; Support,-U.S.-Gov't,-P.H.S.
PT: JOURNAL-ARTICLE
CN: R01DK43841DKNIDDK; RR00240RRNCRR
RN: EC 1.1.1.211; EC 1.1.1.35
NM: long-chain-3-hydroxyacyl-CoA-dehydrogenase; 3-Hydroxyacyl-CoA-Dehydrogenases
AN: 97085264
UD: 9702
TI: Acute fatty liver of pregnancy: is it genetically predetermined? [editorial; comment]
CM: Comment on: Am J Gastroenterol 1996 Nov;91(11):2293-300
AU: Batey-RG
SO: Am-J-Gastroenterol. 1996 Nov; 91(11): 2262-4
ISSN: 0002-9270
PY: 1996
LA: ENGLISH
CP: UNITED-STATES
MESH: Acute-Disease; Cholestasis-etiology; Fatty-Liver-diagnosis; HELLP-Syndrome-genetics; Pregnancy-; Pregnancy-Complications-diagnosis; Pregnancy-Complications-etiology; Recurrence-; Time-Factors
MESH: *Fatty-Liver-genetics; *Pregnancy-Complications; *3-Hydroxyacyl-CoA-Dehydrogenases-deficiency
TG: Female; Human
PT: COMMENT; EDITORIAL
RN: EC 1.1.1.211; EC 1.1.1.35
NM: long-chain-3-hydroxyacyl-CoA-dehydrogenase; 3-Hydroxyacyl-CoA-Dehydrogenases
AN: 97085259
UD: 9702
TI: Fulminant hepatic failure in pregnant women: acute fatty liver or acute viral hepatitis?
AU: Hamid-SS; Jafri-SM; Khan-H; Shah-H; Abbas-Z; Fields-H
AD: Department of Medicine, Aga Khan University Hospital, Karachi, Pakistan.
SO: J-Hepatol. 1996 Jul; 25(1): 20-7
ISSN: 0168-8278
PY: 1996
LA: ENGLISH
CP: DENMARK
AB: BACKGROUND: Hepatitis E virus, which is endemic in our region, can cause severe liver dysfunction in pregnant women and this can be clinically confused with acute fatty liver of pregnancy. METHODS: We studied the clinical and laboratory data as well as the maternal and fetal outcomes of 12 pregnant women presenting with fulminant hepatic failure in order to determine the etiology of the disease. The clinical diagnoses were subsequently correlated with serologic assays for acute HEV infection. All patients were severely ill with deep jaundice, grade 3-4 encephalopathy and abnormal prothrombin times. RESULTS: A clinical diagnosis of acute viral hepatitis was made in nine patients and of acute fatty liver in the other three cases. IgM and IgG antibodies confirmed acute viral hepatitis E in six of the nine patients while one had acute hepatitis A infection. HEV IgM and IgG antibodies were, however, also positive in two of the three patients thought to have acute fatty liver. Maternal and fetal mortality were 16.6% and 50%, respectively. CONCLUSIONS: We conclude that hepatitis E is the usual cause of acute liver failure in our pregnant women and that clinical and laboratory features do not permit accurate distinction between acute HEV infection and acute fatty liver of pregnancy. The prognosis in patients with acute HEV infection is much better than in other groups with severe liver failure (mortality 16% vs 68%).
MESH: Acute-Disease; Adolescence-; Adult-; Antibodies,-Viral-blood; Hepatic-Encephalopathy-pathology; Liver-pathology; Pregnancy-
MESH: *Fatty-Liver-complications; *Hepatic-Encephalopathy-etiology; *Hepatitis-E-complications; *Pregnancy-Complications-etiology
TG: Female; Human
PT: JOURNAL-ARTICLE
RN: 0
NM: Antibodies,-Viral
AN: 96433918
UD: 9702
TI: Immunohistological study in cases of HELLP syndrome (hemolysis, elevated liver enzymes and low platelets) and acute fatty liver of pregnancy.
AU: Halim-A; Kanayama-N; El-Maradny-E; Maehara-K; Takahashi-A; Nosaka-K; Fukuo-S; Amamiya-A; Kobayashi-T; Terao-T
AD: Department of Obstetrics, Hamamatsu University School of Medicine, Japan.
SO: Gynecol-Obstet-Invest. 1996; 41(2): 106-12
ISSN: 0378-7346
PY: 1996
LA: ENGLISH
CP: SWITZERLAND
AB: We immunohistologically studied the hepatic tissue sections in cases with the syndrome of hemolysis, elevated liver enzymes, and low platelets (HELLP syndrome; n = 2) and acute fatty liver of pregnancy (AFLP; n = 2) compared to necropsy controls. Unlike in the AFLP cases, a marked infiltration of neutrophils in liver tissues was found in both cases of the HELLP syndrome. Immunostaining with the antihuman (polyclonal) TNF-alpha, IL-1 beta, IL-8 and antihuman neutrophil elastase (monoclonal antibody) was performed in paraffin-embedded hepatic tissue sections. Liver tissues in HELLP syndrome patients were stained strongly with TNF-alpha and neutrophil elastase antibody. The strongest staining pattern was observed in the eclamptic case, whereas in the AFLP cases, as in the necropsy controls, a very weak staining for anti-TNF-alpha and elastase antibody was found. The liver sections of the HELLP syndrome cases were moderately stained with polyclonal IL-1 beta and IL-8 antibodies whereas AFLP and controls had a very faint staining. Significant correlations were found between the numbers of necrotic hepatocytes and elastase dots in the same microscopic fields (randomly selected) of liver sections from two cases of HELLP syndrome (r2 = 0.63; p < 0.0001), which might suggest a neutrophil-mediated tissue damage in such a disease. This study suggests that a cytokine- and neutrophil-mediated liver injury occurs in the HELLP syndrome but not in AFLP.
MESH: Adult-; Interleukin-1-analysis; Interleukin-8-analysis; Leukocyte-Elastase-analysis; Liver-chemistry; Liver-pathology; Necrosis-; Neutrophils-pathology; Pregnancy-; Tumor-Necrosis-Factor-analysis
MESH: *Cytokines-analysis; *Fatty-Liver-pathology; *HELLP-Syndrome-metabolism; *HELLP-Syndrome-pathology; *Immunohistochemistry-; *Pregnancy-Complications
TG: Female; Human
PT: JOURNAL-ARTICLE
RN: EC 3.4.21.37; 0; 0; 0; 0
NM: Leukocyte-Elastase; Cytokines; Interleukin-1; Interleukin-8; Tumor-Necrosis-Factor
AN: 96436084
UD: 9702
TI: Liver disease in pregnancy.
AU: Wolf-JL
AD: Harvard Medical School, Boston, Massachusetts 02115, USA.
SO: Med-Clin-North-Am. 1996 Sep; 80(5): 1167-87
ISSN: 0025-7125
PY: 1996
LA: ENGLISH
CP: UNITED-STATES
AB: The liver is one of the many organs affected by the physiologic and hormonal changes that occur during pregnancy. Hepatic disorders diagnosed before pregnancy may be unaffected or exacerbated by the pregnant state. Liver disorders that are specific to pregnancy, including hyperemesis gravidarum, intrahepatic cholestasis of pregnancy, acute fatty liver of pregnancy, preeclampsia/ eclampsia, HELLP, and hepatic rupture, may have a profound impact on the morbidity and mortality rates of mother and fetus. Although an unequivocal diagnosis is often difficult to make, it should be attempted in a timely manner so that optimal treatment can be determined. After the diagnosis is made, maximizing the health of the mother and fetus determines future management.
MESH: Diagnosis,-Differential; Liver-Diseases-etiology; Liver-Diseases-therapy; Liver-Function-Tests; Pregnancy-; Pregnancy-Complications-etiology; Pregnancy-Complications-therapy; Pregnancy-Outcome
MESH: *Liver-Diseases-diagnosis; *Pregnancy-Complications-diagnosis
TG: Female; Human
PT: JOURNAL-ARTICLE; REVIEW; REVIEW,-TUTORIAL
AN: 96397485
UD: 9612
SB: AIM
TI: Early occurrence of acute fatty liver in pregnancy.
AU: Buytaert-IM; Elewaut-GP; Van-Kets-HE
AD: Department of Gastroenterology, University Hospital of Ghent, Gent, Belgium.
SO: Am-J-Gastroenterol. 1996 Mar; 91(3): 603-4
ISSN: 0002-9270
PY: 1996
LA: ENGLISH
CP: UNITED-STATES
AB: We report a case of acute fatty liver of pregnancy characterized by its early occurrence in the 26th wk of a twin pregnancy. Usually, this illness begins only late in the third trimester, although onset of disease as early as 26 wk has already been reported. The death of both fetuses also illustrates the high mortality rate and the necessity for prompt diagnosis and treatment.
MESH: Acute-Disease; Adult-; Fetal-Death; Pregnancy-; Pregnancy-Trimester,-Second; Pregnancy,-Multiple; Twins-
MESH: *Fatty-Liver-diagnosis; *Pregnancy-Complications-diagnosis
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 96209604
UD: 9609
TI: Study of the liver changes occurring in preeclampsia and their possible pathogeneti connection with acute fatty liver of pregnancy.
AU: Dani-R; Mendes-GS; Medeiros-J-de-L; Peret-FJ; Nunes-A
AD: Gastroenterology Service, Hospital Israel Pinheiro of the Instituto de Previdencia, Belo Horizonte, Brazil.
SO: Am-J-Gastroenterol. 1996 Feb; 91(2): 292-4
ISSN: 0002-9270
PY: 1996
LA: ENGLISH
CP: UNITED-STATES
AB: OBJECTIVE: The objective of the present study was to investigate liver involvement in preeclampsia on the basis of clinical, laboratory, and histological data and to detect a possible connection with fatty liver of pregnancy by the determination of microvesicular fatty infiltration of the liver. METHODS: The authors studied the liver changes in 10 patients with preeclampsia, observing the clinical and laboratory alterations, the macroscopic liver surface features by laparoscopy, and the presence of microvesicular fatty infiltration by specific lipid staining of hepatic tissue collected by needle biopsy. RESULTS: Macroscopy of the liver surface disclosed some degree of subcapsular liver hemorrhage in all cases; however, the hemorrhage was not related to the clinical and histological severity of the disease. Microvesicular fat droplets were observed in all patients, and the intensity of the fat deposition was not related to pressor levels, laboratory alterations, or the evolution of preeclampsia. CONCLUSIONS: The presence of fatty liver infiltration in all patients studied supports the idea that preeclampsia and acute fatty liver of pregnancy could be components of the same pathologic spectrum, with a probable, but still unproved, pathogenetic connection. The deficiency of the long chain 3-hydroxyacyl-coenzyme A dehydrogenase activity may be the determining factor in the evolution of the disease.
MESH: Acute-Disease; Adolescence-; Adult-; Biopsy-; Cesarean-Section; Infant,-Newborn; Laparoscopy-; Pregnancy-; 3-Hydroxyacyl-CoA-Dehydrogenases-deficiency
MESH: *Fatty-Liver-pathology; *Liver-pathology; *Pre-Eclampsia-pathology;*Pregnancy-Complications-pathology
TG: Female; Human; Male
PT: JOURNAL-ARTICLE
RN: EC 1.1.1.35
NM: 3-Hydroxyacyl-CoA-Dehydrogenases
AN: 96189988
UD: 9607
TI: Disseminated intravascular coagulation and antithrombin III depression in acute fatty liver of pregnancy.
AU: Castro-MA; Goodwin-TM; Shaw-KJ; Ouzounian-JG; McGehee-WG
AD: Department of Obstetrics and Gynecology, University of Southern California Medical Center, Los Angeles, USA.
SO: Am-J-Obstet-Gynecol. 1996 Jan; 174(1 Pt 1): 211-6
ISSN: 0002-9378
PY: 1996
LA: ENGLISH
CP: UNITED-STATES
AB: OBJECTIVE: Acute fatty liver of pregnancy has been associated with a syndrome of marked depression of antithrombin III and disseminated intravascular coagulation. We sought to identify the clinical importance of this accelerated coagulation. STUDY DESIGN: The medical records of patients with acute fatty liver of pregnancy identified during the period of 1982 to 1994 were retrospectively reviewed. RESULTS: Twenty-eight patients with acute fatty liver of pregnancy were identified for an incidence of 1:6692 births. Laboratory evidence of persistent disseminated intravascular coagulation was found in all patients tested. Six patients had clinical bleeding, all associated with genital tract injury. Twenty-three of twenty-three patients tested had markedly decreased antithrombin III levels (average 11%, normal range 80% to 100%). Seven patients received antithrombin III transfusions, which was associated with a significant transient rise in the plasma level. Compared with patients not transfused, however, there was a similar clinical outcome. CONCLUSION: Profoundly depressed antithrombin III levels and laboratory evidence of disseminated intravascular coagulation were present in all cases of acute fatty liver of pregnancy but rarely influenced clinical outcome unless there was concomitant genital tract injury. Antithrombin III transfusions increased plasma levels, but no definite clinical benefit was established in this series because of the small number of cases.
MESH: Acute-Disease; Adolescence-; Adult-; Gestational-Age; Pregnancy-
MESH: *Antithrombin-III-deficiency; *Disseminated-Intravascular-Coagulation-complications; *Fatty-Liver-complications; *Pregnancy-Complications-blood
TG: Female; Human
PT: JOURNAL-ARTICLE; REVIEW; REVIEW-OF-REPORTED-CASES
RN: 9000-94-6
NM: Antithrombin-III
AN: 96148691
UD: 9605
SB: AIM
TI: Recurrence of acute fatty liver of pregnancy.
AU: Visconti-M; Manes-G; Giannattasio-F; Uomo-G
AD: Department of Internal Medicine, Nuovo Pellegrini Hospital, Naples, Italy.
SO: J-Clin-Gastroenterol. 1995 Oct; 21(3): 243-5
ISSN: 0192-0790
PY: 1995
LA: ENGLISH
CP: UNITED-STATES
AB: We report this rare recurrence of biopsy-proven acute fatty liver of pregnancy. In two successive pregnancies, an emergency cesarean section was performed with delivery of healthy babies and rapid maternal recovery with complete normalization of liver function. This is the third such report.
MESH: Acute-Disease; Adult-; Fatty-Liver-pathology; Liver-parasitology; Pregnancy-; Pregnancy-Complications-pathology; Recurrence-
MESH: *Fatty-Liver; *Pregnancy-Complications
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 96116014
UD: 9609
TI: [Uncomplicated second pregnancy following acute fatty liver of pregnancy]
TO: Unkomplizierte Zweitschwangerschaft nach akuter Schwangerschaftsfettleber.
AU: Zurcher-K
AD: Praxis fur Innere Medizin FMH, Bern.
SO: Schweiz-Med-Wochenschr. 1995 May 20; 125(20): 1003-5
ISSN: 0036-7672
PY: 1995
LA: GERMAN; NON-ENGLISH
CP: SWITZERLAND
AB: Acute fatty liver is a rare but usually severe and frightening complication of the last trimester of pregnancy. Many patients therefore do not want or are warned against having another child. This report adds another case of successful consecutive pregnancy to the 28 documented cases in 24 patients. On the other hand, only 2 recurrences have been reported. Therefore, the wish for another pregnancy may be supported, provided the gravida is well informed and the necessary (bi)weekly clinical and laboratory checks are carried out in the last trimester (liver tests, uric acid, prothrombin time and, in case of doubt, further coagulation tests including antithrombin III).
MESH: Adult-; English-Abstract; Infant,-Newborn; Liver-Function-Tests; Pregnancy-; Pregnancy,-Multiple; Prenatal-Care; Twins-
MESH: *Fatty-Liver-complications; *Pregnancy-Complications; *Pregnancy-Outcome
TG: Female; Human
PT: JOURNAL-ARTICLE
AN: 95288616
UD: 9509
TI: Prevalence and nonspecificity of microvesicular fatty change in the liver.
AU: Fraser-JL; Antonioli-DA; Chopra-S; Wang-HH
AD: Department of Pathology, Beth Israel Hospital, Boston, Massachusetts, USA.
SO: Mod-Pathol. 1995 Jan; 8(1): 65-70
ISSN: 0893-3952
PY: 1995
LA: ENGLISH
CP: UNITED-STATES
AB: Hepatic microvesicular fat (MVF) has been associated with clinical syndromes of liver failure (such as acute fatty liver of pregnancy and Reye's syndrome). We performed this pilot study to investigate the specificity and potential risk factors of MVF. Sections of the liver from 17 adult autopsies were snap-frozen, and Oil Red O (ORO)-stained sections were obtained. The presence of MVF in the ORO stains was analyzed in a semiquantitative manner: 3+ = > 90% liver parenchyma with MVF; 2+ = 25-90%; 1+ = 1-24%. H&E-stained liver sections were reviewed independently to determine the presence and extent of inflammation, fibrosis, and necrosis. Autopsy reports were reviewed for the gross findings; medical records were reviewed for demographic data, medical history, and, in particular, medication usage. The study consisted of 10 males and 7 females (15 white, 2 black), with a median age of 76 years (range: 50-88). MVF was identified in 16 patients (94%); it was infrequent (1+, 2+) in the majority, but 4 patients (24%) had 3+ MVF. MVF was not associated with the age or sex of the patients, liver weight, postmortem interval to examination, or other histologic features in the liver. None of the patients, including those with 3+ MVF, had a history of liver disease. All 4 patients with extensive MVF were using salicylates, as opposed to 2 of 5 with 2+ MVF and 1 of 8 with 0 to 1+ MVF (P = 0.015). In this autopsy study, the prevalence of MVF was high, with 24% of patients having extensive
MVF.(ABSTRACT TRUNCATED AT 250 WORDS)
MESH: Aged-; Aged,-80-and-over; Aspirin-adverse-effects; Fatty-Liver-chemically-induced; Middle-Age; Pilot-Projects; Risk-Factors
MESH: *Fatty-Liver-pathology; *Vacuoles-pathology
TG: Female; Human; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
RN: 50-78-2
NM: Aspirin
AN: 95249516
UD: 9508
TI: The molecular basis of pediatric long chain 3-hydroxyacyl-CoA dehydrogenase deficiency associated with maternal acute fatty liver of pregnancy.
AU: Sims-HF; Brackett-JC; Powell-CK; Treem-WR; Hale-DE; Bennett-MJ; Gibson-B; Shapiro-S; Strauss-AW
AD: Department of Pediatrics, Washington University School of Medicine, St. Louis, MO 63110.
SO: Proc-Natl-Acad-Sci-U-S-A. 1995 Jan 31; 92(3): 841-5
ISSN: 0027-8424
PY: 1995
LA: ENGLISH
CP: UNITED-STATES
AB: Mitochondrial long chain fatty acid beta-oxidation provides the major source of energy in the heart. Deficiencies of human beta-oxidation enzymes produce sudden, unexplained death in childhood, acute hepatic encephalopathy, skeletal myopathy, or cardiomyopathy. Long chain 3-hydroxyacyl-CoA dehydrogenase [LCHAD; long-chain-(S)-3-hydroxyacyl-CoA:NAD+ oxidoreductase, EC 1.1.1.211] catalyzes the third step in beta-oxidation, and this activity is present on the C-terminal portion of the alpha subunit of mitochondrial trifunctional protein. We used single-stranded conformation variance analysis of the exons of the human LCHAD (alpha subunit) gene to determine the molecular basis of LCHAD deficiency in three families with children presenting with sudden unexplained death or hypoglycemia and abnormal liver enzymes (Reye-like syndrome). In all families, the mothers had acute fatty liver and associated sever complications during pregnancies with the affected infants. The analysis in two affected children revealed a G to C mutation at position 1528 (G1528C) of the alpha subunit of the trifunctional protein on both alleles. This is in the LCHAD domain and substitutes glutamine for glutamic acid at position 474 of mature alpha subunit. The third child had this G1528C mutation on one allele and a different mutation (C1132T) creating a premature termination codon (residue 342) on the second allele. Our results demonstrate that mutations in the LCHAD domain of the trifunctional protein alpha subunit in affected offspring are associated with maternal acute fatty liver of pregnancy. This is the initial delineation of the molecular basis of isolated LCHAD deficiency.
MESH: Amino-Acid-Sequence; Base-Sequence; Cells,-Cultured; Cloning,-Molecular;Exons-genetics; Fibroblasts-enzymology; Hypoglycemia-; HELLP-Syndrome; Infant;Infant,-Newborn; Molecular-Sequence-Data; Point-Mutation-genetics; Polymorphism,-Single-Stranded-Conformational; Pregnancy-; Sudden-Infant-Death; 3-Hydroxyacyl-CoA-Dehydrogenases-metabolism
MESH: *Fatty-Liver-complications; *Lipid-Metabolism,-Inborn-Errors-genetics; *Multienzyme-Complexes-genetics; *Pregnancy-Complications; *3-Hydroxyacyl-CoA-Dehydrogenases-deficiency; *3-Hydroxyacyl-CoA-Dehydrogenases-genetics
TG: Female; Human; Male; Support,-U.S.-Gov't,-P.H.S.
PT: JOURNAL-ARTICLE
CN: AM20407AMNIADDK; T32HL07081HLNHLBI
RN: EC 1.1.1.211; EC 1.1.1.35; 0; 0
NM: long-chain-3-hydroxyacyl-CoA-dehydrogenase; 3-Hydroxyacyl-CoA-Dehydrogenases; fatty-acid-beta-oxidation-multienzyme-complex; Multienzyme-Complexes
AN: 95148633
UD: 9505
TI: Effects of female sex hormones on mitochondria: possible role in acute fatty liver of pregnancy.
AU: Grimbert-S; Fisch-C; Deschamps-D; Berson-A; Fromenty-B; Feldmann-G; Pessayre-D
AD: Institut National de la Sante et de la Recherche Medicale Unite-24, Clichy, France.
SO: Am-J-Physiol. 1995 Jan; 268(1 Pt 1): G107-15
ISSN: 0002-9513
PY: 1995
LA: ENGLISH
CP: UNITED-STATES
AB: Acute fatty liver of pregnancy occurs in some women. As other cases of microvesicular steatosis are due to impaired mitochondrial oxidation of fatty acids, we investigated the effects of female sex hormones on liver mitochondria in female mice. Three hours after administration of both estradiol (36 mumol/kg) and progesterone (150 mumol/kg), the in vitro beta-oxidation of [U-14C]palmitic acid and the activity of the tricarboxylic acid cycle decreased 49 and 54%, whereas the in vivo oxidation of [U-14C]palmitic acid decreased 38%. One week of treatment with both sex hormones produced ultrastructural lesions of mitochondria, decreased the recovery of mitochondrial proteins by 34%, increased state 4 respiration by 54-77%, and decreased the activities per gram of liver of several enzymes involved in the activation, mitochondrial uptake, and oxidation of fatty acids by 34-54%. We conclude that female sex hormones have deleterious effects on liver mitochondria and suggest that these effects, together with other factors, may contribute to the development of acute fatty liver of pregnancy in some women.
MESH: Acute-Disease; Dose-Response-Relationship,-Drug; Mice-; Mice,-Inbred-Strains; Mitochondria,-Liver-physiology; Mitochondria,-Liver-ultrastructure; Pregnancy-; Time-Factors
MESH: *Estradiol-pharmacology; *Fatty-Liver-etiology; *Mitochondria,-Liver-drug-effects; *Pregnancy-Complications-etiology; *Progesterone-pharmacology
TG: Animal; Female
PT: JOURNAL-ARTICLE
RN: 50-28-2; 57-83-0
NM: Estradiol; Progesterone
AN: 95142173
UD: 9505
TI: Acute fatty liver of pregnancy: an experience in the diagnosis and management of fourteen cases.
AU: Usta-IM; Barton-JR; Amon-EA; Gonzalez-A; Sibai-BM
AD: Department of Obstetrics and Gynecology, University of Tennessee, Memphis 38103.
SO: Am-J-Obstet-Gynecol. 1994 Nov; 171(5): 1342-7
ISSN: 0002-9378
PY: 1994
LA: ENGLISH
CP: UNITED-STATES
AB: OBJECTIVE: Our purpose was to investigate the diagnostic problems and maternal-perinatal outcome in cases of acute fatty liver of pregnancy. STUDY DESIGN: Fourteen cases with acute fatty liver of pregnancy managed during the past 8-years were studied with emphasis on presenting symptoms, admitting diagnosis, laboratory findings, clinical course, maternal complications, and neonatal outcome. RESULTS: The mean gestational age at onset was 34.5 weeks (range 28 to 39). Only seven patients had acute fatty liver of pregnancy as a definite or suspected diagnosis on admission. Computed tomography of the liver was performed on 10 patients, with only two positive results. There were no maternal deaths; however, maternal morbidity was frequent: four patients had hepatic encephalopathy, three pulmonary edema, three ascites, four respiratory arrest, two diabetes insipidus, and 10 had transfusion of blood or blood products to correct either disseminated intravascular coagulation or excessive bleeding. Coagulation abnormalities were common: hypofibrinogenemia (< 300 mg/dl) in 13 patients (93%), prolonged prothrombin time in 12 (86%), and prolonged partial thromboplastin time in 11 (79%). The corrected perinatal mortality was 6.6%. CONCLUSION: Acute fatty liver of pregnancy should be suspected in all patients with symptoms of preeclampsia in the presence of hypoglycemia, low fibrinogen, and prolonged prothrombin time, particularly in the absence of severe abruptio placentae. Computed tomography of the liver has a high false-negative rate in patients with acute fatty liver of pregnancy. In spite of the literature's dismal prognosis, our findings indicate that maternal and perinatal outcomes appear favorable in well-managed patients.
MESH: Acute-Disease; Adolescence-; Adult-; Biopsy-; Cesarean-Section; Fatty-Liver-epidemiology; Fatty-Liver-physiopathology; Liver-pathology; Morbidity-; Pregnancy-; Pregnancy-Complications-epidemiology; Pregnancy-Complications-physiopathology; Pregnancy-Outcome; Puerperium-; Tomography,-X-Ray-Computed; Ultrasonography-
MESH: *Fatty-Liver-diagnosis; *Pregnancy-Complications-diagnosis
TG: Female; Human
PT: JOURNAL-ARTICLE
AN: 95068154
UD: 9502
SB: AIM
TI: Is acute fatty liver of pregnancy a metabolic defect?
AU: Vasiliauskas-E; Rosenthal-P
AD: Department of Pediatrics, Cedars Sinai Medical Center, UCLA School of Medicine.
SO: Am-J-Gastroenterol. 1994 Oct; 89(10): 1908-10
ISSN: 0002-9270
PY: 1994
LA: ENGLISH
CP: UNITED-STATES
MESH: Acute-Disease; Adult-; Fatty-Acids-metabolism; Infant,-Newborn; Liver-metabolism; Pregnancy-; 3-Hydroxyacyl-CoA-Dehydrogenases-deficiency
MESH: *Fatty-Liver-metabolism; *Pregnancy-Complications-metabolism
TG: Female; Human
PT: JOURNAL-ARTICLE
RN: EC 1.1.1.211; EC 1.1.1.35; 0
NM: long-chain-3-hydroxyacyl-CoA-dehydrogenase; 3-Hydroxyacyl-CoA-Dehydrogenases;
Fatty-Acids
AN: 95029214
UD: 9501
TI: Recurrence of acute fatty liver of pregnancy.
AU: MacLean-MA; Cameron-AD; Cumming-GP; Murphy-K; Mills-P; Hilan-KJ
AD: Department of Obstetrics, Queen Mother's Hospital, UK.
SO: Br-J-Obstet-Gynaecol. 1994 May; 101(5): 453-4
ISSN: 0306-5456
PY: 1994
LA: ENGLISH
CP: ENGLAND
MESH: Adult-; Fatty-Liver-pathology; Liver-pathology; Liver-Function-Tests; Pregnancy-; Pregnancy-Complications-pathology; Recurrence-
MESH: *Fatty-Liver-diagnosis; *Pregnancy-Complications-diagnosis
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 94289370
UD: 9410
SB: AIM
TI: Transient diabetes insipidus and acute fatty liver of pregnancy.
AU: Kennedy-S; Hall-PM; Seymour-AE; Hague-WM
AD: Department of Obstetrics and Gynaecology, Lyell McEwin Hospital, Australia.
SO: Br-J-Obstet-Gynaecol. 1994 May; 101(5): 387-91
ISSN: 0306-5456
PY: 1994
LA: ENGLISH
CP: ENGLAND
AB: OBJECTIVE: To review the association of transient diabetes insipidus and acute fatty liver of pregnancy. DESIGN: A retrospective study. SETTING: Six women presenting with polyuria and polydipsia in the third trimester or in the immediate postpartum period, referred over a two and a half year period; five out of six were primigravida. All had raised liver transaminases and biopsy-proven acute fatty liver of pregnancy. Four out of six also had pre-eclampsia. SUBJECTS: Tertiary referral centre. MAIN OUTCOME MEASURES: There were no maternal deaths and only one fetal death. Desamino-cys-1-D-arg-8-vasopressin administration produced a reduction in urine output in all five women to whom it was administered. In all cases symptoms had resolved by the end of the fourth postpartum week. Three of the women have had subsequent pregnancies uncomplicated by either transient diabetes insipidus or acute fatty liver of pregnancy. CONCLUSIONS: The association of transient diabetes insipidus and acute fatty liver of pregnancy appears more common than previously recognised. Both may be part of the spectrum of pre-eclampsia.
MESH: Acute-Disease; Adult-; Diabetes-Insipidus-physiopathology; Diabetes,-Gestational-complications; Fatty-Liver-physiopathology; Polyuria-etiology; Pre-Eclampsia-complications; Pregnancy-; Pregnancy-Complications-physiopathology; Pregnancy-Trimester,-Third; Retrospective-Studies
MESH: *Diabetes-Insipidus-complications; *Fatty-Liver-complications;
*Pregnancy-Complications
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 94289354
UD: 9410
SB: AIM
TI: Acute hepatic failure with swollen mitochondria and microvesicular fatty degeneration of hepatocytes triggered by free radical initiator.
AU: Saibara-T; Himeno-H; Ueda-H; Onishi-S; Yamamoto-Y; Enzan-H; Hara-H; Takehara-Y;
Utsumi-K
AD: First Department of Medicine, Kochi Medical School, Nankoku, Japan.
SO: Lab-Invest. 1994 Apr; 70(4): 517-24
ISSN: 0023-6837
PY: 1994
LA: ENGLISH
CP: UNITED-STATES
AB: BACKGROUND: Reye syndrome and acute fatty liver of pregnancy are characterized by acute hepatic failure associated with characteristic pathologic changes of hepatocytes, i.e., microvesicular fatty degeneration with severe mitochondrial swelling. Several animal models of these conditions have been proposed, although none has demonstrated sufficiently similar laboratory and pathologic features. EXPERIMENTAL DESIGN: A single dose of 2,2'-azobis-(2-amidinopropane) dihydrochloride, a free radical initiator, 100 mg/kg of body weight, injected intraperitoneally to BALB/c mice induced a transient decrease in hepatic energy charge, liver mitochondrial respiratory activity, and succinate dehydrogenase activity and a transient increase in blood ammonia level, suggesting hepatic failure. RESULTS: The hepatocytes displayed microvesicular fatty degeneration with severely swollen mitochondria. CONCLUSIONS: This is the first model of acute hepatic failure with microvesicular fatty degeneration of hepatocytes. It suggests a role of free radicals in the pathogenesis of certain types of acute hepatic failure.
MESH: Adenine-Nucleotides-metabolism; Cholesterol-metabolism; Energy-Metabolism-drug-effects; Free-Radicals; Liver-Failure,-Acute-pathology; Mice-; Mice,-Inbred-BALB-C; Mitochondrial-Swelling; Triglycerides-metabolism
MESH: *Amidines-; *Liver-Failure,-Acute-chemically-induced
TG: Animal; Male; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
RN: 0; 0; 0; 0; 13217-66-8; 57-88-5
NM: Adenine-Nucleotides; Amidines; Free-Radicals; Triglycerides;
2,2'-azobis(2-amidinopropane); Cholesterol
AN: 94231737
UD: 9408
TI: Hepatic disease in pregnancy.
AU: Riely-CA
AD: Department of Medicine, University of Tennessee, Memphis 38163.
SO: Am-J-Med. 1994 Jan 17; 96(1A): 18S-22S
ISSN: 0002-9343
PY: 1994
LA: ENGLISH
CP: UNITED-STATES
AB: Liver disease occurring in pregnancy can be categorized into three groups. The first group includes diseases unique to pregnancy and caused by it. Among these are hyperemesis gravidarum, cholestasis of pregnancy, and disorders associated with preeclampsia. Liver involvement may be expected in 50% of patients with hyperemesis gravidarum. Preeclampsia has been associated with both the HELLP syndrome (hemolysis, elevated liver tests, and low platelets), which includes hepatic infarction and rupture, and with acute fatty liver of pregnancy (AFLP). In patients with HELLP syndrome, liver test abnormalities do not correlate with liver injury. Therefore, this and other disorders associated with preeclampsia require aggressive treatment, primarily with delivery. The second group of liver diseases are those exacerbated by pregnancy. Viral infections involving the liver that are usually benign, such as hepatitis E and herpes simplex, are more likely to be exacerbated in pregnant women and are more likely to lead to fulminant hepatic failure. Cholelithiasis and Budd-Chiari syndrome are more prevalent in pregnant women. The third group is comprised of liver diseases that are preexisting in the pregnant patient and includes autoimmune chronic active hepatitis and Wilson's disease. The number of patients in the last group is small, as chronic liver disease is rare in women who are able to bear children.
MESH: Pregnancy-
MESH: *Liver-Diseases; *Pregnancy-Complications
TG: Female; Human
PT: JOURNAL-ARTICLE; REVIEW; REVIEW,-TUTORIAL
AN: 94152653
UD: 9405
SB: AIM
TI: Acute fatty liver of pregnancy: a clinical study of 12 episodes in 11 patients.
AU: Reyes-H; Sandoval-L; Wainstein-A; Ribalta-J; Donoso-S; Smok-G; Rosenberg-H; Meneses-M
AD: Departamento de Medicina, Hospital del Salvador, Santiago, Chile.
SO: Gut. 1994 Jan; 35(1): 101-6
ISSN: 0017-5749
PY: 1994
LA: ENGLISH
CP: ENGLAND
AB: Twelve episodes of acute fatty liver of pregnancy (AFLP) were diagnosed in 11 patients during the past 18 years in a general hospital in Santiago, Chile, with a prevalence of 1 per 15,900 deliveries. Acute fatty liver of pregnancy started between the 31st and 38th weeks of pregnancy, with malaise, vomiting, jaundice, and lethargy as the main clinical manifestations. Polydipsia (in nine episodes) and skin pruritus (in seven episodes) were unusual clinical findings. In two patients, pruritus started two and four weeks before AFLP, suggesting that an intrahepatic cholestasis of pregnancy preceded AFLP in those patients. Considering the current prevalence of both diseases in Chile, their association should be considered fortuitous. In another patient, two consecutive pregnancies were affected by AFLP, raising to three the number of reported patients with recurrent AFLP. In 11 episodes, liver biopsies supported the diagnosis of AFLP by showing small and midsized vacuolar cytoplasmic transformation as the most prominent histopathological feature. Positive intracellular fat staining was found in the four samples analysed. Studies by electron microscopy showed megamitochondria with paracrystalline inclusions in four samples. All the mothers survived, but fetal mortality was 58.3%. Several extrahepatic complications delayed maternal recovery for up to four weeks after delivery. This study confirms an improvement in maternal prognosis in AFLP, discusses the possibility of an epidemiological association with intrahepatic cholestasis of pregnancy, and increases the number of patients reported with recurrent AFLP.
MESH: Acute-Disease; Adolescence-; Adult-; Age-Factors; Chile-epidemiology; Fatty-Liver-epidemiology; Parity-; Pregnancy-; Pregnancy-Complications-epidemiology; Pregnancy-Outcome; Prevalence-; Recurrence-
MESH: *Fatty-Liver-pathology; *Liver-ultrastructure; *Pregnancy-Complications-pathology
TG: Case-Report; Female; Human; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
AN: 94140191
UD: 9405
SB: AIM
TI: Acute fatty liver of pregnancy and long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency.
AU: Treem-WR; Rinaldo-P; Hale-DE; Stanley-CA; Millington-DS; Hyams-JS; Jackson-S; Turnbull-DM
AD: Division of Pediatric Gastroenterology, Hartford Hospital, Farmington, Connecticut 06115.
SO: Hepatology. 1994 Feb; 19(2): 339-45
ISSN: 0270-9139
PY: 1994
LA: ENGLISH
CP: UNITED-STATES
AB: The pathogenesis of acute fatty liver of pregnancy is unknown, but similarities in the clinical presentation and the histological appearance of the liver with those found in children with metabolic defects in the intramitochondrial beta-oxidation pathway of the liver suggest that a disturbance in hepatic fatty acid oxidation may play a role. We report a woman with acute fatty liver of pregnancy who gave birth to a seemingly normal full-term infant who was seen at 4 mo of age with hypoglycemia, coma and profound hepatic steatosis. The infant had a defect in fatty acid oxidation, long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency, and the mother proved to be heterozygous for this metabolic condition. We hypothesize that the interaction of an affected fetus with a female heterozygous for this defect in fatty acid oxidation in the late third trimester accounts for some cases of acute fatty liver of pregnancy.
MESH: Acute-Disease; Adult-; Carnitine-blood; Cells,-Cultured; Child-; Child,-Preschool; Fibroblasts-enzymology; Heterozygote-; Infant-; Lipid-Metabolism,-Inborn-Errors-enzymology; Oxidation-Reduction; Pregnancy-
MESH: *Fatty-Acids-metabolism; *Fatty-Liver-etiology; *Lipid-Metabolism,-Inborn-Errors-genetics; *Pregnancy-Complications-etiology; *3-Hydroxyacyl-CoA-Dehydrogenases-deficiency
TG: Case-Report; Female; Human; Male; Support,-Non-U.S.-Gov't; Support,-U.S.-Gov't,-P.H.S.
PT: JOURNAL-ARTICLE
CN: R01DK43841DKNIDDK; RR00240RRNCRR
RN: EC 1.1.1.35; 0; 541-15-1
NM: 3-Hydroxyacyl-CoA-Dehydrogenases; Fatty-Acids; Carnitine
AN: 94124113
UD: 9405
TI: Acute fatty liver of pregnancy: the hepatologist's view.
AU: Bacq-Y; Riely-CA
AD: Department of Medicine, University of Tennessee, Memphis.
SO: Gastroenterologist. 1993 Dec; 1(4): 257-64
ISSN: 1065-2477
PY: 1993
LA: ENGLISH
CP: UNITED-STATES
AB: Acute fatty liver of pregnancy is a rare clinical entity unique to pregnancy that can lead to hepatic failure and encephalopathy and, if the diagnosis is delayed, to death for the baby and the mother. The characteristic histological picture demonstrates microvesicular fatty infiltration of hepatocytes. Acute fatty liver of pregnancy is a disease of the third trimester of pregnancy. The most significant clinical findings are nausea or vomiting, abdominal pain, jaundice, hepatic encephalopathy, increased transaminase levels, decreased platelet count, increased prothrombin time, and renal failure. Hypertension and proteinuria are common. Liver biopsy is not always necessary for diagnosis but may be useful in atypical cases. The primary therapy is early delivery and supportive care. Both the obstetric team and the medical consultants must have a high index of suspicion for this disease because early delivery is lifesaving and has transformed the prognosis for the mother and the baby. Collaboration between obstetricians and gastroenterologists is necessary to make the diagnosis and also to improve our understanding of this disease of unknown etiology.
MESH: Acute-Disease; Adult-; Diagnosis,-Differential; Pregnancy-; Pregnancy-Outcome
MESH: *Fatty-Liver; *Liver-pathology; *Pregnancy-Complications
TG: Female; Human
PT: JOURNAL-ARTICLE; REVIEW; REVIEW,-TUTORIAL
AN: 94332531
UD: 9411
TI: [Acute fatty liver of pregnancy: 2 clinical cases]
TO: Higado graso agudo del embarazo: dos casos clinicos.
AU: Kottmann-C
AD: Servicio de Obstetricia y Ginecologia, Hospital Parroquial de San Bernardo.
SO: Rev-Chil-Obstet-Ginecol. 1993; 58(2): 131-4
ISSN: 0048-766X
PY: 1993
LA: SPANISH; NON-ENGLISH
CP: CHILE
AB: The clinical evolution and the laboratory pattern of two patients with histopathologically confirmed fatty liver of pregnancy is described. Both babies were delivered vaginally after oxytocic induction and the mothers recovered completely during the puerperium. There was one fetal death in labor secondary to severe abdominal vascular malformation. The other fetus was born healthy.
MESH: Acute-Disease; Adolescence-; Adult-; English-Abstract; Pregnancy-
MESH: *Fatty-Liver-pathology; *Pregnancy-Complications-pathology
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 94269402
UD: 9409
TI: Acute fatty liver of pregnancy after exposure to toluene. A case report.
AU: Paraf-F; Lewis-J; Jothy-S
AD: Department of Pathology, Royal Victoria Hospital, McGill University, Montreal, Canada.
SO: J-Clin-Gastroenterol. 1993 Sep; 17(2): 163-5
ISSN: 0192-0790
PY: 1993
LA: ENGLISH
CP: UNITED-STATES
AB: We describe a patient in whom acute fatty liver of pregnancy developed after long-term exposure. Acute fatty liver of pregnancy was documented by a liver biopsy specimen stained with oil red O and observed under electron microscopy. Although the mother did well, she gave birth to a stillborn whose autopsy showed visceral congestion and placental infarction. This case raises the hypothesis of a possible relationship between toluene exposure and acute fatty liver of pregnancy.
MESH: Acute-Disease; Adult-; Fatty-Liver-pathology; Pregnancy-; Pregnancy-Complications-pathology; Pregnancy-Outcome
MESH: *Fatty-Liver-chemically-induced; *Pregnancy-Complications-chemically-induced;*Toluene-adverse-effects
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
RN: 108-88-3
NM: Toluene
AN: 94014246
UD: 9401
TI: Pulmonary fat emboli associated with acute fatty liver of pregnancy [letter]
AU: Jones-MB
SO: Am-J-Gastroenterol. 1993 May; 88(5): 791-2
ISSN: 0002-9270
PY: 1993
LA: ENGLISH
CP: UNITED-STATES
MESH: Adult-; Fatty-Liver-epidemiology; HELLP-Syndrome-etiology; Pregnancy-;Puerperal-Disorders-etiology; Risk-Factors
MESH: *Embolism,-Fat-etiology; *Fatty-Liver-complications; *Pregnancy-Complications; *Pulmonary-Embolism-etiology
TG: Case-Report; Female; Human
PT: LETTER
AN: 93243390
UD: 9307
TI: Decreased mitochondrial oxidation of fatty acids in pregnant mice: possible relevance to development of acute fatty liver of pregnancy.
AU: Grimbert-S; Fromenty-B; Fisch-C; Letteron-P; Berson-A; Durand-Schneider-AM; Feldmann-G; Pessayre-D
AD: Unite de Recherches de Physiopathologie Hepatique (Institut National de la Sante et de la Recherche Medicale Unite 24, Hopital Beaujon, Clichy, France.
SO: Hepatology. 1993 Apr; 17(4): 628-37
ISSN: 0270-9139
PY: 1993
LA: ENGLISH
CP: UNITED-STATES
AB: Severe impairment of the beta-oxidation of fatty acids, as a consequence of a single factor or a combination of different causes, leads to microvesicular steatosis of the liver. In an effort to understand the mechanism(s) leading to the development of acute fatty liver of pregnancy in some women, we determined the effects of pregnancy on the mitochondrial oxidation of fatty acids in mice. In vivo, the rate of oxidation of the whole fatty-acid chain length was determined by measuring the rate of exhalation of [14C]CO2 after intragastric administration of a tracer dose of [U-14C]palmitic acid. [14C]CO2 exhalation was not significantly decreased at 14 days of gestation, but it had declined by 40% at 18 days of gestation (i.e., 24 to 48 hr before delivery). The rate of first beta-oxidation cycle was assessed by measuring the rate of [14C]CO2 exhalation after administration of [1-14C]octanoic acid, [1-14C]butyric acid or [1-14C]palmitic acid. [14C]CO2 exhalation had declined by 60%, 46%, and 24% after administration of [1-14C]octanoic acid, [1-14C]butyric acid and [1-14C]palmitic acid, respectively, in 18-day-pregnant mice. Total hepatic lipids and triglycerides, expressed per gram of liver, remained unchanged in 18-day-pregnant mice. In vitro, the rate of mitochondrial beta-oxidation (expressed per milligram of protein) had decreased by 47% at 18 days' gestation with [U-14C]palmitic acid as substrate and by 33% with [1-14C]octanoic acid but remained unchanged with [1-14C]palmitic acid. The activity of the tricarboxylic acid cycle, assessed by the formation of [14C]CO2 from [1-14C]acetic acid, had decreased by 24%. We conclude that the mitochondrial oxidation of fatty acids decreased during late-term pregnancy in mice as a consequence of both decreased mitochondrial beta-oxidation of medium-chain fatty acids, and decreased activity of the tricarboxylic acid cycle. We suggest that this effect, in combination with other factors, may contribute to the development of fatty liver of pregnancy in some pregnant women.
MESH: Body-Weight; Carbon-Radioisotopes; Carnitine-Palmitoyltransferase-metabolism; Coenzyme-A-Synthetases-metabolism; Fatty-Acid-Desaturases-metabolism; Fatty-Liver-metabolism; Isoenzymes-metabolism; Liver-anatomy-and-histology; Liver-ultrastructure; Mice-; Mice,-Inbred-ICR; Microscopy,-Electron; Mitochondria,-Liver-ultrastructure; Organ-Weight; Oxidation-Reduction; Pregnancy-; Pregnancy-Complications-metabolism; Radioisotope-Dilution-Technique; Reference-Values
MESH: *Fatty-Acids,-Nonesterified-metabolism; *Fatty-Liver-physiopathology;*Mitochondria,-Liver-metabolism; *Oxygen-Consumption; *Pregnancy-Complications-physiopathology; *Pregnancy,-Animal-metabolism
TG: Animal; Female; Male
PT: JOURNAL-ARTICLE
RN: EC 1.14.99.-; EC 1.3.-; EC 2.3.1.21; EC 6.2.1.; EC 6.2.1.3; 0; 0; 0
NM: Fatty-Acid-Desaturases; medium-chain-acyl-CoA-dehydrogenase; Carnitine-Palmitoyltransferase; Coenzyme-A-Synthetases; acyl-CoA-synthetase; Carbon-Radioisotopes; Fatty-Acids,-Nonesterified; Isoenzymes
AN: 93239114
UD: 9307
TI: Management of haemorrhage in a case of acute fatty liver of pregnancy by internal iliac artery embolisation.
AU: Kirsop-R; Jakubowicz-D
AD: Department Obstetrics and Gynaecology, Royal North Shore Hospital, St Leonard's NSW,Australia.
SO: Br-J-Obstet-Gynaecol. 1992 Dec; 99(12): 1014-6
ISSN: 0306-5456
PY: 1992
LA: ENGLISH
CP: ENGLAND
MESH: Adult-; Angiography-; Iliac-Artery-radiography; Postpartum-Hemorrhage-radiography; Pregnancy-
MESH: *Disseminated-Intravascular-Coagulation-complications; *Embolization,-Therapeutic; *Fatty-Liver-complications; *Labor,-Premature-etiology; *Postpartum-Hemorrhage-therapy; *Pregnancy-Complications,-Hematologic
TG: Case-Report; Female; Human; Support,-Non-U.S.-Gov't
PT: JOURNAL-ARTICLE
AN: 93119975
UD: 9304
SB: AIM
TI: Fulminant hepatic failure in a young mother.
AU: Gane-EJ; Zwi-LJ; Lane-MR
AD: Department of Gastroenterology, Auckland Hospital, New Zealand.
SO: Aust-N-Z-J-Med. 1992 Apr; 22(2): 142-3
ISSN: 0004-8291
PY: 1992
LA: ENGLISH
CP: AUSTRALIA
AB: We report the case of a mother who developed fulminant hepatic failure withhypoglycaemia, coagulopathy, Grade III hepatic encephalopathy, two days after the delivery of her fourth child. She had complained of pruritus for the final two weeks of pregnancy. She received supportive medical management within a critical care unit, and the hepatic failure resolved completely within 48 hours. Liver biopsy confirmed the diagnosis of acute fatty liver of pregnancy. This case is unusual in that this patient deteriorated markedly following delivery, at a time when spontaneous recovery is normally expected.
MESH: Adult-; Fatty-Liver-pathology; Liver-pathology; Pregnancy-; Puerperal-Disorders-pathology
MESH: *Fatty-Liver-diagnosis; *Puerperal-Disorders-diagnosis
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 92411966
UD: 9212
TI: [Acute hepatopathies in pregnancy: diagnosis and therapy]
TO: Akute Hepatopathien in der Schwangerschaft: Diagnostik und Therapie.
AU: Ochs-A
AD: Medizinische Universitatsklinik II, Freiburg.
SO: Schweiz-Rundsch-Med-Prax. 1992 Aug 18; 81(34): 980-2
ISSN: 0369-8394
PY: 1992
LA: GERMAN; NON-ENGLISH
CP: SWITZERLAND
AB: Severe liver disease complicates pregnancy in only 0.1% of the cases. Viral hepatitis is the most common cause (40%). (Liver cirrhosis usually results in amenorrhea). Liver disease unique to pregnancy comprises "intrahepatic cholestasis of pregnancy" (Increased fetal risk), "acute fatty liver of pregnancy" (AFLP) and "HELLP-syndrome", both with high maternal and fetal risk when untreated. AFLP and HELLP-syndrome are diseases of the third trimester and show similar clinical signs of jaundice, coagulopathy and elevated liver enzymes. The immediate termination of pregnancy preferably by Caesarean section has been shown to improve both, maternal and fetal outcome. Imaging methods like ultrasound are invaluable in the differential diagnosis and detection of complications like subcapsular hematoma in the liver patients with
HELLP-syndrome. Fulminant hepatic failure requires intensive care, liver transplantation is an additional therapeutic option. Recurrent AFLP has been reported recently.
MESH: Acute-Disease; English-Abstract; Fatty-Liver-complications; Hemolysis-;Hepatitis,-Viral,-Human-complications; Hyperemesis-Gravidarum-complications;Liver-enzymology; Liver-Diseases-therapy; Pre-Eclampsia-complications; Pregnancy-;Thrombocytopenia-complications
MESH: *Liver-Diseases-diagnosis; *Pregnancy-Complications-diagnosis
TG: Female; Human
PT: JOURNAL-ARTICLE
AN: 92410135
UD: 9212
TI: [Causes of maternal death. A 10-year case analysis]
AU: Lin-MJ
AD: Second Affiliated Hospital, Chongqing Medical Sciences University.
SO: Chung-Hua-Fu-Chan-Ko-Tsa-Chih. 1992 Jan; 27(1): 12-4, 57
ISSN: 0529-567X
PY: 1992
LA: CHINESE; NON-ENGLISH
CP: CHINA
AB: The causes of maternal deaths in our hospital from 1981 to 1989 were analysed. There were 12,819 live births and 6 maternal deaths during this period, a maternal mortality rate of 46.69/per 100,000. The main cause of maternal deaths was acute fatty liver of pregnancy (50%), and next cardiac disease, acute hemorrhagic necrotic pancreatitis and hemorrhage of subarachnoid space (each 16.67%). There was no death due to obstetric hemorrhage, pregnancy induced hypertension syndrome or ectopic pregnancy. It is suggested that needle biopsy of the liver should be done for pregnant women with jaundice of unknown cause. Pregnant women with cardiac disease should be under the care of both obstetrician and internist in collaboration and cesarean section is indicated when the woman's cardiac function remains at grade 3 or 4.
MESH: Adult-; China-epidemiology; English-Abstract; Pregnancy-; Pregnancy-Complications-mortality; Retrospective-Studies; Rheumatic-Heart-Disease-mortality
MESH: *Cause-of-Death; *Fatty-Liver-mortality; *Maternal-Mortality
TG: Female; Human
PT: JOURNAL-ARTICLE
AN: 92371086
UD: 9211
TI: Magnetic resonance imaging and computed tomography scan for the diagnosis of acute fatty liver in pregnancy [letter; comment]
CM: Comment on: Am J Perinatol 1990 Oct;7(4):316-8
AU: Sherer-DM; Abramowicz-JS
SO: Am-J-Perinatol. 1992 Jul; 9(4): 311
ISSN: 0735-1631
PY: 1992
LA: ENGLISH
CP: UNITED-STATES
MESH: Acute-Disease; Pregnancy-; Tomography,-X-Ray-Computed
MESH: *Fatty-Liver-radiography; *Pregnancy-Complications-radiography
TG: Female; Human
PT: COMMENT; LETTER
AN: 92328899
UD: 9210
TI: [The treatment of acute fatty liver of pregnancy using plasma exchange]
TO: Behandlung der akuten Schwangerschaftsfettleber mit Plasmaaustausch.
AU: Glaser-V; Neumann-M
AD: Medizinischen Klinik, Vogtland-Klinikums Plauen.
SO: Z-Geburtshilfe-Perinatol. 1991 Nov-Dec; 195(6): 272-4
ISSN: 0300-967X
PY: 1991
LA: GERMAN; NON-ENGLISH
CP: GERMANY
AB: A 33-year-old gravida 6, para 5, developed acute fatty liver of pregnancy at 35 weeks' gestation. This clinical picture was seen after caesarean section and delivery of a healthy infant. Post partum hepatic dystrophy associated with coma hepatica, acute renal failure and disseminated, intravascular coagulation was successfully treated with three large-volume plasmaphereses using FFP exchange plasma in combination with haemodialysis. The patient survived and her liver function was restored to normal.
MESH: Adult-; Disseminated-Intravascular-Coagulation-etiology; Disseminated-Intravascular-Coagulation-therapy; English-Abstract; Fatty-Liver-complications; Hemodialysis-; Hepatic-Encephalopathy-etiology; Hepatic-Encephalopathy-therapy; Kidney-Failure,-Acute-etiology; Kidney-Failure,-Acute-therapy; Plasma-Exchange; Pregnancy-
MESH: *Fatty-Liver-therapy; *Plasmapheresis-; *Pregnancy-Complications-therapy
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 92133113
UD: 9205
TI: Acute fatty liver of pregnancy.
AU: Mabie-WC
AD: Department of Obstetrics and Gynecology, University of Tennessee, Memphis.
SO: Crit-Care-Clin. 1991 Oct; 7(4): 799-808
ISSN: 0749-0704
PY: 1991
LA: ENGLISH
CP: UNITED-STATES
AB: AFLP is a syndrome that occurs in the last trimester or postpartum, characterized by jaundice, coagulopathy, and central nervous system disturbances. Renal insufficiency is common. Clinical and laboratory findings are nonspecific. Diagnosis is based primarily on histologic examination of the liver. The cause of AFLP is unknown. The illness has been associated with a high maternal and fetal mortality; with increased awareness and more liberal use of liver biopsy, however, milder forms of the disease are now recognized. Some speculate that AFLP is part of the spectrum of preeclampsia. Liver histology shows microvesicular fat and little or no inflammation or hepatocellular necrosis. Treatment consists of expeditious delivery and maximal supportive care, which may include intensive care unit monitoring, blood component therapy, glucose infusion, sodium restriction, diuretic agents, mechanical ventilation, and dialysis. The role of hepatic transplantation in AFLP appears limited. The risk of mortality during AFLP must be compared with the short- and long-term morbidity and mortality associated with liver transplantation.
MESH: Adult-; Biopsy,-Needle; Diagnosis,-Differential; Fatty-Liver-mortality; Fatty-Liver-therapy; Gestational-Age; Infant,-Newborn; Maternal-Mortality; Pregnancy-; Pregnancy-Complications-mortality; Pregnancy-Complications-therapy; Prenatal-Care
MESH: *Fatty-Liver-diagnosis; *Pregnancy-Complications-diagnosis
TG: Female; Human
PT: JOURNAL-ARTICLE; REVIEW; REVIEW,-TUTORIAL
AN: 92083384
UD: 9203
TI: Acute fatty liver of pregnancy associated with preeclampsia: management of hepatic failure with postpartum liver transplantation.
AU: Amon-E; Allen-SR; Petrie-RH; Belew-JE
AD: Department of Obstetrics and Gynecology, Washington University Medical Center, St.Louis, Missouri.
SO: Am-J-Perinatol. 1991 Jul; 8(4): 278-9
ISSN: 0735-1631
PY: 1991
LA: ENGLISH
CP: UNITED-STATES
AB: Acute fatty liver of pregnancy is a potentially fatal disorder. We report a patient complicated by preeclampsia, coagulopathy, encephalopathy, and hepatorenal syndrome successfully managed by postpartum hepatic transplantation.
MESH: Adult-; Blood-Coagulation-Disorders-etiology; Brain-Diseases-etiology; Fatty-Liver-complications; Hepatorenal-Syndrome-etiology; Liver-Diseases-etiology; Liver-Diseases-surgery; Pregnancy-; Puerperium-
MESH: *Fatty-Liver-surgery; *Liver-Transplantation; *Pre-Eclampsia-complications; *Pregnancy-Complications-surgery
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 92075061
UD: 9203
TI: Intrahepatic cholestasis of pregnancy and acute fatty liver of pregnancy. An unusual but favorable association?
AU: Vanjak-D; Moreau-R; Roche-Sicot-J; Soulier-A; Sicot-C
AD: Service de Reanimation et de Medecine d'Urgence, Centre Hospitalier Emile Roux, Eaubonne, France.
SO: Gastroenterology. 1991 Apr; 100(4): 1123-5
ISSN: 0016-5085
PY: 1991
LA: ENGLISH
CP: UNITED-STATES
AB: During the 26th week of a first pregnancy, a 25-year-old woman presented with pruritus suggesting an intrahepatic cholestasis of pregnancy. The pruritus, however, persisted despite the premature delivery of a normal newborn at the 35th week. Moreover, aspartate aminotransferase activity increased, reaching a maximum of 38 times normal level on the 17th day after the delivery. Thus, an acute fatty liver of pregnancy was suspected and confirmed by liver biopsy. This patient appeared to have both intrahepatic cholestasis of pregnancy and acute fatty liver of pregnancy, an association not previously reported. It is suggested that intrahepatic cholestasis of pregnancy caused premature delivery, which in turn may have prevented the onset of severe maternal and fetal complications caused by acute fatty liver of pregnancy.
MESH: Adult-; Cholestasis,-Intrahepatic-diagnosis; Fatty-Liver-diagnosis; Liver-Function-Tests; Pregnancy-; Pruritus-diagnosis; Pruritus-etiology
MESH: *Cholestasis,-Intrahepatic-complications; *Fatty-Liver-complications;*Pregnancy-Complications-diagnosis
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 91160898
UD: 9106
SB: AIM
TI: Recurrent acute fatty liver of pregnancy associated with a fatty-acid oxidatio defect in the offspring.
AU: Schoeman-MN; Batey-RG; Wilcken-B
AD: Department of Medicine, Westmead Hospital, Australia.
SO: Gastroenterology. 1991 Feb; 100(2): 544-8
ISSN: 0016-5085
PY: 1991
LA: ENGLISH
CP: UNITED-STATES
AB: A case of a 29-year-old woman who has had two episodes both clinically and biochemically consistent with acute fatty liver of pregnancy is described. These episodes occurred in two successive pregnancies, and liver biopsy confirmed the diagnosis in the second pregnancy. Both pregnancies were managed by prompt fetal delivery; on both occasions this led to a complete biochemical resolution of the liver function abnormalities. Two healthy babies were delivered by ceasarian sections. This case is of particular importance because a rapidly progressive and devastating illness developed in both infants, leading to death at 6 1/2 and 6 months, respectively. The illness in both babies was characterized by wide-spread fatty infiltration of several vital organs and a failure of any treatment to influence the outcome of that illness. Studies suggested that the illness in the children was caused by a still ill-defined disorder of fatty acid oxidation. The biochemical disorder evidenced in this family is discussed, in an attempt to shed light on the etiology of acute fatty liver of pregnancy.
MESH: Acute-Disease; Adult-; Cesarean-Section; Infant,-Newborn; Lipid-Metabolism,-Inborn-Errors-pathology; Pregnancy-; Recurrence-; Reye's-Syndrome-genetics; Reye's-Syndrome-pathology
MESH: *Fatty-Acids-metabolism; *Fatty-Liver; *Lipid-Metabolism,-Inborn-Errors-genetics; *Pregnancy-Complications
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
RN: 0
NM: Fatty-Acids
AN: 91085814
UD: 9104
SB: AIM
TI: Magnetic resonance imaging and computed tomography scan for the diagnosis of acute fatty liver of pregnancy [see comments]
CM: Comment in: Am J Perinatol 1992 Jul;9(4):311
AU: Farine-D; Newhouse-J; Owen-J; Fox-HE
AD: Department of Obstetrics and Gynecology, Sloane Hospital for Women, New York, New York.
SO: Am-J-Perinatol. 1990 Oct; 7(4): 316-8
ISSN: 0735-1631
PY: 1990
LA: ENGLISH
CP: UNITED-STATES
AB: 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.
MESH: Adult-; Fatty-Liver-complications; Pre-Eclampsia-etiology; Pregnancy-; Purpura,-Thrombotic-Thrombocytopenic-etiology
MESH: *Fatty-Liver-diagnosis; *Magnetic-Resonance-Imaging; *Pregnancy-Complications-diagnosis; *Tomography,-X-Ray-Computed
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 91025259
UD: 9102
TI: Pregnancy-induced hypertension and acute fatty liver of pregnancy: atypical presentations.
AU: Brown-MA; Passaris-G; Carlton-MA
AD: Department of Renal Medicine and Obstetrics, St. George Hospital, Kogarah, Sydney, Australia.
SO: Am-J-Obstet-Gynecol. 1990 Oct; 163(4 Pt 1): 1154-6
ISSN: 0002-9378
PY: 1990
LA: ENGLISH
CP: UNITED-STATES
AB: Severe pregnancy-induced hypertension and markedly elevated levels of serum alkaline phosphatase developed in a 29-year-old-woman in whom one pregnancy resulted in intrauterine death. Acute fatty liver of pregnancy developed with good fetal outcome in the next pregnancy. This case suggests that pregnancy-induced hypertension and acute fatty liver of pregnancy are part of a clinical spectrum. Moreover, classic abnormalities of liver function tests need not be present in acute fatty liver of pregnancy.
MESH: Acute-Disease; Adult-; Alkaline-Phosphatase-blood; Enzyme-Tests; Liver-Function-Tests; Pregnancy-
MESH: *Fatty-Liver-diagnosis; *Hypertension-diagnosis; *Pregnancy-Complications-diagnosis; *Pregnancy-Complications,-Cardiovascular-diagnosis
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
RN: EC 3.1.3.1
NM: Alkaline-Phosphatase
AN: 91022858
UD: 9101
SB: AIM
TI: Computed tomographic and ultrasonographic findings in women with acute fatty liver of pregnancy.
AU: Van-Le-L; Podrasky-A
AD: Department of Obstetrics, Gynecology and Reproductive Sciences, University of California, San Francisco.
SO: J-Reprod-Med. 1990 Aug; 35(8): 815-7
ISSN: 0024-7758
PY: 1990
LA: ENGLISH
CP: UNITED-STATES
AB: Acute fatty liver of pregnancy is associated with complications that can influence antepartum management and necessitate delivery. To date, liver biopsy has been regarded as the "gold standard" for diagnosis. Radiologic evaluation of the liver has been suggested as a less invasive means of diagnosing this entity. Five patients with acute fatty liver of pregnancy underwent ultrasonography; three underwent concomitant computed tomography (CT) of the liver. The liver appeared to be normal in all the patients undergoing CT evaluation and all undergoing ultrasonography except one. Normal liver examinations are not unusual findings in patients with acute fatty liver of pregnancy.
MESH: Biopsy-; Diagnosis,-Differential; Evaluation-Studies; Fatty-Liver-pathology; Fatty-Liver-radiography; Pregnancy-; Pregnancy-Complications-pathology; Pregnancy-Complications-radiography
MESH: *Fatty-Liver-ultrasonography; *Pregnancy-Complications-ultrasonography; *Tomography,-X-Ray-Computed-standards
TG: Female; Human
PT: JOURNAL-ARTICLE
AN: 91012390
UD: 9101
TI: Liver disease associated with pregnancy.
AU: Anday-EK; Cohen-A
AD: University of Pennsylvania School of Medicine, Department of Pediatrics, Philadelphia 19104.
SO: Ann-Clin-Lab-Sci. 1990 Jul-Aug; 20(4): 233-8
ISSN: 0091-7370
PY: 1990
LA: ENGLISH
CP: UNITED-STATES
AB: Pregnancy is associated with altered liver function, particularly in serum enzymes. Anabolic steroids are responsible to some degree in mediating the physiologic and biochemical changes that occur during an uncomplicated pregnancy. However, several liver disorders are unique to pregnancy and include intrahepatic cholestasis of pregnancy, acute fatty liver of pregnancy, and hepatic dysfunction associated with pre-eclampsia and eclampsia. It is imperative for the clinician to diagnose these liver disorders in a timely manner and to institute appropriate management as maternal and fetal outcome are affected in an adverse manner if these conditions are left untreated.
MESH: Cholestasis,-Intrahepatic-complications; Cholestasis,-Intrahepatic-diagnosis; Cholestasis,-Intrahepatic-prevention-and-control; Liver-Diseases-complications; Liver-Diseases-prevention-and-control; Liver-Function-Tests; Pre-Eclampsia complications; Pre-Eclampsia-diagnosis; Pre-Eclampsia-prevention-and-control; Pregnancy-; Pregnancy-Complications-prevention-and-control
MESH: *Liver-Diseases-diagnosis; *Pregnancy-Complications-diagnosis
TG: Female; Human
PT: JOURNAL-ARTICLE; REVIEW; REVIEW,-TUTORIAL
AN: 90386385
UD: 9012
TI: Acute fatty liver of pregnancy.
AU: Obritsch-JM; Cardwell-MS
AD: Department of Obstetrics and Gynecology, University of Missouri School of Medicine, Columbia 65212.
SO: Mo-Med. 1990 Mar; 87(3): 149-51
ISSN: 0026-6620
PY: 1990
LA: ENGLISH
CP: UNITED-STATES
AB: The authors illustrate the importance of early diagnosis, prompt delivery and intensive management of acute fatty liver of pregnancy (AFLP) in achieving the optimal maternal-fetal outcome.
MESH: Acute-Disease; Adult-; Fatty-Liver-diagnosis; Fatty-Liver-therapy; Infant,-Newborn; Pregnancy-; Pregnancy-Complications-diagnosis; Pregnancy-Complications-therapy
MESH: *Fatty-Liver; *Pregnancy-Complications
TG: Case-Report; Female; Human; Male
PT: JOURNAL-ARTICLE; REVIEW; REVIEW,-TUTORIAL
AN: 90190578
UD: 9006
TI: Diagnosis and management of liver disease in pregnancy.
AU: Wilkinson-ML
AD: Gastroenterology Unit, United Medical School, Guy's Hospital, London, England.
SO: Adv-Intern-Med. 1990; 35: 289-310
ISSN: 0065-2822
PY: 1990
LA: ENGLISH
CP: UNITED-STATES
AB: Liver disease in pregnancy is uncommon, acute viral hepatitis being the most frequent. The latter has a normal prognosis in pregnancy, with the possible exception of NANB hepatitis in India and North Africa. Immunization of neonates born of mothers suffering from acute or chronic HBV is essential and effective. Acute fatty liver of pregnancy has a better prognosis than previously thought, perhaps due to diagnosis of milder cases or improved intensive care. Its etiology is still unknown, but metabolic stress may be important. The confusion and overlap of AFLP, the HELLP syndrome, and liver disease of eclampsia suggest common etiological factors. Urgent delivery of the fetus is recommended in AFLP. The related condition of acute liver rupture may be diagnosed by ultrasound. Successful conservative management has been reported. Estrogens are involved in the pathophysiology of ICP, but this does not explain the profound racial differences in incidence. The nature of the sensitivity to estrogens is not understood, although reduced membrane fluidity, which may be counteracted by S-adenosyl-L-methionine, is one possible explanation. The increased fetal loss associated with ICP suggests that treatment should be more energetic than hitherto. In the worst affected individuals, fetal malnutrition secondary to maternal steatorrhea may be an important factor. In general, patients with chronic liver disease have increased maternal and particularly fetal mortality.
MESH: Hepatitis,-Viral,-Human-diagnosis; Hepatitis,-Viral,-Human-therapy; Liver-Diseases-diagnosis; Liver-Diseases-therapy; Pregnancy-; Pregnancy-Complications-diagnosis; Pregnancy-Complications-therapy; Pregnancy-Complications,-Infectious-diagnosis; Pregnancy-Complications,-Infectious-therapy
MESH: *Liver-Diseases; *Pregnancy-Complications
TG: Female; Human
PT: JOURNAL-ARTICLE; REVIEW; REVIEW,-TUTORIAL
AN: 90144278
UD: 9005
TI: Fulminant hepatic failure caused by acute fatty liver of pregnancy treated by orthotopic liver transplantation.
AU: Ockner-SA; Brunt-EM; Cohn-SM; Krul-ES; Hanto-DW; Peters-MG
AD: Department of Medicine, Washington University School of Medicine, St. Louis, Missouri 63110.
SO: Hepatology. 1990 Jan; 11(1): 59-64
ISSN: 0270-9139
PY: 1990
LA: ENGLISH
CP: UNITED-STATES
AB: A previously healthy 35-year-old woman was seen at 37 weeks' gestation with a 10-day history of fever, vomiting, diarrhea and malaise. Serum laboratory findings included elevation of serum bilirubin and AST, prolongation of serum prothrombin time and a positive monospot. A tentative diagnosis of acute fatty liver of pregnancy was made, and a healthy male infant was delivered by emergency cesarean section because of fetal distress. Over the subsequent 3 days, acute progressive oliguric renal failure, disseminated intravascular coagulation, hypoglycemia requiring intravenous dextrose infusion and pancreatitis developed; her mental status progressed to stage III encephalopathy. Quantitative computed tomography estimated the liver volume to be 770 cm3. The decision to proceed with orthotopic liver transplantation was made on the basis of progressive clinical deterioration despite aggressive support and because of her small liver size. After transplant, the patient's multisystem failure rapidly reversed. Histopathological examination of the native liver demonstrated predominantly zone 3 microvesicular steatosis with characteristic ultrastructural changes consistent with acute fatty liver of pregnancy. Southern blot analysis for Epstein-Barr virus DNA was negative. We conclude that orthotopic liver transplantation should be considered for the small group of patients with fulminant hepatic failure associated with acute fatty liver of pregnancy who manifest signs of irreversible liver failure despite delivery of the fetus and aggresive supportive care.
MESH: Acute-Disease; Adult-; Fatty-Liver-complications; Fatty-Liver-pathology; Hepatic-Encephalopathy-etiology; Liver-pathology; Pregnancy-; Pregnancy-Trimester,-Third
MESH: *Fatty-Liver-surgery; *Hepatic-Encephalopathy-surgery;
*Pregnancy-Complications-surgery
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE; REVIEW; REVIEW,-TUTORIAL
AN: 90109045
UD: 9004
TI: Imaging in acute fatty liver of pregnancy. Case report.
AU: Clements-D; Young-WT; Thornton-JG; Rhodes-J; Hayward-C; Hibbard-B
AD: Department of Medicine, University Hospital of Wales, Heath Park, Cardiff.
SO: Br-J-Obstet-Gynaecol. 1990 Jul; 97(7): 631-3
ISSN: 0306-5456
PY: 1990
LA: ENGLISH
CP: ENGLAND
MESH: Adult-; Fatty-Liver-physiopathology; Infant,-Newborn; Liver-Function-Tests; Pregnancy-; Pregnancy-Complications-physiopathology
MESH: *Fatty-Liver-radiography; *Pregnancy-Complications-radiography; *Tomography,-X-Ray-Computed
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 90359884
UD: 9012
SB: AIM
TI: Recurrent acute fatty liver of pregnancy [see comments]
CM: Comment in: Am J Obstet Gynecol 1991 Jun;164(6 Pt 1):1690-1
AU: Barton-JR; Sibai-BM; Mabie-WC; Shanklin-DR
AD: Department of Obstetrics and Gynecology, University of Tennessee, Memphis.
SO: Am-J-Obstet-Gynecol. 1990 Aug; 163(2): 534-8
ISSN: 0002-9378
PY: 1990
LA: ENGLISH
CP: UNITED-STATES
AB: The first reported case of recurrent acute fatty liver of pregnancy confirmed by biopsy is described. In this case a high index of suspicion led to an early diagnosis and intervention with resultant improved maternal and fetal outcome. Electron microscopic examination of a liver biopsy specimen proved more beneficial in confirmation of the diagnosis compared with routine microscopic examination.
MESH: Acute-Disease; Adult-; Biopsy-; Microscopy,-Electron; Pregnancy-; Recurrence-
MESH: *Fatty-Liver-pathology; *Liver-ultrastructure; *Pregnancy-Complications-pathology
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 90350711
UD: 9011
SB: AIM
TI: [Plasma exchange in acute fatty liver of pregnancy]
TO: Plasmaaustausch bei akuter Schwangerschaftsfettleber.
AU: Sohn-U; Muller-K; Adler-K
AD: Klinik fur Innere Medizin, Bereich II - Kuchwald, Bezirkskrankenhauses, Friedrich Wolf, Karl-Marx-Stadt.
SO: Z-Arztl-Fortbild-Jena. 1990; 84(4): 147-50
ISSN: 0044-2178
PY: 1990
LA: GERMAN; NON-ENGLISH
CP: GERMANY,-EAST
MESH: Acute-Disease; Adult-; Cesarean-Section; Hemodialysis-; Hemoperfusion-; Hepatorenal-Syndrome-therapy; Infant,-Newborn; Pregnancy-; Pregnancy-Trimester,-Third; Pregnancy,-Multiple
MESH: *Fatty-Liver-therapy; *Plasma-Exchange; *Pregnancy-Complications-therapy
TG: Case-Report; Female; Human
PT: JOURNAL-ARTICLE
AN: 90246815
UD: 9008