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    AFLP

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    Bright Kumwenda
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    AFLP

    Uploaded by

    Bright Kumwenda
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    AFLP

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    © © All Rights Reserved

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    AFLP

    Copyright:

    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    6 views50 pages

    AFLP

    Uploaded by

    Bright Kumwenda
    AFLP

    Copyright:

    © All Rights Reserved
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    of 50

    Acute Fatty Liver With

    Pregnancy
    Dr.Mohammed Abdalla
    Egypt. Domiat General Hospital
    Historical points
    • (AFLP) was first identified by Sheehan
    in 1940
    • The name AFLP has replaced
    earlier terminologies, “acute yellow
    atrophy of pregnancy”
    and “acute obstetric fatty metamorphosis
    of liver”
    Incidence and Characteristics

    once in every
    7,000 to 11,000
    deliveries
    Incidence and Characteristics
    • Acute fatty liver of pregnancy most
    frequently complicates the third
    trimester and is commonly associated
    (or complicated ) with preeclampsia (50
    to 100 percent).
    Riely CA. Hepatic disease in pregnancy. Am J Med
    1994;96(1A):18S-22S.

    3. Samuels P, Cohen AW. Pregnancies complicated by


    liver disease and liver dysfunction. Obstet Gynecol Clin
    North Am 1992;19:745-63
    Incidence and Characteristics

    Incidence : 1/7000 -11,000


    Age, (mean, range) 26 (16-39)
    Primiparous (%): 67
    Male baby (%) :60
    Onset week of pregnancy :33% (28-38)
    Mortality (%): ( Maternal )18% - ( Fetal) 47%
    Liver Function Tests
    liver function tests” describes a
    panel of laboratory tests profiling
    discrete aspects of liver function

    No single liver function


    test is available to
    quantify liver disease
    Liver Function Tests
    • aspartate aminotransferase (AST)
    • and alanine aminotransferase (ALT)
    evaluate Liver cell injury or necrosis

    •Marked ALT elevation (viral hepatitis)


    •Moderate ALT elevation (drug-induced
    hepatotoxicity,hyperemesis gravidarum, cholelithiasis,
    HELLP .AFLP.)
    Liver Function Tests

    albumin level
    prothrombin time

    evaluate liver synthetic function


    (are depressed in cirrhosis or
    severe acute liver disease)
    Liver function tests
    alkaline phosphatase,
     bilirubin,
    gamma glutamyl transpeptidase

    evaluate Cholestasis and biliary obstruction

    In normal pregnancies, alkaline phosphatase


    levels may be elevated three- to fourfold,
    secondary to placental alkaline phosphatase
    levels
    Pathogenesis

    The etiology is not


    known precisely.
    Pathogenesis
    •A genetic component has been suggested
    •Recent research suggests that AFLP is
    associated with a Glu474Gln mutation in the
    long-chain 3-hydroxy acyl-coenzyme A
    dehydrogenase (LCHAD), a fatty acid β oxidation
    enzyme.
    •Matern D, Hart P, Murtha AP, Vockley J, Gregersen N,
    Millington DS, et al. Acute fatty liver of pregnancy associated
    with short-chain acyl- coenzyme A dehydrogenase deficiency.
    J Pediatr 2001;138:585-8.
    [76]. Brackett JC, Sims HF, Rinaldo P, et al. Two alpha subunit
    donor splice site mutations cause human trifunctional protein
    deficiency. J Clin Invest 1995;95:2076-82.
    CLINICAL PRESENTATION
    Symptoms/ %
    Signs
    Vomiting 80
    Abdominal pain 52
    Jaundice 93
    Encephalopathy 87
    Polydipsia 80
    Pruritus 60
    Ascitis 47
    ,polydipsia
    with or without polyuria, frequently is
    an early symptom in AFLP.

    •Bourl iere M, Berman J, Ducrotte S, et al: Polyuro-


    polydipsie et steatose hepatique aigue gravidique.
    Discussion a propos d'un cas. J Gynecol Obstet
    Biol Reprod 18:79, 1989
    • Cammu H, Velkeniers B, Charels K, et al:
    Idiopathic acute fatty liver of pregnancy associated
    with transient diabetes insipidus
    ,polydipsia
    The patient may drink 2 or 3 liters
    of liquids overnight. it often
    exceeds the magnitude of
    vomiting. It has been interpreted
    as a transient diabetes insipidus.
    Lethargy and encephalopathy
    • After hours or a few days,
    some patients become
    lethargic and may decline
    into hepatic coma, or milder
    degrees of mental
    impairment.
    ascitis
    Usually transient and
    rarely prominent.
    After delivery, most patients
    improve slowly, and a full
    clinical and laboratory recovery
    may take from 1 to 4 weeks.

    But marked deterioration after


    delivery has been observed
    LABORATORY FEATURES
    • Liver test abnormalities
     conjugated hyperbilirubinemia (usually between
    5 and 15 mg/dL)
     increased alkaline phosphatase (normal <170)
     and modest increases in serum
    aminotransferases normal <50 (usually<1000
    IU/L)
     Leukocytosis occurs commonly
     thrombocytopenia
     decreased clotting factors
     Hypoglycemia and renal dysfunction
    Histopathology
    fatty metamorphosis by liver biopsy:
    The hepatic architecture is intact and the
    lobules are swollen with compressed
    sinusoids
    Centrilobular microvesicular fatty infiltration
    of hepatocytes
    ballooning of hepatocytes

    •Sherlock S. Acute fatty liver of pregnancy and the


    microvesicular fat diseases. Gut 1983;24:265-9.
    Histopathology

    In contrast with viral hepatitis


    and other common causes of
    fulminant hepatic failure,
    necrosis of hepatocytes is
    always minor .
    Vigil-De Gracia P, Lavergne JA. Acute fatty
    liver of pregnancy. Int J Gynaecol Obstet
    2001;72:193-5.
    Complications
    cerebral edema,
    renal failure (60%),
    hypoglycemia (53%),
     infections (45%)
    gastrointestinal hemorrhage (33%),
    coagulopathy (30%),
    fetal death
    severe postpartum hemorrhage
    The upper gastrointestinal hemorrhage may be
    caused by Mallory-Weiss syndrome,
    acute gastric or duodenal lesions (e.g.,
    gastritis, duodenitis, peptic ulcers), or it
    can be a manifestation of a coagulopathy.

    •Cano RI, Delman MR, Pitchumoni CS, et al: Acute fatty liver of pregnancy.
    Complication by disseminated intravascular coagulation
    •Killam AP, Dillard SH, Patton RC, et al: Pregnancy-induced hypertension
    complicated by acute liver disease and disseminated intravascular
    coagulation. Am J Obstet Gynecol 123:823, 1975
    is less severe
    renal involvement
    than with toxemia
    (a mild proteinuria ,mild
    edema and a mild increase
    in blood urea nitrogen and
    creatinine).
    When renal failure is
    aggravated, it usually is
    impossible to distinguish
    from toxemia.
    A severe hypoglycemia often
    appears at any stage of
    the disease, or even
    during clinical recovery.
    detected clinically
    Ascites,
    or by ultrasound, is
    transient and rarely
    prominent.
    (18%) usually is
    Maternal mortality
    attributed to one of its
    complications (gastrointestinal
    hemorrhage, bleeding disorder,
    renal failure, acute pancreatitis)
    but not to liver failure alone.
    It often is impossible to immediately
    perform a liver biopsy in pregnant
    patients with severe coagulation
    abnormalities.
    next
    Therefore, in many cases, it is
    necessary to rely on the clinical
    and laboratory data and, in the
    physician's and obstetrician's
    experience,
    next
    the emergency therapeutic
    decisions usually are made
    without waiting for a
    histologically proven diagnosis.
    Liver biopsy is not
    indicated for
    diagnosis
    Riely CA, Latham PS, Romero R, Duffy TP. Acute
    fatty liver of pregnancy. A reassessment based on
    observations in nine patients. Ann Intern Med
    1987;106:703-6.
    • Ultrasound is most important in the
    exclusion of biliary tract disorders, but its
    value and the value of CT and MR imaging,
    has been considered limited and not
    helpful for the diagnosis and management of
    patients with AFLP.
    •Castro MA, Ouzounian JG, Colletti PM, et
    al: Radiologic studies in acute fatty liver of
    pregnancy. A review of the literature and
    19 new cases. J Reprod Med 41:839, 1996
    The mild jaundice.
    and modest increase in serum
    aminotransferases are important
    signs

    the diagnosis of. fulminant hepatitis


    (viral or toxic).
    the mild increase in blood pressure,
    hyperuricemia, and the intense
    thirst are

    in fulminant hepatitis. and they favor


    the diagnosis of acute fatty liver of
    pregnancy.
    No specific
    treatment
    All patients should be
    hospitalized as
    soon as the diagnosis
    of AFLP is suspected
    Moderate or severely affected patients
    (encephalopathic, deeply jaundiced,
    with a prothrombin time less than
    40% of the control), or with any
    extrahepatic complications, should
    intensive
    be attended in
    care units.
    it seems convenient to
    maintain glucose
    infusions . Because of the
    risk of a sudden hypoglycemia
    until a full metabolic recovery is
    obtained.
    • Two laboratory tests:
    prothrombin time and blood
    glucose, should be repeated at
    least daily, Prothrombin time helps
    to assess the prognosis of liver failure,
    and blood glucose detects a severe
    hypoglycemia.
    Pregnancy termination
    (yes OR no )

    next
    importance of interrupting
    pregnancy may seem
    questionable,
    next
    As it noticed in some patients that the
    disease does not immediately
    improve after delivery

    next
    But also that no patient has
    yet been reported with a
    recovery before delivery.

    next
    •Vanjak D, Moreau R, Roche-Sicot J, et al: Intrahepatic cholestasis
    of pregnancy and acute fatty liver of pregnancy. An unusual but
    favorable association? Gastroenterology 100: 1123, 1991
    • Riely CA: Liver diseases of pregnancy. In Kaplowitz N (ed): Liver
    and biliary diseases, ed 2. Baltimore, Williams & Wilkins, 1996, p
    483
    •Reyes H, Sandoval L, Wainstein A, et al: Acute fatty liver of
    pregnancy: A clinical study of 12 episodes in 11 patients. Gut
    35:101, 1994
    •Hou SH, Levin S, Ahola S, et al: Acute fatty liver of pregnancy.
    Survival with early cesarean section. Dig Dis Sci 29:449,1984
    AFLP should be suspected
    when persistent vomiting,
    malaise, encephalopathy or
    jaundice appear in the final
    weeks of pregnancy or in the
    early puerperium.
    Diagnosis is mainly based
    on clinical and laboratory
    grounds.
    Liver biopsy is usually confirmatory,if done..

    the emergency therapeutic decisions


    usually are made without waiting for a
    histologically proven diagnosis.
    AFLP is a medical and obstetric
    emergency because of the
    metabolic alterations and
    complications and because of
    the impending need to interrupt
    pregnancy.
    close surveillance of future
    pregnancies in patients affected
    previously by this disease is
    recommended.
    an impaired fatty acid metabolism
    during childhood. may affect
    babies born of pregnancies with
    AFLP.
    Thank You
    Dr.Mohammed Abdalla
    EGYPT. Domiat general hospital

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