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Acute Fatty Liver With Pregnancy Dr. Mohammed Abdalla Egypt

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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 ... – PowerPoint PPT presentation

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Title: Acute Fatty Liver With Pregnancy Dr. Mohammed Abdalla Egypt


1
Acute Fatty Liver With Pregnancy
  • Dr. Mohammed Abdalla
  • Egypt, Domiat General Hospital

2
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

3
Incidence and Characteristics
  • once in every 7,000 to 11,000 deliveries

4
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
199496(1A)18S-22S.   3. Samuels P, Cohen AW.
Pregnancies complicated by liver disease and
liver dysfunction. Obstet Gynecol Clin North Am
199219745-63
5
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

6
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
7
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.)

8
Liver Function Tests
  • albumin level
  • prothrombin time
  • evaluate liver synthetic function (are depressed
    in cirrhosis or severe acute liver disease)

9
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
10
Pathogenesis
  • The etiology is not known precisely.

11
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 ß 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
    2001138585-8.   76. Brackett JC, Sims HF,
    Rinaldo P, et al. Two alpha subunit donor splice
    site mutations cause human trifunctional protein
    deficiency. J Clin Invest 1995952076-82.  

12
CLINICAL PRESENTATION
Symptoms/Signs
  • Vomiting
    80
  • Abdominal pain
    52
  • Jaundice
    93
  • Encephalopathy 87
  • Polydipsia
    80
  • Pruritus
    60
  • Ascitis
    47

13
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 1879, 1989
  •   Cammu H, Velkeniers B, Charels K, et al
    Idiopathic acute fatty liver of pregnancy
    associated with transient diabetes insipidus

14
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.

15
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.

16
ascitis
  • Usually transient and rarely prominent.

17
  • 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
18
LABORATORY FEATURES
  • Liver test abnormalities
  • conjugated hyperbilirubinemia (usually between 5
    and 15 mg/dL)
  • increased alkaline phosphatase (normal lt170)
  • and modest increases in serum aminotransferases
    normal lt50 (usuallylt1000 IU/L)
  • Leukocytosis occurs commonly
  • thrombocytopenia
  • decreased clotting factors
  • Hypoglycemia and renal dysfunction

19
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
    198324265-9.  

20
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
200172193-5.  
21
Complications
  • cerebral edema,
  • renal failure (60),
  • hypoglycemia (53),
  • infections (45)
  • gastrointestinal hemorrhage (33),
  • coagulopathy (30),
  • fetal death
  • severe postpartum hemorrhage

22
  • 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
    123823, 1975  

23
  • renal involvement is less severe than with toxemia

(a mild proteinuria ,mild edema and a mild
increase in blood urea nitrogen and creatinine).
24
  • When renal failure is aggravated, it usually is
    impossible to distinguish from toxemia.

25
  • A severe hypoglycemia often appears at any stage
    of the disease, or even during clinical recovery.

26
  • Ascites, detected clinically or by ultrasound, is
    transient and rarely prominent.

27
  • Maternal mortality (18) usually is attributed
    to one of its complications (gastrointestinal
    hemorrhage, bleeding disorder, renal failure,
    acute pancreatitis) but not to liver failure
    alone.

28
Diagnosis and managment
  • It often is impossible to immediately perform a
    liver biopsy in pregnant patients with severe
    coagulation abnormalities.

next
29
Diagnosis and managment
  • 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
30
Diagnosis and managment
  • the emergency therapeutic decisions usually are
    made without waiting for a histologically proven
    diagnosis.

31
Diagnosis and managment
  • 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
1987106703-6.
32
Diagnosis and managment
  • 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 41839, 1996  

33
Diagnosis and managment
  • The mild jaundice.
  • and modest increase in serum aminotransferases
    are important signs

against
the diagnosis of. fulminant hepatitis (viral or
toxic).
34
Diagnosis and managment
  • the mild increase in blood pressure,
    hyperuricemia, and the intense thirst are

uncommon
in fulminant hepatitis. and they favor the
diagnosis of acute fatty liver of pregnancy.
35
TREATMENT
  • No specific treatment

36
TREATMENT
  • All patients should be hospitalized as soon as
    the diagnosis of AFLP is suspected

37
TREATMENT
  • Moderate or severely affected patients
    (encephalopathic, deeply jaundiced, with a
    prothrombin time less than 40 of the control),
    or with any extrahepatic complications, should be
    attended in intensive care units.

38
TREATMENT
  • it seems convenient to maintain glucose infusions
    . Because of the risk of a sudden hypoglycemia
    until a full metabolic recovery is obtained.

39
TREATMENT
  • 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.

40
TREATMENT
  • Pregnancy termination
  • (yes OR no )

next
41
TREATMENT
  • importance of interrupting pregnancy may seem
    questionable,

next
42
TREATMENT
  • As it noticed in some patients that the disease
    does not immediately improve after delivery

next
43
TREATMENT
But also that no patient has yet been reported
with a recovery before delivery.
next
44
TREATMENT
SO ,a prompt delivery is preferable.
  • 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 35101, 1994  
  • Hou SH, Levin S, Ahola S, et al Acute fatty
    liver of pregnancy. Survival with early cesarean
    section. Dig Dis Sci 29449,1984

45
summary
  • AFLP should be suspected when persistent
    vomiting, malaise, encephalopathy or jaundice
    appear in the final weeks of pregnancy or in the
    early puerperium.

46
summary
  • 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.
47
summary
  • AFLP is a medical and obstetric emergency because
    of the metabolic alterations and complications
    and because of the impending need to interrupt
    pregnancy.

48
summary
  • close surveillance of future pregnancies in
    patients affected previously by this disease is
    recommended.

49
summary
  • an impaired fatty acid metabolism during
    childhood. may affect babies born of pregnancies
    with AFLP.

50
Thank You
  • Dr. Mohammed Abdalla
  • EGYPT, Domiat general hospital
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