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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 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.
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Therefore, in many cases, it is
necessary to rely on the clinical
and laboratory data and, in the
physician's and obstetrician's
experience,
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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 )

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

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

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•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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