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Book of Medical Disorders in Pregnancy - Tintash

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curs <strong>in</strong> 60 to 70 percent <strong>of</strong> subsequent<br />

pregnancies.<br />

Preeclampsia:<br />

Hepatic dysfunction with preeclampsia<br />

has long been recognized. 22 more recently,<br />

this dysfunction has been associated<br />

with other f<strong>in</strong>d<strong>in</strong>gs <strong>in</strong> the HELLP<br />

syndrome. This syndrome may complicate<br />

the course <strong>in</strong> 3 to 10 percent <strong>of</strong><br />

patients with preeclampsia and is noted<br />

<strong>in</strong> 0.1 percent <strong>of</strong> all pregnancies. The<br />

pathophysiology <strong>of</strong> HELLP syndrome<br />

reflects that <strong>of</strong> preeclampsia, with<br />

microvascular damage, platelet activation<br />

and vasospasm. Liver biopsy reveals<br />

periportal hemorrhage and fibr<strong>in</strong> deposition.<br />

25 recent data suggest that a defect <strong>in</strong><br />

nitric oxide metabolism may con-tribute<br />

to preeclampsia and HELLP syndrome.<br />

Notable hepatic abnormalities <strong>in</strong> the<br />

HELLP syndrome <strong>in</strong>clude hemolysis<br />

(with elevated bilirub<strong>in</strong> levels and<br />

lactate dehydrogenase levels greater than<br />

600 IU per L), moderately elevated<br />

transam<strong>in</strong>ase levels (AST and ALT<br />

levels <strong>of</strong> 200 to 700 IU per L) and a<br />

platelet count less than 100,000 per mL<br />

(100 × 10 9 per L). 2,3 Patients typically<br />

present with right upper quadrant pa<strong>in</strong><br />

and malaise. 2,3 Sixty percent <strong>of</strong> patients<br />

exhibit significant weight ga<strong>in</strong> or edema;<br />

50 percent have nausea or emesis. 3 No<br />

correlation has been noted between<br />

extent <strong>of</strong> hypertension, liver function test<br />

abnormalities or liver biopsy f<strong>in</strong>d<strong>in</strong>gs. 25<br />

The maternal and fetal complications <strong>of</strong><br />

HELLP syndrome are significant. The<br />

maternal mortality rate is 2 percent, and<br />

the per<strong>in</strong>atal mortality rate is 33 percent.<br />

Among the hepatic consequences are a 2<br />

percent <strong>in</strong>cidence <strong>of</strong> ruptured liver hematoma<br />

(with frequent concomitant mortality)<br />

and a 4 to 38 percent <strong>in</strong>cidence <strong>of</strong><br />

dissem<strong>in</strong>ated <strong>in</strong>travascular coagulation. 3<br />

119<br />

The most effective treatment for HELLP<br />

syndrome is prompt delivery. 2,3 Postpartum<br />

corticosteroids have proved efficacious<br />

<strong>in</strong> improv<strong>in</strong>g maternal platelet<br />

counts, ALT levels and blood pressure. 28<br />

Therapies that have not proved efficacious<br />

<strong>in</strong>clude plasmapheresis, 29 antithrombotic<br />

agents and immunosuppression.<br />

3 Follow<strong>in</strong>g delivery, laboratory<br />

abnormalities peak <strong>in</strong> the first one to two<br />

days postpartum and return to normal<br />

with<strong>in</strong> three to 11 days. The risk <strong>of</strong> recurrence<br />

<strong>of</strong> HELLP syndrome <strong>in</strong> subsequent<br />

pregnancies has been reported as<br />

3.4 percent.<br />

Acute fatty liver <strong>of</strong> pregnancy:<br />

Acute fatty liver <strong>of</strong> pregnancy most<br />

frequently complicates the third trimester<br />

and is commonly associated with<br />

preeclampsia (50 to 100 percent). 2,3 Although<br />

rare (with an <strong>in</strong>cidence <strong>of</strong> one <strong>in</strong><br />

13,000), acute fatty liver <strong>of</strong> pregnancy is<br />

a life threaten<strong>in</strong>g condition, with an 18<br />

percent maternal and a 23 percent fetal<br />

mortality rate.<br />

Symptoms associated with acute fatty<br />

liver <strong>of</strong> pregnancy <strong>in</strong>clude anorexia,<br />

nausea, emesis, abdom<strong>in</strong>al pa<strong>in</strong>, jaundice,<br />

headache and central nervous system<br />

disturbances. Hepatic histopathology<br />

reveals pericentral micro vesicular<br />

fat with m<strong>in</strong>imal <strong>in</strong>flammation or<br />

necrosis. Liver biopsy is not <strong>in</strong>dicated<br />

for diagnosis. 31 The laboratory<br />

abnormalities <strong>in</strong> acute fatty liver <strong>of</strong><br />

pregnancy <strong>in</strong>clude moderate elevations<br />

<strong>of</strong> transa-m<strong>in</strong>ase levels (AST and ALT<br />

less than 1,000 IU per L), prolongation<br />

<strong>of</strong> prothromb<strong>in</strong> time and partial<br />

thromboplast<strong>in</strong> time, decreased<br />

fibr<strong>in</strong>ogen, renal failure, pr<strong>of</strong>ound<br />

hypoglycemia and bilirub<strong>in</strong> levels <strong>of</strong> 1 to

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