Jama 1998;280:559

Cause: PIH/preeclampsia

Pathophys: Unknown; overlaps w acute fatty liver of pregnancy (Ann IM 1987;106:703; Nejm 1985;313:367); both may be a manifestation of a mitochondrial fatty acid metabolizing enzyme, which also leads to liver disease in the infants (Jama 2002;288:2163; Nejm 1999;340:1723)

Sx:Onset after 30th (usually >35th) week of pregnancy. Usually none but may have any sx of PIH and/or headache, confusion; fatigue, malaise; nausea and vomiting; abdominal pain, diffuse or right upper quadrant

Si:Edema, hypertension, proteinuria, jaundice, encephalopathy, seizures, coma; small liver; tender right upper quadrant; preeclampsia in all

85% mortality without rx. Does not seem to recur in future pregnancies

Hypoglycemia, DIC and hemorrhage, renal failure, eclampsia, pancreatitis (back pain), fetal and/or maternal death

Lab:

Chem:AST (SGOT) and ALT (SGPT), uric acid, NH₃ all elevated; bilirubin goes up later; hypoglycemia

Genetic testingof family (Nejm 1999;340:1723)

Hem:Wbc >15 000; microangiopathic anemia/DIC picture w nucleated rbc's, rapidly falling platelet counts; elevated hct, PT, PTT, and fibrin split products, and low fibrinogen levels

Path:Liver bx shows easily missed fat in microvesicles with central (not peripheral) nuclei; r/o Reye's and tetracycline hepatotoxicity

Urine:UA shows proteinuria

Rx:Stabilize, stat delivery, transfuse platelets