I had a patient with Acute Fatty Liver of Pregnancy the other day. Apparently, while doing some follow up research on this complication in pregnancy, I have found it's quite rare. Here is the info I've compiled so far:
Pathophysiology - idiopathic condition, possibly related to pregnancy hormones. Abnormal hepatic mitochondral function, results in buildup of fat droplets in hepatocytes. Minimal inflammation or necrosis of the liver.
S/S - vomiting, epigastric pain, anorexia, jaundice, headache, CNS disturbances, malaise, encephalopathy, polydypsia - transient diabetes insipidus (early symptom), pruritis, ascites (transient, rarely prominent).
Incidence - varying sources state 1 in 7,000 to 1 in 16,000 pregnancies affected. Almost always occurs in third trimester.
Labs - serum bilirubin increased, PT/INR increased, APTT increased, ALT/AST elevated, platelets decreased (thrombocytopenia), fibrinogen decreased, profound hypoglycemia, serum creatinine increased, alk phos increased, leukocytosis, renal dysfunction.
Recovery - infant needs immediate delivery, may take 1-4 weeks for mother to recover, needs ICU care, may be worse initially after delivery, reversal of hypoglycemia via dextrose infusions. Repeat glucose and PT (assesses progress of liver failure) testing daily.
Complications - cerebral edema, renal failure (milder than toxemia - mild proteinuria, mild edema, mild increase in BUN and creatinine), hypoglycemia, infection, GI hemorrhage (caused by Mallory-Weiss syndrome, coagulopathy, acute gastric or duodenal lesions), coagulopathy, fetal death, postpartum hemorrhage.
Maternal mortality - 18% - usually from complications, but not liver failure alone.
Infant mortality - 23%.
Wow - look at the mortality rates!
The patient I was caring for was already post c/s delivery, post op day 1. She was already diagnosed with preeclampsia, and was on magnesium sulfate therapy. When I received report in the morning, looking at her out of whack coagulation (clotting) studies, my first thought was DIC (disseminated intravascular coagulation) -- and getting this sick woman to the ICU.
Creatinine was hanging around 2.5, uric acid at 8.0, fibrinogen was 65, down from 72 the day before. PT/INR/PTT were all prolonged. Bilirubin level above 5 (normal is under 2). Platelets were 150,000 and her WBC count was over 35,000.
She decompensated as the morning progressed, with a decrease in her neurological condition (DTR's and lethargy), and a decline in her renal function. She did not belong in L&D, and was eventually transferred to the ICU with a tentative diagnosis of Acute Fatty Liver of Pregnancy. She was rapidly heading towards hepatic and renal failure. Her magnesium was shut off right before transfer to the ICU. Care to guess on what her Mag level was, before I turned off the Mag? It was a whopping 11.7!! Therapeutic levels while on mag sulfate therapy is around 6. Normal everyday levels for you and me - under 2.5
The last I heard before I left work was that if her labs continued to worsen, she would be transferred out of state to a different hospital and possibly need a liver transplant.
How was her baby you might ask? In the NICU due to fetal distress that led to her emergency c/s delivery.
This is one patient I might never forget.
Oct 29, '06
Scary indeed. Saw this only once in 9 years. I agree, something you never ever forget. Think that pt wound up in ICU and her newborn shipped to Level 3 NICU. It was years ago....
Oct 29, '06
I haven't had a pt with this condition(yet). I say yet, because in the 3.5 years i've been doing L&D at a high risk facility i've seen some pretty crazy things. I have a question...... How did they diagnose this disease? Its seems many of the alterations in lab values are also consistent with very sick PIH pts. Is there a specific lab or physical exam finding that would pull your thought process away from PIH towards this disease? We have some pretty sick PIHers, I want to be prepared....
Oct 29, '06
We recently had a patient come in for 'itching' at 36 weeks. Some very serious eye rolling going on from nurses - 'can you believe it, the doc actually admitted
her'; 'what a whiner, can't even stand a little itching'....
Turned out to have really whacked liver labs, it was idiopathic hepatacholey something or other. Amnio was immature, gave her betamethasone X2 and waited a week, while she stayed with us, then sectioned her. Baby was transferred for immature lungs and mom did great. She would even put herself on and take herself off the monitor for us during her stay.
Just goes to show ya, don't roll your eyes so quickly. AKA judgemental.