Management of Acute Fatty Liver of Pregnancy Complicated by Diabetes Insipidus
Diagnosis and Initial Assessment
- Hypoglycemia is a characteristic finding in patients with acute fatty liver of pregnancy (AFLP) 1
- The Swansea criteria should be used for diagnosis of AFLP, with ≥6 criteria highly correlating with clinical diagnosis 1
Management Algorithm
Step 1: Maternal Stabilization and Monitoring
- Patients with AFLP who develop encephalopathy, elevated serum lactate, MELD score >30, or Swansea criteria >7 should be admitted to intensive care 2, 3, 4
- Correct coagulopathy and metabolic derangements before delivery 2, 3, 4
- Monitor for hypoglycemia, which is common in AFLP 1
Step 2: Expedited Delivery
- Delivery should be expedited once coagulopathy and metabolic derangements have been treated 2, 3, 4
- Decisions about mode of delivery should be made jointly by obstetricians, hepatologists, and the multidisciplinary team 2, 3, 4
- Early recognition of AFLP and prompt delivery are critical for achieving good outcomes 5
Step 3: Post-Delivery Management
- Plasma exchange post-delivery may be considered to improve maternal disease severity and decrease recovery time in severe cases 2, 3, 4
- N-acetylcysteine may be considered in women requiring intensive care admission, although evidence is limited 2, 3, 4
Step 4: Liver Transplantation Consideration
- In the subset of women with AFLP who have severe hepatic impairment, early referral to a transplant center should be made 2, 3, 4
- Postpartum transplantation has occasionally been necessary in severe cases 5
Special Considerations
- Newborns of mothers with AFLP should be screened for long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, as there is a strong association between AFLP and this fetal metabolic disorder 1