Postpartum Acute Fatty Liver of Pregnancy

A Case Report

Naser Al-Husban; Oqba Al-Kuran; Amal Al Helou

Disclosures

J Med Case Reports. 2018;12(67) 

In This Article

Discussion

In our literature research, we did not come across skin rash preceding or being part of an AFLP presentation. Our patient's skin rash was different from pruritic urticarial papules and plaques of pregnancy because it was neither pruritic nor associated with striae, and it involved both upper limbs and the abdomen.[5]

Initially, the diagnosis of AFLP was suspected because of the abrupt onset of feeling very unwell; abrupt onset of abdominal pain, nausea, and vomiting; and the attacks of severe hypoglycemia. The usual presentation of AFLP is nonspecific.[6] The diagnosis of the condition is suggested by jaundice, mild liver enzyme elevation, elevated WBC, disseminated intravascular coagulation (DIC), and a clinically unwell patient.[6] All these features were very apparent and evident in our patient (raised serum bilirubin, raised blood WBC and DIC). The differential diagnosis includes preeclampsia, HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets), viral hepatitis, and obstetric cholestasis.[3–8] Our patient's BP remained normal prior to delivery and all through her hospital stay until discharge. She had no itching to suggest obstetric cholestasis, and her serum bile acid level was normal (6 μmol/L). Her viral hepatitis screen result was negative.

At an early stage, these patients may have an upper gastrointestinal hemorrhage due to coagulation abnormalities, acute renal failure, infection, pancreatitis, or hypoglycemia.[9,10] Our patient had acute renal failure and persistent hypoglycemia with the need for strict input-output observation and intravenous dextrose infusion. She maintained a normal urine output. The association of transient diabetes insipidus and AFLP appears more common than previously recognized. Both may be part of the spectrum of preeclampsia.[11] Hypoglycemia and prolongation of PT helped us to differentiate our patient's presentation from HELLP syndrome. DIC is relatively common in these cases.[12,13] Our patient received appropriate infusions of FFP and cryoprecipitate. She also developed thrombocytopenia, which is a known complication of AFLP.[14] We obtained both a CT scan and a US scan. Both imaging modalities are noninvasive but have limited usefulness in the AFLP diagnosis.[15]

Our patient's blood laboratory test results showed marked elevation of serum bilirubin and jaundice with only mild liver enzyme elevation. She also had leukocytosis and ascites. These results, in addition to the patient's clinical symptoms and hypoglycemia, were consistent with the diagnosis of AFLP.[16]

The patient went through hemostatic dysfunction in the form of hemolytic anemia and DIC, as indicated by the hematological test results. Hemostatic dysfunction started very early in her condition and persisted for a few days thereafter. In those patients who develop AFLP prior to delivery, this dysfunction persists 4–5 days postpartum.[17] Our patient received infusions of PRBCs, FFP, and cryoprecipitate. Severe cases of AFLP can lead to coagulopathy, liver failure, and hypoglycemia. The pathological hepatic condition is usually self-limiting, with liver function returning to normal 7–9 days after delivery.[18,19] Fluid therapy in our patient was very strict to avoid pulmonary edema caused by low plasma oncotic pressure.

In the ICU, our patient was conscious, alert, and did not need ventilator support. Patients who have received ventilator support or encephalopathy and failed to respond to conventional supportive therapy have benefited from plasma exchange alone or in combination with continuous hemodiafiltration.[20–22] We started our patient on an antibiotic and watched her carefully for the development of any sign of adult respiratory distress syndrome (ARDS). On the basis of her CXR, she needed an intravenous diuretic. ARDS might occur as a complication of acute liver failure, septicemia, or transfusion of multiple blood products.[23]

After the fifth day of the clinical onset of our patient's presentation, she started to show clinical and hematobiochemical improvement. In patients who develop AFLP antenatally, clinical recovery typically is seen within 3–4 days; however, laboratory abnormalities can persist much longer.[24]

Our patient made a quick, uncomplicated recovery. We found a case report of massive intrahepatic calcification.[25] AFLP can progress to fulminant hepatic failure with the need for liver transplant, encephalopathy, coma, and death.[26,27] The clinical manifestations of patients with mutations in enzymes of fatty acid metabolism may include AFLP that may mimic severe preeclampsia.[28]

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