Case Study of a Complicated Postpartum Recovery Related to Acute Fatty Liver of Pregnancy

Abstract

Background Acute fatty liver of pregnancy (AFLP) is a fatty acid metabolism disorder that usually occurs when the mother is carrying a male infant and has an associated risk for long‐chain 3‐hydroxyacyl‐coenyme A dehydrogenase (LCHAD) deficiency in the infant. The disease is usually evidenced in the third trimester of pregnancy or postpostpartum but may occur as early as 26 weeks gestation. Researchers have established an AFLP triad: symptoms: nausea/vomiting, jaundice, epigastric pain; laboratory results: renal dysfunction, coagulopathy, liver function abnormalities, hypoglycemia; and complications: renal failure, coagulopathy, ascites, and encephalopathy. Case On Day 2 the woman had a decreased level of consciousness and was “not acting right” according to the nurse. The rapid response team was called, and after a stat head computerized tomography (CT), the woman was transferred to the intensive care unit (ICU). Blood tests at that time revealed low sodium, 124; potassium, 4.8; low chloride, 95; elevated creatinine, 2.3; low glucose, 9; elevated aspartate aminotransferase (AST), 131; elevated alanine transaminase (ALT), 65; elevated alkaline phosphatase, 308; total bilirubin, 9.3; direct bilirubin, 6.1; elevated internationalized normal ratio (INR), 2.6; elevated prothrombin time (PT), 26.4; elevated partial thromboplastin time (PTT), 48.6; elevated ammonia level, 53; low platelets, 52; and low fibrinogen < 50. A physical examination also revealed bilateral scleral icterus with overall jaundice. AFLP was diagnosed, differentiated from hemolysis elevated liver enzymes and low platelets (HELLP) by elevated PT/PTT, INR, and low glucose in addition to the low fibrinogen and elevated ammonia level. The woman also exhibited acute renal failure (ARF) as evidenced by elevated serum creatinine, peripheral edema, and low urine output. Cryoprecipitate and vitamin K were ordered along with serial labs. The woman required intubation and mechanical ventilation due to worsening pulmonary edema. A head CT was ordered due to continued decreased mentation with hepatic encephalopathy. A small subarachnoid hemorrhage (SAH) was noted and neurosurgery and neurology were consulted. A follow‐up magnetic resonance imaging (MRI) was used to further evaluate the SAH and electroencephalogram (EEG) was used to evaluate subclinical seizure activity. The woman was transferred to the neurosurgical ICU for further care and seizure monitoring. The woman remained in status epilepticus with a pentobarbital infusion for 28 days due to encephalopathic state with severe diffuse cerebral dysfunction. Conclusion Interdisciplinary collaboration resulted a full recovery for the woman, and she was discharged on Day 87. Acute fatty liver of pregnancy (AFLP) is a fatty acid metabolism disorder that usually occurs when the mother is carrying a male infant and has an associated risk for long‐chain 3‐hydroxyacyl‐coenyme A dehydrogenase (LCHAD) deficiency in the infant. The disease is usually evidenced in the third trimester of pregnancy or postpostpartum but may occur as early as 26 weeks gestation. Researchers have established an AFLP triad: symptoms: nausea/vomiting, jaundice, epigastric pain; laboratory results: renal dysfunction, coagulopathy, liver function abnormalities, hypoglycemia; and complications: renal failure, coagulopathy, ascites, and encephalopathy. On Day 2 the woman had a decreased level of consciousness and was “not acting right” according to the nurse. The rapid response team was called, and after a stat head computerized tomography (CT), the woman was transferred to the intensive care unit (ICU). Blood tests at that time revealed low sodium, 124; potassium, 4.8; low chloride, 95; elevated creatinine, 2.3; low glucose, 9; elevated aspartate aminotransferase (AST), 131; elevated alanine transaminase (ALT), 65; elevated alkaline phosphatase, 308; total bilirubin, 9.3; direct bilirubin, 6.1; elevated internationalized normal ratio (INR), 2.6; elevated prothrombin time (PT), 26.4; elevated partial thromboplastin time (PTT), 48.6; elevated ammonia level, 53; low platelets, 52; and low fibrinogen < 50. A physical examination also revealed bilateral scleral icterus with overall jaundice. AFLP was diagnosed, differentiated from hemolysis elevated liver enzymes and low platelets (HELLP) by elevated PT/PTT, INR, and low glucose in addition to the low fibrinogen and elevated ammonia level. The woman also exhibited acute renal failure (ARF) as evidenced by elevated serum creatinine, peripheral edema, and low urine output. Cryoprecipitate and vitamin K were ordered along with serial labs. The woman required intubation and mechanical ventilation due to worsening pulmonary edema. A head CT was ordered due to continued decreased mentation with hepatic encephalopathy. A small subarachnoid hemorrhage (SAH) was noted and neurosurgery and neurology were consulted. A follow‐up magnetic resonance imaging (MRI) was used to further evaluate the SAH and electroencephalogram (EEG) was used to evaluate subclinical seizure activity. The woman was transferred to the neurosurgical ICU for further care and seizure monitoring. The woman remained in status epilepticus with a pentobarbital infusion for 28 days due to encephalopathic state with severe diffuse cerebral dysfunction. Interdisciplinary collaboration resulted a full recovery for the woman, and she was discharged on Day 87.