Acute fatty liver of pregnancy
A rare, serious liver disorder occurring in pregnancy
Acute fatty liver of pregnancy | |
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Synonyms | AFLP |
Pronounce | |
Field | Obstetrics, Perinatology, Hepatology |
Symptoms | Nausea, vomiting, abdominal pain (especially in the right upper quadrant), jaundice, headache, malaise, anorexia, confusion |
Complications | Liver failure, renal failure, hypoglycemia, disseminated intravascular coagulation, fetal distress or death |
Onset | Usually in the third trimester of pregnancy |
Duration | Acute onset, resolves after delivery |
Types | |
Causes | Defective mitochondrial fatty acid oxidation; most commonly due to long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD) in the fetus |
Risks | Multiple pregnancy, first pregnancy, male fetus, carrying a fetus with LCHAD deficiency, previous history of AFLP |
Diagnosis | Clinical presentation, liver function tests, coagulation profile, ultrasound, biopsy (rarely needed), Swansea criteria |
Differential diagnosis | HELLP syndrome, preeclampsia, viral hepatitis, intrahepatic cholestasis of pregnancy, acute pancreatitis |
Prevention | No definitive prevention; genetic counseling may be considered for high-risk cases |
Treatment | Immediate delivery of the baby, supportive care (IV fluids, glucose, blood products) |
Medication | Supportive only; no specific drug treatment |
Prognosis | Good with early diagnosis and prompt delivery; maternal mortality <10% with modern care |
Frequency | Rare (1 in 7,000 to 1 in 20,000 pregnancies) |
Deaths | Maternal and fetal mortality can occur if untreated |
Acute fatty liver of pregnancy (AFLP) is a rare but serious condition characterized by the accumulation of fat in the liver of pregnant women. It typically occurs in the third trimester of pregnancy and can lead to liver failure if not promptly diagnosed and treated. AFLP is considered a medical emergency and requires immediate attention to prevent severe complications for both the mother and the fetus.
Pathophysiology
The exact cause of acute fatty liver of pregnancy is not fully understood, but it is believed to be related to a defect in the metabolism of fatty acids. This defect leads to the accumulation of fat in the liver cells, causing liver dysfunction. Genetic factors, such as mutations in the mitochondrial enzyme long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), have been implicated in the development of AFLP. This enzyme is crucial for the oxidation of fatty acids, and its deficiency can result in the accumulation of toxic metabolites.
Clinical Presentation
AFLP typically presents in the third trimester of pregnancy, often between 30 and 38 weeks of gestation. Symptoms can be nonspecific and may include:
- Nausea and vomiting
- Abdominal pain, particularly in the right upper quadrant
- Jaundice
- Malaise and fatigue
- Anorexia
- Headache
- Confusion or altered mental status
In severe cases, AFLP can progress to liver failure, coagulopathy, renal failure, and encephalopathy.
Diagnosis
The diagnosis of acute fatty liver of pregnancy is primarily clinical, supported by laboratory findings and imaging studies. Key laboratory findings include:
- Elevated liver enzymes (AST, ALT)
- Elevated bilirubin levels
- Hypoglycemia
- Coagulopathy (prolonged prothrombin time)
- Leukocytosis
Imaging studies, such as ultrasound or MRI, may show an enlarged liver with increased echogenicity, but these findings are not specific to AFLP.
Management
The primary treatment for AFLP is the prompt delivery of the fetus, as this is the only definitive cure. Supportive care is also crucial and may include:
- Intravenous fluids and glucose to manage hypoglycemia
- Blood products to correct coagulopathy
- Monitoring and support of renal and hepatic function
In severe cases, admission to an intensive care unit may be necessary. Postpartum, liver function typically improves rapidly after delivery.
Prognosis
With early diagnosis and appropriate management, the prognosis for both mother and child can be favorable. However, delayed treatment can lead to significant maternal and fetal morbidity and mortality. Recurrence in subsequent pregnancies is rare but possible, especially if there is an underlying genetic predisposition.
Related pages
External links
Health science - Medicine - Gastroenterology - edit |
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Diseases of the esophagus - stomach |
Halitosis | Nausea | Vomiting | GERD | Achalasia | Esophageal cancer | Esophageal varices | Peptic ulcer | Abdominal pain | Stomach cancer | Functional dyspepsia | Gastroparesis |
Diseases of the liver - pancreas - gallbladder - biliary tree |
Hepatitis | Cirrhosis | NASH | PBC | PSC | Budd-Chiari | Hepatocellular carcinoma | Acute pancreatitis | Chronic pancreatitis | Pancreatic cancer | Gallstones | Cholecystitis |
Diseases of the small intestine |
Peptic ulcer | Intussusception | Malabsorption (e.g. Coeliac, lactose intolerance, fructose malabsorption, Whipple's) | Lymphoma |
Diseases of the colon |
Diarrhea | Appendicitis | Diverticulitis | Diverticulosis | IBD (Crohn's, Ulcerative colitis) | IBS | Constipation | Colorectal cancer | Hirschsprung's | Pseudomembranous colitis |
Pathology of pregnancy, childbirth, and the puerperium | ||||||||||||
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Inborn error of lipid metabolism: fatty-acid metabolism disorders | ||||||||||||||||||||||||
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