Acute fatty liver of pregnancy

Source: Wikipedia, the free encyclopedia.
Acute fatty liver of pregnancy
Clinical history and physical examination
Liver biopsy (rarely needed)
TreatmentPrompt delivery of the infant,
Intensive supportive care
Liver transplantation
Frequency1 in 7,000 to 1 in 15,000 pregnancies
Deaths18%[1]

Acute fatty liver of pregnancy is a rare life-threatening complication of

intravenous fluids and blood products in anticipation of early delivery has improved prognosis.[4]

Signs and symptoms

Acute fatty liver of pregnancy (or hepatic lipidosis of pregnancy) usually manifests in the third trimester of pregnancy, but may occur any time in the second half of pregnancy, or in the

puerperium, the period immediately after delivery.[1] On average, the disease presents during the 35th or 36th week of pregnancy.[5] The usual symptoms in the mother are non-specific including nausea, vomiting, anorexia (or lack of desire to eat) and abdominal pain; excessive thirst may be the earliest symptom without overlap with otherwise considered normal pregnancy symptoms;[5] however, jaundice and fever may occur in as many as 70% of patients.[1][6]

In patients with more severe disease,

acute kidney failure,[7] hepatic encephalopathy,[8] and pancreatitis.[9] There have also been reports of diabetes insipidus complicating this condition.[10]

Many laboratory abnormalities are seen in acute fatty liver of pregnancy.

international normalized ratio, and decreased fibrinogen.[1][4][5] Frank disseminated intravascular coagulation, or DIC, may occur in as many as 70% of people.[1]

Abdominal

ultrasound.[citation needed
]

Pathophysiology

oxidation
and effects of LCHAD deficiency, a hallmark of acute fatty liver of pregnancy

The understanding of the causes of acute fatty liver of pregnancy has been improved by advances in mitochondrial

heterozygous for the affected mutation.[14]

Diagnosis

The diagnosis of acute fatty liver of pregnancy is usually made on clinical grounds by the treating physician, but differentiation from other conditions affecting the liver may be difficult.[1] The diagnosis of acute fatty liver of pregnancy is suggested by jaundice with a lesser elevation of liver enzymes, elevated white blood cell count, disseminated intravascular coagulation, and a clinically unwell patient.[4]

A liver biopsy can provide a definitive diagnosis,[15] but is not always done, due to the increased chance of bleeding in acute fatty liver of pregnancy.[16] Often testing will be done to exclude more common conditions that present in a similar fashion, including viral hepatitis,[17] pre-eclampsia,[5] HELLP syndrome,[4] intrahepatic cholestasis of pregnancy,[1] and autoimmune hepatitis.[3]

Pathology

If a liver

nucleoside reverse transcriptase inhibitors used to treat HIV,[20] and a rare condition known as Jamaican vomiting sickness which is caused by the eating of the unripened Ackee fruit.[21]

Treatment

Acute fatty liver of pregnancy is best treated in a centre with expertise in hepatology, high-risk obstetrics, maternal-fetal medicine and neonatology. The physicians who treat this condition will often consult with experts in liver transplantation in severe cases. Admission to the intensive care unit is recommended.[1]

Initial treatment involves supportive management with

caesarian section may be needed.[1] Often AFLP is not diagnosed until the mother and baby are in trouble, so it is most likely that an emergency C-section is needed.[citation needed
]

The complications of acute fatty liver of pregnancy may require treatment after delivery, especially if pancreatitis occurs.[9] Liver transplantation is rarely required for treatment of the condition, but may be needed for mothers with severe DIC, those with rupture of the liver, or those with severe encephalopathy.[22]

Epidemiology

Acute fatty liver of pregnancy is a rare condition and occurs in approximately one in 7,000 to one in 15,000 pregnancies.[3][19] The mortality from acute fatty liver of pregnancy has been reduced significantly to 18%, and is now related primarily to complications, particularly DIC (Disseminated Intravascular Coagulation) and infections.[1][3] After delivery, most mothers do well, as the stimulus for fatty acid overload is removed. The disease can recur in future pregnancies, with a calculated genetic chance of 25%; the actual rate is lower, however.[12] Mortality of the foetus has also diminished significantly, but still remains 23%,[23] and may be related to the need for premature delivery.[1]

History

The disease was first described in 1940 by H. L. Sheehan as an "acute yellow atrophy" of the liver, then thought to be related to delayed chloroform poisoning.[1][24]

See also

  • Fatty liver

References

  1. ^
    PMID 16432556
    .
  2. S2CID 29356240
    .
  3. ^
    S2CID 24784165
    .
  4. ^
    S2CID 36694459
    .
  5. ^
    PMID 3296215
    .
  6. PMID 3565968
    .
  7. PMID 12032205
    .
  8. PMID 15025263
    .
  9. ^
    PMID 14981397
    .
  10. S2CID 11869406
    .
  11. PMID 8951135
    .
  12. ^
    PMID 10625076
    .
  13. S2CID 23046057.[permanent dead link
    ]
  14. S2CID 35795159.[permanent dead link
    ]
  15. S2CID 39207958
    .
  16. PMID 8572009
    .
  17. PMID 10609353
    .
  18. PMID 9638907
    .
  19. ^
    PMID 8307428
    .
  20. PMID 12800069
    .
  21. PMID 2177300
    .
  22. PMID 9362370
    .
  23. PMID 15902124
    .
  24. S2CID 72493420
    .

External links

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