MATERNAL ACUTE FATTY LIVER OF PREGNANCY AND THE ASSOCIATED RISK FOR LONG‐CHAIN 3‐HYDROXYACYL‐COENZYME A DEHYDROGENASE (LCHAD) DEFICIENCY IN INFANTS

@article{Bellig2004MATERNALAF,
  title={MATERNAL ACUTE FATTY LIVER OF PREGNANCY AND THE ASSOCIATED RISK FOR LONG‐CHAIN 3‐HYDROXYACYL‐COENZYME A DEHYDROGENASE (LCHAD) DEFICIENCY IN INFANTS},
  author={L L Bellig},
  journal={Advances in Neonatal Care},
  year={2004},
  volume={4},
  pages={26–32}
}
  • L. Bellig
  • Published 1 February 2004
  • Medicine
  • Advances in Neonatal Care

Figures and Tables from this paper

Role of 3-Hydroxy Fatty Acid-Induced Hepatic Lipotoxicity in Acute Fatty Liver of Pregnancy

The mechanistic role of increased 3-hydroxy fatty acid in causing lipotoxicity to the liver and in inducing oxidative stress, mitochondrial dysfunction and hepatocyte lipoapoptosis in patients with LCHAD deficiency is discussed.

Pregnancy-Specific Liver Disorders: Acute Fatty Liver

Advances in knowledge of pathogenesis, earlier diagnosis, and improvements in both maternal and neonatal critical care have resulted in increased survival rates and further research is still needed to improve maternal and fetal outcomes and decrease the number of deaths due to this rare condition.

Acute Fatty Liver Disease of Pregnancy: Updates in Pathogenesis, Diagnosis, and Management

Although survival rates have improved in the past 30 years, delay in diagnosis and treatment of AFLP has life-threatening consequences; an algorithmic approach to AFLP may be a valuable resource for clinicians.

Acute fatty liver of pregnancy.

  • H. KoE. Yoshida
  • Medicine
    Canadian journal of gastroenterology = Journal canadien de gastroenterologie
  • 2006
Supportive care and expeditious delivery are essential to optimal maternal-fetal outcomes and remain as the mainstay treatment for AFLP.

Liver disease in pregnancy

P preeclampsia itself is the commonest cause of hepatic tenderness and liver dysfunction in pregnancy, and 2%–12% of cases are further complicated by hemolysis (H), elevated liver tests (EL), and low platelet count (LP)—the HELLP syndrome.

Acute Fatty Liver of Pregnancy - A Review:

AFLP is thought to be a rare variant of pre-eclampsia and can be associated with mild to moderate disease and can cause multiple organ failure in case of delayed diagnosis.

Acute Fatty Liver of Pregnancy: A Clinical-Paraclinical Survey

The clinical and paraclinical signs of AFLP were mostly - liver, coagulation, kidney, and hypoglycemia disorders and no patient had fever, ascites, and polydipsia.

A Case Series of Fatal Fulminant Hepatic Failure in Postpartum Females Secondary to Acute Fatty Liver of Pregnancy

  • V. MohanA. Parasher
  • Medicine, Biology
    Journal of Postgraduate Medicine, Education and Research
  • 2021
Two patients who presented to the hospital with near term pregnancies and features of AFLP rapidly worsened postpartum due to persistent bouts of hypoglycemia and rapidly developing fulminant hepatic failure expire a few days after admission.

A prospective study on maternal and fetal outcome in jaundice complicating pregnancy

A prospective study of maternal and fetal outcome included 51 pregnant women with Jaundice admitted in Institute of Obstetrics and Gynaecology during the period 2011 – 2012 and found that jaundice affects a small percentage of pregnant women, yet it takes a major toll on health of both mother and foetus.

Plasmapheresis in the treatment of fatty liver of pregnancy: A case report

Introduction: Acute fatty liver of pregnancy (AFLP) is a rare but lethal disease with liver involvement that appears in the third trimester of pregnancy. There is no specific treatment for AFLP.
...

References

SHOWING 1-10 OF 27 REFERENCES

Acute fatty liver of pregnancy and long‐chain 3‐hydroxyacyl–coenzyme A dehydrogenase deficiency

It is hypothesized that the interaction of an affected fetus with a female heterozygous for this defect in fatty acid oxidation in the late third trimester accounts for some cases of acute fatty liver of pregnancy.

Dietary management of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD). A case report and survey

A diet low in long-chain fatty acids, supplemented with medium-chain triclyceride oil, decreased the incidence of hypoketotic hypoglycaemia, and improved hypotonia, hepatomegaly, cardiomyopathy, and lactic acidosis, while dietary treatment did not appear to effect peripheral neuropathy, pigmentary retinopathy or myoglobinuria.

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: clinical presentation and follow-up of 50 patients.

LCHAD deficiency often presents with a combination of chronic nonspecific symptoms, and survival can be improved by prompt diagnosis, but morbidity remains alarmingly high despite current therapeutic regimes.

3-Hydroxydicarboxylic aciduria due to long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency associated with sudden neonatal death: protective effect of medium-chain triglyceride treatment

Two siblings were found to be affected by longchain 3-hydroxyacyl-CoA dehydrogenase deficiency, one of which died suddenly and unexpectedly on the 3rd day of life suffering from extreme hypoketotic hypoglycaemia and on this regimen a diet enriched in this type of fat was prescribed.

Fetal genotypes and pregnancy outcomes in 35 families with mitochondrial trifunctional protein mutations.

Fetal mitochondrial trifunctional protein defects should be considered a cause for maternal liver disease, preterm labor, and intrauterine growth retardation.

Pathology of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency caused by the G1528C mutation.

  • T. TyniJ. RapolaA. PaetauA. PalotieH. Pihko
  • Medicine
    Pediatric pathology & laboratory medicine : journal of the Society for Pediatric Pathology, affiliated with the International Paediatric Pathology Association
  • 1997
Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency is a recently discovered disorder affecting the mitochondrial beta-oxidation of fatty acids, and the uniform pattern of histopathologic changes facilitates the diagnostics in this severe disorder, allowing opportunities for therapy and prenatal diagnosis.

Hepatic disease in pregnancy.

  • C. Riely
  • Medicine
    The American journal of medicine
  • 1994