Fatty Liver Disease Of Pregnancy Research Articles

Acute fatty liver disease of pregnancy.

Acute fatty liver disease of pregnancy.

Acute Kidney Injury in Pregnancy.

Acute kidney injury in pregnancy is a public health problem and a significant cause of maternal and fetal morbidity and mortality. The incidence of pregnancy-related acute kidney injury has increased in the developed countries, theorized to be the result of an increase in pregnancies in advanced maternal age, and continues to remain higher in developing countries owing to inadequate antenatal care. While hyperemesis gravidarum is a common cause of pregnancy-related acute kidney injury during the first trimester, complications such as preeclampsia, hemolysis, elevated liver enzymes, and low platelet count syndrome, acute fatty liver disease of pregnancy, thrombotic thrombocytopenic purpura, and hemolytic uremic syndrome are important causes of acute kidney injury later in the pregnancy. Diagnosis of pregnancy-related acute kidney injury can be difficult owing to lack of diagnostic criteria and overlapping clinical features between various causes. General measures to treat pregnancy-related acute kidney injury include identification of the underlying cause of kidney injury, intravenous fluid resuscitation, timely initiation of dialysis if needed, and prompt fetal delivery, if necessary. Specific treatment includes steroid and immunosuppressive therapy for glomerulonephritis; prompt delivery for severe preeclampsia, hemolysis, elevated liver enzymes, and low platelet count syndrome, and acute fatty liver of pregnancy; and plasmapheresis and eculizumab for thrombotic microangiopathies such as thrombotic thrombocytopenic purpura and atypical hemolytic uremic syndrome.

Outcomes of liver transplantation for acute fatty liver disease of pregnancy.

Acute fatty liver of pregnancy (AFLP) often resolves after pregnancy delivery but can progress to acute liver failure necessitating liver transplantation. We performed a retrospective review of the national Scientific Registry of Transplant Recipients (SRTR) data to identify all women in the United States undergoing liver transplantation (LT) for acute liver failure (ALF) from AFLP from 1991 to 2015, and compared to outcomes in women of childbearing age undergoing transplant for ALF from acetaminophen and ALF from other etiologies. Women with AFLP were likely to be on life support at time of LT and had high rates of renal dysfunction (median Cr 2.1, IQR 1.2-2.3), and hyperbilirubinemia (median bilirubin 17.1, IQR 11.0, 19.9). Although their early and late LT survival outcomes were comparable to the other indications for LT, cumulative 5-year graft survival was numerically lower among AFLP patients (54%, 95% CI, 27-76) compared to APAP (70%, 95% CI, 63-77) and "Other ALF" (76%, 95% CI, 72-80) groups. In conclusion, although AFLP is a rare indication for LT, AFLP patients were as sick or sicker than other women of childbearing age undergoing LT for ALF. Worsened graft survival may be related to higher rates of rejection in the AFLP group.

Acute fatty liver of pregnancy: A case report of successfully managed an uncommon catastrophic disease

Acute fatty liver disease of pregnancy has universally high fatality rate. But with modern treatment and early anticipation of diagnosis and complication, and with appropriate supportive management it has improved prognosis.

Acute Fatty Liver Disease of Pregnancy: A Review

Acute fatty liver disease of pregnancy (AFLP) is an uncommon but serious obstetrical emergency, usually occurring during the third trimester. The

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

Acute fatty liver of pregnancy (AFLP) is an obstetric emergency characterized by maternal liver failure and may have complications for the mother and fetus, including death. This review examines recent literature on the epidemiology, pathogenesis, diagnosis, and treatment of acute fatty liver of pregnancy. Pathogenesis of this disease has been linked to defects in fatty acid metabolism during pregnancy, especially in the setting of fetal genetic defects in fatty acid oxidation. The value of screening all patients for these genetic defects remains to be determined. Distinguishing AFLP from other high-risk liver diseases of pregnancy that have overlap features, such as HELLP and preeclampsia, can be challenging. Although sensitive diagnostic tools such as the Swansea criteria have been developed, further work is needed to diagnose AFLP more quickly. 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. Future epidemiological and long-term studies will improve our prediction of women at risk for developing AFLP and determine the long-term consequences of this condition.

Prevalence, Severity, and Impact of Renal Dysfunction in Acute Liver Failure on the US Liver Transplant Waiting List.

Although renal dysfunction is a known complication of acute liver failure (ALF), its frequency, severity, and impact among patients with ALF on the US liver transplant list are not well defined. Organ Procurement and Transplantation data for ALF patients listed as status 1/1A from 2002 to 2012 were analyzed. The frequency and severity of renal dysfunction at the time of listing [the latter was categorized in 5 stages using estimated GFR (eGFR) according to Chronic Kidney Disease Epidemiology Collaboration creatinine 2009 equation] were determined and the association between renal dysfunction and waiting list mortality was assessed using Cox proportional hazard regression analysis. There were a total of 2280 adult patients with ALF, including 56 % with renal dysfunction (defined as eGFR < 60 ml/min/1.73 m(2)) at listing. The highest proportion of patients with renal dysfunction was among those with ALF caused by hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome, fatty liver disease of pregnancy, heat stroke/hyperthermia, hepatitis A virus, and drug-induced liver injury due to acetaminophen APAP, phenytoin, trimethoprim-sulfamethoxazole, and macrolides. Despite the fact that 69 % (468/674) of patients with APAP-induced ALF listed as status 1/1A had renal dysfunction, only 0.9 % underwent simultaneous liver-kidney transplantation. Six-week survival probabilities in patients with ALF on the liver transplant waiting list were 71, 59, 56, 59, and 42 % with renal dysfunction stages of 1, 2, 3, 4, and 5, respectively. Multivariate analysis showed that after controlling for age, etiology of ALF, INR, total bilirubin, and region, the relative risk of death increased progressively as eGFR declined (P < 0.0001). Among patients with ALF on the liver transplant waiting list, renal dysfunction was common (overall prevalence of 56 %). Most importantly, severe renal dysfunction was associated with significantly increased mortality.

A case of acute viral hepatitis interfering with acute fatty liver disease of pregnancy

Acute hepatitis A is a rarely seen infection during preg­nancy. In terms of clinical and laboratory findings, it can be interfere with acute fatty liver disease which can be quite mortal during pregnancy. Since liver function tests are el­evated in both conditions, hepatitis A infection should also be kept in mind in differential diagnosis. We present a 30 year-old pregnant woman with 35 weeks of gestation who presented to our clinic with a suspection of acute fatty liver disease but finally diagnosed as acute hepatitis A in­fection.

Atteinte hépatique gravidique : mode de révélation d’une anomalie de la bêta-oxydation des acides gras chez l’enfant

Acute fatty liver of pregnancy (AFLP) and hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome are serious maternal illnesses occurring in the third trimester of pregnancy with significant perinatal and maternal mortality. AFLP may result from mitochondrial defects in the beta-oxidation of fatty acids, in particular a deficiency of the long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) in the fetus. Clinical findings in AFLP vary and its diagnosis is complicated by a significant overlap in clinical and biochemical features with HELLP syndrome. We report the case of 2 siblings who died, the first one in the neonatal period of asphyxia with multivisceral presentation and the second one from sudden death at 7 months. Autopsy of the latter infant revealed hepatic steatosis associated with cardiomyopathy, which led to suspicion of a fatty acid oxidation deficiency. Mutation analysis demonstrated that both children were homozygous for the common mutation c.1528G>C and the parents were heterozygous for this same mutation. This case demonstrates the importance of screening mothers with acute fatty liver disease of pregnancy and their children at birth for a metabolic disease. This article proposes several metabolic tests for mother and child suspected of having beta-oxidation of a fatty acid disorder.

Pregnancy-associated acute liver disease and acute viral hepatitis: Differentiation, course and outcome

Pregnant women with acute viral hepatitis (VH) and those with pregnancy associated acute liver disease (PAALD) including acute fatty liver disease of pregnancy, hemolysis elevated liver enzyme and low platelet syndrome present with similar clinical features and liver tests abnormalities. Accurate differentiation between the two groups is critical to expedite early delivery in the latter and prevent progressive liver damage. There is scant data in the literature to differentiate between PAALD and VH. We studied the clinical variables, hematological, biochemical and viral serological tests of 87 consecutive pregnant patients with jaundice from 2000 to 2003. There were 46 and 41 patients in PAALD and VH group, respectively. Two-thirds in VH group were due to hepatitis E. Univariate analysis identified hypertension, encephalopathy, oliguria, ascites, serum creatinine, and low platelets as significantly more common in the PAALD group. Multivariate analysis and recursive partitioning identified hypertension and ascites as predictors of PAALD with excellent predictive ability and c value of 0.92. Mortality was 41% in PAALD and 7.5% in VH. Increased bilirubin and oliguria were predictors of mortality in PAALD. Presence of ascites and hypertension differentiates PAALD from VH and should prompt early delivery. Mortality due to hepatitis E is low.

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

Acute fatty liver disease of pregnancy has been recognized as a clinical problem since the 1980s. In the past 8 years, the association of this disease with a genetic inborn error of metabolism in the infant has been recognized. Women who are heterozygous for this disorder are usually asymptomatic until the capacity of their livers to metabolize free fatty acids (FFA) is overwhelmed by a homozygous fetus. The inborn error of metabolism, long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency, may not be immediately recognizable in the infant. Symptoms in the infant are often triggered by an increased long-chain fatty acid load in the diet, or by illness that results in breakdown of endogenous fat. The following case study reviews the clinical pathophysiology of this perinatal health problem and highlights the priorities for the care of infants born to mothers with acute fatty liver disease of pregnancy.

HIV Screening by the Tri-Service Surgical Specialties

A 32-year-old HIV-positive black female underwent 70 days of intensive care after she developed acute fatty liver disease of pregnancy. During her hospitalization, multiple invasive procedures were performed on this patient and many residents, staff, nurses, and technicians were exposed to her body secretions. The patient was identified as HIV positive on a routine screen and denied any risk factors for AIDS. This case served as a catalyst to assess current screening efforts in the military for HIV. A questionnaire was distributed to OB/GYN, general surgery, and urology programs throughout the Army, Navy, and Air Force and the results are presented and discussed.