|
1
|
Ramadan MK, Khaza'al J, Cha'ar D, Bazzi Z, Bachnak R, Haibeh P. Second-trimester acute fatty liver disease of pregnancy: A brief review of the literature and a case report. J Obstet Gynaecol Res 2020; 47:34-43. [PMID: 33230970 DOI: 10.1111/jog.14577] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2020] [Revised: 10/20/2020] [Accepted: 11/03/2020] [Indexed: 12/14/2022]
Abstract
Acute fatty liver disease of pregnancy (AFLP) is a rare life-threatening medical emergency unique to pregnancy. It is characterized by progressive microvesicular fatty infiltration of maternal hepatocytes, but the exact etiology has yet to be elucidated. AFLP typically manifests in late third trimester or immediately postpartum and seldom during second trimester. Prompt delivery, irrespective of gestational age or severity, is crucial for arresting the insult and permitting recovery. We hereby report a 21-year-old Lebanese second-gravid woman at 20 weeks' gestation diagnosed with AFLP depending on clinical features and compatible laboratory studies (score of 8 on Swansea criteria), in spite of early occurrence. A review and analysis of early AFLP (second trimester) compared to late (third trimester) was also presented. AFLP appearing during second trimester is as serious as the disease manifesting in late third trimester, with similar diagnostic difficulties, less association with hypertension, but with greater hesitation of obstetricians to affect prompt delivery and higher adverse perinatal outcome due to added effect of premature delivery in second trimester.
Collapse
Affiliation(s)
- Mohamad K Ramadan
- Department of Obstetrics and Gynecology, Rafik Hariri University Hospital, Beirut, Lebanon.,Department of Obstetrics and Gynecology, Lebanese University, Beirut, Lebanon.,Department of Obstetrics and Gynecology, Makassed General Hospital, Beirut, Lebanon
| | - Janoub Khaza'al
- Department of Obstetrics and Gynecology, Rafik Hariri University Hospital, Beirut, Lebanon.,Department of Obstetrics and Gynecology, Lebanese University, Beirut, Lebanon
| | - Dunia Cha'ar
- Department of Obstetrics and Gynecology, Rafik Hariri University Hospital, Beirut, Lebanon.,Department of Obstetrics and Gynecology, Lebanese University, Beirut, Lebanon
| | - Zeinab Bazzi
- Department of Obstetrics and Gynecology, Rafik Hariri University Hospital, Beirut, Lebanon.,Department of Obstetrics and Gynecology, Lebanese University, Beirut, Lebanon
| | - Ronza Bachnak
- Department of Obstetrics and Gynecology, Rafik Hariri University Hospital, Beirut, Lebanon.,Department of Obstetrics and Gynecology, Lebanese University, Beirut, Lebanon
| | - Pierre Haibeh
- Department of Obstetrics and Gynecology, Rafik Hariri University Hospital, Beirut, Lebanon.,Department of Obstetrics and Gynecology, Lebanese University, Beirut, Lebanon
| |
Collapse
|
|
2
|
Baldini F, Portincasa P, Grasselli E, Damonte G, Salis A, Bonomo M, Florio M, Serale N, Voci A, Gena P, Vergani L, Calamita G. Aquaporin-9 is involved in the lipid-lowering activity of the nutraceutical silybin on hepatocytes through modulation of autophagy and lipid droplets composition. Biochim Biophys Acta Mol Cell Biol Lipids 2019; 1865:158586. [PMID: 31816412 DOI: 10.1016/j.bbalip.2019.158586] [Citation(s) in RCA: 20] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/14/2019] [Revised: 11/26/2019] [Accepted: 12/04/2019] [Indexed: 02/06/2023]
Abstract
Hepatic steatosis is the hallmark of non-alcoholic fatty liver disease (NAFLD), the hepatic manifestation of the metabolic syndrome and insulin resistance with potential evolution towards non-alcoholic steatohepatitis (NASH), cirrhosis and hepatocellular carcinoma. Key roles of autophagy and oxidative stress in hepatic lipid accumulation and NAFLD progression are recognized. Here, we employed a rat hepatoma cell model of NAFLD progression made of FaO cells exposed to oleate/palmitate followed or not by TNFα treatment to investigate the molecular mechanisms through which silybin, a lipid-lowering nutraceutical, may improve hepatic lipid dyshomeostasis. The beneficial effect of silybin was found to involve amelioration of the fatty acids profile of lipid droplets, stimulation of the mitochondrial oxidation and upregulation of a microRNA of pivotal relevance in hepatic fat metabolism, miR-122. Silybin was also found to restore the levels of Aquaporin-9 (AQP9) and glycerol permeability while reducing the activation of the oxidative stress-dependent transcription factor NF-κB, and autophagy turnover. In conclusion, silybin was shown to have molecular effects on signaling pathways that were previously unknown and potentially protect the hepatocyte. These actions intersect TG metabolism, fat-induced autophagy and AQP9-mediated glycerol transport in hepatocytes.
Collapse
Affiliation(s)
| | - Piero Portincasa
- Clinica Medica "A. Murri", Dept. of Biomedical Sciences and Human Oncology, Medical School, University of Bari "Aldo Moro", Italy
| | - Elena Grasselli
- DISTAV, Dept. of Earth, Environment and Life Sciences, Italy
| | | | - Annalisa Salis
- DISTAV, Dept. of Earth, Environment and Life Sciences, Italy
| | - Michela Bonomo
- Dept. of Biosciences, Biotechnologies and Biopharmaceutics, University of Bari "Aldo Moro", Italy
| | - Marilina Florio
- Dept. of Biosciences, Biotechnologies and Biopharmaceutics, University of Bari "Aldo Moro", Italy
| | - Nadia Serale
- DISTAV, Dept. of Earth, Environment and Life Sciences, Italy
| | - Adriana Voci
- DISTAV, Dept. of Earth, Environment and Life Sciences, Italy
| | - Patrizia Gena
- Dept. of Biosciences, Biotechnologies and Biopharmaceutics, University of Bari "Aldo Moro", Italy
| | - Laura Vergani
- DISTAV, Dept. of Earth, Environment and Life Sciences, Italy.
| | - Giuseppe Calamita
- Dept. of Biosciences, Biotechnologies and Biopharmaceutics, University of Bari "Aldo Moro", Italy.
| |
Collapse
|
|
3
|
|
|
4
|
Al-Husban N, Al-Kuran O, Al Helou A. Postpartum acute fatty liver of pregnancy: a case report. J Med Case Rep 2018; 12:67. [PMID: 29855383 PMCID: PMC5984337 DOI: 10.1186/s13256-018-1593-3] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/01/2017] [Accepted: 01/27/2018] [Indexed: 12/27/2022] Open
Abstract
Background Acute fatty liver of pregnancy can be a very dramatic clinical event with significant risk of mortality to healthy women. The pathogenesis is still unknown. It usually occurs in the third trimester or in the immediate postpartum period. The clinical presentation is very variable. Medical staff have to be very cautious even regarding a minor complaint of feeling unwell. Skin rash has not been reported as one of the initial presentations of acute fatty liver of pregnancy. It is best treated in a center with a multidisciplinary approach. Admission to the intensive care unit is recommended. Case presentation We report a case of a 20-year-old Middle Eastern Arabic woman who developed an acute fatty liver of pregnancy. She was not known to have any medical disease. She had had two previous uncomplicated deliveries. She developed acute fatty liver of pregnancy on the first day after an uncomplicated normal vaginal delivery of a healthy male newborn. She started to have nonitchy skin rash over her abdomen and upper limbs. Then she started to feel unwell. Twelve hours later, she developed epigastric and right upper quadrant abdominal pain, followed by jaundice, nausea, and vomiting. She developed recurrent hypoglycemic attacks, hemolytic anemia, coagulopathy, and hepatorenal syndrome. Conclusions The clinical presentation of acute fatty liver of pregnancy is very variable and nonspecific. Skin rash can be a new presenting symptom of acute fatty liver of pregnancy. Immediate suspicion of the diagnosis, appropriate investigations, and urgent initiation of therapy in an intensive care unit and by a multidisciplinary team resulted in a good outcome with no adverse health consequences for our patient.
Collapse
Affiliation(s)
- Naser Al-Husban
- Obstetrics & Gynecology Department, Faculty of Medicine, University of Jordan and Jordan University Hospital, P.O. Box 2194, Amman, 11941, Jordan.
| | - Oqba Al-Kuran
- Obstetrics & Gynecology Department, Faculty of Medicine, University of Jordan and Jordan University Hospital, P.O. Box 2194, Amman, 11941, Jordan
| | - Amal Al Helou
- Obstetrics & Gynecology Department, Faculty of Medicine, University of Jordan and Jordan University Hospital, P.O. Box 2194, Amman, 11941, Jordan
| |
Collapse
|
|
5
|
Anon B, Barbet C, Gendrot C, Labarthe F, Bacq Y. [Acute fatty liver of pregnancy and mitochondrial fatty acid oxidation. Consequences for the offspring]. Arch Pediatr 2017. [PMID: 28647472 DOI: 10.1016/j.arcped.2017.05.012] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Acute fatty liver of pregnancy (AFLP) is a rare liver disease unique to pregnancy that can lead to acute liver failure. The prognosis, initially often fatal for both mother and child, has been improved by prompt delivery. The diagnosis should be highly suspected if the mother presents epigastric pain, nausea and/or vomiting, or polyuria-polydipsia in the third trimester of pregnancy. AFLP has been found associated with a genetic deficiency of fatty acid beta-oxidation, which may cause sudden death in infancy. Consequently, the mother and her newborn should undergo screening for this deficiency.
Collapse
Affiliation(s)
- B Anon
- Service d'hépato-gastroentérologie, hôpital Trousseau, CHRU de Tours, 37044 Tours cedex, France.
| | - C Barbet
- Service de pédiatrie, hôpital Clocheville, CHRU de Tours, 37044 Tours cedex, France
| | - C Gendrot
- Laboratoire de biochimie et biologie moléculaire, hôpital Bretonneau, CHRU de Tours, 37044 Tours cedex, France
| | - F Labarthe
- Service de pédiatrie, hôpital Clocheville, CHRU de Tours, 37044 Tours cedex, France
| | - Y Bacq
- Service d'hépato-gastroentérologie, hôpital Trousseau, CHRU de Tours, 37044 Tours cedex, France
| |
Collapse
|
|
6
|
Abstract
Thrombotic thrombocytopenia purpura (TTP) and the hemolytic uremic syndrome (HUS) are rare thrombotic microangiopathies that can be rapidly fatal. Although the acquired versions of TTP and HUS are generally highest on this broad differential, multiple rarer entities can produce a clinical picture similar to TTP/HUS, including microangiopathic hemolysis, renal failure, and neurologic compromise. More recent analysis has discovered a host of genetic factors that can produce microangiopathic hemolytic syndromes. This article discusses the current understanding of thrombotic microangiopathy and outlines the pathophysiology and causative agents associated with each distinct syndrome as well as the most accepted treatments.
Collapse
Affiliation(s)
- Joseph J Shatzel
- Division of Hematology and Medical Oncology, Knight Cancer Institute, Oregon Health & Science University, 3181 Southwest Sam Jackson Park Road, Portland, OR 97239, USA
| | - Jason A Taylor
- Division of Hematology and Medical Oncology, The Hemophilia Center, Portland VA Medical Center, Knight Cancer Institute, Oregon Health & Science University, 3181 Southwest Sam Jackson Park Road, L586, Portland, OR 97239, USA.
| |
Collapse
|
|
7
|
Abstract
Thrombocytopenia is a common laboratory finding in the intensive care unit (ICU) patient. Because the causes can range from laboratory artifact to life-threatening processes such as thrombotic thrombocytopenic purpura (TTP), identifying the cause of thrombocytopenia is important. In the evaluation of the thrombocytopenia patient, one should incorporate all clinical clues such as why the patient is in the hospital, medications the patient is on, and other abnormal laboratory findings. One should ensure that the patient does not suffer from heparin-induced thrombocytopenia (HIT) or one of the thrombotic microangiopathies (TMs). HIT can present in any patient on heparin and requires specific testing and antithrombotic therapy. TMs cover a spectrum of disease ranging from TTP to pregnancy complications and can have a variety of presentations. Management of disseminated intravascular coagulation depends on the patient’s condition and complication. Other causes of ICU thrombocytopenia include sepsis, medication side effects, post-transfusion purpura, catastrophic anti phospholipid antibody disease, and immune thrombocytopenia.
Collapse
|
|
8
|
Abstract
Thrombocytopenia during pregnancy is quite common. Evaluation of blood counts of pregnant women has shown that thrombocytopenia is the second most common haematological problem in pregnancy, after anaemia. While mostly thrombocytopenia has no consequences for either the mother or the foetus, in some cases it is associated with substantial maternal and/or neonatal morbidity and mortality. It may result from a number of diverse aetiologies. Adequate knowledge of these causes will help the clinicians in making proper diagnosis and management of thrombocytopenia in pregnancy. The evaluation of thrombocytopenia is essential to rule out any systemic disorders that may affect pregnancy management as thrombocytopenia can present as an isolated finding or in combination with underlying conditions. In this concise review, we have provided the overview of thrombocytopenia diagnosed during pregnancy.
Collapse
Affiliation(s)
- A Palta
- a Department of Pathology , Government medical college and hospital , Chandigarh , India
| | - P Dhiman
- b Department of Clinical Hematology , Institute of liver and biliary sciences , New Delhi , India
| |
Collapse
|
|
9
|
Hourigan SK, Torbenson M, Tibesar E, Scheimann AO. The full spectrum of hepatic steatosis in children. Clin Pediatr (Phila) 2015; 54:635-42. [PMID: 25567295 DOI: 10.1177/0009922814566927] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
OBJECTIVE We aim to identify the spectrum of etiologies of steatosis in pediatric liver biopsies. METHODS Information was collected from 155 children with steatosis on liver biopsy, including anthropometrics, laboratory, and radiologic data. Biopsies were reviewed by a liver pathologist. RESULTS The 4 major diagnoses associated with hepatic steatosis were nonalcoholic fatty liver disease (37%), metabolic disease (9%), oncologic disease (8%) and viral hepatitis (7%). Patients with nonalcoholic fatty liver disease were older (P = .0001) and more likely to have a body mass index z score >2 (P < .0001). Patients with a metabolic diagnosis were younger (P = .0002). Radiologic imaging of the liver yielded normal results in 44 of 108 of children (30%); 7 of these had >66% macrovesicular fatty change on biopsy, and 3 had severe fibrosis/cirrhosis. CONCLUSIONS The range of causes of steatosis in pediatric liver biopsies is broad. Not all patients, even with advanced liver disease, had abnormalities with liver imaging, emphasizing the role for liver biopsy in certain cases.
Collapse
Affiliation(s)
- Suchitra K Hourigan
- Division of Pediatric Gastroenterology, Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, MD, USA Division of Pediatric Gastroenterology, Pediatric Specialists of Virginia, Fairfax, VA, USA
| | | | - Eric Tibesar
- Division of Pediatric Gastroenterology, Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, MD, USA
| | - Ann O Scheimann
- Division of Pediatric Gastroenterology, Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, MD, USA
| |
Collapse
|
|
10
|
de Oliveira CV, Moreira A, Baima JP, Franzoni LDC, Lima TB, Yamashiro FDS, Coelho KYR, Sassaki LY, Caramori CA, Romeiro FG, Silva GF. Acute fatty liver of pregnancy associated with severe acute pancreatitis: A case report. World J Hepatol 2014; 6:527-531. [PMID: 25068005 PMCID: PMC4110545 DOI: 10.4254/wjh.v6.i7.527] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/12/2014] [Revised: 05/20/2014] [Accepted: 06/11/2014] [Indexed: 02/06/2023] Open
Abstract
Acute fatty liver of pregnancy is a rare disease that affects women in the third trimester of pregnancy. Although infrequent, the disease can cause maternal mortality. The diagnosis is not always clear until the pregnancy is terminated, and significant complications, such as acute pancreatitis, can occur. Pancreatic involvement typically only occurs in severe cases after the development of hepatic and renal impairment. To date, little knowledge is available regarding how the disease causes pancreatitis. Treatment involves supportive measures and pregnancy interruption. In this report, we describe a case of a previously healthy 26-year-old woman at a gestational age of 27 wk and 6 d who was admitted with severe abdominal pain and vomiting. This case illustrates the clinical and laboratory overlap between acute fatty liver of pregnancy and pancreatitis, highlighting the difficulties in differentiating each disease. Furthermore, the hypothesis for this overlapping is presented, and the therapeutic options are discussed.
Collapse
|
|
11
|
Lima TB, Villar CR, Rodrigues MAM, Baima JP, Yamashiro FDS, Franzoni LC, Caramori CA, Silva GF, Romeiro FG, Sassaki LY. Liver biopsy for visceral leishmaniasis diagnosis in pregnancy: report of 2 cases. World J Clin Infect Dis 2013; 3:20-24. [DOI: 10.5495/wjcid.v3.i2.20] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/20/2013] [Revised: 04/18/2013] [Accepted: 04/29/2013] [Indexed: 02/06/2023] Open
Abstract
Visceral leishmaniasis (VL) or kala-azar is a zoonosis caused by intracellular protozoa of the Leishmania genus and is transmitted to humans by the bite of phlebotomine sandflies. It particularly affects cells in the phagocytic mononuclear system, accompanied by disturbances of cellular and humoral immunity. VL is potentially fatal and is characterized by fever, hepatosplenomegaly, diarrhea, epistaxis, jaundice, anemia, leucopenia, thrombocytopenia, hypoalbuminemia and hyperglobulinemia. Diagnostic suspicion is based on epidemiological, clinical and laboratory data and is confirmed by detecting the parasite in infected tissue. Splenic aspiration is the most sensitive method, followed by bone marrow aspiration (BMA) by sternal puncture, liver biopsy and lymph node aspiration; but, due to safety concerns, BMA is the most recommended method. VL is included as a target disease by players in drug research and development. Severe liver dysfunction associated with VL is uncommon. We report two VL cases in pregnant women from Bauru, Sao Paulo state, Brazil, considered an endemic area. The first of them developed hepatic failure due to fulminant hepatitis. In both cases, BMA was unable to find the protozoan; thus, liver biopsy was the only means of making the diagnosis.
Collapse
|
|
12
|
McCrae KR. Thrombocytopenia in Pregnancy. Platelets 2013. [DOI: 10.1016/b978-0-12-387837-3.00044-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
|
|
13
|
Khellaf M, Loustau V, Bierling P, Michel M, Godeau B. [Thrombocytopenia and pregnancy]. Rev Med Interne 2012; 33:446-52. [PMID: 22742709 DOI: 10.1016/j.revmed.2012.05.011] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2012] [Accepted: 05/11/2012] [Indexed: 11/25/2022]
Abstract
The occurrence of thrombocytopenia during pregnancy is frequent (about 10%). Etiologies of thrombocytopenia are dominated by the gestational thrombocytopenia (>75%), which requires no exploration and no specific treatment; it usually occurs during the last trimester of pregnancy and corrects itself spontaneously after delivery. Other etiologies are: (1) immune thrombocytopenia (ITP) either primary or associated with other pathologies; ITP may appear early in the first trimester of pregnancy, (2) thrombotic microangiopathy syndromes, and (3) obstetric thrombocytopenia: eclampsia and HELLP syndrome (hemolysis elevated liver enzymes, and low platelet count). Treatment of pre-eclampsia and HELLP syndrome is based on resuscitative measures and symptomatic fetal extraction that will be discussed according to the term and severity of the case. The treatment of microangiopathy is based on resuscitation and plasma exchange. For ITP, no specific action is needed during pregnancy and only symptomatic patients with a platelet count less than 30×10(9)/L must receive a treatment. It is important to prepare the childbirth that can be vaginally except if there is an obstetric contraindication. A platelet count of 50×10(9)/L is required for the delivery, and of 75×10(9)/L in case of spinal anesthesia. Treatment implies a short course of corticosteroids associated with infusion of immunoglobulins in the most severe forms or in case of steroids resistance. There is a risk of neonatal thrombocytopenia requiring a control of the blood count for the baby at birth and within 5 days, newborns have to be treated if the platelet count is less than 20×10(9)/L.
Collapse
Affiliation(s)
- M Khellaf
- Service de médecine interne, centre de référence des cytopénies auto-immunes de l'adulte, université PARIS XII, hôpital Henri-Mondor, 51 avenue du Maréchal-de-Lattre-de-Tassigny, Créteil, France.
| | | | | | | | | |
Collapse
|
|
14
|
Disseminated intravascular coagulation in obstetric disorders and its acute haematological management. Blood Rev 2009; 23:167-76. [PMID: 19442424 DOI: 10.1016/j.blre.2009.04.002] [Citation(s) in RCA: 100] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
Abstract
As activation of the coagulation pathway is a physiological response to injury, the development of disseminated intravascular coagulation (DIC) is a warning signal to the clinician that the primary pathological disease state is decompensating. In pregnancy, DIC can occur in several settings, which include emergencies such as placental abruption and amniotic fluid embolism as well as complications such as pre-eclampsia. Whilst the acuteness of the event and the proportionality in the coagulant and fibrinolytic responses may vary between these different conditions, a common theme for pregnancy-associated DIC is the pivotal role played by the placenta. Removal of the placenta is the linchpin to treatment in most cases but appropriate blood product support is also key to management. This is necessary because DIC itself can have pathological consequences that translate clinically into a worse prognosis for affected patients. This article will describe how pregnancy-associated DIC can be diagnosed promptly and how treatment should be managed strategically. It also discusses the latest developments in our understanding of haemostatic mechanisms within the placenta and how these may have relevance to new diagnostic approaches as well as novel therapeutic modalities.
Collapse
|
|
15
|
Abstract
Liver diseases in pregnancy may be categorized into liver disorders that occur only in the setting of pregnancy and liver diseases that occur coincidentally with pregnancy. Hyperemesis gravidarum, preeclampsia/eclampsia, syndrome of hemolysis, elevated liver tests and low platelets (HELLP), acute fatty liver of pregnancy, and intrahepatic cholestasis of pregnancy are pregnancy-specific disorders that may cause elevations in liver tests and hepatic dysfunction. Chronic liver diseases, including cholestatic liver disease, autoimmune hepatitis, Wilson disease, and viral hepatitis may also be seen in pregnancy. Management of liver disease in pregnancy requires collaboration between obstetricians and gastroenterologists/hepatologists. Treatment of pregnancy-specific liver disorders usually involves delivery of the fetus and supportive care, whereas management of chronic liver disease in pregnancy is directed toward optimizing control of the liver disorder. Cirrhosis in the setting of pregnancy is less commonly observed but offers unique challenges for patients and practitioners. This article reviews the epidemiology, pathophysiology, diagnosis, and management of liver diseases seen in pregnancy.
Collapse
|
|
16
|
Bacq Y, Assor P, Gendrot C, Perrotin F, Scotto B, Andres C. [Recurrent acute fatty liver of pregnancy]. ACTA ACUST UNITED AC 2008; 31:1135-8. [PMID: 18176373 DOI: 10.1016/s0399-8320(07)78351-3] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2023]
Abstract
Acute fatty liver of pregnancy (AFLP) is a rare liver disease unique to pregnancy potentially fatal for both mother and child. Only a few cases of recurrence have been published. We report a new case. A 27-year-old primiparous patient presented a first episode of AFLP in 1991 at 37 week's gestation. Diagnosis was suspected because of vomiting, thrombocytopenia, and liver function tests abnormalities. It was confirmed by liver ultrasonography and abdominal computed tomography. Clinical and biological improvement was observed after caesarean delivery. Six years later, the woman began a second pregnancy. Liver function tests and complete blood count were regularly checked. At 30 weeks' gestation, recurrent AFLP occurred and caesarean section was performed. Again, diagnosis was confirmed by both ultrasonography and abdominal computed tomography. In 2006, the mother and the two girls, 15 and 8-year-old respectively, were in good health. The study of the HADHA gene, coding alpha subunit long chain 3-hydroxyacyl coenzyme A dehydrogenase (LCHAD) in the patient failed to find mutations, particularly the common mutation c.1528G>C (Glu474-Gln, p.E474Q). In conclusion, after an episode of AFLP, women should be clearly warned of the risk of recurrence and regularly monitored during the next pregnancy, even if the search of HADHA gene mutation is negative.
Collapse
Affiliation(s)
- Yannick Bacq
- Service d'hépatogastroentérologie, Hôpital Trousseau, CHRU de Tours, Tours.
| | | | | | | | | | | |
Collapse
|
|
17
|
Browning MF, Levy HL, Wilkins-Haug LE, Larson C, Shih VE. Fetal fatty acid oxidation defects and maternal liver disease in pregnancy. Obstet Gynecol 2006; 107:115-20. [PMID: 16394048 DOI: 10.1097/01.aog.0000191297.47183.bd] [Citation(s) in RCA: 75] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
OBJECTIVE The objective was to evaluate the relationships between all types of fetal fatty acid oxidation defects and maternal liver disease, including acute fatty liver of pregnancy and hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome. METHODS This was a case-control study comparing fetal fatty acid oxidation defects to the outcome of maternal liver disease. Fifty case infants with fatty acid oxidation defects were identified, with 25 matched controls collected per case. This generated a total of 50 case infants and 1,250 control infants. Pregnancies were evaluated for the presence of maternal liver disease (comprised of acute fatty liver of pregnancy, HELLP syndrome, and preeclampsia evolving into HELLP syndrome) using a conditional logistic regression model. Subgroup analysis compared long chain to short and medium chain fatty acid defects. RESULTS Maternal liver disease was noted in 16.00% of all fatty acid oxidation defect pregnancies compared with 0.88% in the general population (odds ratio 20.4, 95% confidence interval 7.82-53.2). These pregnancies demonstrated an 18.1-fold increase in maternal liver disease when compared with our matched population controls with unaffected fetuses. All classifications of fatty acid oxidation defects were at high risk of developing maternal liver disease. Long chain defects were 50 times more likely than controls to develop maternal liver disease and short and medium chain defects were 12 times more likely to develop maternal liver disease. CONCLUSION Maternal liver disease is significantly higher across the entire spectrum of fatty acid oxidation defects pregnancies compared with the matched control population. Notably, there is significant risk to the pregnancies with fetuses affected with short and medium chain defects, not just those with fetal long chain fatty acid oxidation defects as previously reported. Future studies should examine the pathophysiology of all infant fatty acid oxidation defects and its implications for maternal liver disease for improved future health outcomes. LEVEL OF EVIDENCE II-2.
Collapse
Affiliation(s)
- Marsha F Browning
- Harvard Medical School, Massachusetts General Hospital, Children's Hospital Boston, Brigham and Women's Hospital, MA 02114, USA.
| | | | | | | | | |
Collapse
|
|
18
|
Abstract
Coagulation problems are very common in intensive care patients. It is important to recognize potential problems, perform a rapid assessment, and start therapy. The author reviews general clinical and laboratory approaches to diagnosis and treatment of the bleeding patient and to correction of coagulopathies. This review outlines a set of often catastrophic coagulation problems, which may present both thrombotic and bleeding challenges. These include heparin induced thrombocytopenia, thrombotic thrombocytopenic purpura, and disseminated intravascular coagulation.
Collapse
Affiliation(s)
- Thomas G DeLoughery
- Oregon Health & Science University, Hematology L586, 3181 SW Sam Jackson Park Road, Portland, OR 97201-3098, USA.
| |
Collapse
|
|
19
|
Yücesoy G, Ozkan SO, Bodur H, Cakiroğlu Y, Calişkan E, Ozeren S. Acute fatty liver of pregnancy complicated with disseminated intravascular coagulation and haemorrhage: a case report. Int J Clin Pract 2005:82-4. [PMID: 15875633 DOI: 10.1111/j.1368-504x.2005.00371.x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/29/2022] Open
Abstract
Acute fatty liver of pregnancy (AFLP) is a rare disorder of unknown aetiology that is diagnosed typically in the third trimester or early postpartum period. The incidence is estimated to be 1/6692-1/13,328. The obstetric team must have a high index of suspicion of this pathology, particularly in the presence of clinical and laboratory findings, such as nausea, vomiting, jaundice, increased serum transaminase levels, increased prothrombin time and hypoglycaemia. Early diagnosis followed by prompt delivery and supportive care provides significantly improved maternal and perinatal outcome. Delay in diagnosis of this obstetric emergency may lead to rapid progression to hepatic failure, disseminated intravascular coagulation (DIC), haemorrhage, encephalopathy, multiple organ failure and finally death. The case of a 34-year-old woman, gravida 3, para 2, with AFLP complicated with DIC is presented herein with a review of literature and discussion of its origin.
Collapse
Affiliation(s)
- G Yücesoy
- Department of Obstetrics and Gynecology, University of Kocaeli, School of Medicine, Kocaeli, Turkey.
| | | | | | | | | | | |
Collapse
|
|
20
|
Abstract
Liver dysfunction during pregnancy can be caused by conditions that are specific to pregnancy or by liver diseases that are not related to pregnancy itself. This review attempts to summarize the epidemiology, pathophysiology, and management of the different pregnancy-related liver diseases, and to review different liver diseases not related to pregnancy and how they may affect or be effected by pregnancy. Some of the liver diseases specific to pregnancy can cause significant morbidity and mortality both to the mother and to the fetus, while most of the liver diseases not specific to pregnancy do not have a deleterious effect on the pregnancy itself.
Collapse
Affiliation(s)
- Fabiana S Benjaminov
- Department of Gastroenterology, Meir Medical Center, University of Tel-Aviv, Kfar-Saba, Israel.
| | | |
Collapse
|
|
21
|
|
|
22
|
Abstract
Thrombocytopenia in pregnant women may result from a number of diverse etiologies. While some of these are not associated with adverse pregnancy outcomes, others are associated with substantial maternal and/or neonatal morbidity and mortality. However, specific therapies, if instituted promptly, may significantly improve the outcomes of affected patients and their offspring. Since the clinical features of many of these disorders often overlap, identifying a specific cause of thrombocytopenia in a pregnant patient may be difficult. However, through familiarity with the more common clinical and laboratory features of each of these disorders, accurate diagnosis may be achieved, and appropriate treatment instituted in most cases. In this review, we discuss the differential diagnosis of the more common causes of pregnancy-associated thrombocytopenia, and provide an overview of approaches to hematologic management.
Collapse
Affiliation(s)
- Keith R McCrae
- Hematology-Oncology, BRB3, Case Western Reserve University School of Medicine and University Hospitals of Cleveland, 10900 Euclid Avenue, OH 44106-4937, USA.
| |
Collapse
|
|
23
|
Watanabe S, Uchida N, Masaki T, Kurokohchi K, Toge T, Kuriyama S, Ohnishi Y, Tanaka H, Kurose T, Hata T. Measurement of left portal vein blood-flow velocity by Doppler sonography in a patient with acute fatty liver of pregnancy. J Med Ultrason (2001) 2003; 30:57-61. [PMID: 27285156 DOI: 10.1007/bf02485171] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2002] [Accepted: 07/04/2002] [Indexed: 11/30/2022]
Abstract
Acute fatty liver of pregnancy was diagnosed in a 32-year-old primipara in her 34th week of gestation. The pulsed Doppler sonogram showed that mean maximum portal blood-flow velocity obtained at the umbilical-point of the left portal vein (the U-point) decreased during the acute phase of the disease and returned to the normal range accompanied by improvement in her condition. Findings of portal blood dynamics obtained by pulsed Doppler sonography and those of the hyperechoic hepatic parenchyma obtained by conventional sonography could prove useful in following up patients in their third trimester of pregnancy.
Collapse
Affiliation(s)
- Seishiro Watanabe
- Third Department of Internal Medicine, Kagawa Medical University, 1750-1 Miki-cho, 761-0701, Kita-gun, Kagawa, Japan
| | - Naohito Uchida
- Third Department of Internal Medicine, Kagawa Medical University, 1750-1 Miki-cho, 761-0701, Kita-gun, Kagawa, Japan
| | - Tsutomu Masaki
- Third Department of Internal Medicine, Kagawa Medical University, 1750-1 Miki-cho, 761-0701, Kita-gun, Kagawa, Japan
| | - Kazutaka Kurokohchi
- Third Department of Internal Medicine, Kagawa Medical University, 1750-1 Miki-cho, 761-0701, Kita-gun, Kagawa, Japan
| | - Tetsuo Toge
- Third Department of Internal Medicine, Kagawa Medical University, 1750-1 Miki-cho, 761-0701, Kita-gun, Kagawa, Japan
| | - Shigeki Kuriyama
- Third Department of Internal Medicine, Kagawa Medical University, 1750-1 Miki-cho, 761-0701, Kita-gun, Kagawa, Japan
| | - Yooichi Ohnishi
- Department of Perinatology and Gynecology, Kagawa Medical University, 1750-1 Miki-cho, 761-0701, Kita-gun, Kagawa, Japan
| | - Hirokazu Tanaka
- Department of Perinatology and Gynecology, Kagawa Medical University, 1750-1 Miki-cho, 761-0701, Kita-gun, Kagawa, Japan
| | - Takaaki Kurose
- Department of Perinatology and Gynecology, Kagawa Medical University, 1750-1 Miki-cho, 761-0701, Kita-gun, Kagawa, Japan
| | - Toshiyuki Hata
- Department of Perinatology and Gynecology, Kagawa Medical University, 1750-1 Miki-cho, 761-0701, Kita-gun, Kagawa, Japan
| |
Collapse
|
|
24
|
Rakheja D, Bennett MJ, Rogers BB. Long-chain L-3-hydroxyacyl-coenzyme a dehydrogenase deficiency: a molecular and biochemical review. J Transl Med 2002; 82:815-24. [PMID: 12118083 DOI: 10.1097/01.lab.0000021175.50201.46] [Citation(s) in RCA: 30] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
Since the first report of long-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency a little more than a decade ago, its phenotypic and genotypic heterogeneity in individuals homozygous for the enzyme defect has become more and more evident. Even more interesting is its association with pregnancy-specific disorders, including preeclampsia, HELLP syndrome (hemolysis, elevated liver enzymes, low platelets), hyperemesis gravidarum, acute fatty liver of pregnancy, and maternal floor infarct of the placenta. In this review we discuss the biochemical and molecular basis, clinical features, diagnosis, and management of long-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency.
Collapse
Affiliation(s)
- Dinesh Rakheja
- Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas 75390, USA.
| | | | | |
Collapse
|
|
25
|
Affiliation(s)
- P Vigil-De Gracia
- Gynecology and Obstetric Unit (Obstetric intensive care), Complejo Hospitalario Metropolitano de la Caja de Seguro Social, Panamá, Panamá.
| | | |
Collapse
|
|
26
|
Affiliation(s)
- S Suzuki
- Department of Obstetrics and Gynaecology, Nippon Medical School, Tokyo, Japan
| | | | | |
Collapse
|
|
27
|
Suzuki S, Watanabe S, Araki T. Acute fatty liver of pregnancy at 23 weeks of gestation. ACTA ACUST UNITED AC 2001. [DOI: 10.1016/s0306-5456(00)00028-0] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
|
|
28
|
McCrae KR, Bussel JB, Mannucci PM, Remuzzi G, Cines DB. Platelets: an update on diagnosis and management of thrombocytopenic disorders. HEMATOLOGY. AMERICAN SOCIETY OF HEMATOLOGY. EDUCATION PROGRAM 2001; 2001:282-305. [PMID: 11722989 DOI: 10.1182/asheducation-2001.1.282] [Citation(s) in RCA: 41] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/23/2023]
Abstract
Thrombocytopenia in the pregnant patient may result from a number of causes, most of which involve either immune-mediated platelet destruction or platelet consumption. Many of these disorders share clinical and laboratory features, making accurate diagnosis difficult. Moreover, uterine evacuation is indicated in the therapy of some disorders, while in others alternative interventions may allow the pregnancy to be carried to term. These and other issues are discussed as part of a comprehensive review of the differential diagnosis and management of thrombocytopenia in pregnancy. The term "refractory ITP" is used with reference to two distinct groups of patients: 1) patients in whom the platelet count cannot be easily increased, including those who are poorly responsive to initial single agent treatment, and 2) those with persistent thrombocytopenia despite the use of conventional therapies. An approach to management of the former group will be presented, followed by a discussion of patients with chronic refractory ITP. The latter will include presentation of new data on the role of Helicobacter pylori in ITP and whether its treatment ameliorates thrombocytopenia, as well as the use of rituximab and other modalities. Thrombotic microangiopathies such as thrombotic thrombocytopenic purpura (TTP) are rare, but life threatening causes of thrombocytopenia. Ultra-large multimers of von Willebrand factor (vWF) aggregate platelets intravascularly, and congenital or immune-mediated deficiencies of a metalloprotease that cleaves these ultra-large multimers may cause TTP. However, little information exists concerning the behavior of this protease in other physiological and pathological conditions. Levels of this protease have now been measured in healthy individuals of different ages, full-term newborns, pregnant women and a patients with variety of pathologic conditions, and these data will be reviewed herein. Heparin-induced thrombocytopenia/thrombosis (HIT/T) remains the most common antibody-mediated, drug-induced thrombocytopenic disorder, and a leading cause of morbidity and mortality. Based on clinical correlations and murine models, there is increasing evidence that antibodies to complexes between platelet factor 4 (PF4) and heparin cause HIT/T, and the molecular composition of the relevant antigen has also become better defined. However, the introduction of sensitive ELISAs to measure anti-PF4/heparin antibodies has complicated diagnosis in some settings in which the incidence of such antibodies in unaffected patients exceeds the incidence of the disease. In addition, the FDA approval of Lepirudin and Argatroban has expanded the repertoire of agents available for therapy of HIT/T and may change the approach to management of asymptomatic patients with thrombocytopenia. However, the optimal use of these drugs in commonly encountered settings remains in evolution, and a need for alternative approaches to prevention and treatment is evident.
Collapse
Affiliation(s)
- K R McCrae
- Department of Hematology/Oncology, Case Western Reserve University, School of Medicine, Cleveland, OH 44107-4937, USA
| | | | | | | | | |
Collapse
|
|
29
|
Brown MA, Hague WM, Higgins J, Lowe S, McCowan L, Oats J, Peek MJ, Rowan JA, Walters BN. The detection, investigation and management of hypertension in pregnancy: full consensus statement. Aust N Z J Obstet Gynaecol 2000; 40:139-55. [PMID: 10925900 DOI: 10.1111/j.1479-828x.2000.tb01137.x] [Citation(s) in RCA: 218] [Impact Index Per Article: 8.7] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Affiliation(s)
- M A Brown
- Australasian Society for the Study of Hypertension in Pregnancy, Sydney NSW, Australia
| | | | | | | | | | | | | | | | | |
Collapse
|
|
30
|
Abstract
Intrahepatic cholestasis of pregnancy is one of the primary disorders of the liver that adversely affects maternal well-being and fetal outcome. Early identification of this condition, careful interdisciplinary monitoring, and prompt delivery at fetal maturity can improve outcomes in the mother and child. Although the cause is unclear, IHCP probably arises from a genetic predisposition for increased sensitivity to estrogens and progestogens and altered membrane composition and expression of bile ducts, hepatocytes, and canalicular transport systems. As a result, the elevations in maternal levels of bile acids and their molar ratios seen in healthy pregnancy rise further in IHCP patients. Also, as the normal fetal-to-maternal transfer of bile acids across the trophoblast is impaired, the excess bile acids with abnormal profiles accumulate and are toxic to the fetus. The management of IHCP is dictated by the increased risks of fetal distress, spontaneous preterm delivery, and sudden death, as well as by alleviating pruritus in the mother. These risks to the fetus rise progressively to delivery, regardless of serum levels of bile acids and ALT. Close monitoring of these markers is essential but does not prevent sudden fetal distress and death. Provision should be made to induce labor as soon as fetal lung maturity has been established. Ursodeoxycholic acid is the only therapy that has proven effective, albeit in small studies, in alleviating pruritus and restoring towards normal the abnormal profiles of bile acids and sulfated steroids in serum and other body fluids. Ursodeoxycholic acid seems to have no obvious adverse effects on the fetus, but experience is insufficient to draw conclusions regarding teratogenicity and prevention of adverse outcomes.
Collapse
Affiliation(s)
- E A Fagan
- Departments of Medicine and Pediatrics, Sections of Hepatology and Pediatric Gastroenterology and Nutrition, Rush Presbyterian-St. Luke's Medical Center, Chicago, Illinois, USA.
| |
Collapse
|
|
31
|
Women's Health LiteratureWatch. J Womens Health (Larchmt) 1998; 7:1299-310. [PMID: 9929864 DOI: 10.1089/jwh.1998.7.1299] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
|