scholarly journals Intra-Hepatic Cholestasis of Pregnancy and Its Consequences: A Review

2021 ◽  
pp. 224-230
Author(s):  
Yugandhara Hingankar ◽  
Vaishali Taksande
Keyword(s):  
Intrahepatic Cholestasis ◽  
Perinatal Death ◽  
Multiple Pregnancy ◽  
Disease Clustering ◽  
Foetal Monitoring ◽  
Elevated Liver Enzymes ◽  
Pregnancy And Postpartum ◽  
Abcb4 Gene ◽  
Acute Fatty Liver ◽  
In Pregnancy

Background: The most common cause of liver illness in pregnancy is intrahepatic cholestasis (IHCP). It has a varying incidence due to geographic variance; factors such as advanced age, multiple pregnancy, family history, and previous pregnancy cholestasis have demonstrated a higher prevalence in these patients. Cholestasis in pregnancy has an aetiology that is currently unknown. It usually occurs after ovarian hyperstimulation syndrome in early pregnancy and coincides with growing oestrogen levels in the second half of pregnancy [1]. The ABCB4 gene mutation is largely associated in a subtype of progressive familial intrahepatic cholestasis, where disease clustering in first-degree relatives increases hereditary predisposition. Itchy palms and soles with elevated liver enzymes and bile acids are the most common symptoms. Some of the reported maternal problems in these patients include preterm labour, HELLP syndrome, acute fatty liver of pregnancy, and postpartum haemorrhage [2]. There are no precise antenatal foetal monitoring tests that can predict foetal fatalities in the womb. To reduce perinatal death with expectant treatment beyond this gestation, it is recommended that a pregnancy be terminated near 36–37 weeks of pregnancy.

scholarly journals Immune thrombocytopenic purpura presenting with spontaneous gingival haemorrhage in pregnancy

2019 ◽  
Vol 12 (1) ◽  
pp. e228309
Author(s):  
Louise Dunphy ◽  
Rhodri Williams
Keyword(s):  
Periodontal Disease ◽  
Immune Thrombocytopenic Purpura ◽  
Medical Intervention ◽  
Acute Onset ◽  
Thrombocytopenic Purpura ◽  
Gingival Bleeding ◽  
Elevated Liver Enzymes ◽  
Acute Fatty Liver ◽  
Periodontal Probing ◽  
In Pregnancy

Gingival bleeding is a common intraoral finding, typically associated with inflamed tissues and periodontal disease. It is easily provoked by periodontal probing or toothbrushing. Spontaneous gingival bleeding rarely occurs and may be the only sign of systemic bleeding problems such as thrombocytopenia, leukaemia or coagulopathy. In pregnancy, acute onset of thrombocytopenia may occur in systemic disorders such as severe pre-eclampsia, HELLP syndrome (haemolysis, elevated liver enzymes, low platelets) or the acute fatty liver of pregnancy. The diagnosis and management of such conditions may challenge physicians. It requires a systematic approach with a comprehensive history to exclude causes of gingival haemorrhage such as periodontal disease, anticoagulant therapy, maxillofacial trauma, haematological disorders or a bacterial infection. The authors describe a case of immune thrombocytopenic purpura presenting with spontaneous gingival haemorrhage in pregnancy. This case highlights the fact that medical intervention to correct the underlying aberration of haemostasis is necessary for local measures to stop the gingival bleeding successfully.

scholarly journals Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group

Blood ◽  
2020 ◽  
Vol 136 (19) ◽  
pp. 2103-2117
Author(s):  
Fadi Fakhouri ◽  
Marie Scully ◽  
François Provôt ◽  
Miquel Blasco ◽  
Paul Coppo ◽  
...  
Keyword(s):  
Thrombotic Microangiopathy ◽  
Working Group ◽  
Current Knowledge ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Specific Treatment ◽  
Clinical Approach ◽  
Elevated Liver Enzymes ◽  
Pregnancy And Postpartum ◽  
Multidisciplinary Working ◽  
In Pregnancy

Abstract Pregnancy and postpartum are high-risk periods for different forms of thrombotic microangiopathy (TMA). However, the management of pregnancy-associated TMA remains ill defined. This report, by an international multidisciplinary working group of obstetricians, nephrologists, hematologists, intensivists, neonatologists, and complement biologists, summarizes the current knowledge of these potentially severe disorders and proposes a practical clinical approach to diagnose and manage an episode of pregnancy-associated TMA. This approach takes into account the timing of TMA in pregnancy or postpartum, coexisting symptoms, first-line laboratory workup, and probability-based assessment of possible causes of pregnancy-associated TMA. Its aims are: to rule thrombotic thrombocytopenic purpura (TTP) in or out, with urgency, using ADAMTS13 activity testing; to consider alternative disorders with features of TMA (preeclampsia/eclampsia; hemolysis elevated liver enzymes low platelets syndrome; antiphospholipid syndrome); or, ultimately, to diagnose complement-mediated atypical hemolytic uremic syndrome (aHUS; a diagnosis of exclusion). Although they are rare, diagnosing TTP and aHUS associated with pregnancy, and postpartum, is paramount as both require urgent specific treatment.

Transient diabetes insipidus with severe maternal and fetal hypernatremia

2014 ◽  
Vol 3 (1) ◽  
Author(s):  
Jennifer K.Y. Ko ◽  
Noel W.M. Shek ◽  
Chin Peng Lee
Keyword(s):  
Diabetes Insipidus ◽  
Twin Pregnancy ◽  
Multiple Pregnancy ◽  
Urine Osmolality ◽  
Presenting Symptoms ◽  
Early Labour ◽  
Acute Fatty Liver ◽  
Paired Serum ◽  
High Index Of Suspicion ◽  
In Pregnancy

AbstractDiabetes insipidus is a condition characterised by polyuria and polydipsia with excretion of diluted urine. Transient diabetes insipidus has been reported in pregnancy and is often associated with acute fatty liver of pregnancy, pre-eclampsia, HELLP syndrome and multiple pregnancy. Presenting symptoms can be vague. In our case, a primigravida with spontaneous monochorionic diamniotic twin pregnancy presented at 36+3 weeks in early labour. Severe hypernatremia was found in the patient and in both twins after a caesarean section was performed. Transient diabetes insipidus was diagnosed based on paired serum and urine osmolality. The hypernatremia was corrected gradually and 1-deamino-8D-arginine vasopressin was given. A high index of suspicion and early involvement of physicians and paediatricians are needed to avoid catastrophic consequences.

scholarly journals A Novel Compound Heterozygous Mutation in ABCB4 Gene Leading to Cholelithiasis, Progressive Familial Intrahepatic Cholestasis (Type 3), and Cirrhosis in a Child

2020 ◽  
Vol 10 (01) ◽  
pp. e134-e136
Author(s):  
Nida Mirza ◽  
Smita Malhotra ◽  
Anupam Sibal
Keyword(s):  
Intrahepatic Cholestasis ◽  
Gall Stone ◽  
Compound Heterozygous Mutation ◽  
Heterozygous Mutation ◽  
Compound Heterozygous ◽  
Initial Symptom ◽  
Progressive Familial Intrahepatic Cholestasis ◽  
Presenting Symptoms ◽  
Abcb4 Gene ◽  
Type 3

AbstractProgressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of autosomal recessive disorders of childhood which presents with intermittent or progressive episodes of cholestasis, with jaundice and pruritus as most common presenting symptoms. PFIC type 3 occurs due to mutations in the ABCB4 gene, mutation in this gene has wide spectrum of features which include intrahepatic stones, cholelithiasis, PFIC type 3, and intrahepatic cholestasis of pregnancy. Here, we are reporting a peculiar case of young male adolescent with novel variant compound heterozygote missense mutation in ABCB4 gene who had gall stone as initial symptom, followed by symptoms of PFIC and eventually decompensated chronic liver disease.

scholarly journals Intrahepatic cholestasis of pregnancy: maternal and fetal outcome and its correlation with serum bile acid levels

2018 ◽  
Vol 7 (11) ◽  
pp. 4403
Author(s):  
Bhawna Sharma ◽  
Neetu Arora ◽  
Kusum Dogra ◽  
Kamal S. Negi
Keyword(s):  
Bile Acid ◽  
Cesarean Delivery ◽  
Intrahepatic Cholestasis ◽  
Perinatal Death ◽  
Tertiary Care ◽  
Neonatal Outcomes ◽  
Serum Bile Acid ◽  
Intrahepatic Cholestasis Of Pregnancy ◽  
Vaginal Deliveries ◽  
Cholestasis Of Pregnancy

Background: Maternal, fetal, and neonatal outcomes in parturients with intrahepatic cholestasis of pregnancy (ICP) have been retrospectively documented. We aimed to present pregnancy outcomes of parturients with ICP who underwent delivery. The study was conducted during a 1-year period in a tertiary care centre.Methods: Data from 1 January to 31 December 2017 were collected to identify parturients with ICP.Results: Almost 3/4th of births came to a vaginal delivery (76.74%) and only 10 parturients had cesarean delivery. 4 of 10 parturients underwent nonelective cesarean section, while 6 had elective cesarean delivery. 15.15 % vaginal deliveries were instrumental. The most common indications for emergency LSCS and instrumental deliveries was fetal distress followed by failure to progress of labour. Most births occurred at or after 37 weeks of gestation (65%).  Regarding neonatal outcomes in terms of birth weight and Apgar scores at 1 and 5 min, they were positive, as well.  None of the babies had Apgar score < 7 at 5 minutes. No case of perinatal death was observed.Conclusions: Although the results were generally positive, larger studies need to be conducted to evaluate the maternal and fetal outcomes in ICP and correlation with serum bile acid levels.

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