scholarly journals Triggers of benign recurrent intrahepatic cholestasis and its pathophysiology: a review of literature

2021 ◽  
Vol 84 (3) ◽  
Author(s):  
A Halawi ◽  
N Ibrahim ◽  
R Bitar

Benign recurrent intrahepatic cholestasis (BRIC) is a rare genetic disorder that is characterized by episodes of cholestasis followed by complete resolution. The episodic nature of BRIC raises concerns about its possible trigger factors. Indeed, case reports of this orphan disease have associated BRIC to some triggers. In the absence of any reviews, we reviewed BRIC trigger factors and its pathophysiology. The study consisted of a systematic search for case reports using PubMed. Articles describing a clear case of BRIC associated with a trigger were included resulting in 22 articles that describe 35 patients. Infection was responsible for 54.3% of triggered episodes, followed by hormonal, drugs, and miscellaneous causes reporting as 30%, 10%, and 5.7% respectively. Females predominated with 62.9%. The longest episode ranged between 3 months to 2 years with a mean of 32.37 weeks. The mean age of the first episode was 14.28 ranging between 3 months to 48 years. Winter and autumn were the major seasons during which episodes happened. Hence, BRIC is potentially triggered by infection, which is most commonly a viral infection, hormonal disturbances as seen in oral contraceptive pills and pregnancy state, and less commonly by certain drugs and other causes. The appearance of cholestasis during the first two trimesters of pregnancy compared to intrahepatic cholestasis of pregnancy could help to differentiate between the two conditions. The possible mechanism of BRIC induction implicates a role of BSEP and ATP8B1. While estrogen, drugs, and cytokines are known to affect BSEP, less is known about their action on ATP8B1.

2005 ◽  
Vol 129 (9) ◽  
pp. 1168-1171 ◽  
Author(s):  
Neda Zarrin-Khameh ◽  
James E. Spoden ◽  
Ruc M. Tran

Abstract A case of angiolymphoid hyperplasia with eosinophilia (ALH) is reported in a 33-year-old woman who developed an auricular nodule during the second trimester of her pregnancy. Angiolymphoid hyperplasia with eosinophilia usually occurs on the head and neck of young adults and is more common in women than in men. Characteristic histologic features of ALH present in this case included proliferation of thick-walled blood vessels lined by prominent endothelial cells, infiltration of the interstitium by chronic inflammatory cells (mainly eosinophils), and presence of lymphoid follicles with germinal centers. The auricular tumor was completely excised. Thirteen months after excision, the patient remains tumor free. Although there are not many case reports on ALH during pregnancy or involving use of oral contraceptive pills, sex hormones may play a role in the pathogenesis of ALH. This hypothesis, in the context of cases previously described in the literature, and the differential diagnosis of ALH are discussed.


1996 ◽  
Vol 24 (3) ◽  
pp. 320-323 ◽  
Author(s):  
Juan G. Mella ◽  
Elke Roschmann ◽  
Juan C. Glasinovic ◽  
Alejandra Alvarado ◽  
Mirella Scrivanti ◽  
...  

Author(s):  
Noor Dharmarha ◽  
Anju Hajari ◽  
Anuradha Konda ◽  
Venkatesa Muni Reddy

Our objective of this case report is to highlight the value of uterine artery embolization as a means of conservative management of uterine arteriovenous malformation in patients wanting preservation of fertility. A 24 years old woman, P1L1A2 presented with complaints of heavy menstrual bleeding for two months. She had a previous term caesarean section in 2014, followed by curettage twice, in 2016 and 2017. The first episode of heavy bleeding occurred two months after the last curettage. Previous menstrual cycles were regular with moderate flow. The patient was started on combined oral contraceptive pills but she did not respond to medical treatment. Transvaginal scan and Doppler showed uterine AVM and endometrial thickness of 7.7mm. Beta hCG was less than 1.2. Digital subtraction angiography and bilateral uterine artery embolization was done. Patient conceived spontaneously a year after the procedure and delivered a healthy baby at term by elective caesarean section. Angiography and uterine artery embolization has revolutionized the management of AVM and contrary to popular belief, the pregnancy outcome after UAE was good.


2015 ◽  
pp. S203-S209 ◽  
Author(s):  
A. PAŘÍZEK ◽  
M. DUŠKOVÁ ◽  
L. VÍTEK ◽  
M. ŠRÁMKOVÁ ◽  
M. HILL ◽  
...  

Intrahepatic cholestasis of pregnancy (ICP) is a disorder of liver function, commonly occurring in the third trimester but sometimes also as soon as the end of the second trimester of pregnancy. Symptoms of this disorder include pruritus, plus abnormal values of bile acids and hepatic transaminases. After birth, symptoms disappear and liver function returns to normal. Though ICP is relatively non-complicated and often symptomatically mild from the point-of-view of the mother, it presents a serious risk to the fetus, making this disease the subject of great interest. The etiology and pathogenesis of ICP is multifactorial and as yet not fully elucidated. Hormonal factors likely play a significant role, along with genetic as well as exogenous factors. Here we summarize the knowledge of changes in steroid hormones and their role in the development of intrahepatic cholestasis of pregnancy. In addition, we consider the role of exogenous factors as possible triggers of steroid hormone changes, the relationship between metabolic steroids and bile acids, as well as the combination of these factors in the development of ICP in predisposed pregnant women.


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