Two cases of progressive familial intrahepatic cholestasis type 2: Role of surgery with brief review of literature

Hinglaj Saha, Ghosh Tapanjyoti, Somak Biswas, Prafulla Kumar Mishra, Kalyani Saha Basu, Uttara ChatterjeeJournal of Indian Association of Pediatric Surgeons 2019 24(1):75-77 Progressive familial intrahepatic cholestasis (PFIC) is a rare bile acid transporter defect and autosomal recessive disorder with type 2 being the most common type. Partial internal or external biliary diversion delays its progression to end-stage liver disease. Here, we discuss two cases of type 2 PFIC.
Source: Journal of Indian Association of Pediatric Surgeons - Category: Surgery Authors: Source Type: research

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AbstractPurpose of ReviewThe purpose of this review is to discuss current and new knowledge regarding liver disease in pregnancy and pregnancy post-liver transplantation.Recent FindingsSevere liver disease associated with pregnancy is rare. Liver biopsy is rarely needed for diagnosis but is safe in selected cases. Intrahepatic cholestasis of pregnancy (ICP) with serum bile acids level>  40 μmol/L is associated with adverse fetal outcomes. Ursodeoxycholic acid should be initiated at diagnosis. Portal hypertension can worsen during pregnancy and screening endoscopy should be performed in the 2nd trimester. ...
Source: Current Gastroenterology Reports - Category: Gastroenterology Source Type: research
ConclusionThese results clearly demonstrated the lignans from Schisandra sphenanthera exert hepatoprotective effects against LCA-induced cholestasis by activation of PXR. These lignans may provide an effective approach for the prevention and treatment of cholestatic liver injury.Graphical abstract
Source: Journal of Ethnopharmacology - Category: Drugs & Pharmacology Source Type: research
CONCLUSION: We present a summary of current advances made in a number of areas relevant to both the classically described FIC1 (ATP8B1), BSEP (ABCB11), and MDR3 (ABCB4) transporter deficiencies, as well as more recently described gene mutations -- TJP2 (TJP2), FXR (NR1H4), MYO5B (MYO5B), and others which expand the etiology and understanding of PFIC-related cholestatic diseases and bile transport. PMID: 31183005 [PubMed]
Source: World Journal of Hepatology - Category: Gastroenterology Tags: World J Hepatol Source Type: research
AbstractIn nearly 50% of patients with drug-induced liver injury, the bile flow is impaired known as cholestasis. Intrahepatic cholestasis of pregnancy (ICP) is the most common liver disease that happens in pregnancy. Some of the clinical symptoms include pruritus, dark urine, and abnormal liver function tests. A rise of serum bile acids is the most accurate diagnostic evidence. ICP may lead to premature birth, fetal distress, and even postpartum hemorrhage or stillbirth in some severe cases. Higher bile acid levels (>  40 μmol/L) are associated with higher rates of adverse fetal outcomes. Due to the mult...
Source: Interdisciplinary Sciences, Computational Life Sciences - Category: Bioinformatics Source Type: research
Progressive familial intrahepatic cholestasis (PFIC) includes autosomal recessive cholestatic rare diseases of childhood.
Source: Digestive and Liver Disease - Category: Gastroenterology Authors: Tags: Review Article Source Type: research
(Digestive Disease Week) A new link has been found between a rare and serious condition that typically presents as itchy palms during pregnancy and the world's most common chronic liver disease, according to research presented at Digestive Disease Week ® (DDW) 2019. Researchers found that intrahepatic cholestasis of pregnancy (ICP), a bile acid disorder in adults that affects roughly one of every 300 pregnancies, may be associated with non-alcoholic fatty liver disease (NAFLD).
Source: EurekAlert! - Medicine and Health - Category: International Medicine & Public Health Source Type: news
Conclusion: Cholestasis resulting from primary duodenal papillary carcinoma is rare in pediatric patients but should be considered in the differential diagnosis.
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
This study was carried out in strict accordance with recommendations of the National Institutes of Health guide for the care and use of Laboratory animals. Before undergoing the experimental work, the protocol was approved by the Comité Institutionnel de Protection des Animaux (CIPA) of UQAM (Permit Number: 0515-R3–759-0516). After a 3-day acclimatization period at UQAM’s animal facility, young (100–125 g; approximately 4 weeks old) male Wistar rats (Charles River, St-Constant, QC, Canada) were randomly fed with a regular chow diet (RCD; n = 13) or HFD (n = 12) for 14&...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
Discussion The liver is one of the largest organs in the body, weighing just over 3 pounds in an adult. It is found in the upper right abdomen, under the right dome of the diaphragm. Grossly, it has asymmetric lobes with the right being larger than the left. The lobes are separated by a fibrous connective tissue band that also anchors the liver in the abdominal cavity. The gallbladder is located on the inferior surface of the liver and stores bile, which is then released into the duodenum. Microscopically, the liver cells are arranged in lobules with canals carrying blood vessels and bile ducts. At any moment about 10-13% ...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news
Progressive familial intrahepatic cholestasis type 3 (PFIC3) is an autosomal-recessive liver disorder that presents with cholestasis at young age and progresses to cirrhosis and end-stage liver disease before adulthood [1 –3]. PFIC3 is caused by impairment of phosphatidylcholine (PC) translocation to bile by the canalicular membrane protein ATP Binding Cassette subfamily B member 4 (ABCB4), formerly known as multidrug resistance protein 3 (MDR3), encoded by the ABCB4 gene [4–6]. In bile, PC is essential in the fo rmation of mixed micelles with bile salts that protect the lining of the biliary tree from their detergent properties.
Source: Journal of Hepatology - Category: Gastroenterology Authors: Source Type: research
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