Surgical Treatment of Progressive Familial Intrahepatic Cholestasis: Comparison of Partial External Biliary Diversion and Ileal Bypass

Kaliciński, Piotr; Ismail, Hor; Jankowska, Irena; Kamiński, Andrzej; J, Pawłowska; Drewniak, Tomasz; Markiewicz, M; Szymczak, Marek

doi:10.1055/s-2003-43570

Cited by 97 publications

(5 citation statements)

References 10 publications

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“…In partial internal biliary drainages, there is no external fistula/stoma but instead a jejunal, ileal or appendix conduit between gallbladder and colon (called cholecystojejunocolic, cholecystoileocolic, cholecystoappendicocolic anastomosis, respectively) or anastomosis between gall bladder and anti-reflux loop of colon (cholecystocolocolic anastomosis) is made either via open surgery or by laparoscopy. 31 – 35 …”

Section: General Approaches To Pficmentioning

confidence: 99%

Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment

Günaydın¹,

Çil²

2018

HMER

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Progressive familial intrahepatic cholestasis (PFIC) is a group of autosomal recessive cholestatic liver diseases which are subgrouped according to the genetic defect, clinical presentation, laboratory findings and liver histology. Progressive liver fibrosis, cirrhosis, and end stage liver disease (ESLD) may eventually develop. PFIC was first described in Amish descendants of Jacob Byler, therefore it was originally called Byler disease. But it can be seen anywhere on the globe. This review summarizes the main features of the subtypes of the disease and discusses the current available diagnosis, conservative and surgical therapeutic options.

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Section: General Approaches To Pficmentioning

confidence: 99%

Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment

Günaydın¹,

Çil²

2018

HMER

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“…Remarkably, PEBD has been recognized to provide an alternative to transplant, with many patients surviving with their native liver. However, complications can occur including recurrent episodes of pruritis, possible need for biliary diversion revision, continued need for aggressive vitamin supplementation, or progressive disease necessitating liver transplant[14,18,19].…”

Section: Resultsmentioning

confidence: 99%

“…This technique bypasses the distal 15% of the ileum to avoid the major site of bile acid reabsorption and is particularly useful in patients without an intact gall bladder[20]. Unfortunately, severe malabsorption can occur and refractory disease has been reported[18,21,22].…”

Section: Resultsmentioning

confidence: 99%

Expanding etiology of progressive familial intrahepatic cholestasis

Henkel

Squires

Ayers

et al. 2019

WJH

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BACKGROUND Progressive familial intrahepatic cholestasis (PFIC) refers to a disparate group of autosomal recessive disorders that are linked by the inability to appropriately form and excrete bile from hepatocytes, resulting in a hepatocellular form of cholestasis. While the diagnosis of such disorders had historically been based on pattern recognition of unremitting cholestasis without other identified molecular or anatomic cause, recent scientific advancements have uncovered multiple specific responsible proteins. The variety of identified defects has resulted in an ever-broadening phenotypic spectrum, ranging from traditional benign recurrent jaundice to progressive cholestasis and end-stage liver disease. AIM To review current data on defects in bile acid homeostasis, explore the expanding knowledge base of genetic based diseases in this field, and report disease characteristics and management. METHODS We conducted a systemic review according to PRISMA guidelines. We performed a Medline/PubMed search in February-March 2019 for relevant articles relating to the understanding, diagnosis, and management of bile acid homeostasis with a focus on the family of diseases collectively known as PFIC. English only articles were accessed in full. The manual search included references of retrieved articles. We extracted data on disease characteristics, associations with other diseases, and treatment. Data was summarized and presented in text, figure, and table format. RESULTS Genetic-based liver disease resulting in the inability to properly form and secrete bile constitute an important cause of morbidity and mortality in children and increasingly in adults. A growing number of PFIC have been described based on an expanded understanding of biliary transport mechanism defects and the development of a common phenotype. 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.

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“…Furthermore, there are reports of late relapse, possibly because of gradual ileum accommodation (8, 9). …”

Section: Discussionmentioning

confidence: 99%

Partial Internal Biliary Diversion in Progressive Familial Intrahepatic Cholestasis: Introduction of a New Approach

Mousavi

Kadı

2014

Hepat Mon

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Introduction:Facilitation of biliary salts secretion represents the mainstay of treatment for progressive familial intrahepatic cholestasis (PFIC). The purpose of this study was to introduce a new approach for the treatment of progressive familial intrahepatic cholestasis (PFIC) to avoid ostoma.Case Presentation:An 11-year-old girl with the diagnosis of PFIC underwent cholecystoappendicostomy with myotomy operation. Because of anastomosis stricture, she was reoperated with cholecystojejunocolic anastomosis and intussuscepted valve surgery. She was followed for 9 months. Despite disappointing outcomes of internal drainage with cholecystoappendicostomy, results of cholecystojejunocolic anastomosis with intussuscepted valve surgery were promising.Discussion:The cholecystojejunocolic anastomosis with intussuscepted valve surgery could be considered as a forthcoming approach in the treatment of intrahepatic cholestasis.

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Surgical Treatment of Progressive Familial Intrahepatic Cholestasis: Comparison of Partial External Biliary Diversion and Ileal Bypass

Abstract: PEBD is more effective than IB for the permanent improvement of symptoms of PFIC. Ileal bypass procedure, although initially effective, does not ensure good long-term results in more than 50 % of patients, probably due to intestinal re-absorption of bile acids increasing over time.

Cited by 97 publications

References 10 publications

Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment

Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment

Expanding etiology of progressive familial intrahepatic cholestasis

Partial Internal Biliary Diversion in Progressive Familial Intrahepatic Cholestasis: Introduction of a New Approach

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