Surgical diversion of enterohepatic circulation in pediatric cholestasis.


:Progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (AS) are conditions caused by either an autosomal recessive or an autosomal dominant genetic defect, and they are both characterized by cholestasis, jaundice, and severe debilitating pruritus refractory to medical management. Before the advent of liver transplantation, most PFIC patients would die from end-stage liver disease in the first decade of life. Although liver transplantation has led to patients' survival, disease recurrence (PFIC-2) and severe extra-hepatic manifestations of the disease (PFIC-1) occurred post transplant. In the late 1980s, Whitington described the use of partial external biliary diversion in PFIC and AS patients as a successful way to improve symptoms and decrease circulating bile acid serum concentrations. Since then, other diversion techniques have been described (ileal exclusion and partial internal biliary diversion). These techniques have the benefit of avoiding a stoma, but equivalent results have not been demonstrated (recurrence of cholestasis after ileal exclusion, limited follow up after internal biliary diversion). Overall, studies have showed that biliary diversions in children with cholestasis are safe procedures with low morbidity and mortality, and that they can reduce inflammation and ongoing liver injury, therefore delaying or avoiding the need for liver transplantation in some patients.


Semin Pediatr Surg


Lemoine C,Superina R




Has Abstract


2020-08-01 00:00:00














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    journal_title:Seminars in pediatric surgery

    pub_type: 杂志文章


    authors: Breech L

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  • Error traps and culture of safety in Hirschsprung disease.

    abstract::Hirschsprung disease affects many children every year around the world. Currently, there is an extensive menu of diagnostic methods, and surgical treatments. This situation compels the physicians to follow the rationale of these interventions. The comprehensive diagnosis and treatment of Hirschsprung disease need sing...

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    authors: De La Torre L,Wehrli LA

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  • Respiratory failure and extracorporeal membrane oxygenation.

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    authors: Frenckner B,Radell P

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  • Delayed fistulization from esophageal replacement surgery.

    abstract::We present two patients who developed delayed fistulization following esophageal replacement surgery. The first is a 13-year-old child who, at the age of 3 years, underwent a trans-mediastinal colonic esophageal replacement for a refractory corrosive injury followed by a retrosternal reverse gastric tube after an earl...

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  • Outcomes in pediatric trauma care.

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    journal_title:Seminars in pediatric surgery

    pub_type: 杂志文章


    authors: Stylianos S,Ford HR

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    abstract::For most children, cancer has become a curable disease. With this increased survival, the late effects of the diagnosis of cancer and its therapy have assumed increased importance for these children. Multiple late effects of essentially all organ systems have been described in selected groups of survivors. These may b...

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    authors: Neglia JP

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    pub_type: 杂志文章,评审


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    pub_type: 杂志文章,评审


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    abstract::Pediatric heart transplantation has undergone major changes over the past two decades, marked by a substantial improvement in survival, reduction in posttransplant complications, and enhancement in quality of life for transplant recipients. Actuarial survival has improved substantially in the last decade. Indications ...

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    authors: Alkhaldi A,Chin C,Bernstein D

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    authors: Reynolds M

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    journal_title:Seminars in pediatric surgery

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    authors: Gramlich T,Petras RE

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    journal_title:Seminars in pediatric surgery

    pub_type: 杂志文章,评审


    authors: Uller W,Fishman SJ,Alomari AI

    更新日期:2014-08-01 00:00:00