Partial internal biliary diversion through a cholecystojejunocolonic anastomosis—a novel surgical approach for patients with progressive familial intrahepatic cholestasis: a preliminary report

Bustorff‐Silva, Joaquim Murray; Neto, Lourenço Sbraggia; Olímpio, Hugo; Alcantara, Roberta Vacari de; Matsushima, Érica; Tommaso, Adriana María Alves De; Brandâo, María Àngela Bellomo; Hessel, Gabriel

doi:10.1016/j.jpedsurg.2007.03.029

Cited by 63 publications

(70 citation statements)

References 17 publications

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“…This suggests that the intestine of PFIC1 patients has increased sensitivity for a restoration of bile salt secretion. Interestingly, PFIC1 patients who receive a liver transplant develop liver steatosis of which the etiology is unknown [10,11]. …”

Section: Atp8b1 Deficiency Causes Intrahepatic Cholestasis In Mice Anmentioning

confidence: 99%

ATP8B1 and ATP11C: Two Lipid Flippases Important for Hepatocyte Function

Naik

Waart²,

Utsunomiya³

et al. 2015

Dig Dis

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P4 ATPases are lipid flippases and transport phospholipids from the exoplasmic to the cytosolic leaflet of biological membranes. Lipid flipping is important for the biogenesis of transport vesicles. Recently it was shown that loss of the P4 ATPases ATP8B1 and ATP11C are associated with severe Cholestatic liver disease. Mutation of ATP8B1 cause progressive familial Intrahepatic Cholestasis type 1 (PFIC1)and benign recurrent intrahepatic cholestasis type 1 (BRIC 1). From our observations we hypothesized that ATP8B1 deficiency causes a phospholipids randomization at the canalicular membrane, which results in extraction of cholesterol due to increase sensitivity of the canalicular membrane. Deficiency of ATP11C causes conjugated hyperbilirubinemia. In our preliminary result we observed accumulation of unconjugated bile salts in Atp11c deficient mice probably because of regulation in the expression or function of OATP1B2. Similar to ATP8B1, ATP11C have regulation on membrane transporters.

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Section: Atp8b1 Deficiency Causes Intrahepatic Cholestasis In Mice Anmentioning

confidence: 99%

ATP8B1 and ATP11C: Two Lipid Flippases Important for Hepatocyte Function

Naik

Waart²,

Utsunomiya³

et al. 2015

Dig Dis

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“…• Normal GGT surgical biliary diversion although limited efficacy is reported in patients with established chronic liver disease [70,71]. Bile salt sequestrants can be used in patients with diarrhoea.…”

Section: Pfic1mentioning

confidence: 99%

The management of childhood liver diseases in adulthood

Joshi

Gupta

Samyn

et al. 2017

Journal of Hepatology

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SummaryAn increasing number of patients with childhood liver disease survive into adulthood. These young adults are now entering adult services and require ongoing management. Aetiologies can be divided into liver diseases that develop in young adults which present to adult hepatologists i.e., biliary atresia and Alagille syndrome or liver diseases that occur in children/adolescents and adults i.e., autoimmune hepatitis or Wilson's disease. To successfully manage these young adults, a dynamic and responsive transition service is essential. In this review, we aim to describe the successful components of a transition service highlighting the importance of self-management support and a multi-disciplinary approach. We will also review some of the liver specific aetiologies which are unique to young adults, offering an update on pathogenesis, management and outcomes. Ó

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“…Some children may benefit from a treatment with partial external biliary diversion (exept those with MDR3 deficiency). Partial responders or nonresponders will require liver transplantation [20][21][22] . If a molecular diagnosis is available for the family, a prenatal diagnosis is possible in some laboratories [23] .…”

Section: Cystic Fibrosismentioning

confidence: 99%

Diagnosis of Neonatal Cholestasis

Girard¹,

Lacaille²

2008

Ann Nestlé [Engl]

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Cholestasis is frequent in neonates (1/2,500 live births) and in young children. It includes many etiologies with sometimes poor prognosis. In case of neonatal cholestasis, the most important point is to look at the stool color and to rule out biliary atresia which needs to be surgically treated before the 45th day of life. Biliary atresia represents almost 50% of cases of neonatal cholestasis, the other causes being numerous. Some cases can be treated with success, such as tyrosinemia type I or inborn errors of bile acid synthesis. However, in the majority of cases, there is no specific treatment, and the evolution of the disease is toward cirrhosis or liver insufficiency leading to liver transplantation. Nowadays, liver transplantation has good results, but it is a difficult procedure with frequent side effects. In the future, analysis and a better understanding of the mechanisms of the different cholestatic diseases could allow the development of other treatments such as liver cell transplantation or gene therapy, bringing new perspectives for children.

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Partial internal biliary diversion through a cholecystojejunocolonic anastomosis—a novel surgical approach for patients with progressive familial intrahepatic cholestasis: a preliminary report

Cited by 63 publications

References 17 publications

ATP8B1 and ATP11C: Two Lipid Flippases Important for Hepatocyte Function

ATP8B1 and ATP11C: Two Lipid Flippases Important for Hepatocyte Function

The management of childhood liver diseases in adulthood

Diagnosis of Neonatal Cholestasis

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