Biliary Atresia

Sanchez‐Valle, Amarilis; Kassira, Noor; Varela, Veronica C.; Radu, Stephanie; Paidas, Charles N.; Kirby, Russell S.

doi:10.1016/j.yapd.2017.03.012

Cited by 66 publications

(31 citation statements)

References 70 publications

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“…In addition, the incidence of NAFLD is increasing dramatically in adolescents and in children[ 1 ]. Biliary atresia (BA) or neonatal hepatitis also can affect the liver in early childhood, and BA is the most common cause of hepatic fibrosis and reason for liver transplantation in young children[ 2 , 3 ]. Therefore, quantitative and non-invasive characterization of the liver conditions is important in pediatric patients.…”

Section: Introductionmentioning

confidence: 99%

Liver intravoxel incoherent motion diffusion-weighted imaging for the assessment of hepatic steatosis and fibrosis in children

Shin¹,

Yoon²,

Kim³

et al. 2018

WJG

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AIMTo evaluate the correlation between intravoxel incoherent motion (IVIM) diffusion-weighted imaging (DWI) parameters and the degree of hepatic steatosis and fibrosis in children.METHODSThis retrospective study was approved by the institutional review board. The children (≤ 18 years) who underwent liver IVIM DWI with 8 b-values under the suspicion of hepatic steatosis or fibrosis from February 2013 to November 2016 were included. Subjects were divided into normal, fatty liver (FAT), and fibrotic liver (FIB) groups. The slow diffusion coefficient (D), fast diffusion coefficient (D*), perfusion fraction (f), and apparent diffusion coefficient (ADC) were measured. MR proton density fat fraction (PDFF), MR elastography (MRE), and IVIM values were compared.RESULTSA total of 123 children (median age of 12 years old, range: 6-18 years) were included, with 8 in the normal group, 93 in the FAT group, and 22 in the FIB group. The D* values were lower in the FIB group compared with those of the normal (P = 0.015) and FAT (P = 0.003) groups. The f values were lower in the FIB group compared with the FAT group (P = 0.001). In multivariate analyses, PDFF value was positively correlated with f value (β = 3.194, P < 0.001), and MRE value was negatively correlated with D* value (β = -7.031, P = 0.032). The D and ADC values were not influenced by PDFF or MRE value.CONCLUSIONIn liver IVIM DWI with multiple b-values in children, there was a positive correlation between hepatic fat and blood volume, and a negative correlation between hepatic stiffness and endovascular blood flow velocity, while diffusion-related parameters were not affected.

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Section: Introductionmentioning

confidence: 99%

Liver intravoxel incoherent motion diffusion-weighted imaging for the assessment of hepatic steatosis and fibrosis in children

Shin¹,

Yoon²,

Kim³

et al. 2018

WJG

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“… Cholangiopathy Prevalence; Sex preponderance Current therapy Genetic cause Ref. Genetic Alagille syndrome (ALGS) 2.2–3.3 in 100,000 live births; no sex preponderance Medical: supportive JAG1 (majority), NOTCH2 [ 83 ] Surgical: liver transplantation Caroli disease (CD) and Caroli syndrome (CS) with congenital hepatic fibrosis 0.1 in 100,000 live births; no sex preponderance Medical: supportive PKHD1 [ 84 ] Surgical: portosystemic shunting, liver transplantation Cystic fibrosis-associated liver disease 12.5 in 100,000 live births Medical: Ursodeoxycholic acid (UDCA), supportive CFTR [ 85 ]; [ 86 ] Surgical: liver transplantation Polycystic liver disease (autosomal dominant polycystic liver disease ADPLD, autosomal dominant polycystic kidney disease ADPKD, autosomal recessive polycystic kidney disease ARPKD) ADPLD: 1–9 in 100,000 live births Medical: supportive ADPLD: PRKCSH, SEC63; ADPKD: PKD1, PKD2, GANAB; ARPKD: PKHD1 [ 84 ]; [ 87 ] ADPKD: 100–250 in 100,000 live births; ARPKD: 5 in 100,000 live births Surgical: aspiration of cyst fluid, liver transplantation (uncommon indication) Idiopathic/multifactorial Biliary atresia 5–14.3 in 100,000 live births; higher prevalence in Asia; female: male ratio 1.4:1 Medical: post-operative systemic corticosteroids, choleretic (agent stimulating bile flow) [ 88 ] Surgical: Kasai portoenterostomy, liver transplantation Primary biliary cholangitis (formerly, primary biliary cirrhosis) 35 in 100,000; female: male ratio 9:1 Medical: UDCA, supportive [ 89 ] Surgical: liver transplantation Primary sclerosing cholangitis …”

Section: Cholangiopathies – An Introductionmentioning

confidence: 99%

Cholangiopathies – Towards a molecular understanding

et al. 2018

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Liver diseases constitute an important medical problem, and a number of these diseases, termed cholangiopathies, affect the biliary system of the liver. In this review, we describe the current understanding of the causes of cholangiopathies, which can be genetic, viral or environmental, and the few treatment options that are currently available beyond liver transplantation. We then discuss recent rapid progress in a number of areas relevant for decoding the disease mechanisms for cholangiopathies. This includes novel data from analysis of transgenic mouse models and organoid systems, and we outline how this information can be used for disease modeling and potential development of novel therapy concepts. We also describe recent advances in genomic and transcriptomic analyses and the importance of such studies for improving diagnosis and determining whether certain cholangiopathies should be viewed as distinct or overlapping disease entities.

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“…Biliary atresia is a rare congenital condition, which is associated with ductopenia and leads to secondary biliary cirrhosis, carrying poor life expectancy in the absence of a Kasai portoenterostomy, followed by liver transplant [13,14]. However, the extent of biliary atresia may vary, both in terms of severity and extension; therefore, biliary hypoplasia may also be diagnosed in newborns with persistent jaundice.…”

Section: Discussionmentioning

confidence: 99%

A Rare Association between Left Lobe Secondary Biliary Cirrhosis and Budd-Chiari Syndrome Secondary to Hepatocellular Carcinoma in the Non-Cirrhotic Right Liver Lobe

Fodor

Fit

Farcau

et al. 2017

JGLD

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Liver cirrhosis is a diffuse chronic liver disease affecting the entire liver. The fibrosis accumulation and distribution in the liver are known to be heterogeneous. „Localized” or „focal” cirrhosis is only anecdotically reported. Acute hepatitis E virus (HEV) infection is uncommon in western countries, especially in temperate climate areas and is very often missed or underdiagnosed. However, it may be responsible of up to 15% of acute-on-chronic liver failure cases. We present the case of a 35-year-old patient with a very uncommon association of Budd-Chiari syndrome secondary to hepatocellular carcinoma (HCC) developed on a non-cirrhotic right liver lobe and secondary biliary cirrhosis of the left liver lobe, that further complicated with acute HEV infection leading to acute-on-chronic liver failure and death.Abbreviations: Alb: Albumin; AFP: alpha feto-protein; ALP: alkaline phosphatase; ALT: alanine aminotransferase; AST: aspartate aminotransferase; BCS: Budd-Chiari syndrome; CK7: cytokeratin 7; CMV: cytomegalovirus; EBV: Epstein-Barr virus; GGT: gamma glutamyl transpeptidase; HBV: hepatitis B virus; HCV: hepatitis C virus; HEV: hepatitis E virus; HSV: hepes simplex virus; HCC: hepatocellular carcinoma; IVC: inferior vena cava; MELD: model for end-stage liver disease; (MD)CT: (multi-detector) computer tomography; PT: prothrombin time; TB: total bilirubin.

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Biliary Atresia

Cited by 66 publications

References 70 publications

Liver intravoxel incoherent motion diffusion-weighted imaging for the assessment of hepatic steatosis and fibrosis in children

Liver intravoxel incoherent motion diffusion-weighted imaging for the assessment of hepatic steatosis and fibrosis in children

Cholangiopathies – Towards a molecular understanding

A Rare Association between Left Lobe Secondary Biliary Cirrhosis and Budd-Chiari Syndrome Secondary to Hepatocellular Carcinoma in the Non-Cirrhotic Right Liver Lobe

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