Can biliary atresia be diagnosed by ultrasonography alone?

Takamizawa, Satoshi; Zaima, Azusa; Muraji, Toshihiro; Kanegawa, Kimio; Akasaka, Yoshinobu; Satoh, Shiiki; Nishijima, Eiji

doi:10.1016/j.jpedsurg.2007.08.032

Cited by 58 publications

(57 citation statements)

References 8 publications

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“…With the intention of finding a non-invasive test that differentiates exactly between hepatocellular and obstructive jaundice, several diagnostic tools have been investigated. Representative studies that refer to sensitivity and specificity of particular examinations are listed in Table 1 [14][15][16][17][18][19][20][21][22][23]. However, all studies contained only small numbers of BA patients (4-48 patients), and the sensitivity and specificity varied widely.…”

Section: Discussionmentioning

confidence: 99%

Endoscopic retrograde cholangiopancreaticography prior to explorative laparotomy avoids unnecessary surgery in patients suspected for biliary atresia

Petersen

Meier

Schneider

et al. 2009

Journal of Hepatology

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Section: Discussionmentioning

confidence: 99%

Endoscopic retrograde cholangiopancreaticography prior to explorative laparotomy avoids unnecessary surgery in patients suspected for biliary atresia

Petersen

Meier

Schneider

et al. 2009

Journal of Hepatology

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“…Lee et al (2003) and Tamazawa et al (2007) reported that the triangular cord sign, identified at the porta hepatis during ultrasound and probably representing fibrosis at the portal plate, was 73 to 100% sensitive and 98 to 100% specific for the diagnosis of biliary atresia. [7][8] Contrary to these findings, Humphrey et al (2007) reported that the "triangular cord sign" has sensitivity of 23% but with a high specificity for diagnosing biliary atresia. mg/kg body weight/day in two equally divided doses, for 5-7 days prior to cholescintigraphy.…”

Section: Utility Of 99mmentioning

confidence: 93%

Utility of 99mTc-mebrofenin hepato-biliary scintigraphy (HIDA scan) for the diagnosis of biliary atresia

Bal¹

2012

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“…Ultrasonographic imaging may reveal an absent or atretic gallbladder, as well as fibrotic regions in the porta hepatis (‘triangular cord') and, intriguingly, increased subcapsular flow [9,14,15,16,17]. The finding of increased subcapsular flow may perhaps provide a readily utilized screening technique to help differentiate biliary atresia from other forms of neonatal cholestasis [14].…”

Section: Biliary Atresia Epidemiology and Salient Clinical Conceptsmentioning

confidence: 99%

Genetic Contributors and Modifiers of Biliary Atresia

Mezina

Karpen²

2015

Dig Dis

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To date, the etiology and pathogenic underpinning of the progression of the most prevalent serious neonatal liver disease, biliary atresia, remains elusive. This disease presents as an aggressive form of neonatal cholestasis characterized by the destruction and obliteration of the extrahepatic bile ducts within the first few weeks of life and a rapid progression of biliary fibrosis, likely due to unremitting cholestasis and retention of biliary constituents including bile acids. In ∼5% of patients, biliary atresia is associated with laterality features, suggesting a genetic underpinning to a disease that begins soon after birth. However, biliary atresia does not occur within families and twins are discordant, indicating an absence of strict mendelian inheritance. Despite this, genes related to bile duct dysmorphogenesis/ciliopathies overlapping with features of biliary atresia in both humans and nonhuman model systems have been proposed. Taken together, strict genetic etiologies leading to a common pathway of a neonatal cholangiopathy resulting in biliary atresia remain elusive. Contributions from fibrogenesis- and inflammation-based studies suggest that early engagement of these pathways contributes to disease progression, but a recent double-blind study did not suggest any benefit from early use of corticosteroids. However, there are genetic contributions to the adaptation and response to cholangiopathies and cholestasis that may be present in certain populations that likely impact upon the response to hepatoportoenterostomy and subsequent biliary tract function. Studies utilizing next generation sequencing technologies (e.g., exome analysis) are ongoing in several laboratories around the world; they are expected to provide insights into genetic contributions to biliary atresia outcomes. Altogether, combinations of exome sequencing and large population studies are expected to reveal causative and modifying genes relevant to patients with biliary atresia as a means to provide therapeutic targets and potential opportunities for genetic screening.

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Can biliary atresia be diagnosed by ultrasonography alone?

Cited by 58 publications

References 8 publications

Endoscopic retrograde cholangiopancreaticography prior to explorative laparotomy avoids unnecessary surgery in patients suspected for biliary atresia

Endoscopic retrograde cholangiopancreaticography prior to explorative laparotomy avoids unnecessary surgery in patients suspected for biliary atresia

Utility of 99mTc-mebrofenin hepato-biliary scintigraphy (HIDA scan) for the diagnosis of biliary atresia

Genetic Contributors and Modifiers of Biliary Atresia

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