Design and validation of a diagnostic score for biliary atresia

El‐Guindi, Mohamed Abdel‐Salam; Sira, Mostafa Mohamed; Sira, Ahmad Mohamed; Salem, Tahany Abdel-Hameed; El-Abd, Osama; Konsowa, Hatem Abdel-Sattar; El-Azab, Dina S; Allam, Alif

doi:10.1016/j.jhep.2014.03.016

Cited by 102 publications

(121 citation statements)

References 32 publications

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“…El-Guindi et al [3] described that GGT was higher in BA than in other cholestatic disorders, with an 85% accuracy for diagnosing BA.…”

Section: Discussionmentioning

confidence: 99%

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Hepatic expression of interferon-γ and CD68 in biliary atresia compared with other neonatal cholestatic disorders

Arafa¹,

Behairy²,

Abdelmotaleb³

et al. 2015

Medical Research Journal

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Objective The diagnosis of biliary atresia (BA) can be challenging as its histopathologic features overlap with those of other pediatric cholestatic liver diseases. We aimed to study the diagnostic value of hepatic interferon-c (IFN-c) and CD68 immunostaining in the differentiation of BA from other causes of neonatal cholestasis (NC).Patients and methods Hepatic IFN-c and CD68 immunostaining were investigated in 69 patients divided on the basis of the diagnosis into the BA group (n = 34) and the non-BA group (n = 35). The semiquantitative assessment included estimating the average of positive staining cells among inflammatory infiltrate in portal tracts of liver biopsy. ResultsThe mean hepatic CD68 and IFN-c immunostaining in patients with BA was significantly higher (41.2 ± 9.2 and 39.3 ± 12.6, respectively) than that in the cholestasis group (20.8 ± 15.2 and 21.5 ± 15.7, respectively). The cutoff values were 24 for CD68 and 22 for IFN-c to differentiate between BA and cholestatic patients, with a sensitivity and specificity of 94.18 and 74.30% for CD68 and 89 and 73.14% for IFN-c, respectively.Conclusion Both IFN-c and CD68 immunostaining of the liver had a diagnostic value in differentiation between BA and other NC disorders and might be useful as an additional stain when investigating infants with NC. Med

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“…El-Guindi et al [3] described that GGT was higher in BA than in other cholestatic disorders, with an 85% accuracy for diagnosing BA.…”

Section: Discussionmentioning

confidence: 99%

“…The dilemma of early diagnosis of BA, particularly distinguishing it from other causes of NC is challenging [3]. There is a high degree of overlap in clinical, biochemical, radiological, and histological characteristics [4].…”

Section: Introductionmentioning

confidence: 99%

Hepatic expression of interferon-γ and CD68 in biliary atresia compared with other neonatal cholestatic disorders

Arafa¹,

Behairy²,

Abdelmotaleb³

et al. 2015

Medical Research Journal

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“…Bilirubin levels were above the upper limit described in reference values as is common in patients with BA [22,23]. Albumin values were within the normal range, reflecting the fact that only a small percentage of patients were severely ill and/or malnourished [22,24].…”

Section: Discussionmentioning

confidence: 99%

Brain-Derived Neurotrophic Factor in Children and Adolescents with Cirrhosis Due to Biliary Atresia

Wilasco

Cruz²,

Santetti³

et al. 2016

Ann Nutr Metab

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Background: The nutritional status in patients with cirrhosis is not so easy to assess properly. Considering the relationship between brain-derived neurotrophic factor (BDNF) and energy homeostasis, the main aim of this study was to evaluate the concentration of BDNF in children and adolescents with cirrhosis due to biliary atresia (BA) and correlate it with their nutritional status. Methods: Fifty-three children and adolescents with cirrhosis due to BA and 33 healthy controls were enrolled in this study. Nutritional status was evaluated using anthropometric parameters, and serum BDNF was measured by ELISA. Spearman coefficient was used to evaluate the correlation between variables. Results: In the cirrhosis group, 28.8% were undernourished and in the control group, 100% were well-nourished. BDNF median values for the control and cirrhosis group were 28.5 and 9.0 pg/ml respectively. BDNF and platelets were positively associated with both Standard Deviation Score (SDS) for height-for-age ratio and SDS for triceps skinfold thickness-for-age ratio. Conclusions: Considering these associations, BDNF may be an indirect biomarker of nutritional status in children and adolescents with chronic liver disease. Further studies must be conducted to clarify the role of BDNF in this population.

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“…Clinically, infants with biliary atresia develop firm hepatomegaly and splenomegaly as young as 4-8 weeks of age, and laboratory tests usually reveal elevations of ALT, AST, and total and direct bilirubin, while GGT and alkaline phosphatase levels often rise to levels >1,000 U/dl [7,8,14]. 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].…”

Section: Biliary Atresia Epidemiology and Salient Clinical Conceptsmentioning

confidence: 99%

“…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]. Notably, infants with biliary atresia exhibit a spectrum of presentations, including some with the presence of a gallbladder, a normal serum GGT, and pigmented stools early in life, which may obscure the diagnosis.…”

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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Design and validation of a diagnostic score for biliary atresia

Cited by 102 publications

References 32 publications

Hepatic expression of interferon-γ and CD68 in biliary atresia compared with other neonatal cholestatic disorders

Hepatic expression of interferon-γ and CD68 in biliary atresia compared with other neonatal cholestatic disorders

Brain-Derived Neurotrophic Factor in Children and Adolescents with Cirrhosis Due to Biliary Atresia

Genetic Contributors and Modifiers of Biliary Atresia

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