Common misdiagnoses of biliary atresia

Sira, Mostafa Mohamed; Taha, Mohammad; Sira, Ahmad Mohamed

doi:10.1097/meg.0000000000000198

Cited by 18 publications

(16 citation statements)

References 30 publications

(33 reference statements)

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“…As reported before in our studies and in the studies of others, the present study showed that different clinical, laboratory, ultrasonographic and histopathological parameters have significant diagnostic values for differentiating BA from non‐BA cholestatic disorders with different specificities and sensitivities for each parameter. No single test showed 100% sensitivity and specificity and it is well documented that the summation of different parameters will give more accuracy in diagnosis, as we reported before in our recently developed BA score …”

Section: Discussionsupporting

confidence: 85%

Urinary urobilinogen in biliary atresia: A missed, simple and cheap diagnostic test

El‐Guindi

El-Said²,

Hussein

et al. 2015

Hepatology Research

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Section: Discussionsupporting

confidence: 85%

Urinary urobilinogen in biliary atresia: A missed, simple and cheap diagnostic test

El‐Guindi

El-Said²,

Hussein

et al. 2015

Hepatology Research

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“…[1][2][3] The incidence ranges from 1 in 5000 to 1 in 15 000 living births depending on the region of the world. [1][2][3] The incidence ranges from 1 in 5000 to 1 in 15 000 living births depending on the region of the world.…”

Section: Introductionmentioning

confidence: 99%

“…Biliary atresia (BA), which rises from a fibro-inflammatory destruction of extrahepatic or intrahepatic bile ducts, is the most common cause of chronic cholestasis in neonates. [1][2][3] The incidence ranges from 1 in 5000 to 1 in 15 000 living births depending on the region of the world. 4,5 If left untreated, patients can die from liver cirrhosis within 2 years of age.…”

Section: Introductionmentioning

confidence: 99%

Gut microbial profile in biliary atresia: a case‐control study

Wang

Tian

Jiang

et al. 2019

J of Gastro and Hepatol

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Background and Aim Biliary atresia (BA) is a progressive fibro‐inflammatory cholangiopathy with an unclear etiology. Various liver disorders are associated with an altered microbiome. However, gut microbiome in BA remains unknown. Here, we performed a case‐control study to investigate the gut microbiota in BA. Methods A cross‐sectional analysis was first conducted for 34 BA patients and 34 healthy controls. Then we investigated the shift in gut microbiota 2 weeks after the Kasai procedure in 16 BA patients. Gut microbiome was initially analyzed using 16S ribosome RNA gene sequencing and further validated by metagenomic sequencing. Fecal bile acids were determined using ultra‐high performance liquid chromatography. Results Compared with healthy controls, BA showed lower diversity and significant structural segregation in the microbiome. At phylum level, Proteobacteria numbers increased, whereas those of Bacteroidetes decreased in BA. At genus level, several potential pathogens such as Streptococcus and Klebsiella thrived in BA, while numbers for Bifidobacterium and several butyrate‐producing bacteria declined. The microbiome was also disturbed after the Kasai procedure. Operational taxonomic units responding to BA showed significant correlation with liver function. Furthermore, the abundance ratio of Streptococcus/Bacteroides showed great promise in distinguishing BA from healthy controls. Intestinal bile acids were dramatically decreased in BA, and Clostridium XIVa positively correlated with the ratio of primary/secondary bile acids. Conclusions Gut microbial dysbiosis, may be caused by decreased bile acids, was associated with liver function and had a good diagnostic potential for BA. Therefore, further exploration of gut microbiota may provide important insights into their potential diagnostic and therapeutic benefits.

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“…The incidence ranges from 1 in 5,000 to 1 in 20,000 living births in different areas of the world. It constitutes nearly one-third of all neonatal cholestasis or more than 90% obstructive cholestastic cases 3 . If untreated, children will die by age 2 years because of progressive liver cirrhosis.…”

Section: Introductionmentioning

confidence: 99%

“…Neonatal hepatitis syndrome (NHS) is another leading cause of neonatal cholestasis and there is great overlap in the clinical, biochemical, imaging, and histopathological characteristics between NHS and BA 3–5 . BA should be differentiated from NHS as soon as possible, since early Kasai operations greatly improve the outcome of BA infants, while NHS infants don’t need surgery and most of which will usually return to normal by one year of age.…”

Section: Introductionmentioning

confidence: 99%

Metabonomics Reveals Metabolite Changes in Biliary Atresia Infants

et al. 2015

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Biliary atresia (BA) is a rare neonatal cholestatic disorder caused by obstruction of extra and intra hepatic bile ducts. If untreated, progressive liver cirrhosis will lead to death within two years. Early diagnosis and operation improve the outcome significantly. Infants with neonatal hepatitis syndrome (NHS) present similar symptoms, confounding the early diagnosis of BA. The lack of non-invasive diagnostic methods to differentiate BA from NHS greatly delays the surgery of BA infants, thus deteriorating the outcome. Here, we performed a metabolomics study in plasma of BA, NHS, and healthy infants using gas chromatography-time-of-flight mass spectrometry. Scores plots of orthogonal partial least square discriminant analysis clearly separated BA from NHS and healthy infants. Eighteen metabolites were found to be differentially expressed between BA and NHS, among which seven (L-glutamic acid, L-ornithine, L-isoleucine, L-lysine, L-valine, L-tryptophan, and L-serine) were amino acids. The altered amino acids were quantitatively verified using ultra-performance liquid chromatography-tandem mass spectrometry. Ingenuity pathway analysis revealed the network of “Cellular Function and Maintenance, Hepatic System Development and Function, Neurological Disease” was altered most significantly. This study suggests that plasma metabolic profiling has great potential in differentiating BA from NHS, and amino acid metabolism is significantly different between the two diseases.

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Common misdiagnoses of biliary atresia

Cited by 18 publications

References 30 publications

Urinary urobilinogen in biliary atresia: A missed, simple and cheap diagnostic test

Urinary urobilinogen in biliary atresia: A missed, simple and cheap diagnostic test

Gut microbial profile in biliary atresia: a case‐control study

Metabonomics Reveals Metabolite Changes in Biliary Atresia Infants

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