Is biliary atresia an autoimmune disease?

A-Kader, H. Hesham; Abdelhameed, Ahmed M.; Al-Shabrawi, Mortada; Mohsen, Nabil; El‐Karaksy, Hanaa; Hassanein, B; El-Sayed, Behairy; Abdel-Khalik, Mohammed K.; Karjoo, Manoochehr

doi:10.1097/00042737-200304000-00020

Cited by 17 publications

(12 citation statements)

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“…Even though no medical therapies exist, sequential treatment strategy involving surgical Kasai portoenterostomy and liver transplantation is the only option for the most affected children. Nonetheless the precise pathogenesis of BA has yet to be determined, a number of theories regarding the etiology of BA include toxin exposure, virus-mediated inflammation, abnormal inflammatory response, defective morphogenesis, genetic mutation, and immunological dysregulation[4]. …”

Section: Introductionmentioning

confidence: 99%

Elevation of serum urokinase plasminogen activator receptor and liver stiffness in postoperative biliary atresia

Udomsinprasert

Honsawek

Jirathanathornnukul

et al. 2016

WJH

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AIMTo investigate serum urokinase-type plasminogen activator receptor (uPAR) and liver stiffness in biliary atresia (BA) and examine the correlation of circulating uPAR, liver stiffness, and clinical outcomes in postoperative BA children.METHODSEighty-five postKasai BA children and 24 control subjects were registered. Circulating uPAR was measured using enzyme-linked immunosorbent essay. Liver stiffness was analyzed using transient elastography.RESULTSBA children had significantly greater circulating uPAR and liver stiffness scores than control subjects (P < 0.001). Circulating uPAR and liver stiffness were substantially higher in jaundiced BA children than non-jaundiced BA children (P < 0.001). In addition, circulating uPAR was positively associated with serum aspartate aminotransferase (r = 0.507, P < 0.001), alanine aminotransferase (r = 0.364, P < 0.001), total bilirubin (r = 0.559, P < 0.001), alkaline phosphatase (r = 0.325, P < 0.001), and liver stiffness scores (r = 0.508, P < 0.001).CONCLUSIONCirculating uPAR and liver stiffness values were greater in BA children than healthy controls. The increased circulating uPAR was associated with liver dysfunction in BA. As a consequence, serum uPAR and liver stiffness may be used as noninvasive biomarkers indicating the progression of liver fibrosis in postKasai BA.

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Section: Introductionmentioning

confidence: 99%

Elevation of serum urokinase plasminogen activator receptor and liver stiffness in postoperative biliary atresia

Udomsinprasert

Honsawek

Jirathanathornnukul

et al. 2016

WJH

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“…Hepatoportoenterostomy or Kasai operation currently remains the standard of care for first line intervention for BA children. Although the etiopathogenic mechanism of BA has not been well established, several theories have been proposed including genetic factors, viral infections, morphogenic defects and immunological dysregulation (A-Kader et al, 2003).…”

Section: Introductionmentioning

confidence: 99%

Elevated serum periostin is associated with liver stiffness and clinical outcome in biliary atresia

Honsawek¹,

Udomsinprasert²,

Vejchapipat³

et al. 2015

Biomarkers

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“…The pathogenesis of BA has remained a mystery. Most of the causal theories include defects resulting from a viral infection or toxin exposure, defects in morphogenesis, genetic predisposition, defects in prenatal circulation and immune dysregulation[3-5]. …”

Section: Introductionmentioning

confidence: 99%

Low bone mineral density and the severity of cholestasis in biliary atresia

Homchan

Chaiwatanarat

Udomsinprasert

et al. 2017

WJH

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AIMTo investigate the prevalence of osteopenia and osteoporosis in postoperative biliary atresia (BA) children and the association of bone mineral density (BMD) and biochemical parameters in postKasai BA subjects.METHODSA total of 70 patients with postKasai BA were enrolled in this prospective study. The patients were classified into two groups according to their jaundice status. BMD of the lumbar spine was analyzed using dual energy X-ray absorptiometry.RESULTSThe prevalence of low bone mass (osteopenia and osteoporosis) in BA patients were 51.4% (36 out of 70). Ten patients (35.7%) in the jaundice group and 8 patients (19.0%) in the non-jaundice group had osteopenia. Sixteen patients (57.1%) in the jaundice group and 2 patients (4.8%) in the no jaundice group had osteoporosis. In addition, lumbar spine BMD Z-score was substantially lower in the jaundice BA patients compared with non-jaundice patients. BA subjects with persistent jaundice had significantly lower serum 25-hydroxyvitamin D than those without jaundice. Further analysis revealed that lumbar spine BMD was correlated with age (r = 0.774, P < 0.001), serum albumin (r = 0.333, P = 0.005), total bilirubin (r = -0.476, P < 0.001), aspartate aminotransferase (r = -0.583, P < 0.001), alanine aminotransferase (r = -0.428, P < 0.001), and alkaline phosphatase(r = -0.456, P < 0.001).CONCLUSIONLow BMD was associated with biochemical parameters reflecting the severity of cholestasis in postKasai BA patients.

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Is biliary atresia an autoimmune disease?

Cited by 17 publications

References 0 publications

Elevation of serum urokinase plasminogen activator receptor and liver stiffness in postoperative biliary atresia

Elevation of serum urokinase plasminogen activator receptor and liver stiffness in postoperative biliary atresia

Elevated serum periostin is associated with liver stiffness and clinical outcome in biliary atresia

Low bone mineral density and the severity of cholestasis in biliary atresia

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