Biliary atresia due to delayed maturation of the gut hormones system? – Introducing a new treatment modality

Michel, Erik; Kilavuz, Ö.; Jäger, Ralph; Nasir, R.

doi:10.1515/jpm.2004.054

Journal of Perinatal Medicine

2004

DOI: 10.1515/jpm.2004.054

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Biliary atresia due to delayed maturation of the gut hormones system? – Introducing a new treatment modality

Erik Michel¹,

Ö. Kilavuz²,

Ralph Jäger³

et al.

Abstract: We hypothesize that dysmaturation may lead to insufficient induction/production/activity of intrinsic gut hormones resulting in prenatally impaired bile flow, or even inspissated bile. Familial occurrence suggests a genetic defect. Exogenous hormone therapy might be an appropriate treatment modality.

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2020

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Functional Role of the Secretin/Secretin Receptor Signaling During Cholestatic Liver Injury

Baiocchi

Zhou

et al. 2020

Hepatology

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iver diseases are a major health concern and affect a large proportion of people worldwide. There are over 100 types of liver disorders, including cirrhosis, cholangiocarcinoma (CCA), hepatocellular carcinoma, and hepatitis. Despite the relevant number of people who are affected by liver diseases, and the increased awareness with regard to these disorders, the number of deaths corresponding to liver injury is expected to increase in the foreseeable future. One of the possible reasons for this is that a complete comprehension of the mechanisms of hepatic damage involving specific liver anatomical districts is lacking, and, as a consequence, current treatments available are suboptimal. A major burden in the clinical setting are chronic cholestatic liver diseases (e.g., primary biliary cholangitis [PBC], primary sclerosing cholangitis [PSC], biliary atresia), which target the biliary epithelium and are characterized by cholestasis. (1,2) Because the secretin (Sct)/secretin receptor (SR) axis (expressed only by cholangiocytes in the liver) (3,4) is the major regulator of ductal bile secretion, (5,6) it is intuitive that this axis plays a key role in the maintenance of biliary homeostasis during the progression of cholangiopathies. For instance, PBC is characterized by reduced bicarbonate secretion, a phenomenon possibly impeding the formation of an HCO − 3 canalicular film ("bicarbonate umbrella") on bile ducts, which has protective properties against highly concentrated bile acids (BAs). (1,7,8) In this review, we examined the molecular mechanisms by which the Sct/SR axis regulates biliary function and the homeostasis of the biliary epithelium in normal and pathophysiological conditions. Sct/SR aXIS: tHe BaSICS Sct, a neuroendocrine, gastrointestinal peptide of 27 amino acids is secreted primarily by the intestinal S cells located in the Lieberkühn crypts. There is evidence that Sct is also secreted by other epithelia in different organs including the pancreas, intestine and liver, thus exerting various biological effects in

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Functional Role of the Secretin/Secretin Receptor Signaling During Cholestatic Liver Injury

Baiocchi

Zhou

et al. 2020

Hepatology

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show abstract

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Biliary atresia due to delayed maturation of the gut hormones system? – Introducing a new treatment modality

Cited by 1 publication

References 27 publications

Functional Role of the Secretin/Secretin Receptor Signaling During Cholestatic Liver Injury

Functional Role of the Secretin/Secretin Receptor Signaling During Cholestatic Liver Injury

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