title: Bile duct enlargement by infusion of L-proline: potential significance in biliary atresia.
abstract: The normal signals of control of bile duct morphogenesis and growth are unknown. Consequently, aberrant development is poorly understood, as for example in those infants with biliary atresia or hypoplasia. A previous report by Isseroff et al., showed that a possible mechanism for the common bile duct enlargement by the parasite Fasciola hepatica was excess L-proline secreted by this parasite. This suggested to us that L-proline might be a specific growth factor for bile duct epithelium, and therefore possibly useful in the understanding and treatment of biliary maldevelopment. We now report an experimental study demonstrating the effect of L-proline on the normal biliary tree of the mouse. We also present a summary to date of an ongoing clinical restrospective study. These experimental findings suggest: (1) there may be a specific requirement for L-proline during the rapid growth of the biliary tract during neonatal life; (2) bile duct hypoplasia and atresia may result from a defect in proline metabolism; (3) proline administration during the early phase of biliary atresia may possibly be therapeutic.
mesh_terms: D000818:Animals; D001652:Bile Ducts / Q000002:abnormalities* / Q000187:drug effects / Q000254:growth & development*; D008297:Male; D051379:Mice; D008815:Mice, Inbred Strains; D011392:Proline / Q000378:metabolism / Q000494:pharmacology*
chemical_list: D011392:Proline