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Good therapeutic effect after PEBD including improved liver function test results and decreased total bile acids in serum, as well as decreased pruritus. The cholic acid (CA)/Chenodeoxycholic Acid(CDCA) ratio in biliary bile also decreased. Patient 2, however, experienced little decrease in pruritus after PEBD, and showed no clear change in the CA/CDCA ratio. Nonetheless, this patient’s course did not rapidly worsen. Increased bile secretion and a decreased CA/CDCA ratio in biliary bile may be the most important responses after PEBD. Relief of pruritus is also an important sign of therapeutic effect. Moreover, progression of symptoms in PFIC1 sometimes can be delayed by PEBD, resulting in a relatively mild BRIC-like course, even when the CA/Chenodeoxycholic Acid ratio and pruritus show little change. However, mechanisms underlying clinical benefits remain obscure.  The above showed the effect after use PEBD.
Detailed explanation follows, chenodeoxycholic acid (CDCA) increased among biliary bile acids while the cholic acid (CA)/CDCA ratio decreased in patient 1. No clear change in the CA/CDCA ratio was apparent after PEBD. When serum bile acids reflected cholestasis (elevated TBA in serum), serum hyocholic acid (HCA) was increased. When TBA were elevated in urine, both urine and serum HCA increased. In addition, in the presence of cholestasis, percentages of 1β-hydroxylated bile acids and ketonic bile acids among TBA rose in the urine.
Chenodeoxycholic Acid is not secreted via bile canaliculi. The BSEP readily secretes monovalent bile acids, including unconjugated CA, glycine-conjugated CA, taurine-conjugated CA, and taurine-conjugated deoxycholic acid. Their findings suggest that the bile acid pool is not greatly altered after PEBD, and that a increased CA/CDCA ratio could account for improvement of PFIC1 patients after PEBD. However, the bile acid pool size and composition of biliary bile was not compared in detail between samples obtained before and after PEBD. In patients obtaining relief after PEBD, the change in bile acid pool size resulting from the treatment is unknown. We suggest that bile acid pool size most likely is decreased, because choletasis is eliminated after successful PEBD. Moreover, in the previous study, Jericho et al did not compare CA/CDCA ratios in samples obtained before and after PEBD. Kurbegov et al have reported, increased total bile acids concentrations and a decrease in the CA/CDCA ratio in biliary bile from patients benefiting from PEBD.

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