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Sunday, September 1, 2019

liver biopsy fibrosis scoring for biliary atresia



Mean fibrosis scores (Ishak) for biliary atresia
(liver biopsy with samples fixed in formalin)
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secondly,
Aspartate Aminotransferase-to-Platelet ratio index (APRi) in infants with biliary atresia: prognostic value at presentation.
Grieve A, Makin E, Davenport M
J Pediatr Surg. 2013 Apr; 48(4):789-95.

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fat soluble vitamin doses in neonatal cholestasis

 Fat-Soluble Vitamin Supplementation in the Cholestatic Infant
Vitamin Laboratory Sign of Deficiency Clinical Sign of Deficiency Treatment/Prevention
Vitamin A Retinol: retinol-binding
protein <0.8 mol/mol
Xerophthalmia, keratomalacia Vitamin A: 3,000–10,000 U/d
Vitamin D 25-Hydroxyvitamin
D <14 ng/mL [ deficiency;
<30 ng/mL [ insufficiency
Rickets, osteomalacia Cholecalciferol: 800–5,000 IU/d;
1,25 OH2 cholecalciferol:
0.05–0.2 mg/kg per d
Vitamin K Prolonged prothrombin time,
elevated protein in
vitamin K absence
Coagulopathy Phytonadione: 2.5–5 mg twice
a week to every day
Vitamin E Vitamin E: total serum
lipid ratio <0.6 mg/g
Neurologic changes,
hemolysis
TPGS: 15–25 U/kg per d; D-a
tocopherol: up to 100 U/kg
per d
TPGS¼D-a-tocopheryl polyethylene glycol 1,000 succinate.

Prematurity and biliary atresia (is prematurity protective) ?

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5668326/

"We hypothesized that correcting for prematurity would have no effect on the degree of fibrosis at surgery, with post-natal age being the most important factor; however, that was not the case. There was indeed good correlation of liver fibrosis scores (in both PBA and control groups) with both post-natal and with post-conceptual age, though some of the infants (predominantly in the PBA group) who were many weeks old had very little liver fibrosis for their post-natal age. This was reflected in the observation that both fibrosis and APRi scores were less in the PBA cohort compared to their controls born around term, implying some kind of “protection” of prematurity perhaps. A possible explanation is that the lack of early enteral feeding seen in some of our groups was in some way contributory."

3 types of biliary atresia