Long-term follow-up after surgery for patients with biliary atresia

The extent of biliary proliferation in liver biopsies from patients with biliary atresia at portoenterostomy is associated with the postoperative prognosis

“…However, when comparing with FS and age in a multivariable analysis, PCK7 was the only independent variable associated with 1-year native liver survival (Tables 2 and 3). Age at the time of surgery is thought to have a detrimental effect on the post-Kasai prognosis [2,[23][24][25][26][27][28][29]. Altman et al [12] found equal outcomes in the younger than 49 days and the 50-to 70-day age groups, which represent an improved prognosis compared with that of the older than 70 days age group.…”

Shear wave elastography helps differentiate biliary atresia from other neonatal/infantile liver diseases

“…Additionally, this imaging test is time-consuming, requiring up to 5 days of pretreatment with phenobarbital and sometimes necessitating 24-h delayed images. Because biliary atresia is a rapidly progressive disease, a delay in diagnosis and surgery could substantially affect the success rate of bile flow restoration with the Kasai portoenterostomy and consequently result in an early need for liver transplantation [2,4,5].…”

A long-term follow-up of five patients with atresia of the common bile duct

“…There are few publications documenting the precise pathology of type 1 BA with information on long-term outcome. [5][6][7][8][9] The relatively mild liver damage estimated by the gross appearance at surgery and the pre-and intraoperative histologic findings, the presence of patent proximal extrahepatic and intrahepatic bile ducts at surgery, the rapid disappearance of jaundice, the nearly full normalization of the preoperative liver pathology, and the fact that only one patient (patient 1) experienced repeated late cholangitis, suggest that the reason for the favorable outcome was not primarily surgical correctability in these cases but a fortunate biology of the anomaly. One might presume that the destructive inflammatory process that characterizes the majority of BA patients did not result in typical pan-involvement in our 5 patients, but it affected only the distal part of the extrahepatic bile ducts causing their obstruction and left the proximal extrahepatic and intrahepatic bile ducts intact or did not effect seriously the patent biliary trees.…”