Treatment
If the intrahepatic biliary tree is unaffected, surgical reconstruction of the extrahepatic biliary tract is possible. This surgery is called a Kasai procedure (after the Japanese surgeon who developed the surgery, Dr. Morio Kasai) or hepatoportoenterostomy. This procedure is not usually curative, but ideally does buy time until the child can achieve growth and undergo liver transplantation.
If the atresia is complete, liver transplantation is the only option. Timely Kasai portoenterostomy (e.g. < 60 postnatal days) has shown better outcomes. Nevertheless, a considerable number of the patients, even if Kasai portoenterostomy has been successful, eventually undergo liver transplantation within a couple of years after Kasai portoenterostomy.
Recent large volume studies from Davenport et al. (Ann Surg, 2008) show that age of the patient is not an absolute clinical factor affecting the prognosis. In the latter study, influence of age differs according to the disease etiology—i.e., whether isolated BA, BASM (BA with splenic malformation ), or CBA(cystic biliary atresia).
It is widely accepted that corticosteroid treatment after a Kasai operation, with or without choleretics and antibiotics, has a beneficial effect on the postoperative bile flow and can clear the jaundice; but the dosing and duration of the ideal steroid protocol have been controversial ("blast dose" vs. "high dose" vs. "low dose"). Furthermore, it has been observed in many retrospective longitudinal studies that steroid does not prolong survival of the native liver or transplant-free survival. Davenport et al. also showed (hepatology 2007) that short-term low-dose steroid therapy following a Kasai operation has no effect on the mid- and long-term prognosis of biliary atresia patients.
Read more about this topic: Biliary Atresia
Famous quotes containing the word treatment:
“The treatment of the incident of the assault upon the sailors of the Baltimore is so conciliatory and friendly that I am of the opinion that there is a good prospect that the differences growing out of that serious affair can now be adjusted upon terms satisfactory to this Government by the usual methods and without special powers from Congress.”
—Benjamin Harrison (1833–1901)
“Our treatment of both older people and children reflects the value we place on independence and autonomy. We do our best to make our children independent from birth. We leave them all alone in rooms with the lights out and tell them, “Go to sleep by yourselves.” And the old people we respect most are the ones who will fight for their independence, who would sooner starve to death than ask for help.”
—Margaret Mead (1901–1978)
“Ambivalence reaches the level of schizophrenia in our treatment of violence among the young. Parents do not encourage violence, but neither do they take up arms against the industries which encourage it. Parents hide their eyes from the books and comics, slasher films, videos and lyrics which form the texture of an adolescent culture. While all successful societies have inhibited instinct, ours encourages it. Or at least we profess ourselves powerless to interfere with it.”
—C. John Sommerville (20th century)