Objectives:
Gamma-glutamyl transferase levels (GGT) are typically elevated in biliary atresia (BA), but normal GGT levels have been observed. This cohort of “normal GGT” BA has neither been described nor has the prognostic value of GGT level on outcomes in BA. We aimed to describe outcomes of a single-centre Australian cohort of infants with BA and assess the impact of GGT level at presentation on outcomes in BA.
Methods:
Infants diagnosed with BA between 1991 and 2017 were retrospectively analysed. Outcomes were defined as survival with native liver, liver transplantation (LT), and death. Patients were categorized into normal (<200I U/L) or high GGT groups based on a mean of 3 consecutive GGT values done before Kasai portoenterostomy (KPE). Baseline parameters, age at surgery, clearance of jaundice (COJ), and outcomes were compared between the 2 groups.
Results:
One hundred thirteen infants underwent KPE at median 61 (30–149) days. At a median follow-up of 14.2 (0.9–26.3) years, 35% (39/113) patients were surviving with native liver, 55% (62/113) underwent LT and 11% (12/113) died pretransplant. 12.3% (14/113) patients had normal GGT. Age at KPE and time to COJ were similar between normal and high GGT groups. Normal GGT group had shorter time from KPE to LT (11 vs 18 months, P = 0.02), underwent LT at a younger age (14 vs 20 months, P = 0.04), and had poorer transplant-free survival (P = 0.04) than high GGT group.
Conclusions:
12.3% of infants with BA had normal GGT levels at diagnosis. Low GGT levels at presentation in BA was associated with a poorer outcome.
465'So knowledge wise, of course there is no harm, as a doctor I want to improve my knowledge, but not desperately. I think there is no harm, but again it comes to the same point, even for the ongoing management, the hospital, they take care of everything, and the patients' have their [telephone] numbers.' [Participant 29] Areas of interest 'I would say more regarding the managing. Because in my practice and for my colleagues as well, we fairly accurately make the diagnosis.' [Participant 7] Format 'I think face to face is better, I don't do well with webinars and stuff like that, I think just meeting where we come that would be good.' [Participant 12] Sydney Child Health Program 'A lot of GPs do the Sydney Child Health Program ... Maybe if we have more information about type 1 diabetes on there because I think a lot of GP registrars enrol for that.' [Participant 3]Diabetes conference 'With type 2 diabetes, we've got a diabetes conference, with the endocrinologist at the hospital. So if we've got patients … the endocrinologist -the staff specialist from Westmead Hospital, and we tell them what our problems are, and how we should be treating each patient. But they come and they sort it all out for us, and there is a lot of education there. We learn a lot. But that doesn't happen with type 1 … Before it would be the patient, the diabetic educator, possibly the dietician and the endocrinologist, and me. We'd all sit down at the practice and discuss that person for 20 minutes.' [Participant 10] GP, general practitioner.
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