Abstract

We aimed to evaluate long-term growth in children and young people with autoimmune liver disease (AILD) treated with daily steroids. This is a retrospective observational cohort study of patients diagnosed between 1992 and 2004 before the age of 16 years. Growth measurements (height, weight and body mass index (BMI)) converted to z-scores were recorded, at diagnosis, 1 and 5 years after commencing treatment and at age 18 years and analyzed together with demographics, disease and treatment related characteristics. Seventy-four patients (35 female) were started on treatment at median age of 12.85 (Inter quartile range (IQR) 9.44, 14.14) years for median duration of 12.07 (IQR 8.68, 13.97) years. At all time-points, the mean z-scores for weight, height and BMI were within the normal range, indicating normal nutritional status. There was no difference in change in z-score for weight, height and BMI from diagnosis until age 18 years when comparing gender (male vs female), ethnicity (Caucasian vs non-Caucasian), diagnosis (AIH vs ASC) and presence of IBD (n = 23). Change in z-score was lower for height and weight for the < 12 years group compared to the ≥12 years age group ( P < 0.05 and P < 0.05, respectively). In addition, change in height z-score correlated positively with age at start of steroid treatment (r = 0.321, P < 0.05) and negatively with duration of steroid treatment (r = -0.321, P < 0.05). Growth of patients with AILD on a daily maintenance dose of steroids remains stable and within normal range during long-term follow up. Small, daily doses are effective in maintaining disease control and minimize the need for high-dose steroid pulses during relapses.

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