Abstract
We addressed growth of biliary atresia (BA) patients living with native livers between ages 0–6 and effects of post-surgical corticosteroid treatment on growth. Growth charts of 28 BA patients born in Finland between 1987 and 2017 were retrospectively evaluated. Dosage and length of corticosteroid treatment and hydrocortisone substitution were reviewed. At birth, BA patients were shorter (median height − 0.6 (interquartile range (IQR) − 1.3 to − 0.1) SDS, n = 28, P < 0.001) than general population. Height remained stable during early childhood (median height − 0.6 (IQR − 1.4 to 0.1) SDS for girls and − 0.4 (IQR − 1.6 to 0.2) SDS for boys at 6 years of age). Patients were of normal height adjusted weight at 6 years with a median age and sex-adjusted body mass index (ISO-BMI) of 20.9 (IQR 19.3 to 25.0) for girls and 22.1 (IQR 20.7 to 25.6) for boys. Higher (≥ 50 mg/kg) cumulative post-portoenterostomy prednisolone dosage resulted in 0.18 SDS lower height per treatment week (β − 0.18, SE 0.04, P < 0.001) compared to lower dosage (< 50 mg/kg).Conclusion: BA patients grow normally during early childhood. As high postoperative corticosteroid dosage has a short-term negative effect on height, very high dosages should be avoided.What Is Known:•Growth of biliary atresia patients has mostly been shown to be within normal limits• Corticosteroids may decrease growth rateWhat Is New:•Biliary atresia patients surviving with their native livers are shorter than general population and their mid-parental target height at birth• A high (> 50 mg/kg) cumulative prednisolone dosage has a negative transitory impact on height gain after portoenterostomy
Highlights
Biliary atresia (BA) is an idiopathic fibro-obstructive cholangiopathy manifesting in infancy [20]
Biliary atresia patients surviving with their native livers are shorter than general population and their mid-parental target height at birth
Most patients were diagnosed with BA type 3 and PE was performed at median age 63 days
Summary
Biliary atresia (BA) is an idiopathic fibro-obstructive cholangiopathy manifesting in infancy [20]. The incidence of BA is varied between 1:8000 and 1:10000 reported in Asia to 1:17000–1:20000 in Northern Europe [26,27,28]. Obstruction of intra- and extrahepatic biliary tracks leads to fibrosis, liver failure, and death in the first 2 years. The current first-line surgical treatment is portoenterostomy (PE). Provided PE restores adequate bile flow, between 23% and 44% of patients survive with native livers until the age of 20 [10, 29, 41]. Liver transplantation (LT) remains a second-line treatment option and BA is the most common indication for childhood LTs [20, 30]
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