Impaired Bone Health in Children With Biliary Atresia

Objectives: To examine the frequency of rickets and bone fractures and to assess areal bone mineral density (aBMD) in childhood among patients with biliary atresia (BA). Methods: We gathered data on all patients diagnosed with BA in Finland that survived to ≥ 1 year of age between 1 January 2000 to 30 June 2018. Data on gestational age, birth weight, post-surgical medications, and history of rickets and bone fractures were collected retrospectively. Serum levels of 25-hydroxyvitamin D [25(OH)D] post-portoenterostomy (PE) were collected. Plain radiographs and dual energy X-ray absorptiometry (DXA) measurements of study subjects were reviewed. Results: Out of 49 patients, 7 (14%) were diagnosed with rickets during infancy. Clearance of jaundice (odds ratio 0.055, 95% CI 0.00266 to 0.393; p < 0.01) was a protective factor against rickets. Sufficient 25(OH)D levels were reached 3 months post-PE. Eleven (22%) patients suffered at least one bone fracture (range 1 to 9) during childhood and adolescence. In DXA measurements, median lumbar spine aBMD anthropometrically adjusted Z-scores were as follows: in native liver survivors 0.8 (interquartile range [IQR] -1.9 to 1.4) at 5 and -0.3 (IQR -1.3 to 0.8) at 10 years and for liver transplanted patients 0.4 (IQR -0.2 to 1.1) at 5 and 0.6 (IQR -0.1 to 1.3) at 10 years. Conclusions: BA patients have an increased risk for rickets and bone fractures compared to the normal population. Most BA patients have aBMD within normal range between 5 to 10 years of age irrespective of liver transplantation status.

DOI: 10.1097/MPG.0000000000002896