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Impaired Bone Health in Children With Biliary Atresia
Satu Ruuska1,2,3, Saila Laakso4,5,6, Outi Leskinen7
1Department of Pediatric Gastroenterology.
Insights
Children with biliary atresia (BA) face higher risks of rickets and bone fractures. Bone mineral density is generally normal between ages 5-10, regardless of liver transplant status.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Pediatric Endocrinology
Background:
- Biliary atresia (BA) is a severe neonatal cholestatic liver disease.
- Early diagnosis and treatment are crucial for outcomes.
- Long-term complications, including bone health issues, require thorough investigation.
Purpose of the Study:
- To investigate the incidence of rickets and bone fractures in children with BA.
- To assess areal bone mineral density (aBMD) in this patient cohort.
- To identify factors influencing bone health in BA survivors.
Main Methods:
- Retrospective data collection from BA patients in Finland (2000-2018).
- Analysis of medical history, including rickets, fractures, and 25-hydroxyvitamin D [25(OH)D] levels.
- Review of plain radiographs and dual energy X-ray absorptiometry (DXA) for aBMD assessment.
Main Results:
- 14% of BA patients were diagnosed with rickets; jaundice clearance was protective.
- 22% experienced at least one bone fracture during childhood/adolescence.
- Median lumbar spine aBMD z-scores were generally within the normal range by age 5 and 10, irrespective of liver transplantation.
Conclusions:
- Biliary atresia patients exhibit an elevated risk for rickets and fractures.
- Bone mineral density in BA patients typically normalizes between 5 and 10 years of age.
- Monitoring bone health is essential for managing long-term outcomes in BA survivors.
Objectives:
The aim of the study was 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, postsurgical medications, and history of rickets and bone fractures were collected retrospectively. Serum levels of 25-hydroxyvitamin D [25(OH)D] postportoenterostomy (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% confidence interval [CI] 0.00266-0.393; 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 1-9) 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 with the normal population. Most BA patients have aBMD within normal range between 5 and 10 years of age irrespective of liver transplantation status.
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