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Published on: January 29, 2018
Deficits in size-adjusted bone mass in children with Alagille syndrome
Irene E Olsen1, Richard F Ittenbach, Alisha J Rovner
1Center for Epidemiology and Biostatistics and Division of Neonatology, Cincinnati Children's Hospital Medical Cente, The University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Insights
Children with Alagille syndrome (AGS) exhibit reduced bone size and mass. Improving fat absorption may help prevent bone fragility in these children.
Area of Science:
- Pediatric Endocrinology
- Bone Metabolism
- Genetic Disorders
Background:
- Alagille syndrome (AGS) is a genetic disorder affecting multiple organs.
- Bone health is a significant concern in children with AGS.
- Previous studies have not fully elucidated the factors influencing bone status in AGS.
Purpose of the Study:
- To assess bone status in children with Alagille syndrome (AGS) compared to healthy controls.
- To identify dietary and AGS-related factors associated with bone status in children with AGS.
Main Methods:
- Dual-energy X-ray absorptiometry (DXA) was used to measure bone mineral content (BMC) and bone area (BA).
- Children with AGS and healthy controls were compared, with data adjusted for age, gender, and height (HT).
- Dietary intake and AGS-specific factors like fat absorption were collected.
Main Results:
- Children with AGS had significantly decreased bone area and bone mineral content adjusted for age and height.
- Low bone mineral content for height z-scores (< -2) were observed in 20% (whole body) and 39% (lumbar spine) of AGS subjects.
- Bone mineralization positively correlated with fat absorption but not with dietary intake.
Conclusions:
- Children with Alagille syndrome demonstrate deficits in bone size and mass relative to body size.
- Addressing malabsorption through targeted interventions may be crucial for preventing bone fragility in AGS.
- Early focus on modifiable factors like fat absorption is recommended for AGS care.
Objectives:
To describe bone status in children with Alagille syndrome (AGS) and healthy control children adjusted for age, gender and height (HT), and to identify dietary intake and AGS-related factors associated with bone status.
Methods:
Prepubertal children with AGS and healthy controls comparable in age and ethnicity were evaluated. Subjects were > or =4 years of age, prepubertal and had whole body (WB) and/or lumbar spine (LS) dual energy X-ray absorptiometry (DXA) scans of acceptable quality. Anthropometric (weight, HT), diet and AGS-specific data (e.g., coefficient of fat absorption, labs, liver transplantation) were also collected. Bone area (BA), bone mineral content (BMC) and HT were log transformed for best fit. Bone data were analyzed unadjusted, adjusted for gender, age and HT, and as HT-specific z-scores.
Results:
AGS and control groups were similar in age, pubertal status and ethnicity. Children with AGS were small-for-age, had decreased BA and BMC-for-age, and decreased WB BA and BMC-for-HT z-scores compared to healthy controls. Prevalence of low BMC-for-HT z-scores (< -2) among AGS subjects was 20% for the WB and 39% for the LS. Bone mineralization was positively related to fat absorption but not dietary intake.
Conclusions:
Children with AGS have deficits in bone size and bone mass relative to body size. Modifiable factors, such as treatment of malabsorption should be explored as an early focus of AGS care to prevent bone fragility.
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