ArticleJournal of inherited metabolic disease2003
Bone mineral density in children, adolescents and adults with glycogen storage disease type Ia: a cross-sectional and longitudinal study.
Article in Journal of inherited metabolic disease, 2003. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 35 citations in OpenAlex.
- Article
- Glycogen Storage Disease Type I and Bone: Clinical and Cellular Characterization.Calcified tissue international · 2024Article
- Endocrine involvement in hepatic glycogen storage diseases: pathophysiology and implications for care.Reviews in endocrine & metabolic disorders · 2024Review
- Article
- Evaluation of Body Composition, Physical Activity, and Food Intake in Patients with Inborn Errors of Intermediary Metabolism.Nutrients · 2021Observational
- ANTHROPOMETRIC AND DIETARY ASSESSMENT OF PATIENTS WITH GLYCOGENOSIS TYPE I.Revista paulista de pediatria : orgao oficial da Sociedade de Pediatria de Sao Paulo · 2021Article
- Over 20-Year Follow-up of Patients with Hepatic Glycogen Storage Diseases: Single-Center Experience.Diagnostics (Basel, Switzerland) · 2020Article
- A patient with glycogen storage disease type Ia combined with chronic hepatitis B infection: a case report.BMC medical genetics · 2019Article
- Non-osteogenic muscle hypertrophy in children with McArdle disease.Journal of inherited metabolic disease · 2018Article
- Bone health in patients with inborn errors of metabolism.Reviews in endocrine & metabolic disorders · 2018Review
- Glycogen storage disease type III: diagnosis, genotype, management, clinical course and outcome.Journal of inherited metabolic disease · 2016Article
- Bone mineral density in glycogen storage disease type Ia and Ib.Genetics in medicine : official journal of the American College of Medical Genetics · 2013Article
- Nutritional issues in treating phenylketonuria.Journal of inherited metabolic disease · 2010Review
- Vitamin E supplementation improves neutropenia and reduces the frequency of infections in patients with glycogen storage disease type 1b.European journal of pediatrics · 2009Article
- The truth of treating patients with phenylketonuria after childhood: the need for a new guideline.Journal of inherited metabolic disease · 2008Review
- A high activity index of stearoyl-CoA desaturase is associated with increased risk of fracture in men.Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA · 2008Article
- Reduction in bone mineral density in glycogenosis type III may be due to a mixed muscle and bone deficit.Journal of inherited metabolic disease · 2008Article
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Authors and funding
7 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The occurrence of (symptoms related to) osteopenia is a known complication in glycogen storage disease type Ia (GSD Ia) patients. However, only limited information is available about bone mineral density (BMD). Using dual energy x-ray absorptiometry, we studied both cross-sectional and longitudinal lumbar spine areal BMD (BMD(areal) in g/cm2), areal BMD corrected for delayed bone maturation (BMD(bone age) in g/cm2), and volumetric BMD (BMD(vol) in g/cm3). Prepubertal GSD Ia patients (n = 8) had normal BMD (median z-scores BMD(areal) -0.6, BMD(bone age) -0.5 and BMD(vol) -0.5), whereas adolescent patients (n = 12) and adult patients (n = 9) had significantly reduced BMD (BMD(areal) -2.3, BMD(bone age) -1.6, BMD(vol) -2.0, and BMD(areal) -1.9, BMD(vol) -1.5, respectively). Our longitudinal study, showing a stable BMD(areal) but a trend to a decrease in BMD(vol) in prepubertal patients during follow-up, did not clarify whether the difference in BMD between prepubertal and adolescent/adult patients reflects a diminished accretion of BMD during childhood or reflects historical differences in treatment. In adolescent and adult GSD Ia patients, BMD(areal) and BMD(vol) were reduced but stable during follow-up. Especially patients with delayed bone maturation were at risk for reduced BMD. No correlation between parameters of metabolic control and BMD could be detected. Daily calcium intake was within recommended allowances ranges. Abnormal biochemical results included hypomagnesaemia (29%), hypercalciuria (34%) and reduced tubular resorption of phosphate (21%). Although the underlying pathophysiology of reduced BMD in GSD Ia remains unsolved, metabolic control should be optimized to correct as much as possible metabolic and endocrine abnormalities that may influence both bone matrix formation and bone mineral accretion.
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