Bone metabolism in galactosemia

Autor: M. J. P. G. van Kroonenburgh, Bianca Panis, M. E. Rubio-Gozalbo, Fred H. M. Nieman, P Ph Forget, Cees Vermeer, Paul P.C.A. Menheere
Přispěvatelé: RS: NUTRIM School of Nutrition and Translational Research in Metabolism, RS: CARIM School for Cardiovascular Diseases, Kindergeneeskunde, MUMC+: DA BV Medisch Specialisten Nucleaire Geneesk (9), Biochemie, MUMC+: DA CDL Algemeen (9), MUMC+: KIO Kemta (9)
Jazyk: angličtina
Rok vydání: 2004
Předmět:
Zdroj: Bone, 35(4), 982-987. Elsevier Science
ISSN: 1873-2763
8756-3282
DOI: 10.1016/j.bone.2004.06.004
Popis: Bone metabolism in galactosemia.Panis B, Forget PP, van Kroonenburgh MJ, Vermeer C, Menheere PP, Nieman FH, Rubio-Gozalbo ME.Department of Pediatrics, Metabolic Diseases, University Hospital Maastricht, 6202 AZ Maastricht, The Netherlands.Classical galactosemia is an autosomal recessively inherited disorder of galactose metabolism. Treatment consists of life-long dietary restriction of galactose. Despite treatment, long-term complications occur such as a decreased bone mineral density (BMD). A decreased BMD might be the result of either dietary deficiencies secondary to the galactose-restricted diet or unknown intrinsic factors. In this study, 40 children with classical galactosemia (13 males and 27 females, aged 3-17 years) on dietary treatment were included to gain insight in the bone metabolism of galactosemics. We found weight and height Z scores significantly decreased in galactosemics. Mean areal BMD Z scores of lumbar spine and of femoral neck as measured by Dual energy X-ray Absorptiometry (DXA) were -0.6 (P < 0.001) and -0.3 (P = 0.066), respectively. Mean volumetric BMD of the femoral neck was significant lower in galactosemics (P < 0.001). The recommended dietary allowances (RDA) for calcium, magnesium, zinc, vitamin D, and protein were met in all patients. Mean serum levels of calcium, phosphate, magnesium, zinc, 1,25-dihydroxy vitamin D (1,25OHD), parathormone (PTH), 17-beta estradiol, bone alkaline phosphatase (BAP), and under-carboxylated osteocalcin (ucOC) were normal. Serum levels of IGF-1 Z score, carboxylated osteocalcin (cOC), N-terminal telopeptide (NTX), and C-terminal telopeptide (CTX) were significantly lower in galactosemics than in control subjects. The different bone markers were strongly correlated. The low levels of IGF-1 Z score, formation marker cOC, and resorption markers NTX and CTX suggest a decreased bone metabolism in galactosemics.
Databáze: OpenAIRE
Externí odkaz: