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Body composition in young patients with galactose metabolic disorders: a preliminary report

  • Artemis Doulgeraki EMAIL logo , Ioannis Monopolis , Domna Deligianni , Maria Kalogerakou und Kleopatra H. Schulpis
Veröffentlicht/Copyright: 13. September 2013
Journal of Pediatric Endocrinology and Metabolism
Aus der Zeitschrift Journal of Pediatric Endocrinology and Metabolism Band 27 Heft 1-2

Abstract

Aim: Body composition evaluation in patients with galactosemia and its variants.

Methods: The body composition of young subjects with galactose metabolic disorders was analyzed with dual X-ray absorptiometry. The subjects were divided into the classic galactosemia (CG; n=14) group and the group with other galactose metabolic disorders (OGMD, e.g., epimerase deficiency; n=8).

Results: In both groups, bone strength and bone mineral density Z-scores were normal. However, CG patients appeared sarcopenic (median muscle mass Z-score=–1.93), whereas OGMD subjects had low-normal muscle mass. In addition, approximately half of all patients were overweight. In CG patients, bone mass was strongly correlated to muscle mass (r=0.81), whereas in OGMD patients it was positively correlated to body mass index (r=0.78).

Conclusions: An imbalance between muscle and fat mass, especially in patients with classic galactosemia, was observed. Given the observed strong correlation between bone and muscle mass, more effective lifestyle counseling is needed.

Keywords: body composition; bone; galactose; galactosemia
Corresponding author: Artemis Doulgeraki, MD, PhD, MRCPCH, Department of Bone and Mineral Metabolism, Institute of Child Health, “Agia Sophia” Children’s Hospital, 115 27, Athens, Greece, Phone: +30 213 2037360, Fax: +30 210 7700111, E-mail:

The authors wish to thank biochemist H. Michelakakis for her contribution in diagnosing galactosemic patients, and also to A. Theodosiadou, radiology technician, for assisting in filing body composition data.

Conflict of interest statement

The authors have no relevant conflict of interest to disclose.

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Received: 2013-4-23
Accepted: 2013-8-1
Published Online: 2013-09-13
Published in Print: 2014-01-01

©2014 by Walter de Gruyter Berlin Boston

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https://doi.org/10.1515/jpem-2013-0161
Schlagwörter für diesen Artikel
body composition; bone; galactose; galactosemia

Artikel in diesem Heft

  1. Masthead
  2. Masthead
  3. Editorials
  4. Editorial
  5. Consensus, guidelines and reviews
  6. Review article
  7. Adaptation of glucose metabolism to fasting in young children with infectious diseases: a perspective
  8. Images in pediatric endocrinology
  9. Coexistence of testicular microlithiasis with parathyroid adenoma in an obese child
  10. Original articles
  11. Remission of hypertension and electrolyte abnormalities following renal transplantation in a patient with apparent mineralocorticoid excess well documented throughout childhood
  12. Effects of metformin in children and adolescents with Prader-Willi syndrome and early-onset morbid obesity: a pilot study
  13. Glycemic control in familial vs. sporadic type 1 diabetes patients over 5 years
  14. Clinical and laboratory features of temporary brittle bone disease
  15. Age of thelarche and menarche in contemporary US females: a cross-sectional analysis
  16. Teriparatide (rhPTH) treatment in children with syndromic hypoparathyroidism
  17. Retinol and retinyl palmitate in fetal mice liver: sexual dimorphism
  18. Skeletal maturation and predicted adult height in children with premature adrenarche
  19. Homozygosity for a DTDST mutation in a child with multiple epiphyseal dysplasia
  20. Body composition in young patients with galactose metabolic disorders: a preliminary report
  21. Detection of vascular risk markers in children and adolescents with type 1 diabetes
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  23. Factors influencing neonatal thyroid-stimulating hormone concentrations as a measure of population iodine status
  24. Pediatric idiopathic intracranial hypertension and the underlying endocrine-metabolic dysfunction: a pilot study
  25. Patient reports
  26. Hypothalamic hamartoma associated with central precocious puberty and growth hormone deficiency
  27. A pediatric patient with Cushing syndrome caused by ectopic ACTH syndrome and concomitant pituitary incidentalomas
  28. A novel genetic mutation in a Portuguese family with GCK-MODY
  29. Permanent neonatal diabetes mellitus in monozygotic twins achieving low-dose sulfonylurea therapy
  30. Hypocalcemia in a paediatric case: from the clinical features to diagnosis
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  32. Iatrogenic acute pancreatitis due to hypercalcemia in a child with pseudohypoparathyroidism
  33. Clinical expression of familial Williams-Beuren syndrome in a Turkish family
  34. The first report of cabergoline-induced immune hemolytic anemia in an adolescent with prolactinoma
  35. Low-level hyperinsulinism with hypoglycemic spells in an infant with mosaic Turner syndrome and mild Kabuki-like phenotype: a case report and review of the literature
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