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Maroteaux-Lamy syndrome associated with growth hormone deficiency.

Abstract
Growth retardation is a common feature of mucopolysaccharide storage disorders, mostly considered to be a consequence of skeletal changes, Maroteaux-Lamy disease is a subtype of mucopolysaccharidosis, demonstrating somatic changes and skeletal deformities. We present a case with Maroteaux-Lamy disease associated with growth hormone deficiency. Magnetic resonance imaging study revealed marked signal changes in white matter due to the storage in brain and empty sella appearance in sellar region. In the presence of empty sella syndrome, hypothalamic-pituitary dysfunction due to the storage material may have led to growth hormone deficiency in this patient. Therefore, we recommend patients with mucopolysaccharidosis, especially those who have growth retardation, to be evaluated by hormonal and radiological studies.
AuthorsB Büyükgebiz, Y Eroğlu, I Kovanlikaya, A Sen, A Büyükgebiz
JournalJournal of pediatric endocrinology & metabolism : JPEM (J Pediatr Endocrinol Metab) 1995 Oct-Dec Vol. 8 Issue 4 Pg. 305-7 ISSN: 0334-018X [Print] Germany
PMID8821911 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Insulin
  • Levodopa
  • Growth Hormone
Topics
  • Child, Preschool
  • Consanguinity
  • Growth Hormone (blood, deficiency)
  • Humans
  • Infant
  • Insulin
  • Levodopa
  • Magnetic Resonance Imaging
  • Male
  • Mucopolysaccharidosis VI (complications, diagnosis)

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