The 4A syndrome association with osteoporosis

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Ozgen A., Ercan E., Ozutemiz O., Hamulu F., Bayraktar F., Yilmaz C.

ENDOCRINE JOURNAL, cilt.46, sa.1, ss.227-230, 1999 (SCI-Expanded, Scopus) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 46 Sayı: 1
  • Basım Tarihi: 1999
  • Doi Numarası: 10.1507/endocrj.46.227
  • Dergi Adı: ENDOCRINE JOURNAL
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.227-230
  • Anahtar Kelimeler: Allgrove syndrome, 4A syndrome, osteoporosis, sensorimotor neuropathy, triple A syndrome
  • Açık Arşiv Koleksiyonu: AVESİS Açık Erişim Koleksiyonu
  • Dokuz Eylül Üniversitesi Adresli: Hayır

Özet

4A syndrome is characterised by adrenocortical insufficiency, achalasia, alacrima, autonomic and other neurological abnormalities. We report an 18-year-old boy with 4 A syndrome and having all classical features of the disease including sensorimotor neuropathy. In addition, the patient had low aldosterone levels and signs of osteoporosis, which apparently developed without glucocorticoid replacement therapy. Although it is speculated that the lack of local growth factors, nutritional deficiency secondary to achalasia or receptor abnormalities regarding bone metabolism contribute to osteoporosis, its etiopathogenesis still needs to be clarified.