The 4A syndrome association with osteoporosis.
Articles > Article · 1999
Authors: Ahmet Gökhan ÖZGEN, E ERCAN, Ahmet Ömer ÖZÜTEMİZ, F HAMULU, F BAYRAKTAR, CENGİZ YILMAZ
ENDOCRINE JOURNAL, Journal Indexed in SCI Expanded, International, 1999, vol 46, pp 227-230
Summary
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.