The 4A syndrome association with osteoporosis

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