The 4A Syndrome Association with Osteoporosis

The 4A Syndrome Association with Osteoporosis

Abstract. 4A syndrome is characterised by adrenocortical insufficiency, achalasia, alacrima, autonomic and other neurological abnormalities. We report an 18-year-old boy with 4A syndrome and having all classical features of the disease including sensorimotor neuropathy. In addition, the patient had...

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Bibliographic Details
Published in:ENDOCRINE JOURNAL Vol. 46; no. 1; pp. 227 - 230
Main Authors: A. GOKHAN OZGEN, ERTUGRUL ERCAN, OMER OZUTEMIZ, FUSUN HAMULU, FIRAT BAYRAKTAR, CANDEGER YILMAZ
Format: Journal Article
Language:Japanese
Published: The Japan Endocrine Society 1999
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Summary:Abstract. 4A syndrome is characterised by adrenocortical insufficiency, achalasia, alacrima, autonomic and other neurological abnormalities. We report an 18-year-old boy with 4A 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.
ISSN:0918-8959