Abstract
An association between achalasia of the cardia and adrenal insufficiency has been found in pairs of siblings in two different families .Endocrine and post mortem studies indicate that mineralocorticoid function was normal in these four children who appear to have selective cortisol deficiency secondary to degeneration of the z.fasciculata and z.reticularis. Two further families with achalasia and adrenal insufficiency have been found in the literature.The condition appears to be inherited as a recessive disorder but the relationship between the adrenal and oesophageal abnormalities is unknown.
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Grant, D., Allgrove, J., Clayden, G. et al. Familial glucocorticoid deficiency and Achalasia of the Cardia. Pediatr Res 12, 1092 (1978). https://doi.org/10.1203/00006450-197811000-00065
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DOI: https://doi.org/10.1203/00006450-197811000-00065
