Federica Gaiani, Pierpacifico Gismondi, Roberta Minelli, Giovanni Casadio, Nicola de'Angelis, Fabiola Fornaroli, Gian Luigi de'Angelis, Marco Manfredi
RATIONALE: Triple-A syndrome, or Allgrove syndrome (AS), is a rare autosomal recessive disorder characterized by the alacrimia, achalasia, and adrenal insufficiency triad. Alacrimia usually starts at early infancy, while achalasia and adrenal insufficiency appear later during childhood or adulthood. Some patients may also present with the so-called Double-A syndrome (i.e., alacrimia and achalasia, or alacrimia and adrenal insufficiency); adrenal insufficiency usually represents a life-threatening event due to severe hypoglycemia...
May 29, 2020: Medicine (Baltimore)