Endocrine Abstracts

The polyglandular autoimmune syndromes (PAS) are rare disorders characterized as multiple autoimmune-mediated organ failure. PAS III includes chronic autoimmune thyroiditis with type 1 diabetes mellitus or pernicious anemia and/or vitiligo or alopecia and many other organ-specific autoimmune diseases, but never involves adrenal cortex. Several patients with PAS III and associated type 1 gastric neuroendocrine tumor (NET) have already been reported. We report probably the first...

Acute intermittent porphyria (AIP) is an uncommon monogenic autosomal dominant disorder with a defect in the haem biosynthesis pathway at the level of the enzyme porphobilinogen deaminase. The disease has low penetrance; most patients could stay asymptomatic throughout life. Clinical manifestation depends on various precipitants, which increase hepatic haem production and cytochrome activity. We report a case of a female patient who had regular moderate-to-severe cyclic AIP at...