Autoimmune polyglandular syndromes. Irene Fung PGY2 Endocrinology rounds January 30, 2009. Objectives. Overview of autoimmune endocrine disease To recognize the clinical presentation of APS-1 and APS-2 To understand work-up and management of primary adrenal insufficiency and hypothyroidism
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Irene Fung PGY2
January 30, 2009
ACTH stimulation test
Providing thyroxine can increase cortisol clearance
Adrenal crisis can be precipitated if hypothyroidism treated without first providing GC replacement!
Hydrocortisone 10 mg/m2/daypo divided TID
Fludrocortisone (Florinef) 0.05-0.3 mg po OD
How does AIRE control expression of a range of genes encoding proteins with divergent transcriptional regulation in their usual cellular locations?
How is expression of AIRE controlled?
Other proteins does AIRE partner with?
Pediatrics in Review. 2005;26:68-74.
17 yo girl w/ pedal edema and fatigue x 2 mths
- due to excessive TRH production and decreased PRL clearance
Elevated liver enzymes, LDH, creatine kinase
The study of AIRE mutations in APS-1 demonstrates mechanisms of self-tolerance during thymic T-cell selection