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E34 Endocrinology
Adrenal Cortex Toronto Notes 2019
Conditions that do NOT Represent True Hirsutism
• Androgen-independent hair
(e.g. lanugo hair)
• Drug-induced hypertrichosis
(e.g. phenytoin, diazoxide, cyclosporine,
Clinical Features
Females
• hirsutism
■ male pattern growth of androgen-dependent terminal body hair in women: back, chest, upper
abdomen, face, linea alba
■ Ferriman-Gallwey scoring system is used to quantify severity of hirsutism
• virilization
■ masculinization: hirsutism, temporal balding, clitoral enlargement, deepening of voice, acne ■ increase in musculature
• defeminization
■ loss of female secondary sex characteristics (i.e. amenorrhea,breast size, infertility)
Males
• minimaleffectsonhair,musclemass,etc.
• inhibitionofgonadotropinsecretionmaycausereductionin:testicularsize,testiculartestosterone
production, and spermatogenesis
Investigations
• testosterone,DHEA-Sasameasureofadrenalandrogenproduction
• LH/FSH(commonlyinPCOS>2.5)
• 17-OHprogesterone,elevatedinCAHdueto21-OHdeficiency;checkonday3ofmenstrualcyclewith
a progesterone level
• forvirilization:CT/MRIofadrenalsandovaries(identifytumours) • ifPCOS,checkbloodglucose,lipids,75gOGTT
Treatment
• discontinuecausativemedications
• antiandrogens, e.g. spironolactone
• oralcontraceptives(increasesexhormonebindingglobulin,whichbindsandrogens>estrogens;reduces
ovarian production of androgens)
• surgicalresectionoftumour
• lowdoseglucocorticoid±mineralocorticoidifCAHsuspected • treatspecificcausativedisorders,e.g.tumours,Cushing’s,etc.
• cosmetictherapy(laser,electrolysis)
Adrenocortical Insufficiency
Definition
• stateofinadequatecortisoland/oraldosteroneproductionbytheadrenalglands
Etiology
PRIMARY (ADDISON’S DISEASE)
Table 25. Etiology of Primary Adrenocortical Insufficiency
minoxidil)
• Topical steroid use
Autoimmune (70-90%) Infection
Infiltrative Vascular
Drugs Others
Isolated adrenal insufficiency
Polyglandular autoimmune syndrome type I and II
Antibodies often directed against adrenal enzymes and 3 cortical zones
TB (7-20%) (most common in developing world) Fungal: histoplasmosis, paracoccidioidomycosis HIV, CMV
Syphilis
African trypanosomiasis
Metastatic cancer (lung>stomach>esophagus>colon>breast); lymphoma Sarcoidosis, amyloidosis, hemochromatosis
Bilateral adrenal hemorrhage (risk increased by heparin and warfarin) Sepsis (meningococcal, Pseudomonas)
Coagulopathy in adults or Waterhouse-Friderichsen syndrome in children Thrombosis, embolism, adrenal infarction
Inhibit cortisol: ketoconazole, etomidate, megestrol acetate Increase cortisol metabolism: rifampin, phenytoin, barbiturates
Adrenoleukodystrophy
Congenital adrenal hypoplasia (impaired steroidogenesis) Familial glucocorticoid deficiency or resistance