Pathology 3500 Lecture 25: Endocrine Disease III
Lecture 010: Endocrine Disease III
Congenital Adrenal Hyperplasia
● Results from a number of autosomal recessive enzymatic defect in the
biosynthesis of cortisol from cholesterol
○ Can’t produce glucocorticoids/ mineralocorticoids
■ Precursors are diverted into sex steroids
○ Deficiency of 21-hydrpyxlages (90% of cases )
■ Convert 17-OH progesterone to 1-deoxycortisol
○ Impaired cortisol synthesis
■ Precursors converted to androgens
○ Enlarged adrenal glands due increased ACTh
■ No negative feedback
○ Virilizing CAH, female infants exhibit pseudohermaphroditism
● Symptoms depend on the enzymatic defect
○ May see salt-wasting CAH if aldosterone synthesis is also impaired
○ Hyponatremia, hyperkalemia, dehydration, gyptian, increase renin secretion, may
be fatal
○ Virilization of newborns
■ Pseudohermaphroditism of baby girls
● Treatment
○ Administration of glucocorticoid and mineralocorticoid to reduced ACTH and
missing hormones
Adrenal insufficiency (hypofunction)
● Acute adrenocortical insufficiency
○ Causes
■ Massive adrenal hemorrhage (Waterhouse-Friderichsen Syndrome)
● Overwhelming sepsis
● Meningococcal associated
● Anticoagulated patients
● Disseminated intravascular coagulation (DIC)
■ Sudden withdrawal of long-term exogenous corticosteroid therapy
■ Stress in patient with chronic adrenal insufficiency
○ May be life-threatening
■ Cardiovascular collapse
■ Marked electrolyte disturbances
■ Requires emergency steroid therapy
● Chronic adrenal insufficiency
○ Autoimmune disease/Addison’s disease (autoimmune adrenalitis) most
common
■ Adrenal becomes small and fibrotic (destruction of cortex)
● Lymphocytic infiltrate
● Only small aggregates of cortical cells remain
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Document Summary
Results from a number of autosomal recessive enzymatic defect in the biosynthesis of cortisol from cholesterol. Deficiency of 21-hydrpyxlages (90% of cases ) May see salt-wasting cah if aldosterone synthesis is also impaired. Hyponatremia, hyperkalemia, dehydration, gyptian, increase renin secretion, may be fatal. Administration of glucocorticoid and mineralocorticoid to reduced acth and missing hormones. Sudden withdrawal of long-term exogenous corticosteroid therapy. Stress in patient with chronic adrenal insufficiency. Autoimmune disease/addison"s disease (autoimmune adrenalitis) most common. Adrenal becomes small and fibrotic (destruction of cortex) Only small aggregates of cortical cells remain. Symptoms due to a lack of drenaocortial hormones. Cortisol decrease leads to increase acth and melanocyte stimulating hormone (msh) Tumors that metastasize to the adrenal gland (lung) There are two main compartments of the pancreas. Island of cell in the middle of purple/blue cells. Cells are organized around a duct where they can secrete the digestive enzymes into. Regulates glucose utilization in cell thereby reducing blood glucose.