Pathology 3500 Lecture Notes - Lecture 29: Glycosuria, Osmoreceptor, Hepatocyte

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Congenital adrenal hyperplasia: results from a number of autosomal recessive enzymatic defects in the biosynthesis of cortisol from cholesterol, deficiency of 21 hydroxylase (90% of cases), this converts 17-oh progesterone to 11- deoxycortisol. Adrenal insufficiency (hypofunction: acute adrenal insufficiency, chronic adrenal insufficiency. Acute adrenal insufficiency: massive adrenal hemorrhage (waterhouse-friderichsen syndrome) Sudden withdrawal of long term exogenous corticosteroid therapy (should be tapered) Stress in patients with chronic adrenal insufficiency: may be life threatening - emergency steroid therapy, causes, overwhelming sepsis (waterhouse-friderichsen syndrome) - often meningococcus associated, anticoagulated patients, disseminated intravascular coagulation. Chronic adrenal insufficiency (addison disease) - causes: autoimmune disease (autoimmune adrenalitis) - most common, progressive destruction of adrenal gland. Infection: tuberculosis, opportunistic infections (hiv associated, metastatic disease (cancer, tumors) Lots of lymphocytes infiltrating the cortex and have small aggregates of cortical cells that remain but a lot are destroyed. Endocrine pancreas pathology: pancreas secretes digestion enzymes into small bowel.

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