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58 Cards in this Set

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Layers of the adrenal gland, superficial to deep, including the hormones made?
Capsule, zona glomerulosa: aldosterone, zona fasciculata: glucocorticoids(cortisol), zona reticularis: sex steriods(estrogen and androgen), Medulla: catecholamines(epinephrine)
Pathogenesis of Cushing syndrome?
Pituitary of hyothalamic(Basophilic adenoma), Adrenal(tumor of hyperplasia), Paraneoplastic(small cell carcinoma of lung: ectopic ACTH), Iatrogenic(exogenous source: most common)
Clinical manifestation of Cushing syndrome?
Central obesity, Moon facies, weakness and fatigability, Hirsutism, acne, Hypertension, plethora, diabetes, osteoporosis, psychosis, purple stria, easy bruising, poor wound healing
Test to determine type of Cushing Syndrome?
Dexamethasone test.
Pituitary: resistant to low dose
Ectopic: Completely insensitive
Adrenal: Low ACTH
Conn syndrome?
Primary hyperaldosteronism, solitary adenoma of the cortex
Clinical Manifestations of Primary hyperaldosteronism?
Hypertension, sodium and water retention, hypokalemia, often with hypokalemic alkalosis, weakness, parasthesia, visual disterbances
Renin level due to Primary hyperaldosteronism?
Renin level decreases with due to negative feedback
Causes of secondary hyperaldosronism serum renin?
Renal ischemia, Renal tumor, edema. Serum renin is increased
Pathogenesis of Adrenal virilism?
Congenital enzyme defects (21/11-hydroxylase), lead to decrease cortisol, leads to increased ACTH, adrenal hyperplasia, increased androgenic steroid produced
Congenital Adrenal Hyperplasia?
AR, 90% of CAH, 21-hydroxylase deficiency (CYP21B), Salt wasting and andrigogenitalism
Waterhouse-Freidrichsen Syndrome?
Hemorrhagic necrosis of adrenals due to Meningococcal meningitis. Associated with Disseminated intravascular Coaggulation(DIC).
Results in catastrophic adrenal insufficiency
Primary acute adrenocortical insufficiency?
Can be caused by rapid withdrawal of steroids when adrenals have been supressed for long time
Primary Chronic adrenocortical insufficiency?
Addison's disease
Etiologies of Addison's disease?
Autoimmune adrenalitis(70%)
Infectios(tuberculosis of fungi)
Cancer Mets(lung, stomach and breast)
Autoimmune adrenalitis?
APS1: AIRE gene, candidiasis
APS2: Thyroiditis and DM type1
Isolated Autoimmine Addison's disease
Layers of the adrenal gland, superficial to deep, including the hormones made?
Capsule, zona glomerulosa: aldosterone, zona fasciculata: glucocorticoids(cortisol), zona reticularis: sex steriods(estrogen and androgen), Medulla: catecholamines(epinephrine)
Pathogenesis of Cushing syndrome?
Pituitary of hyothalamic(Basophilic adenoma), Adrenal(tumor of hyperplasia), Paraneoplastic(small cell carcinoma of lung: ectopic ACTH), Iatrogenic(exogenous source: most common)
Clinical manifestation of Cushing syndrome?
Central obesity, Moon facies, weakness and fatigability, Hirsutism, acne, Hypertension, plethora, diabetes, osteoporosis, psychosis, purple stria, easy bruising, poor wound healing
Test to determine type of Cushing Syndrome?
Dexamethasone test.
Pituitary: resistant to low dose
Ectopic: Completely insensitive
Adrenal: Low ACTH
Conn syndrome?
Primary hyperaldosteronism, solitary adenoma of the cortex
Clinical Manifestations of Primary hyperaldosteronism?
Hypertension, sodium and water retention, hypokalemia, often with hypokalemic alkalosis, weakness, parasthesia, visual disterbances
Renin level due to Primary hyperaldosteronism?
Renin level decreases with due to negative feedback
Causes of secondary hyperaldosronism serum renin?
Renal ischemia, Renal tumor, edema. Serum renin is increased
Pathogenesis of Adrenal virilism?
Congenital enzyme defects (21/11-hydroxylase), lead to decrease cortisol, leads to increased ACTH, adrenal hyperplasia, increased androgenic steroid produced
Congenital Adrenal Hyperplasia?
AR, 90% of CAH, 21-hydroxylase deficiency (CYP21B), Salt wasting and andrigogenitalism
Waterhouse-Freidrichsen Syndrome?
Hemorrhagic necrosis of adrenals due to Meningococcal meningitis. Associated with Disseminated intravascular Coaggulation(DIC).
Results in catastrophic adrenal insufficiency
Primary acute adrenocortical insufficiency?
Can be caused by rapid withdrawal of steroids when adrenals have been supressed for long time
Primary Chronic adrenocortical insufficiency?
Addison's disease
Etiologies of Addison's disease?
Autoimmune adrenalitis(70%)
Infectios(tuberculosis of fungi)
Cancer Mets(lung, stomach and breast)
Layers of the adrenal gland, superficial to deep, including the hormones made?
Capsule, zona glomerulosa: aldosterone, zona fasciculata: glucocorticoids(cortisol), zona reticularis: sex steriods(estrogen and androgen), Medulla: catecholamines(epinephrine)
Autoimmune adrenalitis?
APS1: AIRE gene, candidiasis
APS2: Thyroiditis and DM type1
Isolated Autoimmine Addison's disease
Pathogenesis of Cushing syndrome?
Pituitary of hyothalamic(Basophilic adenoma), Adrenal(tumor of hyperplasia), Paraneoplastic(small cell carcinoma of lung: ectopic ACTH), Iatrogenic(exogenous source: most common)
Clinical manifestation of Cushing syndrome?
Central obesity, Moon facies, weakness and fatigability, Hirsutism, acne, Hypertension, plethora, diabetes, osteoporosis, psychosis, purple stria, easy bruising, poor wound healing
Test to determine type of Cushing Syndrome?
Dexamethasone test.
Pituitary: resistant to low dose
Ectopic: Completely insensitive
Adrenal: Low ACTH
Conn syndrome?
Primary hyperaldosteronism, solitary adenoma of the cortex
Clinical Manifestations of Primary hyperaldosteronism?
Hypertension, sodium and water retention, hypokalemia, often with hypokalemic alkalosis, weakness, parasthesia, visual disterbances
Renin level due to Primary hyperaldosteronism?
Renin level decreases with due to negative feedback
Causes of secondary hyperaldosronism serum renin?
Renal ischemia, Renal tumor, edema. Serum renin is increased
Pathogenesis of Adrenal virilism?
Congenital enzyme defects (21/11-hydroxylase), lead to decrease cortisol, leads to increased ACTH, adrenal hyperplasia, increased androgenic steroid produced
Congenital Adrenal Hyperplasia?
AR, 90% of CAH, 21-hydroxylase deficiency (CYP21B), Salt wasting and andrigogenitalism
Waterhouse-Freidrichsen Syndrome?
Hemorrhagic necrosis of adrenals due to Meningococcal meningitis. Associated with Disseminated intravascular Coaggulation(DIC).
Results in catastrophic adrenal insufficiency
Primary acute adrenocortical insufficiency?
Can be caused by rapid withdrawal of steroids when adrenals have been supressed for long time
Primary Chronic adrenocortical insufficiency?
Addison's disease
Etiologies of Addison's disease?
Autoimmune adrenalitis(70%)
Infectios(tuberculosis of fungi)
Cancer Mets(lung, stomach and breast)
Autoimmune adrenalitis?
APS1: AIRE gene, candidiasis
APS2: Thyroiditis and DM type1
Isolated Autoimmine Addison's disease
Clinical manifestation of Addison's Disease?
Weakness and easy fatigability, GI:NVD, weightloss, Hyperpigmentation(increase ACTH/MSH), hyperkalemia, hyponatremia, volume depetion, hypotension, hypoglycemia
Causes of Secondary Adrenocortical insufficiency?
Pituitary and hypothalamus: mets, cancer, infarction, irradiation, no hyperpigmentation, no hyperkalemia/hyponatremia
Chromaffin cells?
From neural crest cells, synthesize and release catecholamines(EPI and NE) in response to preganglionic sypathetic nerve cells
Pheochromocytoma's rules of 10?
10% familial
10% Extrarenal(paragangliomas)
10% of nonfamilial are bilateral
10% arise in childhood(m>f)
Benign of malignant Pheochromocytoma?
Mets are the only way to tell
Clinicall manifestation of Pheochromocytoma?
Hypertension: sustained of paroxysmal, headache, sweating, anxiety, tremor, acute MI, CHF, V-fib, Stroke
Diagnosis of Pheochromocytoma?
Increased urine excretion of catecholamines and metabolites (Vanillylmandelic acid:VMA and metanephrines)
When do you get a Neuroblastoma?
Early Chidhood
What is a Neuroblastoma?
Malignant catecholamine-producing tumor found in adrenal medula or extra-adrenal paraganglionic tissue
Clinical Manefistation of Neuroblastoma?
90% before age 5, large abdominal mass in younger children, Hypertension, sometimes differentiat into benign cells and stop making N-myc gene
Tumors in MEN-I?
Parathyroid: hyperplasia, adenoma
Pituitary adenoma
Pancreas: Hyperplasia, adenoma, carcinoma
MEN-I Gene
Tumors in MEN-IIa?
Thyroid medullary carcinoma (c-cell)
Pheochromocytoma
Hyperparathyroid
RET gene
Tumors in MEN-IIb?
Thyroid Medullary Carcinoma
Pheochromocytoma
Ganglioneuromas
Marfanoid Habitus