Adrenal Gland

Question 1

Cushing Syndrome

Answer 1

• Excess cortisol due to:
• _{^{ACTH secreting pit adenoma (<u>disease)-</u>Bilateral Hypertrophy}}
• _{^{Adrenal cotical tumor <u>(Zona Fasiculata)-</u>Hypertrophy unilateral w/atrophy on other}}
• _{^{Ectopic ACTH secretion:}}
  
  • ^{_{Small cell carcinoma lung, Carcinoid tumor<strong> (GI),</strong>Medullary carcinoma thyroid}}
• _{^{<strong>Exogenous drug intrake</strong>-<strong><u>Corticosteroids bi-lateral atrophic adrenal</u></strong>}}
• Hyperpigmentation in trunk = Pit onset (disease)
• Atrophy of fast twitch muscles (type2)
• Upregulates Alpha 1 receptors-HTN (neuroepi)
• Delayed wound healing/Abdominal striae
  
  • _{^{(inhibition fibroblast w/low collagen)}}
• Osteroporosis-Lumbar spine
• Assoc w/Hirsuitism in Females
• Diagnose:
• Loss of diurnal variation of cort lvl _{^{(increased lvl in evening)}}
• High cort in serum & unrine
• Immunosupression ^{(inhibits phosphoA2/IL-2/Histamine)}
• Dexa supression test high dose suppress ACTH from Ant. Pit _{^{(1mg @ night & 8mg in morning)}} NO supression to small cell

Question 2

Conn Syndrome

Answer 2

• Excess Aldosterone _{^{(Distal tubule & collecting ducts)}}
• Primary=Adrenal cortical tumor _{^{(adenoma zona glom)}}
• Na+ retention & K+ secretion _^(Principal)
• H+ reabsorp metab alkolosis w/HTN _{^{(alpha-intercalated)}}
• Renin LOW _{^{(supression of renin-angiotensin system)}}
• Muscle weakness/cramps
• Treat: Spironolactone
• Secondary=extra adrenal causes:
• Renal artery stenosis
• CCF _{^{<strong>(chronic cardiac failure)</strong>}}
• Nephrotic syndrome
• Pregers
• Renin HIGH (hypovolemia/hypoxia of kidney)

Question 3

Adrenogenital Syndrome (21 & 11)

Answer 3

• 21 most common (high sex/androgen steroid)
• Sodium wasting w/hyperkalemia (lack of aldosterone)
• Hypotension no vascular tone (lack of cortisol)
• mineralocoticoid & glucocorticoid def.
• 11 Beta dehydrogenase def (high sex steroid)
• Sodium retention & Hypertension (hypokalemia)
• Shared Symptoms:
• Increased LVL of ACTH (unoppesed - feedback)
• Female infants=_{^{Clitoral hypertrophy & pseudohermphrodism}}
• Prepubertal female=_{^{Oligomenorrhea, <u>hirsutism</u>, acne}}
• Prepubertal male=_{^{Precocious puberty}}

Question 4

Adrenogenital Syndrome (17 alpha)

Answer 4

• Excess mineralocorticoids (sodium rentention=HTN)
• Complete lack of Gluco & Androgens
• Female=Lack secondary sexual characteristics
  
  • Eunicoid body shape
  • No mammarian development
  • No pubic hair or axilary
• Male=Pseudohermaphoroditism

Question 5

Primary adrenal Insuff (Addison’s)

Answer 5

• Low cortisol, androgen, aldosterone
• HIGH ACTH
• Chronic = Addison’s
• Def of cortical hormones
• Autoimmune adrenalitis assoc with-
• DM 1, thyroiditis, Polyglandular syndrome
• Metasis _{^{(lung cancer)}} or TB _{^{<strong>(developing countries)</strong>}}
• Treat: Steroid _{^{(adrenal crisis & chronic)}}
• Morphology: Bilateral adrenal ATROPHY
• Test: ACTH stim test _{^{(no cortisol + diagnosis)}}
• Symptoms:
  
  • _{^{Weakness, Hypotension, Skin/mucosa hyper-pigmentation <u>(POM-C)</u>}}
  • _Adrenal crisis=_Sudden intracable vomiting, Ab pain, hypotension (collaspe) caused by hemorrhage in atrophic adrenal
• Lab: _{^{Hyponatremia, hypoglycemia, hyperkalemia (acidosis)}}

Question 6

Waterhouse-Friderichsen Syndrome

Answer 6

• Acute Adrenal Insuff
• Child
• Caused by Neisseria meningitides, Pseudomonas, Haemophilus influenzae
  
  • _{^{Neisseria presents w/petechial hemorrhage}}
• Lab: Hyponatremia, Hypoglycemia, HYPERkalemia
• Morphology: Bilater Adrenal hypertrophy _^(black)
• “Sac of blood”
• Histo: Extensive cortical hemorrhage + DIC

Question 7

Pheochromocytoma (adult)

Answer 7

• Neoplasm of medulla (chromafin cells)
• Rule of 10%- bilateral, children, Malignant, familial
  
  • _{^{Can be located on <em><strong>bladder wall</strong></em> <u><strong>(pt have episodes after urinating)</strong></u>}}
• Excess catecholamines (epineph & norephi)
• Arteriolar constriction
• Clinical: Episodic hypertension, headaches, Tachy
• Morphology: Large adrenal neoplasm (grey/tan)
• Histo: Polygonal or spindle cells w/granular cytoplasm
  
  • _{^{Nest, trabecular, solid/diffuse, or MIXED patterns}}
• Increase in Serum metanephrines _{^{(by-product of epi/nore)}} & Urine metanephrines _^(VMA), Free catecholamines
• Assoc with MEN 2A/B & von-hippel

Question 8

Neuroblastoma (Children)

Answer 8

• Neoplasm of medulla-Solid lobulated malignant tumor in children less than 5
• Genetics-1p deletion (N-myc amplification)
• Symptoms:
• Fever & pain (weight loss)
• “Solid painful Abdominal mass in child”
  
  • _{^{D/D wilms tumor, Lyphoma, Kidney Cyst}}
• Metastasis through blood & lymph _{^{(BM, Liver, Lung)}}
• Histo: Small blue round tumor cell high N/C ratio (Homer Write Pseudorosette)
• Lab: VMA, HVA in blood & Urine

Question 9

MEN (type 1)

Answer 9

• Wermer Syndrome (overexpress of cyclin D-1):
• The 3 P’s
• Prolactinoma (GH adenoma)
• Primary parathyroid hyperplasia _{^{(high PTH & High Ca+2)}}
• Pancreatic tumor (Gastrinoma, Insulinoma, VIPoma)
• Diagnose: Indium 111 octreotide scan

Question 10

MEN (type 2a/b)

Answer 10

• 2A Sipple syndrome (young age):
• Autosomal dominant
• Activation of RET protoncogene
• Medullary carcinoma
• Primary parathyroid Hyperplasia
• Pheochromocytoma
• 2B William Syndrome (young age):
• Medullary CA thryroid
• Pheochromocytoma
• Mucosal neuromas _{^{(GI ganglioneuromas & Marfanoid habitus)}}
  
  • Hirschsprung disease