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163 Cards in this Set
- Front
- Back
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anterior lobe of the pituitary is derived from
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Rathke's puch
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posterior lobe of the pituitary is derived from
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an outpouching of the 3rd ventricle
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supraoptic & paraventricular nuclei produce what?
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ADH & Oxytocin
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blood supply to the posterior pituitary?
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via the inferior hypophysial artery which is a branch from the internal carotid artery
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three types of cells that can be identified in the anterior pituitary on routine H & E staining
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acidophils, basophils & chromophobes
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the 5 cell types in the adenohypophysis by immunostaining
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1) lactotrophs- Prl
2) somatotrophs- GH 3) corticotrophs: ACTH 4) thyrotrophs: TSH 5) gonadotrophs: FSH & LH |
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the precursor for ACTH
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POMC- proopiomelanocortin
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the hypothalamic releasing hormone that inhibits its corresponding anterior pituitary hormone
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dopamine----> inhibits prolactin
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2 types of nonfunctioning pituitary adenomas
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silent-- some hormones, not enough to cause endocrine manifestations
hormone negative- no evidence of any hormone production |
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the MC pleurihormonal adenoma
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somatomanotroph adenomas (GH + Prl)
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stalk effect
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macroadenoma--> compresses the pituitary stalk --> impedes delivery of dopamine from hypothalamus to ant pituitary--> lack of inhibition of prolactin--> freed up lactotrophs--> increase prolactin release--> hyperprolactinemia
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histology of a pituitary adenoma
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monotonously uniform (monomorphic) population of cells--> arranged in solid sheets & are not divided into discrete lobules by fibroconnective tissue trabeculae.
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other causes of hyperprolactinemia besides lactotrophs
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hypothalamic diseases
anti-dopaminergic drugs estrogen therapy renal failure primary hypothyroidism |
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name the oncogene expressed in 40 of somatotroph adenomas. what does it cause?
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gsp- mutation in the alpha subunit that causes it to lose its GTPase activity. this results in persistent binding of GTP to the alpha subunit which in turn results in constitutive activation of adenyl cyclase & unchecked proliferation of somatotrophs
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what tests would be most helpful to confirm dx of somatotroph adenoma/
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IGF-1
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moon face, buffalo hump, & truncal obesity are d/t
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secondary hyperinsulinemia associated w/ cushing's disease.
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what two pathologies that result in cushing's disease cannot be suppressed by dexamethasome?
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cortisol secreting adrenal cortical neoplasm & ACTH secreting nonendocrine neoplasm
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two categories of causes of hypopituitarism & name what is in each category
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hypothalamic lesions: tumor, inflammatory disorders & infections (sarcoid & TB)
pituitary lesions: tumors, surgery/ radiation, inflammatory disorders & infections, SHeehan syndrome (post partum hypopituitarism) & genetic defects |
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genetic defect that results in combined deficiencies of GH, prolactin & TSH?
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POU1F1--> encodes for a pituitary specific transcription factor
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MC presentation of hypopituitarism
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panhypopituitarism
kids: dwarfism & infantilism Adults: hypogonadism, hypothyroidism & hypoadrenalism |
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patient with a macroadenoma would most likely present with:
a) Visual field defects b) hemiparesis c) vertigo d) facial paralysis e) diplopia |
a) visual field defects
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name the 3 hypothalamic (suprasellar) neoplasms
what kind of mass effects do these neoplasms cause? |
craniopharyngioma, gliomas, germ cell tumors
mass effects: hypopituitarism & DI |
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MC tumor in suprasellar region
what is its origin? |
craniopharyngioma
bimodal age distribution (one peak 5-15 yrs, and second peak >65) origin: Rathke's pouch its benign, involving calcifications |
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histology of nonfunctioning adenomas, when are they most common & what percentage of pituitary adenomas are nonfunctioning?
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histo: chromophobic cells or intensely eosinophilic cells, sparsely granular
MC; 4th decade of life percentage: 20-25% |
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sheehan syndrome percentage of gland destroyed before sxs
later morphology of the pituitary in sheehan syndrome |
75%
later: shrunken fibrous scar-- empty sella syndrome |
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what is the body's compensatory mechanism for dealing with SIADH?
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increase in ANP --> but it does not lead to hypervolemia, incr in BP, and peripheral edema
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other causes of SIADH besides small cell carcinoma
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non-neoplastic lung dz (sarcoid, TB), CNS trauma & infections, some drugs & endocrine disorders
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excess descent of the thyroid gland during development leads to--->a
incomplete descent --> b persistence of remnants of thyroglossal duct-->c |
a) substernal thyroid
b) lingual thyroid c) thyroglossal duct cyst |
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colloid is normally rich in _____
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thyroglobulin
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3 most common causes of hyperthyroidism
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graves disease, toxic multinodular goiter, toxic adenoma
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3 sporadic causes of cretinism
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thyroid dysgenesis
inherited defects in thyroid hormone synthesis (dyshormonogenetic goiter) thyroid hormone resistance syndrome---- AD inherited mutations in thyroid hormone receptor--> abolish the ability of the receptor to bind T3 |
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increased susceptibility is associated with ______ in hashimoto thyroiditis
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polymorphism, esp CTLA4 & PTPN22
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hashimoto thyroiditis pts are at risk for what other diseases
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DM, SLE, Myasthenia Gravis, Sjogren's syndrome, and B cell Nonhodgkin lymphoma.
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HLA and other genes assoc w/ graves
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HLA-DR3, polymorphisms in CTLA4 & PTPN22 genes
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scalloping & papillary hyperplasia
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Graves dz
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2 causes for a diffuse nontoxic goiter:
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edemic: via iodine deficiency or goitrogens (cabbage, cauliflower, turnips, cassava root)
or sporadic: via goitrogens, hereditary defect in thyroid hormone synthesis |
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mass effects of multinodular goiter; morphology of goiter
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compression, of trachea, vessels & nerves, & dysphagia
goiter: massive enlargment, nodules w/ a mixture of hyperplastic & dilated follicles, involutional changes: hemorrhage, fibrosis, calcification & cystic degeneration. |
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hyperthyroidism d/t a toxic multinodular goiter can be distinguished from graves by...
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the multinodular goiter does not cause opthalmopathy or dermopathy
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young person & a man w/ a nodule, think _____
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neoplastic
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what is the gross appearance & histology of a thyroid adenomas?
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gross: sharply demarcated solitary nodule
histology: fibrous capsule separates the neoplastic tissue from the surrounding compressed fland. |
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name the histological pattern of these thyroid adenomas:
1) embryonal 2) fetal 3) oncocytic |
1) trabecular
2) microfollicular 3) macrofollicular & hurthle cell these generally come up cold on scintillation scans. |
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genes associated w/ papillary CA
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RET/PTC oncogene (20-40%): constitutively active tyrosine kinase
BRAF gene (30-50%): gain of function mutations--> constitutive activation of MAP kinase pathway--> upregulated growth signal--> correlates w/ prognostic factors like metastasis & extra thyroidal extension |
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oncogene strongly associated with radiation exposure
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RET protoncogene
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name the gross and histological features of papillary CA
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gross: unencapsulated, infiltrative, often cystic w/ foci of fibrosis & calcification
histo: papillary fornds, empty looking nuclei "orphan annie eye" & psammoma bodies |
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name the gross and histological features of follicular CA
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gross: varies; well circumscribed--> extensive invasive
histo: small uniform follicles containing colloid w/ capsular and/ or vascular invasion |
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what genes are involved in follicular CA?
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gain of function mutations of RAS & PIK3CA--> unregulated growth signal
PAX8-PPARG fusion gene |
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what marker is used to monitor for recurrence of follicular CA?
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thyroglobulin
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where can the follicular CA spread to? what is the prognosis for minimally invasive? widely invasive?
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spreads to: bones, lungs, liver
prog: minimally (only invade capsule)= >90% 10 yr survival widely (thru overlying capsule into normal thyroid parenchyma)= 50% 10 yr survival rate |
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what is the gene associated with medullary CA? what is the tumor marker?
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RET protoncogene
tumor marker: calcitonin |
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histology of medullary CA
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cell nests, amyloid deposits in strome, + calcitonin & chromogranin (for thyroglobulin)
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morphological pattern of anaplastic ca
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giant cell & spindle cell types
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histology of parathyroid glands
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cheif cells & fat cells. Chief cells may undergo transition to oxyphil cells (Mitochondria) & water clear cells.
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what are three common causes of hypercalcemia?
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autonomous PTH hypersecretion, osteolytic metastases, PTH-related protein (PTHrP)
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what are the 3 causes associated w/ primary hyperparathyroidism? which one is MC and what genes are associated?
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parathyroid adenoma- MC (overexpression of cyclin D, mutation in MEN 1), primary parathyroid hyperplasia, parathyroid carcinoma
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familial cases associated to primary hyperparathyroidism
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MEN-1, MEN- 2A, & Familial hypocalciuric hypercalcemia
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primary hyperparathyroidism can come in 3 morphologies, what are they?
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adenoma- solitary, encapsulated
hyperplasia- all 4 glands carcinoma- solitayr, dense capsule |
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brown tumors
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osteitis fibrosa cystica
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dissecting osteitis
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osteoclasts actively engaged in bone resorption boring through trabecula of bone.
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2 causes of compensatory hypersecretion of PTH d/t hypocalcemia.
morphology as well |
causes: chronic renal failure, vit D deficiency
morphology: hyperplasia of all 4 parathyroid glands. ---renal osteodystrophy---- |
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calcifications of the basal ganglia and eye lens
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hypoparathyroidism
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what genes are involved in the AD hypoparathyroism?
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gain of fxn mutations in CaSR gene
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hypocalcemi and hyperphosphatemia w/ high serum PTH levels w/ hyperplasia of the parathyroid glands
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pseudohypoparathyroidism
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end organ resistance to PTH; both types
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pseudohypoparathyroidism
type 1a: albright hereditary osteodystrophy --- dec cAMP response to PTH ( def of G salpha) type 1b: normal cAMP response to PTH, but w/ dec response to cAMP |
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short stature, round face, short neck, short metacarpals (4th & 5th digits)
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albright hereditary osteodystrophy
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MC etiology for cushing syndrome? which one has an associated germline mutation?
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exogenous: GC therapy
germline mutation: Adrenal hypersecretion of cortisol (GNAS- constitutively active Gs alpha)-- via an adenoma or carcinoma |
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what tumors besides small cell CA cause an ectopic secretion of CRH or ACTH?
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medullary CA of thyroid
Carcinoid tumors pancreatic islet cell tumors |
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in cushing syndrome:
adenomas are ___ & ____ bland appearing CA are ___ & often ___ |
small & cytologically
large & anaplastic |
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in Cushing disease, what do the adrenals look like? what about in adrenocortical neoplasms?
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cushings- b/l hyperplastic adrenals
neoplasms: uninvolved adrenal cortex = atrophic d/ ACTH suppression |
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MC cause of primary hyperaldosteronism.
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B/L idiopathic hyperaldosteronism
2nd- adrenocortical neoplasm (adenoma-- conn syndrome) or CA |
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Glucocorticoid remediable hyperaldosteronism
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AD disorder
fusion of aldosterone synthase & 11beta hydroxylase genes on chr 8. --> adrenocortical cells secrete hybrid steroids (w/ cortisol & aldosterone) d/t fusion gene tx: w/ GCs .... go figure |
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21- hydroxylase= a
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a) MC b/l adrenocortical hyperplasia, salt wasting, and masculinity
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waterhouse friderichsen syndrome--- what does it look like? what is it? mortality?
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meningococcal septiciemia---DIC w/ widespread purpura
massive b/l adrenal hemorrhage-- acute AI SHOCK--> high mortality rate |
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how much of the adrenal cortex must be destroyed for addison's dz? what are 90% of the causes of Addison's?
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> or = to 90%
causes: AI adrenalitis, TB, AIDS, metastatic cancers |
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Na, K, BP:
Addison's: Cushing: |
A: hypOnatremia, hypERkalemia, hyOtension
C: hypERnatremia, hypOkalemia, hypERtension |
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what are the secondary causes of adrenal insufficiency? how does this differ from primary?
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hypothalamic or pituitary lesions--> dec ACTH
b/l adrenal cortical atrophy, sparing the zone glomerulosa. --- no sodium or potassium abnormalities |
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organs of zuckerkandl is another name for ______
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aorticosympathetic paraganglia--- which are sympathetic and controlled somewhat by the adrenal medulla.
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what are the three groups of the extra-adrenal paraganglia?
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branchiomeric, intravagal, and aorticosympathetic
determined based on their location |
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Pheochromocytoma
name all the 10%'s |
10% extra adrenal
10% b/l in sporadic cases (up 2 50% in familial) 10% in kiddos 10% are malignant (in adrenal cases, up to 40% in extra adrenal cases) |
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which familial syndromes are associated with pheochromocytoma? (4)
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MEN-2A and 2B
neurofibromatosis type 1 Von Hippel-Lindau disease |
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Histo of a pheochromocytoma
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large tumor cells w/ abundant eosinophilic cytoplasm w/ "zeballen" growth pattern--- rounded nests surrounded by delicate fibrovascular tissue trabeculae
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MEN -1
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germline mutations in tumor suppressor gene (MEN 1 gene) on chr. 11q13
- parathyroid hyperplasia or adenoma - pancreatic islet cell tumors (gastrinomas, insulinomas, VIPomas) - pituitary adenoma |
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gastrinomas produce______
insulinomas produce _______ VIPomas produce ________ where is most common site of gastrinomas? |
gastrinomas= peptic ulcers (zollinger ellison syndrome)
insulinomas= hypoglycemia VIPomas = watery diarrhea MC site= duodenum |
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MEN-2A
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germline mutations in RET protooncogene on chr 10q11.2
- C cell hyperplasia & medullar thyroid carcinoma (100%) Pheochromocytoma - often b/l and in extra adrenal paraganglia (paragangliomas) - parathyroid hyperplasia |
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MEN-2B
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same germline mutation as MEN-2A: germline mutations in RET protooncogene on chr 10q11.2
- C cell hyperplasia & medullary thyroid Ca (100%) - Pheochromocytoma - Ganglioneuromas -- skin, eyes, mouth, GI, respiratory & bladder (100%) - Marfanoid body habitus |
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Familial Medullary thryoid cancer
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only strong predispositiont od ev medullary thyroid CA. no other MEN manifestations. FMTC develop cancer later
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name cell type responsible for producing the corresponding hormone:
a) insulin b) glucagon c) somatostatin d) pancreatic polypeptide e) VIP f) serotonin |
a) B-cells
b) alpha cells c) delta cella d) PP cells e) D1 cells f) enterochromaffin cells |
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dx of DM
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random glucose >200 + signs and sxs
fasting glucose > 126 on more than on occasion abnormal glucose tolerance test in which the glucose level is >200 mg/ dl 2 hours after a standard carbohydrate load. |
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what three genetic syndrome are assoc w/ diabetes? they are also associated w/ what thyroid issue?
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Down syndrome, kleinfelter & turner--> also assoc w/ hashimoto thyroiditis
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only glucose can stimulate insulin _____. What is always expressed on Beta cella/
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synthesis... GLUT-2
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what greatly enances transport of glucose into the cell?
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GLUT-4
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in fat cells insulin promotes:
a) dec glucose uptake b) incr lipogenesis c) inc lipolysis d) inc protein synthesis e) incr glycogen synthesis |
b) inc lipogenesis
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what is the HLA haplotype for DM 1? what do the sxs of type 1 DM start to occur?
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HLA-DR3, or DR4; when the B-cells have been destroyed
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what are the three ketones produced in DKA?
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acetoacetic acid, B-hydroxybutyric acid & acetone
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what is the most important even in the pathgenesis of type 2 diabetes?
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development of decreased responsiveness of the target cells to insulin.
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T/F
Genetic factor is more important in DM 1 than DM 2. |
FALSE
DM2 has a HUUUGGGEE Genetic component |
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what does obesity result in in relation to adipokines?
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incr secretion of adipokines that promotes insulin resistance, coupled w/ dec secretion of adipokines that promote insulin sensitivity
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what would be the insulin reading on a pt in the early stage of B cell failure?
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hyperinsulinemia--> so low blood sugar.
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four factors contributing to Beta cell failure?
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genetics
apoptosis- if p53 is intact, the some of those damaged cells are undergoing apoptosis amyloidosis: amylin is cosecreted & copackaged w/ insulin--- inc insulin output= inc amyloid output inc FFA |
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what does DM1 pt and their islet cells look like clinically, genetically & pathologically?
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young, <20
normal weight dec blood insulin anti islet cell abs DKA common HLA-D linked AI driven insulinitis early, marked atrophy & fibrosis and beta-cell depletion of islet cells |
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what does DM2 pt and their islet cells look like clinically, genetically & pathologically?
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older >40
obese eu or hyperglycemic no antiislet cell abs DKA rare no HLA association insulin resistance no insulinitis focal atrophy & amyloid deposits, mild Beta-cell depletion |
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Which of the following statements regarding Type 1 diabetes is correct?
onset >40 obese insulin resistant DKA rare anti-islet cell abs |
anti-islet cell abs
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MODY
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maturity onset diabetes of the young--AD, onset before 25, impaired B cell fxn, normal weight no autoabs against islet, no insulin resistance, NOT AI.
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which MODY is this:
mutations in glucokinase, mild hyperglycemia. 50% of pt develop gestational diabetes |
MODY 2
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which MODY is this:
mutations in insulin promoter factor 1---> decreased B cell mass |
4
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which MODY is this:
mutations in neurogenic differentiation factor---> decreased B cell mass |
6
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what are the 3 Mody's with severe B cell secretory defect?
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1 (transcription factor 4 alpha), 3 (transcription factor 1 alpha), 5 (hepatocyte nuclear factor 1beta)
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what do the AGEs induce in DM 2 pt? what can they do to the heart?
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crosslinking of type IV collagen in BM --> BM becomes more leaky
heart: trap LDL particles in artery walls--> atherosclerosis. and contribute to hyaline arteriolosclerosis. |
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PKC is activated by what? what does that result in?
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PKC is activated by increase in intracellular glucose... PKC activation causes incr VEGF (angiogenesis factor), incr endothelin & dec NO (ischemia), incr profibrogenic molecules, incr plasminogen activator inhibitor-1, incr pro-inflammatory cytokines
bottom line: neovascularization, ischemia, clotting, & further damage. |
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how does hyperglycemia cause oxidative damage?
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by being converted to sorbitol & using NADPH... a dec in NADPH will cause an increase in oxidative cell damage d/t oxidize glutathione.
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Which of the following statements regarding MODY is correct?
a) an acquired form of diabetes b) pts exhibit insulin resistance c) pts have insulitis d) pts are usually obese e) pts have impaire beta-cell function |
e) impaired beta cell fxn.
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How does a DM dev neovascular glaucoma?
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VEGF in anterior chamber forms neovascular membrane over the surface of the iris which can then occluse the outflow of aqueous humor through trabecular meshwork --> increased intraocular pressure --> damage to optic nerve (glaucoma)
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what are the diabetic ocular complications?
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retinopathy:
d/t incr capillary permeability, dec pericytes (loss of additional support that are contigous to the capillaries)--> hemorrhages, ischemia (d/t hyaline arteriosclerosis & VEGF ) |
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Which of the following is a pathologic feature of diabetic microangiopathy?
a-Thinning of the capillary basement membrane b- Apoptosis of the capillary endothelial cells c-inc permeability of the capillary basement membrane d-Development of fenestrations in the capillary endothelium |
c- inc permeability of the capillary basement membrane
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MC type of islet cell tumor
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insulinomas-- 90%= benign
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clinical manifestation: Hyperglycemia/mild diabetes, anemia, necrolytic migratory erythema (erythematous rash around mouth or anal genital area– erosion and crusting)
name the neoplasm |
glucagonoma
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clinical manifestation
Hyperglycemia/ mild diabetes, cholelithiasis, steatorrhea, hypochlorhydria name the neoplasm |
somatostatinoma: Somatostatin inhibits release of cholecystokinin and insulin. Cholecystokinin is the major hormone responsible for gallbladder contraction and pancreatic enzyme secretion. Somatostatin also inhibits release of gastrin resulting in hypochlorhydria. Somatostatins inhibit release of lipase by inhibiting CCK which can cause steatorrhea.
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clinical manifestation:
facial flushing, hypotension periorbital edema, lacrimation what neoplasm caused this? |
enterochromaffin cell (carcinoid) tumor
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Which of the following islet cell tumors is responsible for the Zollinger-Ellison syndrome?
a-insulinoma b- gastrinoma c- glucagonoma d- somatostatinoma e- vipoma |
b- gastrinoma
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what changes will you see histologically in benign nephrosclerosis?
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hyaline arteriolosclerosis & fibroelastic hyperplasia
(occurs in the interlobular arteries & arcuate arteries & consist of hypertrophy of the media, duplication of the elastic lamina & increased myofibroblastic tissue in the intima). |
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what changes will you see histologically in Malignant nephrosclerosis?
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diastolic pressure >120
necrotizing glomerulitis & cortical infarcts with hyperplastic arteriolosclerosis!!!!! |
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in Renal artery stenosis, surgery cures up to 80% of pts if performed before ________ develops in the opposite kidney
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hyaline arteriolosclerosis
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what type of fibromuscular dysplasia is the MC type?
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medial
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what are thrombotic microangiopathies? what organs are principally involved?
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Group of conditions having in common thrombosis in small vessels, microangiopathic hemolytic anemia (MHA- characterized by schistocytes--- fragmented RBCs in peripheral blood smear), thrombocytopenia
HUS (kidneys-- endothelial injury= initiating event) & TTP (CNS- platelet activation & aggregation is the initating event) |
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what is morphology of HUS/TTP?
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fibrinoid necrosis, thrombi in small vessels, cortical infarcts, hyperplastic arteriolosclerosis & cortical scars in chronic cases
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what is deficient in familial HUS?
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Factor H which when deficient causes an incr in the alternative pathways C3 convertase activity
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what conditions are under the antiphospholipid ab syndrome umbrella?
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scleroderma & malignant HTN, postpartum renal failure, chemotherapeutic & immunosuppressive drugs.
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what is the deficiency in TTP? autoantibody is against what?
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ADAMTS13 which is a protease that cleaves very large vWF multimers. a deficiency in ADAMTS-13 --> accumulation of large vWFs --> platelet aggregaion --> microthrombi. Most have an autoab against ADAMTS-13
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how does pancreatitis result in ATN?
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causing "third spacing" of fluid within the abdomen which leads to hypovolemia --> reduces renal blood flow & can cause ATN.
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4 mechanisms for dec urine output?
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obstruction, backleak, sublethal endothelial injury, tubuloglomerular feedback.
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tubuloglomerular feedback
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incr delivery of Nacl to distal tubule--> incr release of adenosine from macula densa --> constriction of afferent arteriole --> dec blood flow to glomerulus --> dec GFR.
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what are the three phases of ATN?
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1) initiation phase: slight dec urine output, incr BUN & Cr
2) maintenance phase: oliguria, incr BUN & Cr, salt & H20 retention, hyperkalemia & metabolic acidosis 3) recovery phase; polyuria & electrolyte loss- hypokalemia. |
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UA results
a) Degenerating epithelial cells & “dirty brown” granular casts b)Red blood cell casts & proteinuria c) Pyuria & white blood cell casts |
a) Acute Tubular Necrosis
b)Acute Glomerulonephritis c)Acute Tubulointerstitial Nephritis |
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chronic pyelonephritis is an important cause of what?
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ESRD
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what is the morphology of chronic Pyelonephritis?
|
THYROIDIZATION!!!!!!!!!!
coarse scars overlying blunted or deformed calyces, chronic interstitial inflammation & fibrosis, tubular atrophy |
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what condition is associated with Associated with an increased risk of transitional cell carcinoma of the renal pelvis?
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analgesic nephropathy
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what gene is lost or inactivated or mutation in clear cell carcinoma?
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1 of the VHL genes is lost and the other VHL gene is mutation or inactivated by hypermethylastion.
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what is the gene associated with the familial form of papillary carcinoma?
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MET protooncogene
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what is the histology of chromophobe renal carcinoma?
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Sheets of cells with pale eosinophilic cytoplasm & perinuclear halos
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renal cell CA paraneoplastic syndromes:
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polycythemia (erythropoietin),
hypercalcemia (PTH-related protein), Cushing syndrome (ACTH), hypertension (renin), amyloidosis, eosinophilia |
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Triphasic pattern: blastema, stroma & epithelial elements.
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Wilms tumor
mc primary renal tumor in children |
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syndromes associated with incr risk of wilms tumor
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WAGR syndrome, denys-drash syndrome, beckwith wiedemann syndrome
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WAGR syndrome
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aniridia, genital anomalies, &mental retardation
germline deletion at 11p13--- loss of PAX6 & WT-1 |
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denys-drash syndrome
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gonadal dysgenesis, nephropathy, gemline missense mutation of WT-1=== 90% will develop wilms tumor
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beckwith-wiedemann syndrome:
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organomegaly, hemihypertrophy, macroglossia... overexpressionf of IGF2 gene. Affected region is WT-2
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Michaelis gutmann bodies
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malacoplakia--- reflects an acquired defect in macrophage degradative function
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risk factors for bladder carcinoma
|
Cigarette smoking
Exposure to arylamines (esp. 2-naphthylamine) Schistosoma haematobium infection (~ 70% of the tumors are squamous) Analgesic abuse Cyclophosphamide exposure Bladder irradiation |
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papilloma
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papillae lined by normal appearing urothelium--- do not recur or invade
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PUNLMP
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papillae lined by thicker than normal urothelium- may recur but do not invade
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LG papillary urothelial carcinoma
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papillae lined by thick urothelium w/ mild atypia-- may recur <10% invade
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HG papillary urothelial carcinoma
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papillae lined by urothelium exhibiting marked atypia w/ freq mitoses-- freq recur ~80% invade
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carcinomas arising in the posterior urethra are most commonly _______ carcinomas
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transitional cell
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carcinomas arisng in the anterior urethra are most commonly _______ carcinomas
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squamous cell
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Fabry disease
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x linked inherited disorder resulting in a deficiency of the enzyme alpha galactosidase A--> accumulation of glucosphingolipids in the glomeruli --> chronic renal failure.
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hypercellular glomeruli, granular deposits of IgG, IgM & C3 in mesangiium and along GBM. electron dense deposits-- "humps"
GN? |
poststreptococcal glomerulonephritis
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type I RPGN?
type II RPGN? Type II RPGN? |
1) anti-GBM dz (goodpasture)
2)immune complex mediated (PSGN, lupus henoch schonlen) 3) pauci immune (wegener G, microscopic) |
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Type II MPGN is associated with what factor
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C3 nephritic factor (C3NeF) which stabilizes alternative pathways C3 convertase.
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which MPGN is assoc w/ HBV & HCV?
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secondary
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nerve deafness + eye disorder + GN
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alport syndrome
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where is the defect in the x-linked version of alport syndrome?
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alpha 5 chain of collagen type 4
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where is the mutation in thin basement membrane lesion?
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genes encoding alpha 3 or alpha 4 of type 4 collagen
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PKHD1 encodes?
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fibrocystin (ARPKD)
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MC genetic cause of ESRD in children and young adults
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nephronophthisis
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immature mesenchyme, cartilage & immature collecting ducts + enlarged cysts
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multicystic renal dysplasia
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