Term
Congenital malformation of kidney characterized by cysts & abnormal tissue (e.g. cartilage)?
is this an inherited disorder? |
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Definition
Dysplastic kidney. No
It can be bilateral --> must be distinguished from ARPKD
if bilateral, renal failure may result |
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Term
| Hepatic manifestations of ARPKD |
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Definition
| congenital hepatic fibrosis & hepatic cysts (leading to portal HTN) |
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Term
| 3 extrarenal manifestations of ADPKD? |
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Definition
1) Berry aneurisms
2) Hepatic cysts
3) Mitral valve prolapse |
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Term
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Definition
| acute tubular necrosis (from sloughed off, necrotic tubular cells) |
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Term
| areas of tubule affected by ischemic ATN? Toxic ATN? |
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Definition
proximal tubule & medullary segment of TAL
Proximal tubule (causes include: aminoglycosides, metals (e.g. lead), myoglobinuria, ethylene glycol (+oxylate crystals), radiocontrast dye, & urate (tumor lysis syndrome) |
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Term
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Definition
Hyperkalemia & metabolic acidosis (also fluid retention)
in recover phase (2-3 weeks after insult), excessive diuresis makes pt susceptible to HYPOkalemia (torsades) |
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Term
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Definition
| acute interstitial nephritis (drug-induced hypersensitivity; T-cell mediated) |
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Term
| 4 causes of papillary necrosis |
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Definition
1) Analgesics (chronic phenacetin & aspirin)
2) Sickle-cell trait or disease
3) Diabetes
4) Severe acute nephritis |
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Term
| name 6 etiologies of nephrotic syndrome (remember categorization in 2's) |
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Definition
1) 2 have foot process effacement --> MCD & FSGS
2) 2 have immune complexes: membranous & MPGN (type I & II)
3) 2 are associated w/ systemic diseases: diabetic nephtropathy & systemic amyloidosis |
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Term
| which nephrotic syndrome responds well to steroids? |
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Definition
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Term
where are the immune deposits in:
1) Membranous nephropathy
2) MPGN type I
3) MPGN type II |
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Definition
1) Subepithelium
2) Subendothelium (more common to have tram-tracks w/ this one)
3) Inside BM (AB stabilizes C3 nephritic factor) |
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Term
| Etiologies of FSGS? (remember the 3 H's) |
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Definition
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Term
| Nephrotic syndrome associated w/ cancer? |
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Definition
membranous nephropathy (subepithelial "spike-and-dome")
also Hep B&C, SLE, Drugs (e.g. NSAIDS, penicillamine) |
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Term
| most common organ involved in systemic amyloidosis? |
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Definition
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Term
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Definition
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Term
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Definition
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Term
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Definition
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Term
| 4 things that characterize nephritic syndrome: |
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Definition
1) Limited proteinuria (<3.5g/day)
2) Oliguria/azotemia
3) Salt retention --> Edema (periorbital) & HTN
4) RBC casts & dysmorphic RBCs in urine |
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Term
Name disease:
1) 2-3 weeks after infection, cola colored urine, oliguria, HTN, periorbital edema
2) Concurrent illness, or recently overcome illness --> nephritic syndrome |
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Definition
1) PSGN
2) IgA nephropathy |
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Term
| what are the crescents made up of in RPGN? |
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Definition
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Term
| where do you see the EDD in PSGN? |
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Definition
| subepithelial, however they deposit subendo, and then work their way subepithelial where they aggregate together |
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Term
| differentiate Churge-strauss syndrome from microscopic polyangiitis: |
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Definition
Both have: pANCA, are vasculitides, & can cause pauci-immune RPGN
CSS: eosinophilia, granulomas, & asthma |
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Term
Inheritence of Alport?
Triad of Alport? |
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Definition
X-linked; defect in TypeIV collagen
1) Isolated hematuria
2) Hearing problems
3) Ocular disturbances (lens dislocation, posterior cataracts, corneal dystrophy) |
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Term
| pyuria w/ sterile culture? |
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Definition
| urethritis. Probably chlamydia or gonorrhea |
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Term
| 2 UTIs that are nitrite negative |
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Definition
| staph saprophyticus & enterococcus |
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Term
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Definition
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Term
Kidney stones:
1) Kidney stone that is radiolucent?
2) Most common kidney stone?
3) Cystinuria |
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Definition
1) Uric acid stone --> alkalinize urine & allopurinol
2) Calcium oxalate (e.g. hypercalcemia, idiopathic hypercalciuria, crohns) --> HCTZ
3) Cysteine stone --> alkalize urine
alkalinization of urine is accomplished by KHCO3 administration. |
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Term
| What is the only stone that forms better in alkaline environment? |
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Definition
| Magnesium-ammonium-phosphate stone (proteus, staghorn calculus, etc) |
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Term
Give pathologic mechanism for each manifestation of chronic renal failure:
1) Anorexia, pericarditis, platelet dysfunction, encephalopathy (w/ asterixis)
2) HTN
3) Hyperkalemia w/ Metabolic acidosis
4) Anemia
5) Hypocalcemia
6) Osteomalacia & osteoporosis |
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Definition
1) uremia
2) Na+/H2O retention
3) tubular dysfunction (esp distal tubule/collecting duct)
4) Decreased EPO production by peritubular interstitial cells
5) Decreased 1-alpha hydroxylation in PT
6) Secondary hyperparathyroidism from decreased 1,25OH-VitD |
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Term
| Renal cancer associated w/ tuberous sclerosis |
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Definition
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Term
| Mutation in this gene predisposes to renal cell carcinoma |
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Definition
VHL --> activates IGF1 & HIF --> increase VEGF & PDGF. Familial form is bilateral & multiple.
Sporadic form is usually unilateral & single in a old male smoker |
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Term
| Why can RCC Px w/ a left-sided varicocele? |
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Definition
| it likes to invade veins (along w/ HCC, these 2 carcinomas are notorious for invading vessels... which is uncommon for carcinomas in general) |
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Term
| VHL gene mutation is associated w/ these 2 cancers |
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Definition
hemangioblastoma of cerebellum & renal cell carcinoma
Also, they have cavernous hemangiomas of skin, mucosa, and organs
VHL is AD and on Chr3 |
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Term
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Definition
Wilms tumor
Aniridia
Genital abnormalities
Retardation (mental & motor) |
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Term
| Beckwith-Wiedemann syndrome |
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Definition
Wilms tumor (mutation in "WT2" not WT1)
Neonatal hypoglycemia
Muscular hemihypertrophy
Organomegaly (including tongue) |
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Term
| 4 paraneoplastic syndromes w/ renal cell carcinoma? |
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Definition
1) EPO (2ndry PCV0
2) Renin (HTN)
3) PTHrP (hypercalcemia)
4) ACTH (cushings) |
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Term
| What mutation occurs early in the "flat" type of transitional cell carcinoma? |
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Definition
| p53 (this type has no low-grade stage; to goes straight to HG to invasion) |
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