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F 6/2 Flashcards

1
Q

Where do horseshoe kidneys get “caught?”

A

On IMA

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2
Q

How does AR PCKD present?

A

Infants w worsening RF and HTN
Newborns may present w Potter sequence
Assoc w cong hep fibrosis (leads to portal HTN) and hepatic cysts

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3
Q

How does AD PCKD present?

A

Young adults w HTN (inc renin), hematuria and worsening RF

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4
Q

What is the gene in AD PCKD?

A

APKD1, APKD2

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5
Q

What are non-renal associations w AD PCKD?

A

Berry aneurysm (COD), hepatic cysts, MV prolapse

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6
Q

What is medullary cystic kidney disease and how is it inherited?

A

Cysts in medullary CD
Parenchymal fibrosis - shrunken kidneys, RF
AD

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7
Q

How is azotemia defined?

A

Inc BUN, Inc Cr

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8
Q

What causes prerenal azotemia

A

Dec BF to kidneys

Dec GFR, azotemia, oliguria

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9
Q

What is the BUN:Cr in pre-renal azotemia?

A

> 15

Reabs of fluid and BUN - tubular function intact

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10
Q

What causes postrenal azotemia?

A

Downstream obstruction of UT

Dec GFR, azotemia, oliguria

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11
Q

What is the BUN:Cr in longstanding postrenal azotemia?

A

<15

Tubular damage

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12
Q

What is the most common cause of acture renal failure?

A

ATN

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13
Q

Which areas of the nephron are particularly susceptible to ischemia?

A

PT

Medullary segment of TAL

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14
Q

What area of the nephron is particularly susceptible to toxins?

A

PT

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15
Q

What are the lab findings in ATN?

A

Oliguria w brown granular casts
Inc BUN, Inc Cr
HK (dec renal exc)
Met acidosis (inc anion gap)

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16
Q

What is acute interstitial nephritis?

A

Drug-induced hypersensitivity involving interstitiom and tubules?

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17
Q

What are causes of AIN?

A

NSAIDs, penicillin, diuretics

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18
Q

How does AIN present?

A

Oliguria, fever, rash - days to wks after starting drug

Eosinophils in urine

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19
Q

What is renal papillary necrosis and how does it present?

A

Necrosis of renal papillae

Gross hematuria, flank pain

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20
Q

What are some causes of RPN?

A

Chronic analgesic use (phenacetin, aspiriin)
DM
SCT or SCD
severe acute pyelonephritis

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21
Q

What are the features of nephrotic syndrome?

A 
Proteinuria > 3.5 g/d
hypoalbuminemia - pitting edema
hypogammaglobulinemia - risk inf
hypercoaguable state - loss of AT III
HL, Hchol - fatty casts in urine
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22
Q

What is the most common cause of nephrotic syndrome in children? What is its cause?

A

MCD
Usually idiopathic
may be associated w HL

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23
Q

How does MCD appear on HE, EM, IF?

A

HE - N
EM - effacement of foot processes
IF - N

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24
Q

How does MCD respond to steroids?

A

well

damage is mediated by cytokines from T cells

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25
Is there inc risk of inf in MCD?
No - selective proteinuria | loss of albumin but not Ig
26
What is the most common cause of nephrotic syndrome in Hispanics and AAs?
FSGS
27
What causes FSGS?
Usually idiopathic | may be assoc w HIV, heroin use, SCD
28
What are the findings in FSGS on HE, EM, IF?
HE - focal and segmental sclerosis EM - effacement of foot processes IF - N
29
How does FSGS respond to steroids?
Not well; progresses to CRF
30
What is the most common cause of nephrotic syndrome in caucasian adults?
Membranous nephropathy
31
What causes MN?
Usually idiopathic | assoc w HBV, HCV, solid tumors, SLE, drugs (NSAID, penicillamine)
32
MN - HE, IF, EM
HE - thick GBM IF - granular (IC depo) EM - subepithelial deposits w spike and dome appearance
33
How does MPGN look on HE and IF?
HE - Thick GBM w tram-track appearance | IF - granular (IC depo)
34
Where are the IC deposits located in Type I and II MPGN?
Type I - subendothelial Assoc w HBV, HCV Type II = dense deposit disease - intramembranous assoc w C3 nephritic factor
35
What is C3 nephritic factor?
AutoAb that stabilizes C3 convertase, leading to overactivation of complement, inflammation, and low levels of circulation C3
36
What happens to the kidney in DM?
NEG of vasc BM leads to hyaline arteriolosclerosis
37
What is more affects in DM - aff or eff arteriole?
Efferent Leads to high GFP Hyperfiltration injury leads to microalbuminuria
38
What is the first indication of kidney damage in DM?
Albuminuria
39
What are Kimmelstiel-Wilson nodules?
Sclerosis of the mesangium in diabetic nephropathy
40
What is the most commonly involved organ in systemic amyloidosis?
Kidney
41
Where does amyloid deposit in the kidney?
Mesangium
42
What are the features of nephritic syndrome?
``` proteinuria < 3.5 g/d Oliguria, azotemia salt retention - periorbital edema, HTN RBC casts/dysmorphic RBCs in urine Hypercellular, inflamed glomeruli ```
43
What is the inciting event for PSGN?
GAS skin inf or pharyngitis
44
What are the histological findings of PSGN?
IC depo - granular IF | subepitheial humps - EM
45
What is the IF patterin in Goodpasture?
Linear (anti-BM Ab)
46
What causes Goodpasture?
Ab against collagen in glomerular and alveolar BM
47
How does Goodpasture present?
Hematuria | Hemoptysis young adult males
48
What is the most common type of renal disease in SLE?
Diffuse proliferative GN
49
How does Wegener present on IF, what is it associated w?
Negative (pauci-immune) | c-ANCA
50
How do Churg-Strauss and microscopic polyangiitis present on IF, what is it associated w?
``` Negative IF (pauci-immune) p-ANCA ```
51
What distinguishes Churg-Strauss from microscopic polyangiitis?
Granulomatous inflammation, eosinophilia, arthma
52
What is RPGN?
Nephritic syndrome that progresses to RF in w to m
53
What is found in Bowman space in RPGN? What are they composed of?
Crescents | Fibrin and macrophages
54
What is the most common nephropathy worldwide?
Iga ( Berger)
55
How does IgA nephropathy present?
During childhood as episodic gross or microscopic hematuria w RBC casts Usually following mucosal infection
56
Where does IgA deposit in IgA nephropathy?
Mesangium
57
What is Alport syndrome?
``` Inherited defect in type IV collagen Most commonly X-linked Thinning, splitting GBM Isolated hematuria sensory hearing loss ocular disturbances ```
58
What causes "thyroidization" of the kidney?
Chronic pyelonephritis
59
Scarring at what location is characteristic of VER?
Upper and lower poles
60
What is the most common type of nephrolithiasis?
Calcium oxalate/phosphate
61
What are causes of Ca stones?
Idiopathic hypercalciuria HyperCa Crohn disease
62
What is tx for Ca stones?
HCT
63
What is the most common cause of Ammonium Mg PO4 stones?
Inf w urease pos organisms (Proteus, Klebsiella) | alkaline urine leads to formation of stone
64
What type of stone is radiolucent?
Uric acid
65
What are risk factors for uric acid stones?
``` hot arid climates low urine vol acidic pH gout hyperuricemia (leukemia, MPD) ```
66
What is the tx for uric acid stones?
Hydration | alkalinization of urine (K bicarb)
67
What type of nephrolithiasis is mostly seen in children?
Cystine
68
What is the result of loss of VHL tumor suppressor gene
Increased IGF-1 Inc HIF TF Increased VEGF, PDGF
69
What is Wilms tumor?
Malignant kidney tumor comprised of blastema (immature kidney mesenchyme) primitive glomeruli and tubules stromal cells Most common malignant renal tumor in children avg age = 3y
70
How does Wilms tumor present?
Lg, unilateral flank mass w hematuria and HTN
71
What is WAGR syndrome?
``` Wilms tumor Aniridia Genital abN Mental and motor retardation del of WT1 tumor suppressor gene ```
72
What is Denys-Drash syndrome?
Wilms tumor Prog glomerular disease male pseudohermaphroditism assoc w muts of WT1
73
What is Beckwith-Wiedemann syndrome?
``` Wilms tumor neonatal hypoglycemia muscular hemihypertrophy organomegaly assoc w muts in WT2 gene cluster, particularly IGF1 ```
74
What are the 2 types of urothelial carcinomas and their progression?
Flat - high grade, invades, early p53 muts | Papillary - low grade, prog to high grade then invades
75
What is nimodipine and what is used for following subarachnoid hemorrhage?
CCB | Prevent cerebral vascular spasm
76
What is the most common primary cerebral neoplasm in adults?
Glioblastoma
77
What does cleft 1 become?
External auditory meatus
78
What CNs are associate with arch 1?
V2, V3
79
What CNs are associated with arch 2?
VII
80
What CNs are associated w arch 3?
IX - stylopharyngeus
81
What CNs are associated w arch 4 (and 6)
X
82
What does pouch 3 become
Thymus and inferior PTs
83
What does pouch 4 become?
Super PT
84
What does pouch 5 become/
C cells of thyroid
85
What are the clefts derived from?
Ectoderm
86
What are the arches derived from?
Mesoderm and neural crests
87
What are the pouches derived from?
Endoderm
88
What is a branchial cleft cyst?
Cleft 2, lateral neck | Can have pharyngeal fistula
89
What pharyngeal arches are disrupted in MEN 2A?
PT - 3rd, 4th | Parafollicular cells of thyroid - 4th, 5th
90
What is aberrand in Digeorge?
Aberrant 3rd and 4th pouch deveopment T cell deficiency (athymic) hCa (aPT)
91
What pharyngeal arch is associated with the carotid arteries?
3
92
What pharyngeal arch is associated with the aortic arch?
4
93
What is a cleft lip?
Failure of the fusion of the maxillary and medial nasal processes to fuse
94
What is cleft palate?
Failrue of fusion of lateral palatine process, nasal septum, and/or median palatine process
95
What is Treacher-Collins syndrome?
Failure of the 1st arch to migrate | Mandibular hypoplasia, facial abN
96
What is the msot common congenital malformation of the head and neck?
Unilateral cleft
97
What is the most common arterial malformation in infants?
PDA

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