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DT3-Exam 3 > IL Renal Review & Cystic Diseases--Leah** > Flashcards

IL Renal Review & Cystic Diseases--Leah** Flashcards

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1
Q

Vertebral level of the kidneys?

A

T12-L3
ONE TWO THREE; THERES THE KIDNEE!
*Note that left kidney is slightly higher than right and that left renal vein is longer than right.

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2
Q

Renal Blood Flow is ______% of total cardiac output?

A

20%

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3
Q

Outermost two parts of kidney

A

-cortex, then columns which are BETWEEN the pyramids.

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4
Q

Describe flow of urine from the medullae to the ureters

A

medullas –> pyramids –> calyces –> pelvis –> ureter

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5
Q

Within the renal pelvis there are _____.

A

renal papillae

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6
Q

Describe the 7 parts of a nephron:

A

-glomerulus/ bowmans capsule –> PT –> thin descending loop –> thick asecending loop –> Distal convoluted tubule –> connecting tubule –> collecting duct

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7
Q

Vascular system surrounding the loops of henle:

A

vasa recta, collects any H2O secreted from thin descending loop

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8
Q

artery that feeds into the afferent arteriole?

A

arcuate artery

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9
Q

Vessels that enter and exit the glomerulus?

A
  • afferent enters glomerulus

- efferent exits

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10
Q

microscopic structures seen in the renal cortex?

A

glomeruli

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11
Q

microscopic structures seen in the renal medullae?

A

straight portions of tubules and collecting ducts

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12
Q

Definition of azotemia

A

-decreased GFR –> elevated Cr and BUN

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13
Q

Definition of uremia

A

-essentially azotemia, but must have manifestation of renal disease in OTHER organ systems as well

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14
Q

three types of azotemia

A

pre-renal, renal, post-renal

  • pre: reduced renal perfusion, not renal malfunction
  • renal: intrinsic renal disease
  • post: downstream urine obstruction
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15
Q

Two examples of post-renal azotemia

A

-BPH, stone

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16
Q

Two examples of pre-renal azotemia

A

-hypovolemic shock, CHF

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17
Q

Example of uremia

A

fibrinous pericarditis secondary to presence of circulating nitrogenous compounds in blood

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18
Q

Rapidly progressive glomerulonephritis is assc with what change?

A

-rapid GFR decrease

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19
Q

How does acute kidney injury manifest?

Reversible?

A
  • rapid GFR decrease
  • oliguria, anuria
  • azotemia
  • usually reversible
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20
Q

Chronic Kidney Disease definition

A
  • common end point of renal disease

- GFR less than 60 for at least 3 months

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21
Q

End Stage Renal Disease definition

A

GFR five or less, will need dialysis

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22
Q

Renal Tubular Diseases cause

A

electrolyte derangement, polyuria

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23
Q

What is a UTI called if it effects the kidney? The bladder?

A
  • kidney: pyelonephritis

- bladder: cystitis

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24
Q

What is Nephrolithiasis?

How does it present?

A
  • kidney stone

- pain and hematuria

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25
Four tissue types that may be effected by renal disease?
- tubules - glomeruli - interstitium - vasculature
26
Diffuse, focal, segmental, and global are used to describe?
- location of disease involvement - diffuse vs focal refers to location in kidney - segmental vs global refers to location in glomeruli
27
Three methods of examining renal tissue and their uses?
- light micro: determines disease process/ cells present - ImmunoF: determines etiology; ie presence of Abs - electron micro: determines structural changes
28
What do the kidneys look like in fetal life/ childhood? Adulthood?
- kids: lobulated kidneys - adult: smooth * Asx if adult retains lobules, NOT the same as ADPKD
29
Bilateral renal agenesis is not compatible with life. Describe the outcome of unilateral renal agenesis.
- compatible with life BUT | - get compensatory hypertrophy and possible glomerulosclerosis --> chronic disease
30
What is oligohydraminos and what can cause it?
- low levels of amniotic fluid - fluid is either LEAKING or not being produced - not being produced= baby isn't peeing = kidney's absent or not functional
31
What are symptoms of oligohydraminos?
``` POTTER P- pulm hypoplasia O- oligohydraminos T- twisted face T- twisted skin E- extremity defects R- renal agenesis *Also: Breech Position ```
32
What is dangerous to patients with unilateral renal agenesis?
``` Any systemic disease with potential for renal damage -DM, pregnancy, HTN, CHF, others -chemo, drugs -renal disease (many others) ```
33
Ddx for a small kidney?
- congenital hypoplasia | - atrophy secondary to some systemic disease
34
In horseshoe kidney, where do the kidneys fuse? | How common is it?
- inferior pole 90%, upper in 10% | - 1:500-1k
35
Where does horseshoe kidney get stuck in development? | Is horseshoe kidney clinically relevant?
- IMA (L3) | - not clinically relevant, usually incidental finding
36
Disease to assc with horseshoe kidney?
Turners, although definitely not all horseshoe kidney patients have Turners, incidence is just higher in that population.
37
Cause of 5-10% chronic kidney disease?
ADPKD, just as common as horseshoe kidneys
38
polycystin 1 + 2 are what type of proteins and cause which diseases?
polycsystin 1 mutation = PKD type 1 polycystin 2 mutation = PKD type 2 *Both code for integral membrane proteins and lead to poor cell-cell/ cell-matrix interactions = POOR TUBULAR GROWTH + ABNORMAL ECM = TUBULAR CYSTS
39
What chromosome is assc with PKD1? PKD2?
PKD1-chromosome 16p (more common, higher number) | PKD2- chromosome 4 (2X2=4)
40
PDK1/2 Which is more severe? More common? Has older age of onsent?
PKD1: earlier onset severe disease (50s), more common AND more severe PKD2 severe disease presents in late 60s
41
Result of tubular cysts in ADPKD (3)
- interstitial inflammation - vascular damage - enlarged cystic kidneys w/ functioning tubules b/n cysts * Cysts seen on EXTERNAL surface
42
ADPKD PE findings: | *What is the cause of the pain?*
- palpable kidneys - hematuria (hemorrhaging into cysts) and CVA tenderness - pain is caused by enlarging/ clot passing cysts
43
Three extrarenal findings that MAY BE assc with ADPKD
-liver cysts -Berry aneurysms -heart valve anomalies (only in smaller percentages of patients)
44
Gene assc with childhood polycystic disease/ ARPKD
PKHD1, fibrocystin, chromosome 6
45
Describe the appearance of childhood ARPKD:
- enlarged SMOOTH kidneys - COLLECTING DUCT cysts = SPONGY cut surface - ALWAYS liver cysts/ fibrosis assc (40% in ADPKD)
46
Four categories of ARPKD? Which are more common? What is the result?
- perinatal, neonatal, infantile, juvenile - *more common are perinatal, neonatal - see death in utero/ sometimes at birth due to - **PULMONARY HYPOPLASIA ensues
47
Medullary Sponge Kidney: two changes in the kidney
- medullary cysts | - collecting duct dilation
48
Medullary Sponge Kidney clinical findings:
- asx, calculi, infections, hematuria etc | - No impairment of kidney function
49
Most common cause of genetic renal disease in children and young adults
Nephronophthisis | group of disorders
50
Three variants nephronophthisis: | Which is most common?
``` sporadic, familial juvenile, renal-retinal #1 = familial juvenile ```
51
nephronophthisis: inheritance pattern number of genes involved? genes assc with familial juvenile form?
AR 7 genes NPH1-3 assc with most common form, familial juvenile
52
Where are cysts located in nephronophthisis? Gross appearance of kidneys? End result?
- SMALL kidneys - corticomedullar jxn cysts - causes chronic renal failure
53
Three histo findings assc with nephronophthisis
- tubular atrophy - BM thickening in Distal + proximal tubules - interstitial fibrosis
54
Three early clinical findings assc with nephronophthisis
polyuria (dilute urine), tubular acidosis, sodium wasting
55
How long after onset does nephronophthisis cause renal failure?
5-10 years
56
Four extra-renal findings assc with nephronophthisis
- liver fibrosis - cerebellar and ocular abnormalities (extra ocular mm's) - retinal dystrophy
57
Describe the appearance of multicystic renal dysplasia (2): | Treatment and prognosis if unilateral/ bilateral
- islands of undifferentiated mesenchyme/ cartilage - immature collecting ducts - baby gets renal failure if bilateral, can surgically remove affected kidney if unilateral
58
Acquired cystic kidney disease: - cause - location of cysts - symptoms
- chronic dialysis --> tubular obstruction by fibrosis or oxalate crystals - cortical and medullary cysts - asx or hematuria
59
Rare outcome of acquired cystic kidney disease
-7% get renal squamous cell carcinoma
60
Two renal cystic disease effecting children
ARPKD, nephronophthisis
61
How to distinguish tumor v cyst on imaging
cysts always AVASCULAR, have fluid lines, and appear smooth
62
``` Where are cysts located in: ADPKD ARPKD Sponge Kidney Nephronophthisis Cystic Kidney Disease ```
-ADPKD: tubular (Note: tubular can also be called "cortex + medulla", because that's where tubules are.) -ARPKD: collecting ducts -Sponge: medulla -Nephronophthisis:corticomedullar jxn -Cystic Kidney Disease: cortex AND medulla
63
Renal condition causing ocular/ retinal anomalies?
Nephonrophthisis, also causes cerebellar and hepatic anomalies.
64
Disease assc with pulmonary hypoplasia and neonatal death?
ARPKD
65
Disease that causes renal failure within 5-10 years of onset?
nephronophthisis
66
Baby with Portal HTN should make you put what cystic kidney disease on your Ddx?
ARPKD- almost always presents with hepatic cysts/ fibrosis --> portal HTN

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