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Renal Flashcards

1
Q

nephrogenesis:
from which age to which in gestation?

  1. which structure degenerates?
  2. then which structure function as interim kidney, until when? then become what?
  3. which structure is permanent? include which 2 major structures? 这两互动不良会有什么后果？
  4. which structure is the last to canalize? 特点？
A

nephrogenesis:
from which age to which in gestation: continue thru 32-36 wks

  1. which structure degenerates: pronephros [原肾,前肾]
  2. then which structure function as interim kidney: mesonephrons 中肾
    until when: 1st trimester
    then become what: male genital system
  3. which structure is permanent: –metanephros 后肾
    include which 2 major structures: 1) ureteric bud 输尿管芽; 2) metanephric mesenchyme

这两互动不良会有什么后果： Multicystic dysplastic kidney 多囊肾发育不良

  1. which structure is the last to canalize?
    ureteropelvic junction: 输尿管肾盂连接
    特点：MC site of hydronephrosis in fetus
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2
Q

Potter syn:

口诀？
Causes?
Cx?
MCC of death?

A

口诀: Babies who can Pee in the utero develop Potter syn (oligohydraminios 羊水过少）

Causes： ARPKDs, posterior urethral valves 后尿道瓣膜, bilateral renal agenesis

Cx: “POTTER”

  • Pulmonary hypoplasia (MCC of death)
  • Ooligohydraminios (trigger)
  • Twisted face
  • Twisted skin
  • Extremity defects
  • Renal failure (in utero)
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3
Q

horseshoe kidney:

  1. what deficit?
  2. kidneys trapped between?
  3. association?
A
  1. what deficit: inferior poles of both kidneys fuse
  2. kidneys trapped between: aorta and IMA (Inferior mesenteric A)
  3. association: Turner Sym (XO性染色体）
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4
Q

Multicystic dysplastic kidney:

Cause?
双侧还是单侧？

A

多囊肾发育不良

Cause： abnormal interaction between ureteric bud and metanephric mesenchyme

双侧还是单侧：单侧

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5
Q

kidney transplantation:

取哪侧？

A

left - longer vein

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6
Q

Ureters: course:

female? male?

A

Water under the bridge

in female: under uterine artery 子宫动脉
in male: ductus deferens 输精管

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7
Q

Rule of body fluid (% of body weight)?

How to measure:

1) Plasma volume?
2) ECF (extracellular fluid)?

normal osmolarity?

A

Rule of body fluid (% of body weight): 60-40-20

60% total body water (40% is non-H2O mass)
40% ICF
20% ECF

How to measure:

1) Plasma volume - radiolabeled albumin
2) ECF (extracellular fluid) - inulin 菊粉

normal osmolarity: 290 mOsm/L

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8
Q

glomerular filtration barrier由哪几种机制构成？

如果其中一种barrier lost, 导致？

A

glomerular filtration barrier由哪几种机制构成：

  1. size barrier (fenestrated cap. endothelium)
    2) charge barrier (fused base mem with heparin sulfate)
    3) epithelial layer of podocyte foot process

如果charge barrier lost, 导致 nephrotic syn 肾病综合征:
albuminuria, hypoproteinemia (generalized edema), hyperlipidemia

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9
Q

对物质X的renal clearance怎么计算？

以下情况意味着：

  1. Cx < GFR
  2. Cx > GFR
  3. Cx = GFR
A

Cx = Ux*V/Px

meaning: volume of plasma from which X is completely cleared per unit time (单位是mL)

Ux = urine [X]
V = urine flow rate (mL/mim）
Px = plasma [X]

以下情况意味着：

  1. Cx < GFR: net tubular reabsorption of X
  2. Cx > GFR: net tubular secretion of X
  3. Cx = GFR: no net tubular reabsorption or secretion of X
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10
Q

Best substance used to calculate GFR?

What’s used to estimate GFR? is it over- or under-estimate?

normal GFR?

A

Best substance used to calculate GFR：
Insulin (it’s freely filtered, neither reabsorbed nor secreted)

GFR = Uinsulin * V/Pinsulin

What’s used to estimate GFR: creatinine

is it over- or under-estimate: over, because creatinine is slightly secreted

normal GFR: 100 mL/min

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11
Q

how to estimate ERPF (effective renal plasma flow?

A

PAH (para-aminohippuric acid 对氨基马尿酸)

原因：

1) PAH is both filtered AND actively secreted in the proximal tubule
2) Almost ALL PAH is excreted

ERPF underestimates the true RPF by ~ 10% (PAH毕竟不是100%被清除）

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12
Q

define FF? how to calculate?

Which substance affects mostly afferent arteriole, and how to change FF? - inhibited by which drugs?

Which substance affects mostly efferent arteriole, and how to change FF? - inhibited by which drugs?

A

FF (filtration fraction) = GFR/RFP [肾小球率过滤/肾血流】

calculation = Creatinine clearance / PAH clearance

Which substance affects mostly afferent arteriole, and how to change FF? - inhibited by which drugs?

  • PGs dilates afferent arteriole: ↑ GFP, ↑ RFP, so FF no change
  • inhibited by NSAIDs

Which substance affects mostly efferent arteriole, and how to change FF? - inhibited by which drugs?

  • AT II preferentially constricts efferent arteriole: ↑ GFP, ↓ RFP, so FF ↑
  • inhibited by ACE inhibitors
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13
Q

↑ [plasma protein] 对GFR, RPF, FF的影响？

↓ [plasma protein] 对GFR, RPF, FF的影响？

A

↑ [plasma protein] 对GFR, RPF, FF的影响：（血浆蛋白质上升，相当于血管内水减少）
GFR ↓, RPF 不变, FF ↓

↓ [plasma protein] 对GFR, RPF, FF的影响？（血浆蛋白质下降，相当于血管内水增多）
GFR↑ , RPF 不变, FF ↑

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14
Q

constriction of ureter对GFR, RPF, FF的影响？

A

输尿管狭窄，鲍曼囊压力retrograde 升高，尿滤不过

GFR ↓, RPF 不变, FF ↓

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15
Q

定义: Filter load (how to calculate)?

A

Filter load (mg/min，每分钟有多少蛋白质被滤过) = GFR (mL/min) * plasma conc. of X (mg/mL)

Filter load = GFR * Px

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16
Q

Glucose: 在肾脏哪个节段、以什么机制清除？

when will glucosuria show?
when will transporters be saturated?

A

completely reabsorbed at proximal tubule (近端肾小管）by Na+/Glucose co-transporter?

when will glycosuria show: ~ 200 mg/dL
when will transporters be saturated: ~ 375 mg/dL

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17
Q

aa: 在肾脏哪个节段、以什么机制清除？

associated with which disease?

A

aa: re-absorbed by sodium-dependent transporters at proximal tubule (近端肾小管）

associated with which disease - Hartnup disease (AR)

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18
Q

Hartnup disease:

genetic pattern?
Cause?
Cx?
Rx?

A

genetic pattern: AR

Cause: deficiency of neutral aa (e.g., tryptophan) re-absorption in proximal tubule and from gut: neural aminoaciduria

Cx: pellagra-like sym (3 D’s: diarrhea, dermatitis, dementia)

Rx: high-protein diet + nicotinic acid

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19
Q

Nephron segment physio:

PCT:

  1. functions?
  2. regulators?
A

PCT (proximal convoluted tubule):

  1. functions:
    1) reabsorb ALL glucose + aa
    2) reabsorb most H2O, Na+ (~80%), K+, HCO3-, CL-, PO4(3-) [note: isotonic absorption]
    3) generate and secrete NH3
  2. regulators:
    1) PTH: 排磷
    2）AT II: stimulate Na+/H+ exchange, 保钠保水， 保HCO3- : “contraction alkalosis”
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20
Q

Nephron segment physio:

descending loop of Henle (thin):
1. functions?

A
  1. functions:

被动重吸收水- 尿液浓缩

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21
Q

Nephron segment physio:

ascending loop of Henle （thick):

  1. functions?
  2. regulators?
  3. sites for which drugs?
A
  1. functions:
    1) actively reabsorb Na+, K+ and Cl-
    2) indirectly reabsorb Mg2+, Ca2+
    3) impermeable to H2O!!!! make sure urine less concentrated (与thin descending loop of Henle正相反）
  2. regulators：
    1） loop diuretics (inhibit Na+/K+/2Cl- co-transporter)
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22
Q

Nephron segment physio:

DCT:

  1. functions?
  2. regulators?
  3. sites for which drugs?
A

DCT (distal convoluted tubule):

  1. functions:
    1) actively reabsorb Na+, Cl-
    2) make urine hypotonic
  2. regulators
    1) PTH: 排磷 (PCT也受PTH调控）
  3. sites for which drugs： thiazide diuretics
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23
Q

Nephron segment physio:

collecting tubule:

  1. functions?
  2. regulators?
  3. sites for which drugs?
A
  1. functions:
    在Aldosterone作用下保钠排钾
  2. regulators：
    1) Aldosterone: ↑ Na+ channel on luminal side
    2) ADH: ↑ aquaporin H2O channel on luminal side
  3. sites for which drugs：
    1） amiloride 阿米洛利 [利尿药]
    2) triamterene 三氨喋呤
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24
Q

renal tubule defects: 记忆口诀？

A

“The kidney put out FABulous Glitting Liquid”

FAnconi syn： PCT
Bartter syn: thick ascending loop of Henle
Gitelman syn: DCT
Liddle syn: collecting duct

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25
Q

Fanconi syn:

cause?
affect region?
Cx?

A

“The kidney put out FABulous Glitting Liquid”
FAnconi syn： PCT

cause: any disease damaging PCT (Wilson disease), ischemia, nephrotoxins, drugs

Cx: 
1. metabolic acidosis (PCT acidosis - lost HCO3)
2. 血尿4大变化：
1） 氨基酸尿 animoaciduria
2) 尿糖 glycosuria
3) 低磷酸血症 hypophosphatemia
4) 低尿酸血症 hypouricemia
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26
Q

Gitelman syn：

genetic pattern?
affect?
Cx?

A

AR,
affect DCT, [ less severe than Bartter syn in ascending loop of Henle]];

导致低钾血症和代碱，和Bartter syn的区别在于没有高氯离子尿）

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27
Q

Liddle syn:

genetic pattern?
affect?
Cx?
Rx?

A

“The kidney put out FABulous Glitting Liquid”

genetic pattern: AD

affect: renal collecting tube: ↑ Na+ reabsorption

Cx: HTN, hypokalemia, 代碱，↓ aldosterone

Rx: Amiloride

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28
Q

“relative conc. along proximal tubules”这个概念是在测量什么？

TF [tubular fluid] / P [plasma]:
> 1 意味着？ 哪些物质？
= 1 意味着？ 哪些物质？

A

“relative conc. along proximal tubules”这个概念是在测量什么:
- 原尿沿着近端卷曲管PCT流动时，相对于水的重吸收，物质X的吸收速度

TF [tubular fluid] / P [plasma]:
> 1 意味着：solute is reabsorbed slower than H2O
 哪些物质: 
-PAH, Creatinine, insulin (几乎全是排出）
- Urea, Cl- (都有plateau)

= 1 意味着: solute and H2O are reabsorbed at same rate
哪些物质: K+, Na+

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29
Q

AT II: acts on which systems/organs?

A
  1. VSMCs: ↑ BP
  2. Kidney: constricts efferent arteriole of glomerulus: ↑ FF to preserve GFR in low-volume status
  3. Kidney: proximal tubule Na+/H+ activity (促进水、钠、HCO3-重吸收， 可能引起“收缩性碱中毒” contraction alkalosis)
  4. Adrenal gland: 分泌aldosterone: 促进水、钠重吸收
  5. posterior pituitary: ADH (促进水重吸收)
  6. stimulate hypothalamus - thirst
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30
Q

ANP:

made by ?
functions?
Mechanism:
作用位点？

A

made by atria in response to ↑ volume/atrial pressure

functions: 心房利钠因子，排钠排水降压，主要是对血容量升高的调控机制
may act as “check” to RSS system

Mechanism: relax VSMCs via cGMP - ↑ GFR, ↓ renin

作用位点： 1) afferent arteriole. 2) distal nephron

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31
Q

ADH：

made by ?
functions?
Mechanism:

A

Vasopressin/antidiuretic hormone [血管加压素/抗利尿激素】

made by posterior pituitary

functions: regulate osmolarity; also respond to low blood volume states 例如失血过多时

Mechanism: act at V2 receptor in principle cells in collecting tubule - insert aquaporin H2O channels on luminal side (principle cells), ↑ H2O reabsorption

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32
Q

JGA (juxtaglomrular apparatus):

由什么细胞组成？
对什么反应？
功能？

A

由什么细胞组成：
1）JG cells (modified SMCs of afferent arteriole)
2) macula densa (NaCL sensor, part of the DCT)

对什么反应: secrete renin in response to

1) ↓ renal blood pressure,
2) ↓ NaCL delivery to distal tubule
3) ↑ sympathetic tone (b1) - BB 降血压的机理一部分就是inhibit b1-R of the JGA, causing ↓ renin release

功能: defends GFR via RAA

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33
Q

EPO:

made by?
function?

A

made by: renal interstitial cells in the peritubular cap. bed

function: in response to hypoxia

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34
Q

what effect does the kidney has to VitD?

this step is regulated by which hormone?

A

proximal tubule cells use 1a-hydroxylase to convert 25-OH VD to 1, 25-(OH)2 VD (active)

this step is regulated by which hormone: PTH

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35
Q

prostaglaindins:
对肾脏血流的影响？
PG本身被什么药物抑制？

A

prostAglaindins: paracrine secretion, vasodilates the Afferent arterioles to ↑ RBF

PG本身被什么药物抑制： NSAID (这种药物引起急性肾衰的原因）

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36
Q

Mx for NSAIDs to cause ARF (acute renal failure)?

A

NSAIDs inhibits renal-protective PG synthesis - constricts the afferent arteriole and ↓ GFR

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37
Q

AT II:

synthesized in response to ?
sits of action?
对GFR, FF, [Na+]的影响？
net effects?

A

synthesized in response to： ↓ BP

sits of action: efferent arteriole

对GFR, FF, [Na+]的影响: ↑ GFR, ↑ FF, compensatory Na+ reabsorption in both proximal and distal nephron

net effects:

1) ↑ FF (preserve renal function) in low-volume state
2) simultaneously Na+ reabsorption to maintain circulating volume [这一点和ANP相反】

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38
Q

PTH:

synthesized in response to ?
sits of action?
net effects?

A

synthesized in response to:
1) ↓ 血钙
2）↑ 血磷
3）↓ 血浆1, 25-(OH)2-VD浓度

sites of action：
1） DCT: ↑ 钙重吸收
2）PCT: ↓磷重吸收
3) ↑ 1, 25-(OH)2-VD conversion at proximal tubule

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39
Q

Aldosterone:

synthesized in response to ?
sits of action?
Mx?
net effects?

A

在肾上腺合成，synthesized in response to: ↓ BP ( 被AT II刺激合成）and ↑ 血钾

sits of action：principle cells in collecting tubules

Mx: act on mineralocorticoid R - insert Na+ channel and Na+/K+ pump on luminal side

net effects: Na+ and H2O reabsorption, K+ and H+ secretion

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40
Q

potassium shifts:

哪些情况钾shift out from cells?
哪些情况钾shift into cells?

A

口诀：

  1. Pts with hyperkalemia? DO insulin Lab work.
  2. Insulin shifts K+ into cells
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41
Q

低钾引起？

高钾引起？

A

低钾引起：U wave, flattened T waves

高钾引起: wide QRS, peaked T waves

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42
Q

Winters formula:

to calculate respiratory compensation for a simple metabolic acidosis

如果得出的数值和实际相差太大，意味着？

A

Winters formula:

pCO2 = 1.5 [HCO3-] + 8 ± 2

(to calculate respiratory compensation for a simple metabolic acidosis)

如果得出的数值和实际相差太大，意味着: not a simple metabolic acidosis, likely a mixed acid-base disorder

43
Q

respiratory acidosis: define

which conditions cause respiratory acidosis?

A

pH < 7.4
PCO2 > 40 mmHg

which conditions cause respiratory acidosis:
hypoventilation (无法把CO2呼出去，聚集在体内造成呼酸）

  1. airway obstruction
  2. acute/chronic lung disease
  3. opioids, sedatives
  4. weakening of respiratory muscles
44
Q

metabolic acidosis: define

which conditions cause respiratory acidosis?

A

pH < 7.4
PCO2 < 40 mmHg (with compensation from hyperventilation)

  • 再分类需要check anion gap
45
Q

define: anion gap
正常值？

↑ 见于哪些疾病？
正常却有酸中毒时，见于哪些疾病？

A

anion gap = Na+ - (Cl- + HCO3-)

正常值： 8-12 mEq/L

↑ 见于哪些疾病：MUDPILES

正常却有酸中毒时，见于哪些疾病: HARD ASS

46
Q

respiratory alkalemia: define

which conditions cause respiratory alkalemia?

A

respiratory alkalemia: pH > 7.4, PCO2 < 40 mmHg

caused by hyperventilation (CO2呼出过多，体内酸少了就会碱中毒）

“HHSTP”

  • hysteria
  • hypoxemia
  • Salicylates (early stage)
  • Tumor
  • Pulmonary embolism
47
Q

metabolic alkalemia: define

which conditions cause respiratory alkalemia?

A

pH > 7.4, PCO2 > 40 mmHg

(with compensation from hypoventilation)

“LA-VH”

  • loop diuretics
  • antacid use
  • vomiting
  • hyperaldosteronism
48
Q

RTA (renal tubule acidosis) 肾小管性带酸

现代分型？
病因？

各自affected segment of nephron? K+ change? Cx?

A

病因: 尿液酸化不足，其余肾功能受损不太严重；酸化不足要么是近端肾小管不能重吸收碱（HCO3-), 要么是远端肾小管无法分泌足够的酸

现代分型：
Type 1： Distal RTA （pH > 5.5)
Type 2 : proximal RTA (pH < 5.5）
Type 4: adrenal insufficiency (pH < 5.55)

现代分型不包括type 3 (Combined proximal and distal RTA)

49
Q

哪些情况有hematuria, no casts?

A

bladder cancer

kidney stones

50
Q

哪些情况有pyuria (脓尿）, no cast？

A

cystitis 膀胱炎

51
Q

RBC casts indicate?

A

ARF

  1. ongoing glomerulonephritis
  2. ischemia
  3. malignant HTN
52
Q

WBC casts indicate?

A
  1. tubulointersitial inflammation
  2. acute pyelonephritis
  3. transplantation rejection
53
Q

fatty casts indicate?

A

“oval fat bodies” - nephrotic syn

54
Q

granular casts indicate?

A

“muddy brown” casts - acute tubular necrosis

55
Q

waxy casts?

A

CRF (chronic renal failure)

advanced renal disease

56
Q

Define/各自哪些代表疾病？

Nephritic syn
Nephronic syn

A

NephrItic syn = (Inflammation) 肾炎综合征

NephrOnic syn = 肾病综合征 (O= prOteriuria, > 3.5 g/day)

57
Q

focal segmental glomerulosclerosis:

Nephritic syn or Nephronic syn?

特点？

A

局灶性节段性肾小球硬化症, Nephronic syn (大量蛋白尿）

特点
1. MC for nepronic syn in AA and hispanics
2. LM: segmental sclerosis and hyalinosis
3。 EM: effacement of foot process similar to minimal change disease

58
Q

肾病Dx “buss words”:

LM - diffuse cap. and GBM thickening
IF: granular (immune complex deposition)
EM: “spike and dome”

Dx?
Nephritic syn or Nephronic syn?
特点？

A

Dx: membranous nephropathy

Nephritic syn or Nephronic syn: MC for Nephronic syn in caucasian adults (Nephronic presentation of SLE)

59
Q

肾病Dx “buss words”:

LM - normal
IF: negative
EM: “effacement of foot process”

Dx?
Nephritic syn or Nephronic syn?
特点

A

Dx: minimal change disease （Diff with membranous nephropathy)

Nephritic syn or Nephronic syn:
MCC for Nephronic syn in children

特点: 对激素反应性好

60
Q

MPGN (membrano-proliferative glomerulonephritis):

Nephritic syn or Nephronic syn?

分型？各自LM, IF特点? association?

A

Nephritic syn, but can present as nephritic syn !!!

以GBM增生为特点（看名字）

分型？各自LM, IF, EM特点? association?

Type I:

  • sub endothelial IC (immune complex) deposits with granular IF
  • “tram-track” appearance due to GBM splitting caused by mesangial ingrowth (肾小球系膜)
  • association: HBV, HCV, can be idiopathic

Type II:

  • intramembranous IC deposits: “dense deposits”
  • association: C3 nephritic factor (stablize C3 convertase - ↓ serum C3 levels)
61
Q

diabetic glomerulonephropathy:

Nephritic syn or Nephronic syn?

LM： patho的特征结构？

损伤哪2个结构？后果？

A

Nephronic syn

LM: Kimmelstiel-Wilson lesion (mesangial expansion, GBM thickening, eosinophilic nodular glomerulosclerosis)

  1. non-enzymatic glycosylation of GBM: ↑ permeability, thickening
  2. non-enzymatic glycosylation of efferent arteriole- ↑ GFR, - mesangial expansion
62
Q

Nephritic syn特点？

代表疾病？

A

NephrItic syn =Inflammation

hematuria, RBC casts in urine, azotemia, oliguria, HTN (salt retention), proteinuria < 3.5 g/d (蛋白尿的程度低，与肾病综合征区别）

代表疾病：”ABDRP”

  1. PSGN (acute poststreptococcal glomerulonephritis) 急性链球菌感染后肾小球肾炎
  2. RPGN (rapid progressive glomerulonephritis)
  3. DPGN (diffuse proliferative glomerulonephritis)
  4. Berger disease (IgA nephropathy)
  5. Alport syn
63
Q

PSGN:

  1. 好发人群？
  2. Cx?
  3. LM? IF? EM?
  4. lab?
A
  1. 好发人群： children, ~ 2wks after group A streptococcal infection (pharynx or skin) - spontaneously resolve
  2. Cx: peripheral and periorbital edema, dark urine (cola-colored), HTN
  3. LM: glomeruli enlarged and hyper cellular
    IF: “starry sky”, “lumpy bumpy” - deposits of IgM, IgG, C3 deposition along GBM
    EM: subepithelial IC humps
  4. lab: ↑ anti-DNase B titer, ↓ complement levels
64
Q

RPGN:

哪些疾病可能result in this pattern?
LM, IF特点？
Prognosis?

A

rapid progressive glomerulonephritis 快速进行性肾小球肾炎

进展很快，预后很差，days to wks 就会出现肾功能急剧恶化

哪些疾病可能result in this pattern：

  1. Goodpasture Syn
  2. Wegener (granulomatosis with polyangiitis)
  3. microscopic polyangiitis

LM, IF特点: crescent-moon shape (fibrin, C3b, monocytes, macrophages)

65
Q

Dx:

~ 40 yr, 血尿+HTN, 肾脏活检见“linear IF”?

哪一型hypersensitivity?

A

Goodpasture syn (Type II hypersensitivity)

RPGN一种

Ab against GBM (里面的type IV collagen)， 形成典型的linear IF

66
Q

Wegener disease: 什么病？
Cx?
Ab marker?

A

necrotizing granulomatous polivasculitis

Cx: 累及3大系统：

  1. 肺部症状：咳血 he’moptysis
  2. 上呼吸道：肉芽肿+溃疡
  3. 肾脏：RPGN

Ab marker： c-ANCA

67
Q

microscopic polyangiitis: 又名？

最显著的lab marker?

A

Churg-Strauss syn

最显著的lab marker: p-ANCA (anti-neutrophil Ab)

68
Q

DPGN (diffuse proliferative glomerulonephritis):

是什么疾病的表现？
Nephritic syn or Nephronic syn?
LM, EM, IF特点？

A

是什么疾病的表现：
1. SLE (MCC for death)
2. MPGN (membrano-prolif
erative glomerulonephritis)

Nephritic syn or Nephronic syn： 常常both

LM, EM, IF特点:

  • LM： “wire looping” of cap
  • EM: IgG-based ICs, C3 deposition
  • IF: granular
69
Q

Berger disease: 又名？

LM, EM, IF特点？

Cx：

seen with which disease?

A

IgA nephropathy (和Buerger disease - “smoker disease”别混淆了）

LM, EM, IF特点:

  • LM: mesangial proliferation
  • EM: mesangial IC deposition
  • IC: IgA based IC deposition in mesangium

Cx：URI or acute gastroenteritis, episodic hematuria with RBC casts

seen with which disease: Henoch-Schonlein purport HSP
(HSP4大症状：3-7 yr kid, 1）腹痛，血便， 2）血尿，3）皮疹，4）关节痛）

70
Q

Alport Syn:

在肾脏的表现？
其他系统的表现？
病因？

A

在肾脏的表现：glomerulonephritis

其他系统的表现: sensorineural deafness, less commonly eye problems (cataracts)

病因: mutation in type IV collagen (basement mem) (X-linked)

71
Q

Dx:

unilateral flank tenderness
colicky pain radiating to groin
hematuria

A

kidney stone

72
Q

MC presentation for kidney stone?

cause?
XR?
urine crystal?

Rx: thiazides, citrate

A

calcium oxalate 草酸钙 stone in pt with hypercalciuria, normocalcemia

cause:

  1. hypercalciuria (idiopathic, or 2° to conditions causing hypercalciuria: cancer, ↑ PTH, etc)
  2. ethylene glycol (乙二醇, antifreeze)
  3. VC abuse
  4. crohn disease

XR: radiopaque (X光上不透明）

urine crystal：envelop, or dumbbell

73
Q

Dx: “coffin lid” radiopaque kidney stone?

由什么组成？
cause?
容易引起什么？Rx?

A

coffin lid” radiopaque kidney stone由什么组成: ammonium magnesium phosphate

cause: infection with urease + bugs (尿素酶+的细菌，包括proteus mirabilis 奇异变形杆菌， staphylococcus, Klebsiella) - hydrolyze urea to ammonia → urine alkalization

容易引起什么: staghorn calculi ⟹ UITs

Rx: control underlying infection + surgical removal of stone

74
Q

the only radiolucent kidney stone?

CT, ultrasound, XR?

association?

Rx?

A

Uric acid stone (5%) - radiolUcent

visible on CT, ultrasound;
not on XR

association:
1) gout (hyperuricemia)
2) ↑ cell turnover (leukemia)

Rx: alkalization of urine

75
Q

Dx: hexagonal urine crystal?

MC seen in which population? 2° to ?
哪种test +?
RX?

A

cystine 胱氨酸 (占肾结石1%），radiopaque

MC seen in which population: children
2° to cystinuria

哪种test +: sodium nitroprusside test

Rx: alkalization of urine + hydration

76
Q

MC 1° renal malignancy:

  1. 好发人群？
  2. 组织学起源？病理所见？
  3. Cx?
  4. 转移途径？转移到什么器官？
  5. association with gene deletion on which chr?
  6. Rx?
A

renal cell carcinoma (RCC):

  1. 好发人群：50-70 yr male, ↑ incidence with smoking and obesity
  2. 组织学起源-proximal tubule cells
    病理所见: polygonal clear cells, filled with lipids and carbohydrates
  3. Cx: chronically (“silent cancer”), hematuria, flank pain, fever, weight loss
  4. 转移途径: 血行（invade renal v - IVC), metastasizes to lung and bone
  5. if not sporadic, association with
    1) gene deletion on which chr3 (von Hippel-Lindau syn)
    2) paraneoplastic syn (ectopic EPO, ATCH, PTHrP)
  6. Rx: resection (if local) or immunotherapy

resistant to chemo or radiation

77
Q

Dx:

Cx: plainless hematuria, flank pain,
abdominal mass

肾切片 H&E stain shows polygonal cellos with eosinophilic cytoplasm, abundant mitochondria without perinuclear clearing
[diff with which disease?]

Rx?

A

Dx: renal oncocytoma 嗜酸细胞瘤 (benign)

diff with which disease： chromophobie renal cell carcinoma

Rx: nephrectomy

78
Q

Define: Wilms tumor

发生年龄？
组织学见？
Cx?
gene mutation on which chr?
part of which syn?
A

Wilms tumor: nephroblastoma

发生年龄: MC renal malignancy of early childhood (2-4 yr)

组织学见: contain embryonic glomerular structure

Cx: huge, palpable flank mass and/or hematuria

gene mutation: Loss-of-fuction mutation of WT1 or WT2 (tumor suppressor genes) on chromosome 11

part of which syn: “Beckwith-Wiedenmann syn” - “WAGR”

  • Wilms tumor
  • Aniridia 无虹膜,虹膜缺失
  • Genitourinary malformation
  • Retardation
79
Q

Dx: 60-80 yr pt, painless hematuria, no casts?

  1. associated with?
  2. 组织学所见？
A

Transitional cell carcinoma (MC tumor of urinary tract system)

1. associated with: 考的是环境因素 
“problem in your Pee SAC"
- Phenacetin 非那西汀;[解热镇痛药]
- Smoking
- Aniline dyes  苯胺染料
- Cyclophosphamide

UW: 接触rubber, leather, plastics, aromatic amine-containing dye的病史

  1. 组织学所见：papillary growth lined by transitional epithelium with mild nuclear atypic and pleomorphism
80
Q

squamous cell carcinoma of the bladder:

病因、病理？
Risk factors? 和哪个地区有关？
Cx:

A

病因、病理：chronic irritation of bladder - squamous metaplasia - dysplasia and squamous cell carcinoma

Risk factors：

  1. 和哪个地区有关： middle east (Schistosoma haematobium 埃及裂体吸虫)
  2. chronic cystitis, smoking, chronic nephrolithiasis 肾石病

Cx: painless hematuria

81
Q

acute infectious cystitis:

Cx? 有无systemic signs (fever, chills)?

Risk factors?

MCC?

If lab + for leukocyte esterase， nitrites + for gram-?

If sterile pyuria, urine culture negative?

A

Cx: suprapubic pain, dysuria, urinary frequency, urgency

有无systemic signs (fever, chills): 无

Risk factors：

  1. female (尿道短）
  2. sex (“honeymoon cystitis”)
  3. indwelling catheters

MCC:

  1. E. coli (无论哪个病人群体里都是最常见的病因）
  2. Staphylococcus saprophyticus 腐生性葡萄球菌 (esp in sexually active females)
  3. Klebsiella
  4. Proteus mirabilis 奇异变形杆菌 [urine has ammonia scent)
  5. adenovirus - 出血性膀胱炎

If lab + for leukocyte esterase， nitrites + for gram-： E.coli

If sterile pyuria, urine culture negative: Neisseria gonorrhoeae, or Chlamydia trachomatis 沙眼衣原体

82
Q

Acute vs. chronic pyelonephritis:

Acute:
Cx?
affect which area of kidney?
Urine casts是哪一种？
病理见什么细胞infiltration?
CT所见？
A

Acute:

Cx: dysuria, fever, costrovertebral angle tenderness, nausia, vomitting

affect which area of kidney: cortex [CT所见]

Urine casts是哪一种: WBC casts

病理见什么细胞infiltration: neutrophil 【vs. chrnoic: lymphocytic infiltration - “thyrodiazation of kidney”]

83
Q

drug-induced interstitial nephritis (tubulointerstitial nephritis):

lab?
哪些药物引起？时间点？
Cx?

A

lab: pyuria （脓尿， classically eosinophils) + azotemia

哪些药物引起？时间点？

  1. 1-2 wks after: diuretics, penicillin derivates, sulfonamides, rifampin
  2. months after NSAIDs

Cx: fever, rash, hematuria, costoverterbral angle tenderness, can be asymptomatic

84
Q

Diffuse cortical necrosis: 单侧还是双侧肾脏？

可能病因？
association?

A

Diffuse cortical necrosis: 单侧还是双侧肾脏: 双侧

可能病因: likely due to combination of vasospasm and DIC

association with obstetric catastrophes (abrupt placentae, 胎盘早期脱离) or septic shock

85
Q

Acute tubular necrosis:

  • Key lab finding?
  • MCC for which disease?
  • stages?
  • death most commonly occurs at which stage?
  • 主要病因？在每个病因中which segment is particularly susceptible to injury?
A

Key lab finding: granular “muddy brown” casts

  • MCC for which disease: intrinsic renal failure
  • stages:
    1. inciting event
    2. maintenance phase: oliguria (1 - 3 wks); risk for hyperkalemia, metabolic acidosis
    3. recovery phase: polyuria, ↓ BUN and creatinin; risk for hypokalemia
  • death most commonly occurs at which stage: the initial oliguric phase
  • 主要病因:
    1. ischemic - 2° to hypotension, shock, sepsis, hemorrhage, CHF
  • proximal tubule + thick ascending limb are particularly susceptible to ischemia
    2. nephrotoxic - 2° to toxic substance (aminoglycosides, cisplatin, lead, radiocontrast), crush-caused myoglobinuria, hemoglobinuria.
  • proximal tubule is particularly susceptible to ischemia
86
Q

renal papillary necrosis 肾乳头坏死

Cx?
病因？
trigger?
association (尤其是和药物的关系）?

A

Cx: gross hematuria and proteinuria

病因: sloughing of renal papillae

trigger: resent infection or immune stimulus

association：

  1. DM
  2. acute pyelonepphritis
  3. chronic phe’nacetin use (非那西汀, 解热镇痛药: 包括acetaminophen 扑热息痛,)
  4. sickle cell anemia
87
Q

Acute renal failure (ARF): 定义？

病因上要区分为肾前、肾脏本身、肾脏后因素：

  1. prerenal azotemia: 病因？Na+, H2O的变化，导致BUN/creatinineratio变化？
  2. intrinsic RF:
    病因？导致BUN/creatinineratio变化？
  3. postrenal azotemia: 病因？导致BUN/creatinineratio变化？
A

Acute renal failure (ARF): 定义： abrupt decline in renal function within a few days + ↑ creatinine and ↑ BUN (正常情况下BUN 被重吸收，但creatinine is not)

病因上要区分为肾前、肾脏本身、肾脏后因素：

  1. prerenal azotemia:
    病因: ↓ RBF (hypotension) - ↓ GFR
    Na+, H2O的变化，导致BUN/creatinine ratio变化: kidney attempts to conserve volume - retain Na+/H2O and urea - BUN/creatinine ↑
  2. intrinsic RF:
    病因: 最常见的是acute tubular necrosis (ischemia / or toxins); 其次是RPGN (acute glomerulonephritis); patchy necrosis leads to debris obstructing tubule and fluid back flow - ↓ GFR

肾脏本身出问题，BUN reabsorption impaired - BUN/creatinine ↓

  1. postrenal azotemia:
    病因: 肾脏后梗阻，（stones, BPH, neoplasia, congenital）但必须是双侧梗阻

导致BUN/creatinineratio变化：BUN/creatinine ↑

88
Q

renal failure consequences: 口诀？

A

MAD HUNGER

89
Q

renal osteodystrophy 病理？

Cx?

A
  1. failure of VitD hydroxylation: ↓ intestinal Ca2+ reabsorption

hypocalcemia + hyperphosphatemia - 2° hyperparathyrodism

  1. hyperphosphatemia - tissue calcification - ↓ serum Ca2+

Cx: subperiosteal 骨膜下 thinning of bones

90
Q

ADPKD:

遗传特征？mutation?
cx?
cause of death?
Association?

A

遗传特征: AD
mutation:
1. PKD1 mutation (chr 16) > 85%
2. PKD2 mutation (chr 4) 15% case

cx: 起病很慢，常常需要10-20年积累，半数病人都无症状； 有症状的话flank pian, hematuria, HTN, urinary infection, progressive renal failure

cause of death: complication of chronic kidney disease, or HTN

Association:

  1. berry aneurysms
  2. MVP 二尖瓣脱垂
  3. benign hepatic cysts
91
Q

ARPKD: 原名？

遗传特征？mutation?
cx?
cause of death?
Association?

A

infantile PKD

92
Q

medullary cystic disease :

定义？
B超见？is medullary cyst visible?
Px?

A

(肾)髓质囊性病

inherited tubulointerstitial fibrosis; progressive renal insufficiency, cannot concentrate urine

B超见: shrunken kidneys; medullary cysts not visible

Px: poor

93
Q

simple vs. complex renal cyst:

哪种病require follow-up or removal?

A
  1. simple renal cyst:

very common, 最多见的renal masses , 多发于outer cortex;
typically asymtomactic, found incidentally

  1. complex renal cyst:
    require follow-up or removal, due to risk of RCC
94
Q

Mannitol:

site of action?
Mx?
Use?
Toxicity/contraindication?

A

site of action: 肾小管

Mx：osmotic diuretics, ↑ tubular fluid osmolarity, ↑ urine flow - ↓ intracranial /intraocular pressure

Use:

1) drug overdose
2) ↑ intracranial /intraocular pressure

Toxicity:

1) pulmonary edema
2) dehydration

contraindication: anuria, CHF

95
Q

acetazolamide

site of action?
Mx?
Use?
Toxicity/contraindication?

A

乙酰唑胺

site of action: PCT

Mx: carbonic anhydrase [碳酸酐酶 ] inhibitor: cause self-limited NaHCO3 diuretics, ↓ total-body HCO3- stores

Use:

  1. glaucoma (在青光眼中考过机制：inhibit aqumous humor secretion by the ciliary epithelium
  2. urinary alkalization
  3. metabolic alkalosis 代碱
  4. altitude sickness
  5. pseudotumor cerebri

Toxicity/contraindication:
“ACID”azolamide causes ACIDosis

  1. hyperchloremic acidosis 高氯血症性酸中毒
  2. paresthesia: 感觉异常
  3. NH3 toxicity
  4. sulfa allergy [出现对磺胺过敏时，换用ethacrynic acid 利尿酸】
96
Q

furosemide

site of action?
Mx?
Use?
Toxicity?

A

呋塞米, 速尿

site of action: sulfonamide loop diuretics, act on thick ascending loop

Mx:

1) inhibit Na+/K+/2CL- co-transporters
2) stimulate PGE release - vasodilator of afferent arteriole [so inhibited by NSAIDs - 考点： 如果速尿和NSAID联用，利尿效果减弱的机制
3) ↑ Ca2+ excretion (“Loops Lose calcium”)

Use:

  1. edematous states (CHF, pulmonary edema, cirrhosis, nephrotic syn)
  2. HTN
  3. hypercalcemia
Toxicity: "OH DANG!"
Ototoxicity  (中)耳毒性
Hypokalemia
Dehydration
Allergy (sulfa)[出现对磺胺过敏时，换用ethacrynic acid 利尿酸】
Nephritis (interstitial)
Gout
97
Q

‘ethacrynic acid

site of action?
Mx?
Use?
Toxicity/contraindication?

A

利尿酸,天尼酸[利尿药]

Mx: loop diuretics, but not a sulfonamide!!!
a derivative of phenoxyacetic acid ( 苯氧基乙酸) ; 因此可以用于 对速尿furosemide过敏的病人

Use: Action similar to furosemide

  1. edematous states (CHF, pulmonary edema, cirrhosis, nephrotic syn)
  2. HTN
  3. hypercalcemia

Toxicity/contraindication?

  • Similar to furosemide (OH DANG!)
  • among the loop diuretics, 最容易引起Ototoxicity (中)耳毒性
  • can cause hyperuricemia!! never use in gout
98
Q

hydrochlorothiazide (HCTZ)

site of action?
Mx?
Use?
Toxicity/contraindication?

A

氢氯噻嗪, thiazide diuretics

site of action: early DCT

Mx: inhibit NaCL reabsorption, ↓ diluting capacity of the nephron, ↓ Ca2+ excretion

Use:

  1. HTN
  2. CHF
  3. idiopathic hypercalciuria
  4. nephrogenic diabetes insipidus
  5. osteoporosis
Toxicity/contraindication:
(3低4高）：
- 低钾、低钠、低镁血症
- hyperGLUC: hyperGlycemia (高血糖），hyperLipidemia (高血脂）， hyperUricemia （高尿酸血症）， hyperCalcemia (高钙血症）
- sulfa allergy
99
Q

K+- sparing diuretics: 代表药物？

site of action?
Mx?
Use?
Toxicity/contraindication?

A

K+- sparing diuretics: 代表药物：”K+ STAys”

  • Spironolactone 螺内酯, 安体舒通(醛甾酮拮抗药,利尿药)
  • eplerenone 依普利酮
  • triamterene 三氨喋呤
  • Amiloride 阿米洛利

site of action: CCT (cortical collecting tube)

Mx:

  1. Spironolactone , eplerenone: competitive AR antagonists
  2. triamterene, Amiloride: block Na+ channels

Use:

  1. hyperaldeosteronism
  2. K+ depletion
  3. CHF

Toxicity:

  1. hyperkalemia - can cause arrhythmia !!!
  2. Spironolactone由于拮抗雄激素受体而出现的内分泌症状：gynecomastia, antiandrogen effects
100
Q

Diuretics:
how to change urine NaCL?

the only exception?

A

↑ NaCl

the only exception: acetazolamide 乙酰唑胺

site of action: PCT
Mx: carbonic anhydrase [碳酸酐酶 ] inhibitor: cause self-limited NaHCO3 diuretics, ↓ total-body HCO3- stores

101
Q

Diuretics:

how to change urine K+?

A

Loop diuretics (Furosemide, ethacrynic acid) and thiazides ↑ urine K+;

K+-sparing diuretics may ↓ urine K+ (同时升高血钾）
"K+ STAys"
- Spironolactone   螺内酯, 安体舒通(醛甾酮拮抗药,利尿药)
- eplerenone  依普利酮
- triamterene 三氨喋呤
- Amiloride   阿米洛利
102
Q

Diuretics:

which ones ↓ blood pH (acidemia)?
which ones ↑ blood pH (alkalemia)?

Mx?

A

↓ blood pH (academia):

1) : CA inhibitor (acetazolamide 乙酰唑胺)
2) K+-sparing diuretics (Spironolactone, eplerenone)

which ones ↑ blood pH (alkalemia)?

1) loop diuretics (furosemide)
2) thiazides

103
Q

Diuretics:

which ones ↑ urine Ca2+ (同时 ↓ 血钙）？

which ones ↓ urine Ca2+ (同时 ↑ 血钙）？

Mx?

A

which ones ↑ urine Ca2+ (同时 ↓ 血钙）： Loop diuretics (Furosemide, ethacrynic acid)

which ones ↓ urine Ca2+ (同时 ↑ 血钙）: thiazides

104
Q

ACE inhibitors:

代表药物？
机制？
Use?
Toxicity?
一个最著名的毒副作用怎么避免？
A

ACE inhibitors:

代表药物：-pril (Captopril, enalapril, lisinopril)

机制:
inhibit ACE - ↓ AT II - ↓ GFR (所以在bilateral renal artery stenosis里要避免，否则会引起肾衰）
- 同时inhibit inactivation of bradykinin and substance P (vasodilators) - 引起臭名昭著的副作用：咳嗽

Use：

  1. HTN
  2. CHF (prevent unfavorable heart remodeling)
  3. proteinuria
  4. diabetic nephropathy

Toxicity: “Captopril’s CATCHH”

  • Cough
  • Angioedema
  • Teratogen
  • Creatinine ↑ (因为↓ GFR）
  • Hyperkalemia 高钾血症
  • hypotension

最著名的毒副作用（cough)怎么避免: 用AT II inhibitors (-sartans)

Step 1 notes (22 decks)

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