Renal Flashcards
nephrogenesis:
from which age to which in gestation?
- which structure degenerates?
- then which structure function as interim kidney, until when? then become what?
- which structure is permanent? include which 2 major structures? 这两互动不良会有什么后果？
- which structure is the last to canalize? 特点？
nephrogenesis:
from which age to which in gestation: continue thru 32-36 wks
- which structure degenerates: pronephros [原肾,前肾]
- then which structure function as interim kidney: mesonephrons 中肾
until when: 1st trimester
then become what: male genital system - 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
- which structure is the last to canalize?
ureteropelvic junction: 输尿管肾盂连接
特点：MC site of hydronephrosis in fetus
Potter syn:
口诀？
Causes?
Cx?
MCC of death?
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)
horseshoe kidney:
- what deficit?
- kidneys trapped between?
- association?
- what deficit: inferior poles of both kidneys fuse
- kidneys trapped between: aorta and IMA (Inferior mesenteric A)
- association: Turner Sym (XO性染色体）
Multicystic dysplastic kidney:
Cause?
双侧还是单侧？
多囊肾发育不良
Cause： abnormal interaction between ureteric bud and metanephric mesenchyme
双侧还是单侧：单侧
kidney transplantation:
取哪侧？
left - longer vein
Ureters: course:
female? male?
Water under the bridge
in female: under uterine artery 子宫动脉
in male: ductus deferens 输精管
Rule of body fluid (% of body weight)?
How to measure:
1) Plasma volume?
2) ECF (extracellular fluid)?
normal osmolarity?
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
glomerular filtration barrier由哪几种机制构成？
如果其中一种barrier lost, 导致？
glomerular filtration barrier由哪几种机制构成：
- 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
对物质X的renal clearance怎么计算？
以下情况意味着：
- Cx < GFR
- Cx > GFR
- Cx = GFR
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：
- Cx < GFR: net tubular reabsorption of X
- Cx > GFR: net tubular secretion of X
- Cx = GFR: no net tubular reabsorption or secretion of X
Best substance used to calculate GFR?
What’s used to estimate GFR? is it over- or under-estimate?
normal GFR?
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
how to estimate ERPF (effective renal plasma flow?
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）
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?
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
↑ [plasma protein] 对GFR, RPF, FF的影响？
↓ [plasma protein] 对GFR, RPF, FF的影响？
↑ [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 ↑
constriction of ureter对GFR, RPF, FF的影响？
输尿管狭窄，鲍曼囊压力retrograde 升高，尿滤不过
GFR ↓, RPF 不变, FF ↓
定义: Filter load (how to calculate)?
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
Glucose: 在肾脏哪个节段、以什么机制清除？
when will glucosuria show?
when will transporters be saturated?
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
aa: 在肾脏哪个节段、以什么机制清除？
associated with which disease?
aa: re-absorbed by sodium-dependent transporters at proximal tubule (近端肾小管）
associated with which disease - Hartnup disease (AR)
Hartnup disease:
genetic pattern?
Cause?
Cx?
Rx?
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
Nephron segment physio:
PCT:
- functions?
- regulators?
PCT (proximal convoluted tubule):
- 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 - regulators:
1) PTH: 排磷
2）AT II: stimulate Na+/H+ exchange, 保钠保水， 保HCO3- : “contraction alkalosis”
Nephron segment physio:
descending loop of Henle (thin):
1. functions?
- functions:
被动重吸收水- 尿液浓缩
Nephron segment physio:
ascending loop of Henle （thick):
- functions?
- regulators?
- sites for which drugs?
- 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） - regulators：
1） loop diuretics (inhibit Na+/K+/2Cl- co-transporter)
Nephron segment physio:
DCT:
- functions?
- regulators?
- sites for which drugs?
DCT (distal convoluted tubule):
- functions:
1) actively reabsorb Na+, Cl-
2) make urine hypotonic - regulators
1) PTH: 排磷 (PCT也受PTH调控） - sites for which drugs： thiazide diuretics
Nephron segment physio:
collecting tubule:
- functions?
- regulators?
- sites for which drugs?
- functions:
在Aldosterone作用下保钠排钾 - regulators：
1) Aldosterone: ↑ Na+ channel on luminal side
2) ADH: ↑ aquaporin H2O channel on luminal side - sites for which drugs：
1） amiloride 阿米洛利 [利尿药]
2) triamterene 三氨喋呤
renal tubule defects: 记忆口诀？
“The kidney put out FABulous Glitting Liquid”
FAnconi syn： PCT
Bartter syn: thick ascending loop of Henle
Gitelman syn: DCT
Liddle syn: collecting duct
Fanconi syn:
cause?
affect region?
Cx?
“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
Gitelman syn：
genetic pattern?
affect?
Cx?
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）
Liddle syn:
genetic pattern?
affect?
Cx?
Rx?
“The kidney put out FABulous Glitting Liquid”
genetic pattern: AD
affect: renal collecting tube: ↑ Na+ reabsorption
Cx: HTN, hypokalemia, 代碱，↓ aldosterone
Rx: Amiloride
“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？
“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+
AT II: acts on which systems/organs?
- VSMCs: ↑ BP
- Kidney: constricts efferent arteriole of glomerulus: ↑ FF to preserve GFR in low-volume status
- 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)
- Adrenal gland: 分泌aldosterone: 促进水、钠重吸收
- posterior pituitary: ADH (促进水重吸收)
- stimulate hypothalamus - thirst
ANP:
made by ?
functions?
Mechanism:
作用位点？
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
ADH：
made by ?
functions?
Mechanism:
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
JGA (juxtaglomrular apparatus):
由什么细胞组成？
对什么反应？
功能？
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
EPO:
made by?
function?
made by: renal interstitial cells in the peritubular cap. bed
function: in response to hypoxia
what effect does the kidney has to VitD?
this step is regulated by which hormone?
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
prostaglaindins:
对肾脏血流的影响？
PG本身被什么药物抑制？
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）
Mx for NSAIDs to cause ARF (acute renal failure)?
NSAIDs inhibits renal-protective PG synthesis - constricts the afferent arteriole and ↓ GFR
AT II:
synthesized in response to ?
sits of action?
对GFR, FF, [Na+]的影响？
net effects?
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】
PTH:
synthesized in response to ?
sits of action?
net effects?
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
Aldosterone:
synthesized in response to ?
sits of action?
Mx?
net effects?
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
potassium shifts:
哪些情况钾shift out from cells?
哪些情况钾shift into cells?
口诀：
- Pts with hyperkalemia? DO insulin Lab work.
- Insulin shifts K+ into cells
低钾引起？
高钾引起？
低钾引起：U wave, flattened T waves
高钾引起: wide QRS, peaked T waves
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？
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
respiratory acidosis: define
which conditions cause respiratory acidosis?
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）
- airway obstruction
- acute/chronic lung disease
- opioids, sedatives
- weakening of respiratory muscles
metabolic acidosis: define
which conditions cause respiratory acidosis?
pH < 7.4
PCO2 < 40 mmHg (with compensation from hyperventilation)
- 再分类需要check anion gap
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？
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
respiratory alkalemia: define
which conditions cause respiratory alkalemia?
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
metabolic alkalemia: define
which conditions cause respiratory alkalemia?
pH > 7.4, PCO2 > 40 mmHg
(with compensation from hypoventilation)
“LA-VH”
- loop diuretics
- antacid use
- vomiting
- hyperaldosteronism
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?
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)
哪些情况有hematuria, no casts?
bladder cancer
kidney stones
哪些情况有pyuria (脓尿）, no cast？
cystitis 膀胱炎
RBC casts indicate?
ARF
- ongoing glomerulonephritis
- ischemia
- malignant HTN
WBC casts indicate?
- tubulointersitial inflammation
- acute pyelonephritis
- transplantation rejection
fatty casts indicate?
“oval fat bodies” - nephrotic syn
granular casts indicate?
“muddy brown” casts - acute tubular necrosis
waxy casts?
CRF (chronic renal failure)
advanced renal disease
Define/各自哪些代表疾病？
Nephritic syn
Nephronic syn
NephrItic syn = (Inflammation) 肾炎综合征
NephrOnic syn = 肾病综合征 (O= prOteriuria, > 3.5 g/day)
focal segmental glomerulosclerosis:
Nephritic syn or Nephronic syn?
特点？
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
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？
Dx: membranous nephropathy
Nephritic syn or Nephronic syn: MC for Nephronic syn in caucasian adults (Nephronic presentation of SLE)
肾病Dx “buss words”:
LM - normal
IF: negative
EM: “effacement of foot process”
Dx?
Nephritic syn or Nephronic syn?
特点
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
MPGN (membrano-proliferative glomerulonephritis):
Nephritic syn or Nephronic syn?
分型？各自LM, IF特点? association?
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)
diabetic glomerulonephropathy:
Nephritic syn or Nephronic syn?
LM： patho的特征结构？
损伤哪2个结构？后果？
Nephronic syn
LM: Kimmelstiel-Wilson lesion (mesangial expansion, GBM thickening, eosinophilic nodular glomerulosclerosis)
- non-enzymatic glycosylation of GBM: ↑ permeability, thickening
- non-enzymatic glycosylation of efferent arteriole- ↑ GFR, - mesangial expansion
Nephritic syn特点？
代表疾病？
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”
- PSGN (acute poststreptococcal glomerulonephritis) 急性链球菌感染后肾小球肾炎
- RPGN (rapid progressive glomerulonephritis)
- DPGN (diffuse proliferative glomerulonephritis)
- Berger disease (IgA nephropathy)
- Alport syn
PSGN:
- 好发人群？
- Cx?
- LM? IF? EM?
- lab?
- 好发人群： children, ~ 2wks after group A streptococcal infection (pharynx or skin) - spontaneously resolve
- Cx: peripheral and periorbital edema, dark urine (cola-colored), HTN
- LM: glomeruli enlarged and hyper cellular
IF: “starry sky”, “lumpy bumpy” - deposits of IgM, IgG, C3 deposition along GBM
EM: subepithelial IC humps - lab: ↑ anti-DNase B titer, ↓ complement levels
RPGN:
哪些疾病可能result in this pattern?
LM, IF特点？
Prognosis?
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：
- Goodpasture Syn
- Wegener (granulomatosis with polyangiitis)
- microscopic polyangiitis
LM, IF特点: crescent-moon shape (fibrin, C3b, monocytes, macrophages)
Dx:
~ 40 yr, 血尿+HTN, 肾脏活检见“linear IF”?
哪一型hypersensitivity?
Goodpasture syn (Type II hypersensitivity)
RPGN一种
Ab against GBM (里面的type IV collagen)， 形成典型的linear IF
Wegener disease: 什么病？
Cx?
Ab marker?
necrotizing granulomatous polivasculitis
Cx: 累及3大系统：
- 肺部症状：咳血 he’moptysis
- 上呼吸道：肉芽肿+溃疡
- 肾脏：RPGN
Ab marker： c-ANCA
microscopic polyangiitis: 又名？
最显著的lab marker?
Churg-Strauss syn
最显著的lab marker: p-ANCA (anti-neutrophil Ab)
DPGN (diffuse proliferative glomerulonephritis):
是什么疾病的表现？
Nephritic syn or Nephronic syn?
LM, EM, IF特点？
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
Berger disease: 又名？
LM, EM, IF特点？
Cx：
seen with which disease?
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）
Alport Syn:
在肾脏的表现？
其他系统的表现？
病因？
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)
Dx:
unilateral flank tenderness
colicky pain radiating to groin
hematuria
kidney stone
MC presentation for kidney stone?
cause?
XR?
urine crystal?
Rx: thiazides, citrate
calcium oxalate 草酸钙 stone in pt with hypercalciuria, normocalcemia
cause:
- hypercalciuria (idiopathic, or 2° to conditions causing hypercalciuria: cancer, ↑ PTH, etc)
- ethylene glycol (乙二醇, antifreeze)
- VC abuse
- crohn disease
XR: radiopaque (X光上不透明）
urine crystal：envelop, or dumbbell
Dx: “coffin lid” radiopaque kidney stone?
由什么组成？
cause?
容易引起什么？Rx?
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
the only radiolucent kidney stone?
CT, ultrasound, XR?
association?
Rx?
Uric acid stone (5%) - radiolUcent
visible on CT, ultrasound;
not on XR
association:
1) gout (hyperuricemia)
2) ↑ cell turnover (leukemia)
Rx: alkalization of urine
Dx: hexagonal urine crystal?
MC seen in which population? 2° to ?
哪种test +?
RX?
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
MC 1° renal malignancy:
- 好发人群？
- 组织学起源？病理所见？
- Cx?
- 转移途径？转移到什么器官？
- association with gene deletion on which chr?
- Rx?
renal cell carcinoma (RCC):
- 好发人群：50-70 yr male, ↑ incidence with smoking and obesity
- 组织学起源-proximal tubule cells
病理所见: polygonal clear cells, filled with lipids and carbohydrates - Cx: chronically (“silent cancer”), hematuria, flank pain, fever, weight loss
- 转移途径: 血行（invade renal v - IVC), metastasizes to lung and bone
- if not sporadic, association with
1) gene deletion on which chr3 (von Hippel-Lindau syn)
2) paraneoplastic syn (ectopic EPO, ATCH, PTHrP) - Rx: resection (if local) or immunotherapy
resistant to chemo or radiation
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?
Dx: renal oncocytoma 嗜酸细胞瘤 (benign)
diff with which disease： chromophobie renal cell carcinoma
Rx: nephrectomy
Define: Wilms tumor
发生年龄？ 组织学见？ Cx? gene mutation on which chr? part of which syn?
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
Dx: 60-80 yr pt, painless hematuria, no casts?
- associated with?
- 组织学所见？
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
- 组织学所见：papillary growth lined by transitional epithelium with mild nuclear atypic and pleomorphism
squamous cell carcinoma of the bladder:
病因、病理？
Risk factors? 和哪个地区有关？
Cx:
病因、病理：chronic irritation of bladder - squamous metaplasia - dysplasia and squamous cell carcinoma
Risk factors：
- 和哪个地区有关： middle east (Schistosoma haematobium 埃及裂体吸虫)
- chronic cystitis, smoking, chronic nephrolithiasis 肾石病
Cx: painless hematuria
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?
Cx: suprapubic pain, dysuria, urinary frequency, urgency
有无systemic signs (fever, chills): 无
Risk factors：
- female (尿道短）
- sex (“honeymoon cystitis”)
- indwelling catheters
MCC:
- E. coli (无论哪个病人群体里都是最常见的病因）
- Staphylococcus saprophyticus 腐生性葡萄球菌 (esp in sexually active females)
- Klebsiella
- Proteus mirabilis 奇异变形杆菌 [urine has ammonia scent)
- 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
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？
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”]
drug-induced interstitial nephritis (tubulointerstitial nephritis):
lab?
哪些药物引起？时间点？
Cx?
lab: pyuria （脓尿， classically eosinophils) + azotemia
哪些药物引起？时间点？
- 1-2 wks after: diuretics, penicillin derivates, sulfonamides, rifampin
- months after NSAIDs
Cx: fever, rash, hematuria, costoverterbral angle tenderness, can be asymptomatic
Diffuse cortical necrosis: 单侧还是双侧肾脏？
可能病因？
association?
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
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?
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
renal papillary necrosis 肾乳头坏死
Cx?
病因？
trigger?
association (尤其是和药物的关系）?
Cx: gross hematuria and proteinuria
病因: sloughing of renal papillae
trigger: resent infection or immune stimulus
association：
- DM
- acute pyelonepphritis
- chronic phe’nacetin use (非那西汀, 解热镇痛药: 包括acetaminophen 扑热息痛,)
- sickle cell anemia
Acute renal failure (ARF): 定义？
病因上要区分为肾前、肾脏本身、肾脏后因素：
- prerenal azotemia: 病因？Na+, H2O的变化，导致BUN/creatinineratio变化？
- intrinsic RF:
病因？导致BUN/creatinineratio变化？ - postrenal azotemia: 病因？导致BUN/creatinineratio变化？
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：
- 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 ↑ - 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 ↓
- 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 ↑
renal failure consequences: 口诀？
MAD HUNGER
renal osteodystrophy 病理？
Cx?
- failure of VitD hydroxylation: ↓ intestinal Ca2+ reabsorption
hypocalcemia + hyperphosphatemia - 2° hyperparathyrodism
- hyperphosphatemia - tissue calcification - ↓ serum Ca2+
Cx: subperiosteal 骨膜下 thinning of bones
ADPKD:
遗传特征？mutation?
cx?
cause of death?
Association?
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:
- berry aneurysms
- MVP 二尖瓣脱垂
- benign hepatic cysts
ARPKD: 原名？
遗传特征？mutation?
cx?
cause of death?
Association?
infantile PKD
medullary cystic disease :
定义？
B超见？is medullary cyst visible?
Px?
(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
simple vs. complex renal cyst:
哪种病require follow-up or removal?
- simple renal cyst:
very common, 最多见的renal masses , 多发于outer cortex;
typically asymtomactic, found incidentally
- complex renal cyst:
require follow-up or removal, due to risk of RCC
Mannitol:
site of action?
Mx?
Use?
Toxicity/contraindication?
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
acetazolamide
site of action?
Mx?
Use?
Toxicity/contraindication?
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:
- glaucoma (在青光眼中考过机制：inhibit aqumous humor secretion by the ciliary epithelium
- urinary alkalization
- metabolic alkalosis 代碱
- altitude sickness
- pseudotumor cerebri
Toxicity/contraindication:
“ACID”azolamide causes ACIDosis
- hyperchloremic acidosis 高氯血症性酸中毒
- paresthesia: 感觉异常
- NH3 toxicity
- sulfa allergy [出现对磺胺过敏时，换用ethacrynic acid 利尿酸】
furosemide
site of action?
Mx?
Use?
Toxicity?
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:
- edematous states (CHF, pulmonary edema, cirrhosis, nephrotic syn)
- HTN
- 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
‘ethacrynic acid
site of action?
Mx?
Use?
Toxicity/contraindication?
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
- edematous states (CHF, pulmonary edema, cirrhosis, nephrotic syn)
- HTN
- 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
hydrochlorothiazide (HCTZ)
site of action?
Mx?
Use?
Toxicity/contraindication?
氢氯噻嗪, thiazide diuretics
site of action: early DCT
Mx: inhibit NaCL reabsorption, ↓ diluting capacity of the nephron, ↓ Ca2+ excretion
Use:
- HTN
- CHF
- idiopathic hypercalciuria
- nephrogenic diabetes insipidus
- 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
K+- sparing diuretics: 代表药物？
site of action?
Mx?
Use?
Toxicity/contraindication?
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:
- Spironolactone , eplerenone: competitive AR antagonists
- triamterene, Amiloride: block Na+ channels
Use:
- hyperaldeosteronism
- K+ depletion
- CHF
Toxicity:
- hyperkalemia - can cause arrhythmia !!!
- Spironolactone由于拮抗雄激素受体而出现的内分泌症状：gynecomastia, antiandrogen effects
Diuretics:
how to change urine NaCL?
the only exception?
↑ 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
Diuretics:
how to change urine K+?
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
Diuretics:
which ones ↓ blood pH (acidemia)?
which ones ↑ blood pH (alkalemia)?
Mx?
↓ 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
Diuretics:
which ones ↑ urine Ca2+ (同时 ↓ 血钙）？
which ones ↓ urine Ca2+ (同时 ↑ 血钙）？
Mx?
which ones ↑ urine Ca2+ (同时 ↓ 血钙）： Loop diuretics (Furosemide, ethacrynic acid)
which ones ↓ urine Ca2+ (同时 ↑ 血钙）: thiazides
ACE inhibitors:
代表药物？ 机制？ Use? Toxicity? 一个最著名的毒副作用怎么避免？
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：
- HTN
- CHF (prevent unfavorable heart remodeling)
- proteinuria
- 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)
