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GIR Test 3 > B5.045 Renal Physiology V: Renal Tubular Diseases > Flashcards

B5.045 Renal Physiology V: Renal Tubular Diseases Flashcards

1
Q

overview of tubular function

A

specialized transport proteins move electrolytes, organic solutes, and water in both absorptive and secretory direction

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2
Q

proximal tubule function

A

reabsorption of solutes/fluid

secretion of organic anions/cations

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3
Q

ascending loop of henle function

A

reabsorption of Na+, Cl-, K+

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4
Q

distal tubule function

A

reabsorption of Na+, Cl- and water

secretion of K+ and H+

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5
Q

collecting duct function

A

reabsorption of Na+, Cl- and water

secretion of K+ and H+

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6
Q

2 sources of tubulopathies

A
  1. mutation of renal transport systems, inherited in both dominant and recessive manner
  2. acquired defects
    - induced by pharm agents
    - injury and inflammatory processes
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7
Q

primary transporters in proximal tubule

A

all transport depends on Na,K ATPase

  • glucose, AA, phosphate absorbed by Na+ co-transporters
  • protons secreted by Na+ proton exchanger
  • chloride follows paracellular pathways and base-dependent exchangers
  • organic acids are secreted by Na+ dependent and independent OAT transporters
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8
Q

apical OA transporter

A

secretes OA via exchange with Cl-

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9
Q

basolateral OA transporter

A

absorb OA via exchange with Na+

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10
Q

effect of transport defects in proximal tubule

A

Fanconi syndrome
kidney reabsorptive condition
whole proximal tubule is effectively shut down

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11
Q

signs and symptoms of Fanconi syndrome

A 
polyuria, polydipsia, and dehydration
hypophoshatemic rickets or osteomalacia
growth failure
metabolic acidosis
hypokalemia
hyperchloremia
hypophosphatemia/phosphaturia
glucosuria, aminoaciduria
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12
Q

why do you get hyperchloremia in Fanconi

A

due to acidosis

bicarb is lost and exchanged for Cl-

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13
Q

variability of symptoms in Fanconi

A

depend on extent of compromise in proximal tubule

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14
Q

genetic diseases associated with Fanconi syndrome

A 
cystinosis
galactosemia
glycogen storage disease
Lowe syndrome
Wilson disease
tyrosinemia
Dent's disease
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15
Q

environmental assaults that can cause Fanconi’s syndrome

A

exposure to nephrotoxic heavy metals
expired tetracyclines, gentamycin
toluene and derivatives (paint factories)

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16
Q

what causes the genetic forms of Fanconi

A

accumulation of metabolic products and/or metals in proximal tubule cells leads to cellular dysfunction and necrosis

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17
Q

primary transport systems in ascending loop of henle

A

Na+, K+, and Cl- are all cotransported on apical side by NKCC (important for countercurrent exchange system)
K+ and Cl- reabsorbed together at basolateral side or independently through channels
K+ also leaks to tubular side via ROMK1

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18
Q

function of ROMK1

A

secretes K+ back into lumen

helps K+ recycling and maintains function of NKCC

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19
Q

what leads to transport defects in ascending loop of henle

A

alterations in ROMK1, NKCC, and Barttin

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20
Q

what is Barttin

A

basolateral Cl- channel in ascending loop of henle

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21
Q

clinical signs and symptoms of bartter’s syndrme

A 
polyuria, polydipsia
poor muscle tone
heart repolarizing abnormalities
hyponatremia
hypokalemia
hypochloremia
ECF volume contraction
high renin-aldosterone
metabolic alkalosis, high urine H+
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22
Q

what is the source of the electrolyte abnormalities associated with bartter’s

A

NKCC dysfunction

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23
Q

why do you get ECF volume contraction in bartter’s

A

NKCC involved in Na+ sensing in the macula densa

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24
Q

which symptoms are associated with hypokalemia

A

poor muscle tone

heart repolarizing abnormalities

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25
Q

why do you get metabolic alkalosis in bartters

A

lots of aldosterone leads to excessive H+ secretion

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26
Q

genetic mutations that can lead to Bartter’s

A

ROMK1
NKCC2
Barttin
autosomal recessive

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27
Q

environmental causes of Bartter’s

A

inhibitors of NKCC:
furosemide
bumetanide

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28
Q

transport mechanisms of distal tubule

A

Na+ and Cl- are cotransported on apical side and reabsorbed basolaterally
K+ is secreted to tubular fluid via K+ channels
Ca2+ is reabsorbed via channels and the Na/Ca exchanger

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29
Q

what is TSC

A

thiazide sensitive co-transporter

Na+/Cl- transporter on apical membrane

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30
Q

what is TRPM6

A

Mg2+ channel in apical membrane

31
Q

what is the mechanism of action of most diuretics

A

inhibit Na+ channels to induce solute diuresis

32
Q

causes of transport defects in distal tubule

A

alterations in TSC or TRPM6

Gitelman’s syndrome

33
Q

clinical signs and symptoms of Gitelman’s

A 
hyponatremia
hypokalemia, high urine K+
hypomagnesemia, high urine Mg2+
hypocalciuria
ECF volume contraction
normotensive
34
Q

why is Gitleman’s less associated with polyuria than other tubular defects

A

a lot of compensatory mechanisms from proximal tubule/loop of henle mask the effects

35
Q

how can you differentiate Gitleman’s from Bartter’s

A

Bartters has higher R-A-A activation and more trouble concentrating urine

36
Q

genetic mutations associated with Gitelmans

A

TSC
TRPM6
autosomal recessive

37
Q

environmental assaults causing Gitelmans

A

inhibitors of TSC:

thiazides

38
Q

transport mechanisms of collecting tubule

A

Na+ is reabsorbed apically by ENac
K+ is secreted to tubular fluid by K+ channels
water reabsorbed via aquaporins

39
Q

what leads to transport defects in the collecting tubule

A

alterations in ENac (constantly activated) - Liddle’s syndrome
defects of aquaporins or their regulation- nephrogenic diabetes insipidus

40
Q

signs and symptoms of Liddle’s syndrome

A 
increased ECF volume
hypertension, refractory to drugs
decreased R-A-A activation
hypokalemia
metabolic acidosis
41
Q

cause of Liddles syndrome

A

genetic mutations in the ENac subunits that avoids their cell internalization and degradation
maintains ENac activity uncontrolled
autosomal dominant

42
Q

what is nephrogenic diabetes insipidus

A

collecting ducts cells resistant to ADH

43
Q

clinical signs and symptoms of nephrogenic diabetes insipidus

A 
polyuria
polydipsia
risk of dehydration
dilute urine with low osmolarity
sometimes hypernatremia
44
Q

how do you distinguish nephrogenic from non-nephrogenic diabetes insipidus

A 
give desmopressin (exogenous ADH)
will improve if non-nephrogenic
45
Q

what causes non-nephrogenic diabetes insipidus

A

defect in ADH production

excessive water intake (psychogenic)

46
Q

genetic mutations associated with nephrogenic diabetes insipidus

A 
mutations in vasopressin receptor (V2)
defective AQP
failure in AQP targeting mechanisms
X-linked inheritance patter
independent of ADH levels and not responsive to ADH
47
Q

environmental assaults that can lead to nephrogenic diabetes insipidus

A

drugs, lithium

48
Q

process of proton secretion in nephron

A

in proximal tubule, H+ is secreted by the Na/H exchanger

in distal and collecting tubules H+ is secreted by ATPases

49
Q

effect of defects in proton secretion

A

renal tubular acidosis

classified as proximal or distal renal tubular acidosis

50
Q

clinical signs and symptoms of renal tubular acidosis

A 
muscle weakness
cardiac repolarization problems
growth retardation
kidney stones
hypokalemia
hyperchloremia
hypercalciuria
metabolic acidosis, low excretion of H+
51
Q

what is hypercalciuria and its effect

A

calcium collects in kidneys and urine

causes kidney stones

52
Q

what causes growth retardation in renal tubular acidosis

A

metabolic acidosis activates osteoclasts
moves calcium out of bone
bones look radiolucid on x-ray (dark due to lack of calcium)
bones have a bent, elastic shape

53
Q

mutations in proximal RTA

A

apical Na/H exchanger

basolateral Na/bicarb exchanger

54
Q

mutations in distal RTA

A

apical proton ATPases
basolateral anion exchanger
carbonic anhydrase

55
Q

effect of ischemia or toxins on the renal tubules

A

acute tubular necrosis

56
Q

clinical course of acute tubular necrosis

A

initial phase with aliguria
recovery phase with increase in urine but loss of electrolytes
can progress to loss of renal function/acute renal failure depending on cause

57
Q

effect of inflammation of tubules

A

acute or chronic
inability to concentrate urine
polyuria, salt wasting
metabolic acidosis

58
Q

cystic disease of the kidney

A

polycystic kidney disease
medullary cystic disease
acquired cystic disease
localized simple cysts

59
Q

types of PKD

A

autosomal dominant

autosomal recessive

60
Q

types of medullary cystic disease

A

medullary sponge kidney

nephronophthisis

61
Q

genes involved in adult PKD

A

PKD1 - polycystin 1

PKD2- polycystin 2

62
Q

genes involved in child PKD

A

PKHD1 - fibrocystin

63
Q

genes involved in nephronophthisis

A

NPH1
NPH2
NPH3

64
Q

most common monogenic disorder of the kidney

A

autosomal dominant polycystic kidney disease

65
Q

characterize autosomal dominant polycystic kidney disease

A

growth of multiple fluid-filled cysts that affect kidney structure and function
affects all ethnic groups, 1:500- 1:1000
progress to chronic renal failure (8-10% of all cases of end stage renal failure)

66
Q

manifestations of ADPKD

A

cardio alterations
hypertension
extrarenal cysts in pancreas and liver

67
Q

severity of different forms of ADPKD

A

PKD1 more common (85%) and more severe than PKD2

68
Q

function of polycystins

A

control normal kidney development and function
flow triggers polycystin complex to transfer Ca2+ into cells
Ca2+ is used in transcriptional control of genes

69
Q

phenotypic changes related to polycystin alterations

A 
transepithelial fluid secretion
cell proliferation
alteration in cell planar polarity
remodeling of ECM
cell cilia alterations
70
Q

renal symptoms of ADPKC

A 
hematuria
decrease in concentrating ability of kidney
infections
hypertension w increased renin
GFR reduces late in disease
kidney 10x normal side
compression of vena cava could lead to lower extremity edema
ESRD by age of 60
71
Q

extrarenal symptoms of ADPKD

A

cerebral aneurysms
hepatic and pancreatic cysts
LVH, mitral valve disease

72
Q

diagnosis of ADPKD

A

urine analysis normal at first
microscopic to gross hematuria, proteinuria
serum creatinine and BUN progressively rise
GFR not good indicator
imaging best for diagnostics

73
Q

treatment of ADPKD

A

ACE inhibitors
pain management, nephrectomy
dialysis or kidney transplant

GIR Test 3 (19 decks)

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