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Renal > Pediatric Nephrology > Flashcards

Pediatric Nephrology Flashcards

1
Q

What is the first embryonic kidney?

A

pronephros

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2
Q

When does the pronephros develop?

A

3 weeks gestation

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3
Q

What is the second embryonic kidney?

A

mesonephros

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4
Q

When does the mesonephros develop?

A

4 weeks gestation

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5
Q

What does the mesonephros become?

A

it degenerates into the wolffian/mesonephric duct and will become the ureteric bud

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6
Q

What establishes the structure and number of nephrons in the kidney during development?

A

branching of the ureteric bud

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7
Q

When do the first glomeruli form?

A

at 9 to 10 weeks gestation

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8
Q

When during pregnancy is nephron number increasing, and when is it completed?

A

late second and third trimesters, done by 32 to 36 weeks

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9
Q

What does CAKUT stand for?

A

congenital abnormalities of the kidney and urinary tract

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10
Q

What is the most common form of CAKUT?

A

hydronephrosis, which may be associated with obstruction/obstructive uropathy or vesicoureteral reflux/reflux nephropathy

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11
Q

What group of disorders contain the primary causes of CKD and ESKD in the pediatric population?

A

CAKUT

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12
Q

What proportion of CKD/ESKD etiologies in pediatrics are due to hypertension and diabetes?

A

less than 1%

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13
Q

In adults who were born with or had pediatric CKD, what is the primary concern?

A

cardiovascular risks

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14
Q

What are the main complications of pediatric CKD?

A 
impaired growth 
anemia 
hypertension 
MBD 
CV mortality 
cognitive development
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15
Q

Who’s growth is most affected in pediatric CKD?

A

infants with CKD, who lose the 1/3 of total growth that occurs during the first 2 years of life

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16
Q

What are causes of growth impairment in CKD in kids?

A

oral aversion, feeding intolerance, metabolic acidosis, mineral and bone disorders

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17
Q

How do you treat growth impairment in pediatric CKD?

A

treat acidosis
nutritional support/G tube
growth hormone therapy

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18
Q

When is anemia a big problem in pediatric CKD?

A

during CKD stages 3 to 5, where there is a huge increase in prevalence

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19
Q

What causes anemia in pediatric CKD?

A

decreased EPO production, iron deficiency and dysregulation

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20
Q

How is anemia treated in pediatric CKD?

A

iron/vitamin supplementation

recombinant human EPO (rHuEPO)

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21
Q

What proportion of kids with CKD have HTN?

A

half

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22
Q

What is HTN in kids with CKD a risk factor for?

A

LVH and cardiovascular mortality

23
Q

What is the treatment goal for HTN in pediatric CKD?

A

BP < 50th percentile for age and height using RAAS inhibitors

24
Q

What is particularly important in assessing HTN in pediatric CKD patients?

A

evaluating for masked hypertension and white coat hypertension

25
Q

What is a cardiovascular risk associated with CKD MBD in childreN?

A

extra skeletal and vascular calcification

26
Q

What are treatment goals for managing MBD in pediatric CKD?

A

lowering serum phos and PTH while avoiding hypercalcemia

27
Q

How is MBD managed in pediatric CKD?

A

diet
phos binders
activated vit D supplements
** avoid over suppression of PTH for growing children**

28
Q

What is the leading cause of death in the pediatric CKD population?

A

cardiovascular disease

29
Q

Does cardiovascular risk improve in pediatric CKD following renal transplant?

A

yes but not entirely

30
Q

What risk group are kids with CKD in for cardiovascular disease?

A

highest risk, which includes familial hypercholesterolemia, type I DM, heart transplant, and Kawasaki disease)

31
Q

what is an important diagnosis to rule out when evaluating UTI’s in childreN?

A

posterior urethral valves - changes bladder structure and development, with very significant long term consequences

32
Q

What should be ruled out when evaluating a child for isolated persistent hematuria?

A

wilms tumors, nephrolithiasis/hypercalciuria

33
Q

What are significant risks in actively nephrotic children?

A

infection and thrombi

34
Q

What is the most common nephrotic syndrome in children?

A

minimal change disease

35
Q

Does a lack of Lack of hematuria, hypertension, hematuria, and azotemia support or refute a diagnosis of minimal change disease?

A

it supports it

36
Q

If there is no response/remission of proteinuria in minimal change disease following standard therapy, what is the next step?

A

evaluate for other causes of NS such as FSGS or membranous nephropathy

37
Q

Which glomerulonephritis have low complement levels? (5)

A
  1. PSGN
  2. MPGN
  3. Lupus nephritis
  4. bac endocarditis or VA shunt nephritis
  5. C3 GN
38
Q

What glomerulonephritis’ have normal complement levels? (2)

A
  1. IgA nephropathy

2. Alport syndrome/thin basement membrane disease

39
Q

What is the most common childhood vasculitis?

A

henoch-schonlein purpura (HSP)

40
Q

What nephropathy is HSP biopsy similar to?

A

igA nephropathy

41
Q

Which of the vasculitides is very rare in pediatrics?

A

anti-GBM/goodpasture’s

42
Q

Which of the cystic kidney diseases has no functional tissue?

A

multicystic dysplastic kidney

43
Q

What is Caroli’s disease?

A

ARPKD with congenital hepatic fibrosis

44
Q

What is nephronophthisis?

A

AR ciliopathy which causes interstitial fibrosis and corticomedullary cysts

45
Q

Which childhood renal disease can have eye disease or neurologic syndromes along with it?

A

nephronophthisis

46
Q

What is pediatric renal disease that currently requires both a liver and kidney transplant?

A

primary hyperoxaluria

47
Q

Proximal RTA/Fanconi syndrome, polyuria and failure to thrive at a very early age can be followed by ESKD if this disease isn’t diagnosed.

A

nephropathic cystinosis

48
Q

A prenatal ultrasound reveals that the fetus has 2 kidneys that are both measuring large for gestational age and are echogenic. What is the most likely diagnosis?

a. Multicystic Dysplastic Kidney
b. ADPKD
c. ARPKD
d. Renal Agenesis
e. Hydronephrosis

A

c- ARPKD

49
Q

A 16 year old boy with no past medical history presents with clearly chronic, end-stage kidney disease (ESKD) of unknown etiology. Which is the most likely underlying diagnosis?

a. ADPKD
b. Renal Dysplasia
c. ARPKD
d. Lupus Nephritis
e. Diabetic Nephropathy

A

b - renal dysplasia

50
Q

Which complication of CKD is unique to the pediatric population?

a. CKD-MBD (mineral bone disorder)
b. Anemia
c. Hypertension
d. Growth Impairment
e. Psychosocial impairment

A

d- growth impairment

51
Q

A 7 year old boy presents with brown urine, hematuria and proteinuria on UA, and hypertension. Complement level C3 is low. What is the most likely diagnosis?

a. Lupus nephritis
b. Post-infectious glomerulonephritis
c. Membranoproliferative glomerulonephritis (MPGN)
d. IgA nephropathy
e. Focal segmental glomerulosclerosis (FSGS)

A

b - PSGN

52
Q
  1. What is the cut off for a normal protein-to-creatinine spot ratio in the urine?
A

0.2

53
Q

Above what number is a protein-to-creatinine ratio considered to be in the nephrotic range?

A

2-3

54
Q

What is the number of RBCs to be seen on a microscopic urinalysis that can be considered/defined as microscopic hematuria?

A

> 5 RBCs per high powered field (RBC/hpf)

Renal (28 decks)

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