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Renal > SM 217 Pediatric Nephrology > Flashcards

SM 217 Pediatric Nephrology Flashcards

1
Q

What is the Pronephros in development?

A

Simple tubes

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2
Q

When is the Pronephros present in development?

A

The Pronephros is present 3 weeks EGA

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3
Q

What is the Mesonephros in development?

A

Filtering units and glomeruli with tubules that eventually degenerate into the Wolffian duct; formed caudal to the Pronephros at 4 weeks EGA

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4
Q

What forms the Ureteric bud?

A

Mesonephros

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5
Q

When is the Mesonephros present in development?

A

The Mesonephros is present 4 weeks EGA

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6
Q

What does the Ureteric bud do and when?

A

At 5 weeks EGA, the Ureteric bud invades the surrounding mesenchyme and begins signaling at the tips of it’s expansion to convert Metanephric Mesenchyme into renal epithelia

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7
Q

What is reciprocal signaling in the developing signaling?

A

Bidirectional signaling between the Ureteric Bud and the Metanephric Mesenchyme that allows for renal branching and the development of collecting ducts, renal pelvis, etc.

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8
Q

What embryonic structure forms the renal collecting ducts, pelvis, and ureters?

A

The Ureteric bud

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9
Q

What embryonic structure forms the glomeruli, proximal tubule, loop of Henle, and distal tubules?

A

The Metanephric Mesenchyme

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10
Q

When do the first glomeruli form and when have they all formed?

A

The first glomeruli forms at 9-10 weeks, and they form exponentially until development is complete at 32-36 weeks

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11
Q

What are CAKUT?

A

Congenital Anomalies of the Kidney and Urinary Tract - the main cause of ESRD in pediatric populations

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12
Q

What is renal agenesis?

A

Failure of the Ureteric bud to form or induce the Metanephric Mesenchyme, leading to Apoptosis

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13
Q

What is the most common CAKUT?

A

Hydronephrosis

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14
Q

What causes Hydronephrosis?

A

Vesicoureteral reflux

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15
Q

What are other, less common CAKUTs?

A

Renal cysts and renal dysplasia

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16
Q

What are the major causes of CKD/ESRD in adults?

A

Diabetic Nephropathy, HTN, ADPKD

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17
Q

Are the major causes of CKD/ESRD the same in adults and kids?

A

Nope

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18
Q

What are the major causes of CKD/ESRD in kids?

A

Wide differential, including: Renal aplasia/dysplasia, FSGS, Obstructive Uropathy

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19
Q

What are the complications of CKD in kids?

A 
Impaired growth
Anemia
HTN
Bone Disorders
CV Risk
Cognitive Development/Transition to Adult Care
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20
Q

How does CKD impair growth in kids?

A

Since 1/3rd of growth occurs in the first 2 years of life, infants with CKD have stunted growth due to malnutrition, metabolic acidosis, and mineral/bone disorders

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21
Q

How is impaired growth in kids with CKD treated?

A

Treatment of acidosis
Nutritional support via G-tube feeds
Growth Hormone therapy

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22
Q

How does anemia progress in kids with CKD?

A

Higher stages of CKD have a higher prevalence of anemia in CKD

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23
Q

What causes anemia in CKD in kids?

A

Decreased production of Erythropoietin and iron dysregulation in the kidney

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24
Q

How is anemia in CKD in kids treated?

A

Iron supplementation + recombinant human erythropoietin

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25
Q

How does HTN manifest in kids with CKD?

A

HTN effects more than half of kids with CKD and resists anti-hypertensive medications, is often masked, and is a major risk factor for CV mortality and LV Hypertrophy

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26
Q

How should HTN be managed in kids with CKD?

A

Intensified BP control to < 50th percentile for age/height is better, and ambulatory blood pressure monitoring is required

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27
Q

How do mineral bone disorders manifest in CKD in kids?

A

Dysregulation of the Ca/Phos/PTH/FGF23 axis leads to bone growth issues and vascular calcifications

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28
Q

How are bone disorders in kids with CKD managed?

A

Try and lower serum Phosphorus and PTH levels with:

Dietary restrictions
Phosphorus binders (taste awful)
Vit D Supplements
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29
Q

What causes CV risk and mortality in kids with CKD?

A

Compounded effects of other CKD manifestations such as HTN/LV Hypertrophy, bone disorders, and anemia

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30
Q

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

A

Cardiovascular mortality

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31
Q

What forms the comma-shaped body and s-shaped body during kidney development?

A

The Metaneprhic Mesenchyme

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32
Q

What is Renal Dysplasia?

A

Irregular signaling between the Metaneprhic Mesenchyme and the Ureteric Bud

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33
Q

What could cause Renal Hypoplasia?

A

Premature birth

34
Q

What can cause hydronephrosis?

A

UVJ obstruction

35
Q

What could cause bilateral hydronephrosis?

A

A posterior uretrhal valve in the bladder

36
Q

How do genetic factors contribute to the development of CAKUT?

A

Large combination of genetic factors of varying modes of inheritance

37
Q

What determines the severity of CKD in kids?

A

Age of onset, primary renal disease (systemic disease is worse), metabolic acidosis, and malnutrition + anemia/bone disorder/growth distrubances

38
Q

Why is sodium tricky in kids with CKD?

A

Traditional CKD management in adults focuses on low sodium, but in kids, some sodium is needed for development

39
Q

How do CV causes of death differ between adults and kids with CKD?

A

Adults with CKD have CHF and atherosclerosis

Kids with CKD have arrythmyias, cardiomyopathy, medial calcifications

Kids with CKD have CV risk with less warning signs

40
Q

How does CKD effect cognitive development in kids?

A

CKD leads to low cognitive ability and quality of life, as well as low social development due to short stature

41
Q

Why is there a high risk period of transition to adult care in kids with CKD?

A

Adolescents have poor judgment as is, and this can lead to treatment non-adherence with most kidney transplants failing between 17 - 24 years of age

42
Q

What are renal ultrasound and VCUG used for?

A

Searching for hydronephrosis and renal enlargement

43
Q

Why might UTI’s be concerning in kids with CKD?

A

UTI may indicate urinary tract obstruction or posterior urethral valves, which can have long term consequences such as repeated infections

44
Q

What is VUR?

A

Vesicoureteral Reflux, the retrograde flow of urine from the bladder into the ureters which predisposes UTI and hydronephrosis

45
Q

What are consequence of recurrent UTI in kids with CKD?

A

Renal scars, hypertension, and CKD (due to whatever is causing the recurrent UTI’s, not the UTI’s themselves)

46
Q

What factors should be considered in interpreting gross hematuria?

A

Timing: part of stream = urinary tract, full stream = kidney
Color: Red/Pink = urinary tract, brown = kidney
Pain: Painful = urinary tract, painless = kidney

47
Q

How should microscopic hematuria be interpreted in kids with CKD?

A

More concerning in the presence of proteinuria and a family history of kidney failure/kidney stones

48
Q

What defines remission of nephrotic syndrome?

A

Proteinuria < 3.5mg/day for 3 consecutive days with resolution of edema

49
Q

What defines relapse of nephrotic syndrome?

A

Proteinuria > 3.5mg/day for 3 consecutive days with resolution of edema

50
Q

What is the most common cause of nephrotic syndrome in kids?

A

Minimal Change Disease

51
Q

What are the types of Steroid Sensitive Nephrotic Syndrome?

A

Steroid Dependent NS

Frequently Relapsing NS

52
Q

What is Steroid Resistant Nephrotic Syndrome?

A

Failure to achieve remission after 4 weeks of prednisone, suggesting steroid resistant MCD or something else like Membranous Nephropathy

53
Q

What is the DDx for kids with Nephrotic Syndrome?

A 
MCD
FSGS
Membranous Nephropathy
Congenital Nephrotic Syndromes
Familial Nephrotic Syndromes
54
Q

What are complications of Nephrotic Syndrome in kids?

A

Hypogammglobulinemia
Loss of anticoagution factors
AKI

55
Q

What infections are kids with Nephrotic Syndrome predisposed to and why?

A

Loss of immunoglobulins = risk for Pneumococcal infections

56
Q

How do complement levels change in PSGN?

A

Low C3, normal C4

57
Q

How do complement levels change in Lupus and MPGN?

A

Low C3, Low C4

58
Q

How could PSGN and IgA Nephropathy be differentiated by timing?

A

IgA Nephropathy occurs during or shortly after a mucosal infection, while PSGN occurs 2 weeks after a pneuomococcal infection

59
Q

What is an abscess?

A

Chronic bacterial infection

60
Q

Which GN have low complement levels?

A

PSGN
MPGN
Lupus

61
Q

Which GN have normal complement levels?

A

IgA Nephropathy

ANCA-associated GN

62
Q

What is Henoch Schonlein Purpura?

A

A vascular infection that presents with normal complement levels and hematuria and nephrotic range proteinuria

63
Q

How do MPA and GPA differ with respect to granulomas?

A

GPA has necrotizing granulomas in the airways + kidneys while MPA is non-granulomatous in the vasculature

64
Q

What can suggest Hemolytic Uremic Syndrome in kids?

A

Bloody diarrhea

65
Q

Is Eculizumab indicated for D-HUS or aHUS

A

Eculizumab is a C5 convertase inhibitor, so it is indicated for aHUS (driven by defects in the CRP) and not D-HUS

66
Q

What diseases can cause a HUS-like syndrome in kids (ie DDx)?

A 
Pneumococcal-associated HUS (due to Neuraminidase)
Atypical HUS (recurrent episodes)
67
Q

Which type of HUS uses the MAC to cause damage?

A

aHUS, since it is driven by complement and can form the MAC to damage cells

68
Q

How does medullary sponge kidney present?

A

Neprholithiasis, collecing duct dilation

69
Q

Is Multycystic Dyplastic Kidney Disease symptomatic and bilateral?

A

Nope, unilateral and asymptomatic

70
Q

What gene mutation and gene product cause ARPKD?

A

ARPKD is driven by mutations in PKHD1 and result in altered production of Fibrocystin, with the different mutations setting severity of disease

71
Q

Are ADPKD and ARPKD unilateral or bilateral?

A

ADPKD and ARPKD are bilateral

72
Q

Which causes hepatic fibrosis and portal hypertension, ADPKD or ARPKD?

A

ARPKD effects the liver and causes fibrosis as well as hepatic hypertension

73
Q

Which causes compressed lungs, ADPKD or ARPKD?

A

ARPKD has much larger kidneys and causes compressed lungs

74
Q

Which causes ESKD earlier, ADPDK or ARPKD?

A

ARPKD causes ESKD much earlier, in utero or early childhood, while ADPKD causes kidney failure in adulthood

75
Q

How is ADKPD diagnosed?

A

Ultrasound

76
Q

What is Nephronophtisis?

A

An Autosomal Recessive ciliopathy caused by mutation in NPHP genes, associated with eye disease and leads to ESKD

77
Q

What is Primary Hyperoxaluria?

A

Mutation in AGXT gene that leads to systemic overproduction of oxalate and kidney failure

78
Q

What is Nephropathic Cystinosis?

A

Autosomal Recessive lysosomal storage disorder caused by mutation in CTNS gene that leads to proximal RTA, treated with Cysteamine

79
Q

What characterizes Multicystic Dysplastic Kidney?

A

No functional kidney tissue

80
Q

How many RBC’s need to be seen on a microscopic urinalysis to define microscopic hematuria?

A

5+

81
Q

What defines a nephrotic range protein-creatinine ratio?

A

2-3 or more

82
Q

How can IgA Nephropathy and PSGN be differentiated?

A

IgA Nephropathy does not effect Complement levels while PSGN has low C3

Renal (19 decks)

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