Pediatric Nephrology Flashcards

1
Q

How much of the cardiac output does the nephron in the neonate receive ?

A

20-30ml/min

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2
Q

What are the 5 functions of the kidney ?

A
Waste handling (Urea and creatinine)
Water handli9Sodium and potassium)ng 
Salt balance 
Acid base balance
Endocrine (Red cells/blood pressure/bone health)
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3
Q

In glomerular filtration what is the purpose of the endothelial cells ?

A

Fenestrated
Filter
Vulnerable to immune mediated injury

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4
Q

What 2 proteins are found in the basement membrane ?

A

Type IV collagen (COL4) and Laminin

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5
Q

What cells in the basement membrane of the glomerulus synthesize the proteins ?

A

Podocytes and endothelial cells

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6
Q

What is the role of mesangial cells in the glomerulus ?

A

They are smooth muscle cells within the basement membrane which are structural support of the glomerulus.

They regulate blood flow of the glomerular capillaries.

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7
Q

Proteinuria signifies what ?

A

Glomerular injury

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8
Q

What is the cause of Haematuria and what is the name of the syndrome ?

A

Increasing haematuria and intravascular overload - Nephritic syndrome

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9
Q

What is the cause of Proteinuria and what is the name of the syndrome?

A

Increasing proteinuria and intravascular depletion - Nephrotic syndrome

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10
Q

In an acquired Glomerulopathy which disease/condition attacks the epithelial ells (podocytes) ?

A

Lupus
Focal segmental glomerulitis
Minimal change disease (MCD)

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11
Q

In an acquired Glomerulopathy which disease/condition attacks the basement membrane ?

A

Membranous glomerulopathy
Membranoproliferative Glomerulonephritis.
Post infectious Glomerulonephritis.

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12
Q

In an acquired Glomerulopathy which disease/condition attacks the endothelial cells ?

A

Infection associated glomerulonephritis (PIGN), Haemolytic Uraemic Syndrome (HUS), Membranoproliferative Glomerulonephritis (MPGN), Lupus

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13
Q

In an acquired Glomerulopathy which disease/condition attacks the mesangial cells ?

A

Henoch-Schonlein purpura (HSP)
IgA nephropathy
Lupus

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14
Q

What is nephrotic syndrome?

A

Nephrotic range proteinuria which leads to Hypoalbuminaemia and finally oedema

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15
Q

What are the 3 ways in which we would test for proteinuria in children ?

A

Dipstix
Protein Creatinine Ratio (Early morning urine is best)
24 hour urine collection

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16
Q

What are the clinical signs and symptoms of nephrotic syndrome ?

A

Oedema
Proteinuria
Increased protein creatinine ratio
Low albumin

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17
Q

What are the typical features of minimal change disease?

A

Age 2-5
Normal BP
Resolving microscopic haematuria
Normal renal function

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18
Q

What are the atypical features of Minimal change syndrome ?

A

Suggestions of autoimmune disease
Abnormal renal function
Steroid resistance

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19
Q

What would be the treatment of minimal change disease if the child present with typical signs and symptoms ?

A

Prednisalone for 8 weeks

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20
Q

What are some of the symptoms to glucocorticoid treatment ?

A
GI side effects
Glucose intolerance 
Hypertension
Risk of adrenal crisis
Growth issues
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21
Q

What is the pathological process of nephrotic syndrome ?

A

Interaction between lymphocytes and podocytes - The glomerular filtration barrier changes size (shrinks) .

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22
Q

What is the second line treatment for nephrotic syndrome ?

A

Immunosupression

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23
Q

What is a form of acquired steroid resistant nephrotic syndrome ?

A

Focal segmental Glomerulosclerosis (FSGS)

Podocyte loss
Progressive inflammation and sclerosis

24
Q

What is a forms/s of congenital steroid resistant nephrotic syndrome ?

A

Presents <3 months of age
Mutations in nephrin and podocin genes
i.e. HPHS 1 and 2

25
Q

What are the types of idiopathic nephrotic syndromes ?

A

Minimal change
FSGS
MPGN

26
Q

What are the types of acquired nephrotic syndromes ?

A

HSP/IgA
Lupus
Post infectious

27
Q

What are the clinical signs of glomerulonephritis ?

A
Haematuria and proteinuria
Reduced GFR
Oliguria
Fluid overload (Raised JVP and oedema)
Hypertension
28
Q

What are the causes of GN ?

A
Post infectious GN
HSP/IgA nephropathy
Membraniproliferative GN
Lupus nephritis
ANCA +ve vasculitis
29
Q

What are the causes of Acute Post Infectious Golmerulonephritis ?

A

Group A Strep (Throat)

Beta hemolytic

30
Q

How do you diagnose acute post-infectious glomerulonephritis ?

A

Bacterial culture
Positive ASOT
Low C3 normaises

31
Q

What is the treatment for acute post-infectious glomerulonephritis ?

A

Antibiotic
Support renal function
Overload/hypertension

32
Q

What is the most common glomerulonephritis ?

A

IgA Nephropathhy

33
Q

What are the clinical signs and symptoms of IgA nephropathy ?

A
Usually occurs 1-2 days after URTI
Usually in older children and adults
Recurrent macroscopic haematuria
Chronic microscopic haematuria
Varying degree of proteinuria
34
Q

What is the pathogenesis/steps of IgA nephropathy ?

A
  1. Increasing IgA in circulation
  2. Production of Anti-IgA antibodies
    3a. Immune complexes form in the circulation.
    3b. Immune complexes form in situ
  3. Immune complexesin the mesangium cause local activation and injury
35
Q

How do you confirm the diagnosis of IgA nephropathy ?

A

Confirmation via biopsy

36
Q

What is the treatment for IgA nephropathy ?

A

Mild disease - Proteinuria with ACEi

Moderate/severe disease - Immunosuppression

37
Q

What is the clinical picture for a child with IgA related vasculitis ?

A
Age of onset 5-15
Mandatory palpable purpura and one of the following 4:
1. Abdominal pain
2. Renal involvement 
3. Arthritis or arthralgia
4. Biopsy (IgA deposition)
38
Q

What is HSP ?

A

IgA vasculitis with Nephritis

39
Q

What is the treatment for IgA Vasculitis ?

A

Symptomatic (Joints and gut)
Glucocorticoid therapy
Immunosuppression
Hypertension and proteinuria screening

40
Q

Define acute kidney injury (AKI)

A

abrupt loss of kidney function, resulting in the retention of urea and other nitrogenous waste products and in the dysregulation of extracellular volume and electrolytes

41
Q

What are the 3 cardinal symptoms of an AKI ?

A

Anuria/oliguria
Hypertension with fluid overload
Rapid rise in plasma creatinine

42
Q

What is the management of AKI ?

A

3 M’s

Monitor (Urine output)
Maintain (good hydration)
Minimise (Drugs)

43
Q

What are the causes of AKI ?

A

Pre-renal

Perfusion problem

44
Q

What are some of the intrinsic renal problems which cause AKI ?

A
Glomerular disease (HUS and GN)
Tubular injury (Acute tubular necrosis)
Interstitial nephritis (NSAID or autoimmune)
45
Q

What is a post-renal cause of an AKI ?

A

Obstructive

46
Q

When is Haemolytic-Uraemic Syndrome (HUS) typical ?

A

Post-diarrhoea (Caused by E.coli)

47
Q

What are the presenting symptoms of HUS ?

A
Bloody diarrhoea
Triad of:
1. Microangiopathic 
 haemolytic anaemia
2. Thrombocytopenia
3. Acute renal faulire/AKI
48
Q

What is the treatment of HUS ?

A
3 M's:
1. Monitor (5 kidney functions)
Fluid balance
Electrolytes
Acidosis
Hypertension
2. Maintain
IV Normal saline and fluid
Renal relacement therapy
3. Minimise
No antibiotics
49
Q

What are the long-term consequences of AKI ?

A

Blood pressure
Proteinuria monitoring
Evolution to CKD

50
Q

What are the congenital causes of CKD ?

A

Congenital Anomalies of the Kidney and Urinary Tract (CAKUT)

Reflux nephropathy
Dysplasia

51
Q

What kinds of investigations are appropriate in analyzing the kidneys ?

A

USS
DMSA (Isotope scan)
MAG 3 Scan

52
Q

What is the treatment for lower tract infection of the urinary tract?

A

3 days PO antibiotics

53
Q

What is the treatment for upper tract infections/pyelonephritis ?

A

Antibiotics for 7-10 days

54
Q

How do we prevent urinary infections in children ?

A

Fluids
Hygiene
Relive constipation
Voiding dysfunctin

55
Q

What is the management of CKD ?

A
Waste handling
Water handling
Salt balance
Acid base control
Endocrine