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RACP Paediatrics Exam > Nephrology > Flashcards

Nephrology Flashcards

1
Q

What does the ureteric bud form?

A

Renal pelvis, infundiublae, calyces and collecting ducts

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2
Q

What does the metenephric mesenchyme form?

A

Glomerulus, PCT, DCT, Loop of hence

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3
Q

In a drowning incident with hypoxia which areas of the kidney will be affected the most?

A

Loop of henle, PCT

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4
Q

What conditions can cause neonatal onset of fanconi syndrome?

A

Tyrosinaemia, Galactosemia, Mitochondrial disease

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5
Q

How do you test for cystinosis?

A

Elevated cystine levels in WBC and corneal deposits seen by ophthalmology

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6
Q

Treatment for corneal deposits in Cystinosis?

A

Cysteamine (mercaptamine)

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7
Q

Which part of the nephron does acetazolamide work?

A

PCT

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8
Q

Which part of the nephron does amiloride work?

A

CD

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9
Q

Which part of the nephron does chlorothiazide work?

A

DCT

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10
Q

Which part of the nephron does spironolactone work?

A

CD

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11
Q

What channel does amiloride work on?

A

ENAC on the Collecting duct

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12
Q

What electrolyte disturbance does Gitelman Syndrome cause?

A

Hypokalaemia, hypomagnesaemia, Alkalosis, Hypocalciuria

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13
Q

What electrolyte disturbance does Bartter syndrome cause?

A

Polyuria, hypochloraemia, hypokalaemia, alkalosis, hypercalciuria - nephrocalcinosis

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14
Q

Equation for Fraction of excreted Na+?

A

(Urine Na x Plasma Cr)/(Urine Cr x Plasma Na) x 100
Pre-renal <1%
Renal >2%

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15
Q

What is the gene for genetically x-linked diabetes insipidus?

A

AVPR2 gene coding for the ADH receptor

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16
Q

Treatment of nephrogenic DI?

A

Low solute diet
Water
Diuretics - tricks kidney to reabsorb at the PCT

17
Q

What is Liddle Syndrome?

A

Gain in function of the aldosterone system causing increased Na and excretion of K+ with resulting hypertension.
Treatment low salt diet and amiloride

18
Q

How long can nephritis develop after HSP condition?

A

3 months

19
Q

What does split thickened glomerular basement membrane with basket weave appearance indicate on histology renal biopsy?

A

Alport syndrome

20
Q

What is the gene and inheritance for Alport syndrome?

A

COL4A5 - X linked 80%. Encodes for Type IV collagen which makes up the framework of the basement membrane.
Can be AD and AR - less severe forms

21
Q

What are the extra-renal features of alport syndrome?

A

Anterior lenticonus, hearing loss, macular abnormalities

22
Q

What is linear IgG staining pathognomonic for on renal biopsy?

A

Anti-GDM

23
Q

Definition of nephrotic syndrome?

A

Nephrotic range proteinuria and hypoalbuminaemia or oedema where serum albumin can’t be measured.

24
Q

What is nephrotic range

A

> 40mg/m2/hr (>1000mg/m2/day)
Urine protein to creatinine ratio >200mg/mmol

25
Q

Which autoantibody is associated with idiopathic membranous nephropathy?

A

PLA2R- Targets the M type phospholipase A2 receptor (surface of podocyte cell membranes)

26
Q

What is the gene mutation in Gitelman syndrome?

A

SLA12A3 (NCCT) - Autosomal recessive

27
Q

What test can measure GFR in the hospital?

A

Plasma disappearance of radio-isotope study - Cr-EDTA

28
Q

What is the Schwartz equation for eGFR

A

eGFR = 36.5 x height (cm) = serum creatinine (umol/L)

29
Q

What can overestimate your creatinine clearance?

A

Low muscle mass
Tubular secretion of creatinine (eg.nephrotic syndrome/CKD)

30
Q

What gene and inheritance is related to nephropnopthisis?

A

NPHP - Autosomal recessive
Nephrocystin gene

31
Q

What is the classical presentation of nephropnopthisis?

A

Polyuria
Polydipsia
Enuresis
Bland urine
Disproportionate anaemia for degree of CKD
Short stature
Small kidneys with poor CM differentiation
Extra-renal manifestations - Retinitis pigmentosa, visual and developmental delay, cerebellar abnormalities, obesity, polydactyly

32
Q

What is the most common organism involved in spontaneous peritonitis in a nephrotic patient?

A

Strep. Pneumoniae

33
Q

What electrolyte disturbance can Tacrolimus cause?

A

hypomagnesaemia, hyperkalaemia

34
Q

Best study modality to look for ectopic ureters or duplex collecting system?

A

IVU

35
Q

What is the treatment for children on PD who present with suspected peritonitis?

A

Cefazolin and Ceftazidime (need to cover for gram negative and gram positive organisms until culture returns)
If MRSA known vancomycin and ceftazidime

36
Q

What is the gene mutation for congenital nephrotic syndrome?

A

NPHS1 and NPHS2

RACP Paediatrics Exam (25 decks)

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