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Paeds BPT Written Exam > Renal > Flashcards

Renal Flashcards

1
Q

Hepatic fibrosis occurs universally in which inherited renal disease?

A

Autosomal recessive PKD

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2
Q

Presentation of AR polycystic kidney disease

A
  • Often present at birth with oligohydramnios and Potter facies, or
  • Bilateral palpable enlarged kidneys
  • HTN
  • UTIs
  • Hepatic fibrosis -> oesophageal varices
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3
Q

Presentation of AD polycystic kidney disease

A
  • Often found incidentally on imaging
  • May have HTN and abdominal mass (less common than ARPKD in children - usually presents later in life)
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4
Q

Gene causing AD polycystic kidney disease?

A

PKD1 gene (encoding polycystin 1) on chr 16 or
PKD2 gene (encoding polycystin 2) on chr 4

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5
Q

Gene causing AR polycystic kidney disease?

A

PKHD1 gene

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6
Q

Presentation of nephronopthisis?

A
  • Polyuria
  • Polydipsia
  • Salt-losing nephropathy (without nephritis or nephrotic syndrome)
  • Anaemia and FTT
  • Extra renal manifestations incl: retinitis pigments, cerebellar aplasia with coloboma, liver fibrosis, inability to perform horizontal eye movements
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7
Q

Features of Bardet-Biedyl syndrome?

A

All the things you don’t want…
- obesity
- intellectual disability/GDD
- hypogenitalism
- extra digits (polydactyly)
- cystic dysplasia of the kidneys
- Polyuria, polydipsia and UTIs
- retinitis pigmentosa
- macrocephaly with macrotia

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8
Q

Most severe form of cystic renal disease?

A

Multicystic dysplastic kidney
- non inherited
- usually unilateral, fatal if bilateral
- causes involution of affected kidney and compensatory hypertrophy of other kidney

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9
Q

Embryological development of kidneys in early gestation

A

Pronephros (appears wk 3)
Mesonephros (appears wk 4)
Metanephros (appears wk 5) - when it appears the others regress

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10
Q

What does the metanephric blastema and ureteric bud form?

A

Metanephric blastema -> glomeruli and interstitial cells

Ureteric bud -> collecting ducts, renal pelvis and ureter

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11
Q

What is the triad of features in Prune Belly Syndrome?

A
  1. Abdominal muscle deficiency (“prune belly” appearance)
  2. Undescended testes
  3. Dilation of prostatic urethra, bladder and ureters

Other associations: ASD, TetOF, Imperforate anus, intestinal malrotation, poly/syndactyly

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12
Q

Most likely cause of incontinence if child has continuous voiding (“always wet”)?

A

Ectopic ureter

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13
Q

Which direction do testes usually twist in torsion?

A

Medially (so must be twisted laterally for detorsion)

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14
Q

What is reabsorbed in the proximal convoluted tubule?

A

Glucose (~100%)
Amino acids (~100%)
HCO3 (90%)
Na+ (65-80%)
Cl-
PO4
K+
H2O
Ca2+

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14
Q

Which diuretic acts on the proximal convoluted tubule?

A

Acetazolamide (carbonic anhydrase inhibitor)

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15
Q

What is reabsorbed in the loop of Henle?

A

Na+ (10-20%)
Cl-
H2O
K+
Mg++ and Ca++

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16
Q

Which diuretic acts on the thick ascending loop of Henle?

A

Loop diuretics (block the Na+/K+/2Cl- channel)

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17
Q

What is reabsorbed in the early distal convoluted tubule?

A

Na+ and Cl

(K+ and H+ are excreted)

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18
Q

Which diuretics act on the early distal convoluted tubule?

A

Thiazide diuretics (block Na+/Cl- channel)

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19
Q

What effect do thiazides have on urinary calcium excretion?

A

They reduced urinary calcium excretion (unlike loop diuretics)

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20
Q

Where does ADH act?

A

Principal cells of the collecting duct: insertion of AQ channels on apical side -> H2O reabsorption

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21
Q

What does aldosterone do?

A

In the principal cells of the CD:
- Increases epithelial Na+ channel activity
- increases Na+/K+ pump -> Na+ reabsorption, K+ secretion

In alpha intercalated cells
- Increases H+ secretion and increases HCO3/Cl pump -> HCO3 reabsorption

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22
Q

Which diuretics act on collecting duct?

A

Amiloride
Spironolactone (aldosterone antagonist)

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23
Q

Causes of glomerulonephritis with low complement?

A

PIGN (resolves within 12wks)
Membranoproliferative GN
SLE nephritis
Atypical HUS

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24
Q

Causes of glomerulonephritis with normal complement?

A

RPGN
IgA nephropathy
HSP nephritis
Granulomatosis with polyangiitis and Goodpastures

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25
Q

Best test for detecting recent strep infection?

A

Anti-DNAse B
(more sensitive and specific)

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26
Q

Mutation causing Alports syndrome

A

COL4A gene mutation encoding type 4 collagen
(Mostly X-linked inheritence)

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27
Q

Features of Alports syndrome

A
  1. Sensorineural hearing loss
  2. Renal impairment: haematuria, HTN, progressive renal impairment
  3. Eye: anterior lenticonus, retinal changes
  4. Leiomyomatosis
28
Q

Urine osmolality in pre-renal AKI vs ATN?

A

Pre-renal AKI: >500 (ie concentrated)

ATN <350

29
Q

Urine fractional excretion of Na+ (FeNa) in pre-renal AKI vs ATN?

A

Pre-renal AKI <1%

ATN >1%

30
Q

Poor prognostic factors for Haemolytic Uraemic Syndrome?

A
  • Anuria >2/52
  • Initial neutrophil count >20
  • Coma or CNS involvement
  • Atypical HUS (ie diarrhoea negative)
31
Q

Labs in Haemolytic Uraemic Syndrome

A

Anaemia
Thrombocytopenia
Blood smear: burr cells (echinocytes) - elongated or oval shaped RBCs, helmet shaped schistocytes, other fragmented RBCs
Reticulocytosis
Elevated creat
Urine: blood and protein
Elevated LDH, bilirubin, low haptoglobin
Normal coags (fibrinogen can be normal or increased)

32
Q

3 features of haemolytic uraemia syndrome

A
  1. Microangiopathic haemolytic anaemia
  2. Thrombocytopenia
  3. Renal involvement (haematuria, proteinuria, elevated creatinine)
33
Q

Management of complement mediated HUS?

A

Eculizumab (a mab to complement factor C5) may be used for complement mediated HUS or
STEC HUS with severe CNS involvement

34
Q

Causes of Haemolytic Uraemic Syndrome

A

D+ (90%)
- Shiga toxin E coli
- Shigella

D-
- Pneumococcal
- HIV
- Complement mediated
- Drug related

35
Q

WCC and white cell casts in urine think

A

Acute interstitial nephritis

36
Q

Causes of acute interstitial nephritis

A

Drugs (90%)
- commonly NSAIDs, diuretics and antibiotics (penicillin, cephalosporin)

Infections (Eg Hep B, HIV, CMV)

SLE, sarcoidosis

37
Q

Causes of acute tubular necrosis

A
  1. Kidney ischaemia eg due to trauma, burns, cirrhosis
  2. Sepsis
  3. Nephrotoxins eg aminoglycosides, contrast, amphotericin, cisplatin, vancomycin
38
Q

Urinalysis in ATN

A

Muddy brown granular, epithelial cell casts, and free renal tubular epithelial cells

39
Q

Indications for dialysis in acute setting

A
  • Metabolic/ electrolyte imbalances not amendable to medical therapy eg hyperkalemia
  • symptomatic uraemia (eg bleeding, pericarditis, encephalopathy)
  • severe fluid overload
40
Q

Management of Alport Disease

A
  • ACE/ARB has been shown to slow progression of renal disease
41
Q

What drives ultrafiltration (fluid removal) in peritoneal dialysis?

A

Osmotic gradient from dextrose in dialysate

42
Q

What drives solute removal (clearance) in peritoneal dialysis?

A

Most diffusion down concentration gradient
Some via convection (“solute drag”)

43
Q

Most common organisms causing peritonitis

A

In PD patients- staph epidermidis/ CoNS

In NS - S pneumoniae, then E coli

44
Q

Side effects of tacrolimus?

A

Low mag
Nephrotoxicity!!
Hepatic dysfunction
Glucose intolerance
Tremors
Diarrhoea

45
Q

MOA of tacrolimus

A

Blocks IL-2
Reduces T cell activation and proliferation

46
Q

Side effects of mycophenolate?

A
  • GI upset
  • Leukopenia
  • Thrombocytopenia
  • Anorexia
47
Q

Renal diseases that can recur after transplant and lead to graft loss?

A
  • Membranoproliferative GN
  • FSGS
  • Atypical HUS
48
Q

Most common form of renal stone in children

A

Calcium oxalate > calcium phosphate > struvite or uric acid

49
Q

Inhibitors of renal stone formation

A

Citrate
Magnesium
Pyrophosphate

50
Q

Non-nephrotic glycosuric dwarfing with hypophosphatemic rickets in early childhood

A

Fanconi syndrome

51
Q

Congenital causes of fanconi syndrome in neonate?

A
  • Galactosemia
  • Tyrosinemia type 1
  • Mitochondrial
52
Q

Congenital causes of fanconi syndrome in infants?

A
  • Cystinosis
  • Fructosemia
  • Lowes syndrome (X-linked)
  • Fanconi-Bickel syndrome
53
Q

Congenital causes of fanconi syndrome in children

A
  • Cystinosis
  • Dent’s disease (X-linked)
  • Wilsons disease
54
Q

How to differentiate Bartter from Gitelman syndrome

A
  • Barrters usually presents early / perinatal whereas Gitelmans presents later (age >5)
  • Gitelmans = little mag (hypomagnesemia) *though can get this with B type 4
  • Hypocalciuria is seen in Gitelmans
55
Q

Features of type 1 (distal) RTA?

A

Hyperchloremic normal anion gap metabolic acidosis
Hypokalemia
Hypercalciuria +/- nephrocalcinosis (stones)**
Elevated urine pH*

56
Q

Hypokalemic metabolic alkalosis and hypetension is seen in which tubulopathy?

A

Liddle syndrome

57
Q

What is the cause of type 2 (proximal) RTA?

A

Impaired HCO3- reabsorption in the PCT -> Bicarbonate wasting

58
Q

Features of type 2 (proximal) RTA?

A

Hyperchloremic NAGMA - that doesn’t respond well to bicarb
Low urine pH (<5.5)
Low-normal K+

May be associated with Fanconi syndrome

59
Q

Causes of type 2 (proximal) RTA?

A

Idiopathic
Fanconi syndrome
Cystinosis
Drug incl acetazolamide, 6MP, heavy metal poisoning

60
Q

Features of type 4 RTA?

A

NAGMA
Hyperkalemia ***
hyperchloremia
Urine pH <5.5

61
Q

What is the cause of type 4 RTA?C

A

Aldosterone deficiency or resistance (commonly due to obstructive uropathy or interstitial renal disease)

62
Q

AR PKD is associated with what liver disease?

A

Caroli disease (dilation of biliary tree, can present with recurrent cholangitis)

63
Q

What is ‘pseudohypoaldosteronism’ type 1?

A

A genetic condition in which there is aldosterone RESISTANCE
- aldosterone levels will be high
- hyperkalemia
- normal - low Na+

64
Q

Natural history of HSP

A
  • If renal complications develop, it usually does so in first 6 months (97%)
  • First episode (without renal disease) usually resolves within 4 weeks
  • Recurrence occurs in 25-35% within first 4 months
65
Q

Adverse effects of cyclosporin?

A

Gum hypertrophy
Excess hair growth
HTN
Hepatotoxicity
Paraesthesias

66
Q

Features of Fraser syndrome

A

Cryptopthalmos
Syndactyly
Renal agenesis or hypoplasia
Genital abnormalities eg hypospadias, cryptorchidism

67
Q

What happens if BP cuff too small?

A

Will overestimate systolic BP (and vice versa)

Bladder width should be approx 40% of arm circumference

68
Q
A

Paeds BPT Written Exam (18 decks)

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