Renal Flashcards

1
Q

What does a DMSA scan detect?

A

Static scan of the renal cortex

Detects functional defects e.g. scars

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2
Q

What do you need to remember when ordering a DMSA post UTI?

A

○ Very sensitive so need to wait at least 2 months after a UTI to avoid diagnosing false ‘scars’

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3
Q

What happens in an MCUG?

A

Contrast introduced into bladder through urethral catheter

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4
Q

What can an MCUG tell you?

A

Can visualise bladder and urethral anatomy

Detects vesicoureteric reflux and urethral obstruction

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5
Q

What’s the downside of an MCUG?

A

High radiation dose

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6
Q

What happens in a MAG3?

A

○ Dynamic scan

MAG3 (isotope labelled substance) is excreted from the blood into the urine

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7
Q

What can a MAG3 tell you?

A

Measures drainage, best performed with high urine flow

In children old enough to cooperate (>4 years), scan during micturition is used to identify VUR

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8
Q

What is a duplex kidney?

A

Two ureters drain from two separate pelvicalyceal systems

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9
Q

What are the common complications of a duplex kidney?

A

Obstructed hydronephrotic upper moiety and ureter, associated with bladder ureterocele
Ectopically inserted upper pole ureter entering urethra or vaginal, causing true continual incontinence
VUR into lower pole ureter, sometimes causing infection and scarring

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10
Q

What is a horseshoe kidney?

A

Two renal segments fused across midline, usually at lower poles

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11
Q

What is horseshoe kidney associated with?

A

Turner and Laurence-Moon-Biedl syndromes

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12
Q

What are the clinical features of horseshoe kidney?

A

Usually asymptomatic but increased incidence of PUJ obstruction and VUR so can develop UTI

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13
Q

What is the main defect in Potter syndrome?

A

Bilateral renal agenesis or bilateral multicystic dysplastic kidneys
Reduced fetal urine excretion leads to oligohydramnios, which causes fetal compression

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14
Q

What are the classical facies of Potter syndrome?

A

Low set ears
Beaked nose
Prominent epicanthic folds
Downward slant to eyes

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15
Q

What are the non-renal features of Potter syndrome?

A

○ Pulmonary hypoplasia causing respiratory failure

Limb deformities e.g. severe talipes

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16
Q

What’s the prognosis for Potter syndrome?

A

Fatal if not stillborn

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17
Q

What causes multi cystic dysplastic kidney disease?

A

failure of union of the ureteric bud with the nephrogenic mesenchyme

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18
Q

What is the resulting kidney like in multi cystic dysplastic kidney?

A

Non-functioning structure with large fluid-filled cysts with no renal tissue and no connection with the bladder

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19
Q

How is multi cystic dysplastic kidney managed?

A

Only needs nephrectomy if it remains very large or if hypertension develops

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20
Q

What are the features of AR polycystic kidney disease?

A

Diffuse bilateral enlargement of both kidneys

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21
Q

What are the features of AD polycystic kidney disease?

A

Separate cysts of varying size between normal renal parenchyma
Kidneys are enlarged
Causes hypertension and haematuria in childhood, and renal failure in late adulthood
Associated with cerebral aneurysms and subarachnoid haemorrhage

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22
Q

What are the cardinal features of Alport syndrome?

A

Hereditary nephritis with sensorineural deafness and anterior lenticonus (conical deformity of lens of eye seen with slit lamp)

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23
Q

What is the main defect in Alport syndrome?

A

Type IV collagen

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24
Q

How is Alport syndrome inherited?

A

X linked or AR

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25
Q

How does Alport syndrome present and progress?

A
Incidental microscopic haematuria or episode of macroscopic haematuria
Deafness around 10 years
Hypertension in mid teens
Eye signs in mid-late teens
Average age for ESRF is 21 years
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26
Q

What are the features of testicular torsion?

A
Acute abrupt onset of often severe pain
Early puberty
Swelling of testis and hemiscrotum often with erythema/discolouration
Negative urinaylsis
Needs urgent surgery
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27
Q

What are the features of torsion of the appendix of the epididymus?

A
More common than testicular tosion
Subacute onset of pain over hours
Pre pubertal
Localised pain at upper pole of testis
Can be managed conservatively but difficult to distinguish from torsion of testicle
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28
Q

What are the features of epididymitis?

A

Gradual insidious onset of discomfort or pain
Adolescence
Epididymal tenderness
Urinalysis often positive

29
Q

What is the defect in cystinuria?

A

Defect in reabsorption of, and hence excessive excretion of, cystine, ornithine, arginine and lysine

30
Q

How is cystinura inherited?

A

AR

31
Q

What is the consequence of cystinuria?

A

Recurrent urinary stone formation which are extremely hard and densely radio opaque

32
Q

How is cystinuria treated?

A

high fluid intake and alkalinisation or urine with oral potassium citrate

33
Q

What is the mutation in x linked hypophosphataemic rickets?

A

PEX gene on the X chromosome

34
Q

What is the defect in x linked hypophosphataemic rickets?

A

Isolated defect in phosphate reabsorption

35
Q

What are the clinical features of x-linked hypophosphaaemic rickets?

A

ALP rises before phosphate becomes low

Causes delayed growth, rickets, delayed dentition and dental abscesses

36
Q

How are X linked hypophosphataemic rickets treated?

A

Supplementation

37
Q

What is the defect in proximal RTA?

A

Failure to reabsorb filtered HCO3 but can still acidify urine

38
Q

What are the symptoms in proximal RTA?

A

Faltering growth
Vomiting
Short stature

39
Q

How is proximal RTA treated?

A

High doses of alkali

40
Q

What is the defect in cystinosis?

A

AR defect in transport of cystine out of lysosomes

41
Q

What are the early clinical features of cystinosis?

A

Fanconi syndrome
Photophobia
Hypothyroidism

42
Q

What are the late features of cystinosis?

A
Renal failure at 8-10 years
Pancreatic involvement with DM
Liver involvement with hepatomegaly
Reduced fertility
Neurological deterioration and cerebral atrophy
43
Q

How is cystinosis diagnosed?

A

○ Cystine crystals in cornea on slit lamp
Peripheral blood white cell cystine level
Antenatal diagnosis possible

44
Q

How is cystinosis treated?

A

Supportive - supplements, high fluid intake
Cysteamine, which increases cystine transport out of lysosome
Indometacin reduces GFR and hence helps with severe polyuria and secondary polydipsia, and electrolyte wasting

45
Q

What is glomerulonephritis??

A

Inflammation of the glomeruli leading to:

  • Haematuria and/or proteinuria
  • Nephrotic syndrome
  • Acute nephritic syndrome
  • Rapidly progressive crescentic glomerulonephritis
46
Q

What are the main causes of glomerulonephritis

A

Normal complement:

  • FSGS
  • IgA nephropathy
  • HSP

Reduced complement

  • Acute post-streptococcal
  • Mesangiocapillary GN
  • SLE
  • Shunt nephritis
47
Q

What is the defect in Bartter syndrome?

A

Inborn AR defect in the sodium/potassium/chloride co transporter in the thick ascending limb, leading to sodium chloride and water wasting

48
Q

What are the symptoms of Bartter syndrome?

A
Polyuria
Polydipsia
Episodes of dehydration
Faltering growth
Constipation
Maternal polyhydramnios
49
Q

How is Bartter syndrome diagnosed?

A

Hypochloraemic hypokalaemic alkalosis
Inappropriately high levels of urinary chloride and sodium
Normal or high urinary calcium

50
Q

How is Bartter syndrome treated?

A

Potassium supplementation and indometacin

51
Q

What is pseudo-Batter syndrome?

A

Same plasma biochemistry with low chloride and potassium but inappropriately low levels of urine chloride and sodium

52
Q

What are the causes of pseudo Bartter syndrome?

A

Cystic fibrosis
Congenital chloride diarrhoea
Laxative abuse
Cyclical vomiting

53
Q

What is the triad of nephrotic syndrome?

A

Oedema
Proteinuria
Hypoalbuminaemia

54
Q

What are the causes of nephrotic syndrome?

A
Minimal change disease
FSGS
Mesangial proliferative glomerulonephritis
Membranous nephropathy
SLE
55
Q

What are the possible complications of nephrotic syndrome?

A

Infections with encapsulated bacteria
Thrombosis
Hypovolaemia
Drug toxicity

56
Q

How is nephrotic syndrome managed?

A
Steroids for initial presentation
Steroids for first relapse
For frequent relapses
-Cyclophosphamide
-Tacrolimus
-Cyclosporin
-Mycophenolate mofetil
-Levamisole
Refer to renal service if steroid resistant
57
Q

What is the defect in Gitelman syndrome?

A

Inborn AR defect in the distal tubule sodium and chloride cotransporter

58
Q

What are the symptoms of Gitelman syndrome?

A

Often asymptomatic

Or, transient episodes of tetany and weakness with abdominal pain and vomiting

59
Q

What are the biochemical features of Gitelman syndrome?

A

Hypokalaemic metabolic alkalosis
Raised renin and aldosterone
Hypomaynesaemia
Hypocalciuria (distinguishes from Bartter, where there is normal or high urinary calcium)

60
Q

Where are the ureters placed in VUR?

A

Ureters are displaced laterally and enter directly into the bladder rather than at an angle, with a shortened or absent intramural course

61
Q

What defines severe VUR?

A

reflux during bladder filling and voiding with distended ureter, renal pelvis, and clubbed calyces
High risk of renal scarring

62
Q

What are the important consequences of VUR?

A

Urine returning to the bladder from the ureters after voiding results in incomplete bladder emptying, which encourages infection
Kidneys may become infected with intrarenal reflux especially
Bladder voiding pressure is transmitted to the renal papillae, which may contribute to renal damage if voiding pressures are high

63
Q

What is Fanconi syndrome?

A

Generalised proximal tubular dysfunction

64
Q

How does Fanconi syndrome present?

A
Polydipsia and polyuria
Salt depletion and dehydration
Hyperchloraemic metabolic acidosis
Rickets
Failure to thrive/poor growth
65
Q

Which substances are lost in the urine in Fanconi syndrome?

A
Amino acids
Glucose
Phosphate
Bicarbonate
Sodium
Calcium
Potassium
Urate
66
Q

What are the causes of secondary Fanconi syndrome?

A
Cystinosis
Glycogen storage disorders
Lowe syndrome (oculocerebrorenal dystrophy)
Galactosaemia
Fructose intolerance
Tyrosinaemia
Wilson disease
67
Q

What are the acquired causes of Fanconi syndrome?

A

Heavy metals
Drugs and toxins
Vitamin D deficiency

68
Q

What is the raid of haemolytic uraemic syndrome?

A

Acute renal failure
Microangiopathic haemolytic anaemia
Thrombocytopenia