Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Paediatrics RACP Exam Study > Paediatric Renal 1 > Flashcards

Paediatric Renal 1 Flashcards

1
Q

When do nephrons finish growing?

A
  • Formation of nephrons 36-40 weeks gestation
  • GFR per 1.73m^2 is improving until age 2/3rd year
  • Functional maturation with tubular growth and elongation 1st 10 years
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What mutation is implicated in Alport Syndrome?

A
  • Mutations in Type IV collagen

- Lots of different genes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the causes of acute nephritis in childhood?

A
  • Post-infectious
  • Vasculitis (HSP), rarely SLE, Wegener granulomatosis, microscopic polyarteritis, polyarteritis nodosa
  • IgA nephropathy and mesangiocapillary glomerulonephritis
  • Anti-GBM disease (Goodpasture syndrome) – very rare
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the expected time course to resolution of the signs / findings of acute glomerulonephritis?

A
  • gross haematuria 2-4 weeks
  • hypertension 4-6 weeks
  • low C3 8-12 weeks
  • proteinuria 6/12 (may be intermittent for up to 1 year)
  • microscopic haematuria up to 2 years
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What infectious diseases are associated with secondary membranous nephropathy?

A
  • Congenital syphilis
  • Chronic hepatitis B
  • Malaria
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What renal disease is most likely to present with renal vein thrombosis?

A
  • nephrotic syndrome - specifically membranous nephropathy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Anion gap formula:

A

Na - (bicarb + chloride)

Normal is 8-12

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the increased anion gap causes of metabolic acidosis?

A 
M — Methanol
U — Uremia 
D — Diabetic ketoacidosis
P — Paracetamol, Propylene glycol, Paraldehyde
I — Infection, Iron, Isoniazid (lactic acidosis in overdose), Inborn errors of metabolism
L — Lactic acidosis
E — Ethylene glycol, ethanol 
S — Salicylates
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the normal anion gap causes of metabolic acidosis?

A

Addisons (adrenal insufficiency)
Bicarbonate loss (GI or Renal)
Chloride excess
Diuretics (Acetazolamide)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the aetiology of Goodpasture disease?

A

Pulmonary haemorrhage and glomerulonephritis due to antibodies to certain epitopes of Type IV collagen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What are the most common causes of HUS?

A
  • Western countries - verotoxin or Shiga-like toxin producing E. coli (STEC), 0157:H7 in Europe/Americas
  • Asian subcontinent/southern Africa- toxin of Shigella dysenteriae type 1
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are non-gut bug infectious causes of HUS?

A
  • Neuraminidase (Strep pneumoniae)
  • HIV
  • Apparently diptheria can
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Chemotherapy causes of renal Fanconi syndrome?

A
  • Cisplatin

- Ifosfamide

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Why can Cr be low (overestimate GFR)?

A

Decreased muscle mass = decreased Cr

Increased tubular secretion in CKD, nephrotic syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How much does tubular secretion contribute to renal Cr clearance?

A

~10-15% (normal circumstances - in CKD etc. may well be higher percentage affecting estimated GFR)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Cystatin C can be influenced by what factors?

A

Steroid use and thyroid function

17
Q

What is the name of the lethal ciliopathy with

  • occipital encephalocele
  • enlarged cystic kidneys
  • postaxial polydactyly
A

Meckel-Gruber syndrome

18
Q

A child presents with kidney stones, low K and Cl, metabolic alkalosis, FTT with tubular dysfunction, What syndrome is most likely?

A
  • Bartter - similar to having frusemide!!
  • Na-K-Cl cotransporter in loop of Henle
  • Urinary calcium excretion is normal-high since calcium reabsorption in the thick ascending limb requires normal sodium chloride reabsorption
19
Q

What are the genes encoding nephrin and podocin?

A

NPHS1 is nephrin

NPHS2 is podocin

20
Q

Which gene is most implicated in congenital nephrotic syndrome?

A 
NPHS1 nephrin (40-80%) 
NPHS2 podocin is as well, but is more likely to be steroid resistant childhood nephrotic syndrome.
21
Q

How do you treat rickets in primary RTA?

A
  • Bicarbonate first

- Then phosphate and vitamin D

22
Q

Renin release is stimulated by…

A

Low sodium - macula densa detects low sodium with hypovolemia.

23
Q

Tacrolimus side effects include…

A 
Everything has GI side effects
Important: 
High BSL, diabetes
Nephrotoxicity with hyperkalemia
Neurotoxicity with parasthesia, 
LESS likely to cause high cholesterol
24
Q

Excretion of excess sodium when the dietary intake of salt is high is primarily driven by:

A

ANP/ANF

25
Q

Which RTA gives you HYPERkalemia?

A

RTA 4

26
Q

Which syndromes are associated with Wilms tumour?

A
  • WAGR (Wilms tumour, Aniridia, GU malformation, retardation)
  • Denys-Drash syndrome
  • Beckwith-Wiederman / hemihypertrophy
  • Frasier syndrome (kidneys and genitalia)
  • Simpson-Golabi-Behmel syndrome (overgrowth)
27
Q

How much should your BP dip overnight?

A

10%

28
Q

What is the most common inherited cause of Fanconi syndrome?

A

Cystinosis

29
Q

What is the typical phenotype of Fanconi?

A
  • Fanconi is RTA 2
  • Growth failure, <1yo
  • Polyuria/polydipsia
  • Rickets
  • Bloods show hyperchloraemic metabolic acidosis with hypokalemia and hypophosphataemia
  • urinary acidification with glycosuria, aminoaciduria, hyperphosphaturia
30
Q

A fair-skinned 18 month old presents with fevers, polyuria, growth failure since 6/12, and not walking. She has been previously treated for constipation which was thought to have caused her to vomit. On further questioning, Mum says she seems unable to see very well and does not like going outside to play. You think she has…

A

Cystinosis - build up of cystine which can’t get out of the lysosomes. Brain, eyes, liver and kidney!

If younger BOY, he could have Lowe syndrome which is congenital cataracts, mental retardation/behavioural issues, and Fanconi syndrome.

31
Q

Which gene is associated with Lowe syndrome?

A

OCRL1 gene, which encodes the phosphatidylinositol polyphosphate 5-phosphatase protein.
X-linked recessive

32
Q

A 3 week old baby boy is noted to have cataracts, has only just regained birth weight, is floppy and seems to pass a lot of urine. He has heavy proteinuria and mild glycosuria on dipstick. What renal associated syndrome could he have?

A

Lowe syndrome - cataracts, progressive growth failure, hypotonia, and Fanconi syndrome with significant proteinuria

  • Blindness and renal insufficiency
  • Characteristic behavioral abnormalities are also seen, including tantrums, stubbornness, stereotypy (repetitive behaviors), and obsessions.
  • Kidneys show nonspecific tubulointerstitial changes.
  • Thickened GBM and change in proximal tubule mitochondria also seen.
33
Q

What is the difference between RTA 1 and 2?

A
  • RTA 1 = distal, can’t get rid of acid
  • RTA 2 = proximal, can’t keep bicarbonate
  • Kidney stones and calcium in urine with distal RTA
34
Q

What do you see in bloods and urine with Renal Fanconi?

A
  • RTA2 - proximal
  • Bloods show normal anion gap, hyperchloraemic metabolic acidosis with hypokalemia and hypophosphataemia
  • urinary acidification with glycosuria, aminoaciduria, hyperphosphaturia
35
Q

What do you see in bloods and urine with RTA 1?

A
  • RTA1 - distal
  • Bloods show normal anion gap metabolic acidosis, varying electrolytes but usually normal
  • Calciuria, pH>5.5, stones

NB severe recessive form gives hyperchloremic metabolic acidosis (sometimes bicarb<10), hypokalemia, stones

36
Q

What is MOSTLY re absorbed in the proximal tubule?

A
  • glucose and AA
  • phosphate
  • citrate
  • 2/3 salt and H20
37
Q

What does the distal tubule re absorb?

A
  • calcium
  • bicarbonate
  • some salt and water (ADH receptor 2 lives here)

Paediatrics RACP Exam Study (30 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions