Nephrology

Question 1

what is the GFR of a neonate and at what age does it mature to its full function?

Answer 1

20-30

reaches adult function of >90 by 2 years old

Question 2

what are the 5 main kidney functions?

Answer 2

waste handling 
water handling 
salt balance 
acid base control
endocrine - red cells, blood pressure, bone health

Question 3

how does glomerulopathy present?

Answer 3

proteinuria

haematuria

Question 4

does nephrotic or nephritic syndrome cause intravascular overload?

Answer 4

nephritic = intravascular overload 
nephrotic = intravascular depletion

Question 5

what component of the glomerular filtration barrier is affected in minimal change disease?

Answer 5

epithelial cell (podocyte)

Question 6

what component of the glomerular filtration barrier is affected in post-infective glomerulonephritis

Answer 6

basement membrane & endothelial cell

Question 7

what component of the glomerular filtration barrier is affected in haemolytic uraemia syndrome?

Answer 7

endothelial cell

Question 8

what component of the glomerular filtration barrier is affected in IgA nephropathy?

Answer 8

mesangial cell

Question 9

how would you test for proteinuria?

Answer 9

dipsix
protein creatinine ratio
24 hour urine collection

Question 10

what is the normal protein creatinine ratio and what is the nephrotic range?

Answer 10

< 20mg/mmol

>250mg/mmol

Question 11

what is the typical presentation of a child with nephrotic syndrome?

Answer 11

swollen face in the mornings
periorbital and eyelid swelling in the morning 
pitting oedema 
ascites 
pale, dehydrated, low BP

Question 12

what are the clinical signs of nephrotic syndrome in a child?

Answer 12

frothy urine

ascites, small pleural effusion, pitting oedema

Question 13

a patient presents with signs of nephrotic syndrome. what investigations would you carry out?

Answer 13

urine dipstick
protein creatinine ration
urine Na (low)

blood;

• Albumin (low)
• Creatinine (normal)

Question 14

when would you consider a renal biopsy in a child who presented with nephrotic or nephritic syndrome?

Answer 14

steroid resistant

Question 15

what is the treatment for nephrotic syndrome in a child?

Answer 15

prednisolone for 8 weeks

Question 16

what are the side effects from high dose steroids?

Answer 16

behaviour change 
sleep disturbance 
increased infection risk 
hypertension 
cushings

Question 17

after how long of no response are they considered steroid resistant?

Answer 17

> 4 weeks

Question 18

what is responsible for steroid resistance in nephrotic syndrome?

Answer 18

interaction between lymphocytes and podocytes

Question 19

what is the 1st and 2nd line treatment for nephrotic syndrome in children?

Answer 19

1st line - prednisolone

2nd line - immunosuppression

Question 20

when would you change treat with immunosuuppresion in nephritic syndrome?

Answer 20

steroid dependant and frequent relapses (>4/year)

Question 21

name an acquired cause of steroid resistant nephrotic syndrome?

Answer 21

focl segmental glomerulosclerosis (FSGS)

Question 22

what is the treatment for focal segmental glomerulonephritis (FSGF)?

Answer 22

immunosuppression i.e. cyclophosphamide

Question 23

how would you differentiate haemaglobinuria from microscopic haematuria?

Answer 23

haemaglobinuria is dipstick positive but microscopy negative

Question 24

what are causes of haematuria?

Answer 24

systemic - clotting factors 
renal - glomerulonephritis 
tumour - wilm's nephroblastoma 
kidney cysts 
malignancies i.e. bladder cancer 
bladder/kidney stones 
UTI 
trauma 
urethritis

Question 25

what are the features of nephritic syndrome?

Answer 25

``` haematuria and proteinuria hypertension reduced GFR - oliguria - fluid overload (raised JVP, oedema) - hypertension - AKI ```

Question 26

what are the features of nephrotic syndrome?

Answer 26

hypoalbuminaemia hypoproteinuria oedema

Question 27

is intravascular overload associated with nephrotic or nephritic syndrome?

Answer 27

nephritic syndrome

Question 28

a patient presents with haematuria. he was found to have nephritic syndrome. what immunological tests will you carry out to approach a diagnosis?

Answer 28

renal USS throat swab and ASOT (anti-streptolysin O test) ANA, ANCA complement C3 and C4

Question 29

a patient presents with haematuria. he was found to have nephritic syndrome. what tests would you carry out to approach a diagnosis?

Answer 29

``` FBC U&E creatinine albumin urine culture (exclude UTI) protein creatinine ratio (exclude nephrotic syndrome) ```

Question 30

what immunological test is diagnostic for post-streptococcal glomerulonephritis?

Answer 30

ASOT | anti-streptolysin O test

Question 31

what change in complement C3 and C4 is found in post-streptococcal glomerulonephritis ?

Answer 31

low C3 | normal C4

Question 32

what is the treatment for post- infectious glomerulonephritis?

Answer 32

antibiotics support electrolyte and/or acid base imbalance correct overload/hypertension with diuretics

Question 33

what is the cause of IgA related vasculitis?

Answer 33

non-streptococcal post infectious glomerulonephritis | - any virus can cause this

Question 34

what are the diagnostic features of IgA related vasculitis?

Answer 34

``` palpable purpura + 1 of the following - abdominal pain - renal involvement - arthritis or arthralgia - biopsy (IgA deposition) ```

Question 35

is IgA vasculitis granulomatous or non-granulomatous inflammation?

Answer 35

non-granulomatous

Question 36

what is the treatment for IgA vasculitis?

Answer 36

treat symptomatically for mild disease severe disease - immunosuppression with prednisolone or cyclophosphamide

Question 37

what are the clinical features of IgA nephropathy?

Answer 37

recurrent macroscopic haematuria +/- chronic microscopic haematuria varying degree of proteinuria negative autoimmune workup normal compliment levels confirmation biopsy if unsure

Question 38

what is the treatment for mild and severe IgA nephropathy?

Answer 38

mild - ACE inhibitors (for proteinuria) | moderate/severe - immunosuppression

Question 39

mutations in which genes are responsible for acquired FSGS?

Answer 39

NPHS1 - nephrin | NPHS2 - podocin