RENAL

Question 1

Which kidney is higher than the other?

Answer 1

The left is higher than right

Question 2

Which kidney is biopsied?

Answer 2

generally lower pole of right kidney

Question 3

What is the function of the thick ascending loop of henle?

Answer 3

Sodium, potassium, chloride transport

Question 4

What is the function of the proximal tubules?

Answer 4

Glucose transport
Phosphate transport
Amino acid transport

Question 5

What is the function of the collecting ducts?

Answer 5

Water transport
Sodium, potassium transport

Question 6

What is the function of the distal tubules?

Answer 6

Proton (H+) secretion
Sodium chloride transport

Question 7

What are the disorders affecting the ascending limb of henle?

Answer 7

Bartter syndrome

Question 8

What are the disorders affecting collecting ducts?

Answer 8

Nephrogenic diabetes insipidus
Pseudohypoaldosteronism
Liddle syndrome

Question 9

What are the disorders affecting the proximal convoluted tubules?

Answer 9

Renal glycosuria
Hypophosphataemic rickets
Isolated, generalized aminoaciduria
the last two combined make fanconi syndrome

Question 10

What are the disorders affecting the distal tubules?

Answer 10

Distal renal tubular acidosis
Gitelman syndrome

Question 11

Describe the clinical features of fanconi syndrome + what fanconi syndrome is

Answer 11

Genralized proximal tubular disorder with initally well preserved glomerular function.
Growth faltering, polyuria and rickets in association with a normal plasma anion gap, metabolic acidosis, hypophosphataemia, hypokalaemia and generalized aminoaciduria.

Question 12

What is the commonest cause of fanconi syndrome in Europe?

Answer 12

Nephropathic cystinosis
Disorder of lysosomal cystine transport of , resulting in excessive intracellular accumulation of free cystine in many organs including the kidney, eyes and thyroids.

Question 13

What is inheritance of nephropathic cystinosis?

Answer 13

AR

Question 14

What is the treatment for nephropathic cystinosis?

Answer 14

Mercaptamine, which prevents accumulation of lysosomal cystine.

Question 15

hypokalaemic, hypochloraemic metabolic alkalosis with salt wasting + hypocalcurea + hypomagnesaemia

Answer 15

Gitelman Syndrome

Question 16

hypokalaemic, hypochloraemic metabolic alkalosis with salt wasting + hypercalcurea + normal Mg2+

Answer 16

Bartter Syndrome

Question 17

Damage to what mutation and where is difference between Gitelman and Bartter syndrome?

Answer 17

Bartter- Ascending loop of henle furosemide-sensitive sodium–potassium–chloride channel (NKCC2)
Gitelman- distal convoluted tubule thiazide-sensitive sodium–chloride channel (NCCT)

Question 18

What is classical clinical presentaation of Bartter vs Gitelman?

Answer 18

Barter - early childhood, faltering growth, poor feeding, dehydration + lethargy
Gitelman teenager, muscle weakness and cramps, and short stature

Question 19

RTA type 1 can be characterised as what?
What are lab findings?

Answer 19

failure to secrete H+ ions in distal convoluted tubule
metabolic acidosis, hyPOkalaemia urine pH>5.5

Question 20

RTA type 2 can be characterised as what? What are lab findings?

Answer 20

Bicarbonate wasting in proximal convoluted tubule
metabolic acidosis hyPOkalaemia, urine pH<5.5 + Urine has rasied bicarb, glucose and amino acids (Like fanconi!)

Question 21

RTA type 4 can be characterised as what?
What are lab findings?

Answer 21

Impairment in formation of ammonia
metabolic acidosis, hyPERkalaemia

Question 22

How does RTA type 1 typically present?

Answer 22

renal colic, renal stones, faltering growth

Question 23

How does RTA type 2 typically present?

Answer 23

Faltering growth and rickets
Fanconi syndrome

Question 24

What is the commonest abnormality of renal tract?

Answer 24

Hydro­nephrosis secondary to pelvi-ureteric junction obstruction (PUJO)

Question 25

Define nephrotic syndrome

Answer 25

proteinuria, oedema and hypoalbuminaemia together with hyperlipidaemia.

Question 26

What is nephrotic syndrome most commonly secondary to?

Answer 26

minimal change disease

Question 27

What is the percentage of patients whose nephrotic syndrome relapses?

Answer 27

70%

Question 28

define nephrotic syndrome relapse

Answer 28

Urine albumin 3+ or 4+ (or proteinuria >4 mg/m2/h) in three consecutive early morning specimens having been in remission previously

Question 29

Define nephrotic syndrome remission

Answer 29

Urine albumin nil or trace (or proteinuria <4 mg/m2/hour) in three consecutive early morning urine specimens

Question 30

Define frequent relapse in relation to nephrotic syndrome

Answer 30

Two or more relapses in initial six months or more than three relapses in any twelve months

Question 31

Define steroid dependence in relation to nephrotic syndrome

Answer 31

Two consecutive relapses when on alternate day steroids or within 14 days of its discontinuation

Question 32

Define steroid resistance in relation to nephrotic syndrome

Answer 32

Absence of remission despite therapy with daily prednisolone at a dose of 2 mg/kg/day for 4 weeks

Question 33

Describe minal change disease

Answer 33

Fuckery of the podocyte on the parietal epithelial cells of the basement membrane

Question 34

What is the treatment for nephrotic syndrome?

Answer 34

prednisolone (60 mg/m2/day for four weeks followed by 40 mg/m2/day) for at least another four weeks. Penicillin prophylaxis

Question 35

What are the chickenpox rules for nephrotic syndrome?

Answer 35

should receive varicella zoster immunoglobulin within 96 hours of exposure to chickenpox. If a child with nephrotic syndrome develops chickenpox, they should receive treatment with intravenous aciclovir.

Question 36

What are the types of immune-complex mediated glomerulonephritis?

Answer 36

Post-infectious, usually post streptococcal Other post-infectious causes – bacterial, viruses, rickettsiae, fungal, parasites Henoch–Schönlein purpura (HSP)/IgA vasculitis Systemic lupus erythematosus (SLE) IgA nephropathy (IgAN) Membranoproliferative glomerulonephritis (MPGN)/C3 glomerulopathy

Question 37

What type of glomerulonephritis is commonest? And what is it associated with?

Answer 37

post group A haemolytic strep Low C3

Question 38

How does IgA nephritis normally present?

Answer 38

Macroscopic haematuria following URTI/ upper GI illness. Occurs 1-2 days after rather than a week seen in post-strep

Question 39

subepithelial immune deposits or humps are seen in what?

Answer 39

post-strep glomerulonephritis

Question 40

mesangial deposits are a feature of what ?

Answer 40

IgA nephritis

Question 41

Subendothelial depostis are a feature of what?

Answer 41

Membranoproliferative glomerulonephritis AND in class III +IV lupus

Question 42

Diffuse thinning and splitting of glomerular basement membrane/ lamina densa is a feature of what?

Answer 42

Alport syndrome

Question 43

What are the features of Alport syndrome

Answer 43

haematuria 1-2 days post URTI + bilateral sensorinueral hearing loss and anterior lenticonus