Correct

1- A 25-year-old man has a renal biopsy due to worsening renal function. This
reveals linear IgG deposits along the basement membrane. What is the most
likely diagnosis?ia

A.A Systemic lupus erythematousia

B.A IgA nephropathyia
C.A Minimal change diseaseia
D.A Post-streptococcal glomerulonephritisia
E.A Goodpasture's syndromeia

(e)
These changes are characteristic of Goodpasture's syndrome

Goodpasture's syndrome
sqweqwesf erwrewfsdfs adasd dhe
Goodpasture's syndrome is rare condition associated with both pulmonary
haemorrhage and rapidly progressive glomerulonephritis. It is caused by anti-
glomerular basement membrane (anti-GBM) antibodies against type IV
collagen. Goodpasture's syndrome is more common in men (sex ratio 2:1) and
has a bimodal age distribution (peaks in 20-30 and 60-70 age bracket). It is
associated with HLA DR2
he earaer aeraer asdsadas eerw dssdfsselleds
Features

 pulmonary haemorrhage he
 followed by rapidly progressive glomerulonephritishe

he earaer aeraer asdsadas eerw dssdfsselleds

Factors which increase likelihood of pulmonary haemorrhage
 young maleshe
 smokinghe
 lower respiratory tract infectionhe
 pulmonary oedemahe
 inhalation of hydrocarbonshe

he earaer aeraer asdsadas eerw dssdfsselleds

Investigations
 renal biopsy: linear IgG deposits along basement membranehe
 raised transfer factor secondary to pulmonary haemorrhageshe

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 plasma exchangehe
 steroidshe
 cyclophosphamidehe

Correct

1
2- Which one of the following is the most common cause of nephrotic syndrome
in children?ia

A.A Minimal change diseaseia

B.A IgA nephropathyia

C.A Focal segmental glomerulosclerosisia
D.A Chronic pyelonephritisia
E.A Infantile microcystic diseaseia
(a)
Minimal change glomerulonephritis nearly always presents as nephrotic
syndrome, accounting for 75% of cases in children and 25% in adults. The
majority of cases are idiopathic and respond well to steroids

Glomerulonephritides
sqweqwesf erwrewfsdfs adasd dhe
Knowing a few key facts is the best way to approach the difficult subject of
glomerulonephritis:
he earaer aeraer asdsadas eerw dssdfsselleds
Membranous glomerulonephritis

 presentation: proteinuria / nephrotic syndrome / CRFhe

 cause: infections, rheumatoid drugs, malignancyhe
 1/3 resolve, 1/3 respond to cytotoxics, 1/3 develop CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

IgA nephropathy - aka Berger's disease, mesangioproliferative GN
 typically young adult with haematuria following an URTIhe

he earaer aeraer asdsadas eerw dssdfsselleds

Diffuse proliferative glomerulonephritis
 classical post-streptococcal glomerulonephritis in childhe
 presents as nephritic syndrome / ARFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Minimal change disease
 typically a child with nephrotic syndrome (accounts for 80%)he
 causes: Hodgkin's, NSAIDshe
 good response to steroidshe

he earaer aeraer asdsadas eerw dssdfsselleds

Focal segmental glomerulosclerosis
 may be idiopathic or secondary to HIV, heroinhe
 presentation: proteinuria / nephrotic syndrome / CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Rapidly progressive glomerulonephritis - aka crescentic glomerulonephritis
 rapid onset, often presenting as ARFhe
 causes include Goodpasture's, ANCA positive vasculitis, SLEhe

he earaer aeraer asdsadas eerw dssdfsselleds

Mesangiocapillary glomerulonephritis (membranoproliferative)

2
  type 1: cryoglobulinaemia, hepatitis Che
 type 2: partial lipodystrophyhe

External links

Postgraduate Medical Journal

Acute glomerulonephritis

Correct

3- A 64-year-old female is brought to the Emergency Department by her family,

who are concerned about her increasing confusion over the past 2 days. On
examination she is found to be pyrexial at 38ºC. Blood tests reveal:
he earaer aeraer asdsadas eerw dssdfsselleds

Hb 9.6 g/dl
Platelets 65 * 109/l
WCC 11.1 * 109/l
Urea 23.1 mmol/l
Creatinine 366 µmol/l

he earaer aeraer asdsadas eerw dssdfsselleds

What is the most likely diagnosis?ia

A.A Wegener's granulomatosisia

B.A Thrombotic thrombocytopenic purpuraia

C.A Haemolytic uraemic syndromeia

D.A Idiopathic thrombocytopenic purpuraia
E.A Rapidly progressive glomerulonephritisia

(b)

HUS or TTP? Neuro signs and purpura point towards TTP

The combination of neurological features, renal failure, pyrexia and

thrombocytopaenia point towards a diagnosis of thrombotic thrombocytopenic
purpura

Thrombotic thrombocytopenic purpura

sqweqwesf erwrewfsdfs adasd dhe
Pathogenesis of thrombotic thrombocytopenic purpura (TTP)

 abnormally large and sticky multimers of von Willebrand's factor cause

platelets to clump within vesselshe
 in TTP there is a deficiency of caspase which breakdowns large
multimers of von Willebrand's factorhe
 overlaps with haemolytic uraemic syndrome (HUS)he

3
he earaer aeraer asdsadas eerw dssdfsselleds
Features
 rare, typically adult femaleshe
 feverhe
 fluctuating neuro signs (microemboli)he
 microangiopathic haemolytic anaemiahe
 thrombocytopeniahe
 renal failurehe

he earaer aeraer asdsadas eerw dssdfsselleds

Causes
 post-infection e.g. urinary, gastrointestinalhe
 pregnancyhe
 drugs: ciclosporin, oral contraceptive pill, penicillin, clopidogrel,
aciclovirhe
 tumourshe
 SLEhe
 HIVhe

External links

British Committee for Standards in Haematology

2003 TTP/HUS guidelines
Correct

4- What is the most common site for extra-renal cysts in a patient with
autosomal dominant polycystic kidney disease (ADPKD)?ia

A.A Pancreasia
B.A Brainia
C.A Liveria

D.A Spleenia
E.A Thyroidia

(c)

Most common location of extra-renal cysts in ADPKD is liver

Liver cysts are present in 70% of patients with ADPKD. Around 8% of patients
have berry aneurysms

ADPKD: features
sqweqwesf erwrewfsdfs adasd dhe
Features

 hypertensionhe
 recurrent UTIshe
 abdominal painhe
 renal stoneshe

4
  haematuriahe
 CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Extra-renal manifestations
 liver cysts (70%)he
 berry aneurysms (8%)he
 CVS: mitral valve prolapse, mitral/tricuspid incompetence, aortic root
dilation, aortic dissectionhe
 cysts in other organs: pancreas, spleen, thyroidhe

Correct

5- A 65-year-old female with a 20 year history of rheumatoid arthritis is referred

to the acute medical unit with bilateral leg oedema. The following results are
obtained:
he earaer aeraer asdsadas eerw dssdfsselleds

Urea 11.2 mmol/l

Creatinine 205 µmol/l
Albumin 26 g/l
Bilirubin 12 mmol/l
ALP 120 IU/l
Urine protein 6.2 g/24 hours

he earaer aeraer asdsadas eerw dssdfsselleds

Which investigation is most likely to lead to the correct diagnosis?ia

A.A CT abdomenia
B.A Plasma magnesiumia
C.A Intravenous urogramia
D.A Rectal biopsyia

E.A Renal angiogramia

(d)
This rather odd question fooled most candidates when it appeared. The chronic
inflammatory process (rheumatoid) predisposes to amyloidosis which in turn
can cause nephrotic syndrome. Rectal biopsy is an (infrequent) test done to look
for amyloidosis.
he earaer aeraer asdsadas eerw dssdfsselleds
Rheumatoid drugs such as gold may cause nephrotic syndrome but none of the
other options point to this as an answer

Amyloidosis: types
sqweqwesf erwrewfsdfs adasd dhe
AL amyloid

 L for immunoglobulin Light chain fragmenthe

 due to myeloma, Waldenstrom's, MGUShe

5
  features include: cardiac and neurological involvement, macroglossia,
periorbital eccymoseshe

he earaer aeraer asdsadas eerw dssdfsselleds

AA amyloid
 A for precursor serum amyloid A protein, an acute phase reactanthe
 seen in chronic infection/inflammationhe
 e.g. TB, bronchiectasis, rheumatoid arthritishe
 features: renal involvement most common featurehe

he earaer aeraer asdsadas eerw dssdfsselleds

Beta-2 microglobulin amyloidosis
 precursor protein is beta-2 microglobulin, part of the major
histocompatibility complexhe
 associated with patients on renal dialysish

Correct

6- A 5-year-old boy is seen in the Emergency Department due to lethargy and

pallor. There is no recent history of diarrhoea. The following results are
obtained:
he earaer aeraer asdsadas eerw dssdfsselleds

Hb 8.4 g/dl
Platelets 30 * 109/l
Urea 24 mmol/l
Creatinine 164 µmol/l

he earaer aeraer asdsadas eerw dssdfsselleds

Urinalysis reveals proteinuria and haematuria. What is the most appropriate
management?ia

A.A IV cyclophosphamideia
B.A Ciprofloxacinia
C.A Oral prednisoloneia
D.A IV methylprednisolone followed by oral prednisoloneia
E.A Plasma exchangeia

(e)
There is no role for antibiotics, steroids or immunosuppressants in haemolytic
uraemic syndrome (HUS). Plasma exchange may be indicated, particularly in
severe cases of HUS not associated with diarrhoea

Haemolytic uraemic syndrome

sqweqwesf erwrewfsdfs adasd dhe
Haemolytic uraemic syndrome is generally seen in young children and produces
a triad of:

 acute renal failurehe

 microangiopathic haemolytic anaemiahe
 thrombocytopeniahe

6
he earaer aeraer asdsadas eerw dssdfsselleds
Causes
 post-dysentery - classically E coli 0157:H7 ('verotoxigenic',
'enterohaemorrhagic')he
 tumourshe
 pregnancyhe
 ciclosporin, the Pillhe
 systemic lupus erythematoushe
 HIVhe

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 treatment is supportive e.g. fluids, blood transfusion and dialysis if
requiredhe
 there is no role for antibiotics, despite the preceding diarrhoeal illness in
many patientshe
 the indications for plasma exchange in HUS are complicated. As a
general rule plasma exchange is reserved for severe cases of HUS not
associated with diarrhoeahe

Correct

7- Which of the following types of renal tubular acidosis is associated with

hyperkalaemia?ia

A.A Type 1 renal tubular acidosisia

B.A Type 2 renal tubular acidosisia
C.A Type 3 renal tubular acidosisia
D.A Type 4 renal tubular acidosisia

E.A Type 5 renal tubular acidosisia

(d)
Type 4 renal tubular acidosis is associated with hyperkalaemia

Renal tubular acidosis

sqweqwesf erwrewfsdfs adasd dhe
All three types of renal tubular acidosis (RTA) are associated with
hyperchloraemic metabolic acidosis (normal anion gap)
he earaer aeraer asdsadas eerw dssdfsselleds
Type 1 RTA (distal)

 inability to generate acid urine (secrete H+) in distal tubulehe

 causes hypokalaemiahe
 complications include nephrocalcinosis and renal stoneshe
 causes include idiopathic, RA, SLE, Sjogren'she

he earaer aeraer asdsadas eerw dssdfsselleds

Type 2 RTA (proximal)
 decreased HCO3- reabsorption in proximal tubulehe
 causes hypokalaemiahe
 complications include osteomalaciahe

7
  causes include idiopathic, as part of Fanconi syndrome, Wilson's disease,
cystinosis, outdated tetracyclineshe

he earaer aeraer asdsadas eerw dssdfsselleds

Type 4 RTA (hyperkalaemic)
 causes hyperkalaemiahe
 causes include hypoaldosteronism, diabeteshe

Correct

8- Which one of the following types of glomerulonephritis is most

characteristically associated with partial lipodystrophy?ia

A.A Minimal change diseaseia

B.A Diffuse proliferative glomerulonephritisia
C.A Mesangiocapillary glomerulonephritisia

D.A Membranous glomerulonephritisia

E.A Rapidly progressive glomerulonephritisia
(c)
Type 2 mesangiocapillary glomerulonephritis is associated with partial
lipodystrophy. Type 1 is seen in association with hepatitis C and
cryoglobulinaemia

Glomerulonephritides
sqweqwesf erwrewfsdfs adasd dhe
Knowing a few key facts is the best way to approach the difficult subject of
glomerulonephritis:
he earaer aeraer asdsadas eerw dssdfsselleds
Membranous glomerulonephritis

 presentation: proteinuria / nephrotic syndrome / CRFhe

 cause: infections, rheumatoid drugs, malignancyhe
 1/3 resolve, 1/3 respond to cytotoxics, 1/3 develop CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

IgA nephropathy - aka Berger's disease, mesangioproliferative GN
 typically young adult with haematuria following an URTIhe

he earaer aeraer asdsadas eerw dssdfsselleds

Diffuse proliferative glomerulonephritis
 classical post-streptococcal glomerulonephritis in childhe
 presents as nephritic syndrome / ARFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Minimal change disease
 typically a child with nephrotic syndrome (accounts for 80%)he
 causes: Hodgkin's, NSAIDshe
 good response to steroidshe

8
he earaer aeraer asdsadas eerw dssdfsselleds
Focal segmental glomerulosclerosis
 may be idiopathic or secondary to HIV, heroinhe
 presentation: proteinuria / nephrotic syndrome / CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Rapidly progressive glomerulonephritis - aka crescentic glomerulonephritis
 rapid onset, often presenting as ARFhe
 causes include Goodpasture's, ANCA positive vasculitis, SLEhe

he earaer aeraer asdsadas eerw dssdfsselleds

Mesangiocapillary glomerulonephritis (membranoproliferative)
 type 1: cryoglobulinaemia, hepatitis Che
 type 2: partial lipodystrophyhe

Correct

9- A 54-year-old woman with a history membranous glomerulonephritis

secondary to systemic lupus erythematous is admitted to hospital. Her previous
stable renal function has deteriorated rapidly. The following blood tests were
obtained:
he earaer aeraer asdsadas eerw dssdfsselleds

Na+ 139 mmol/l

K+ 5.8 mmol/l
Urea 44 mmol/l
Creatinine 867 µmol/l
Albumin 17 g/l
Urinary protein 14 g/24 hours
Urine dipstick protein +++
blood ++

he earaer aeraer asdsadas eerw dssdfsselleds

What has likely caused the sudden deterioration in renal function?ia

A.A Exacerbation of SLEia

B.A Renal vein thrombosisia

C.A Bilateral hydronephrosisia

D.A Acute interstitial nephritisia
E.A Analgesic nephropathyia
(b)
Nephrotic syndrome predisposes to thrombotic episodes, possibly due to loss of
antithrombin III. These commonly occur in the renal veins and may be bilateral.
Common symptoms include loin pain and haematuria

Nephrotic syndrome: complications

sqweqwesf erwrewfsdfs adasd dhe
Complications

9
  increased risk of infection due to urinary immunoglobulin losshe
 increased risk of thromboembolism related to loss of antithrombin III
and plasminogen in the urinehe
 hyperlipidaemiahe
 hypocalcaemia (vitamin D and binding protein lost in urine)he
 acute renal failurehe

Correct

10- A 27-year-old man is diagnosed with Goodpasture's syndrome. Which one

of the following does not increase the likelihood of a pulmonary haemorrhage?ia

A.A Smokingia
B.A Inhalation of hydrocarbonsia
C.A Male genderia
D.A Dehydrationia

E.A Lower respiratory tract infectionia

(d)
Dehydration may decrease the likelihood of a pulmonary haemorrhage.
Pulmonary oedema is associated with an increased risk

Goodpasture's syndrome
sqweqwesf erwrewfsdfs adasd dhe
Goodpasture's syndrome is rare condition associated with both pulmonary
haemorrhage and rapidly progressive glomerulonephritis. It is caused by anti-
glomerular basement membrane (anti-GBM) antibodies against type IV
collagen. Goodpasture's syndrome is more common in men (sex ratio 2:1) and
has a bimodal age distribution (peaks in 20-30 and 60-70 age bracket). It is
associated with HLA DR2
he earaer aeraer asdsadas eerw dssdfsselleds
Features

 pulmonary haemorrhage he
 followed by rapidly progressive glomerulonephritishe

he earaer aeraer asdsadas eerw dssdfsselleds

Factors which increase likelihood of pulmonary haemorrhage
 young maleshe
 smokinghe
 lower respiratory tract infectionhe
 pulmonary oedemahe
 inhalation of hydrocarbonshe

he earaer aeraer asdsadas eerw dssdfsselleds

Investigations
 renal biopsy: linear IgG deposits along basement membranehe
 raised transfer factor secondary to pulmonary haemorrhageshe

he earaer aeraer asdsadas eerw dssdfsselleds

Management

10
  plasma exchangehe
 steroidshe
 cyclophosphamidehe

Correct

11- A 10-year-old boy is taken to see the GP by his mother. For the past two
days he has had a sore throat associated with blood in his urine. There is no
significant past medical history. The GP suspects glomerulonephritis and refers
the patient to hospital. What would a renal biopsy most likely show?ia

A.A Proliferation of endothelial cellsia

B.A No changeia
C.A Mesangial hypercellularityia

D.A Basement membrane thickeningia

E.A Capillary wall necrosisia
(c)
This boy is likely to have IgA nephropathy. Histological features include
mesangial hypercellularity and positive immunofluorescence for IgA & C3

IgA nephropathy
sqweqwesf erwrewfsdfs adasd dhe
Basics

 also caused Berger's disease or mesangioproliferative

glomerulonephritishe
 commonest cause of glomerulonephritis worldwidehe
 pathogenesis unknown, ?mesangial deposition of IgA immune
complexeshe
 histology: mesangial hypercellularity, positive immunofluorescence for
IgA & C3he

he earaer aeraer asdsadas eerw dssdfsselleds

Differentiating between IgA nephropathy and post-streptococcal
glomerulonephritis
 post-streptococcal glomerulonephritis is associated with low
complement levelshe
 main symptom in post-streptococcal glomerulonephritis is proteinuria
(although haematuria can occur)he
 there is typically an interval between URTI and the onset of renal
problems in post-streptococcal glomerulonephritishe

he earaer aeraer asdsadas eerw dssdfsselleds

Presentations
 young male, recurrent episodes of macroscopic haematuriahe
 typically associated with mucosal infections e.g., URTIhe
 nephrotic syndromehe
 renal failurehe

11
he earaer aeraer asdsadas eerw dssdfsselleds
Associated conditions
 alcoholic cirrhosishe
 coeliac disease/dermatitis herpetiformishe

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 steroids/immunosuppressants not be shown to be usefulhe

he earaer aeraer asdsadas eerw dssdfsselleds

Prognosis
 25% of patients develop ESRFhe

Correct

12- Alport's syndrome is due to a defect in:ia

A.A Type I collagenia

B.A Type II collagenia
C.A Type III collagenia
D.A Type IV collagenia

E.A Type V collagenia

(d)

Alport's syndrome
sqweqwesf erwrewfsdfs adasd dhe
Alport's syndrome is a hereditary condition, usually X-linked dominant but may
be autosomal recessive or dominant. It is due to a defect in the gene which codes
for type IV collagen resulting in an abnormal glomerular-basement membrane
(GBM). The disease is more severe in males with females rarely developing
renal failure
he earaer aeraer asdsadas eerw dssdfsselleds
A favourite question in the MRCP is an Alport's patient with a failing renal
transplant. This may be caused by the presence of anti-GBM antibodies leading
to a Goodpasture's syndrome like picture
he earaer aeraer asdsadas eerw dssdfsselleds
Alport's syndrome usually presents in childhood. The following features may be
seen:

 microscopic haematuriahe
 progressive renal failurehe
 bilateral sensorineural deafnesshe
 retinitis pigmentosahe
 lenticonus: protrusion of the lens surface into the anterior chamberh

Correct

13- Each one of the following is a recognised side-effect of erythropoietin,

except:ia

12
 A.A Urticariaia
B.A Hypertensionia
C.A Bone achesia
D.A Long bone fracturesia

E.A Pure red cell aplasiaia

(d)

Erythropoietin
sqweqwesf erwrewfsdfs adasd dhe
Erythropoietin is a haematopoietic growth factor that stimulates the production
of erythrocytes. The main uses of erythropoietin are to treat the anaemia
associated with chronic renal failure and that associated with cytotoxic therapy
he earaer aeraer asdsadas eerw dssdfsselleds
Side-effects of erythropoietin

 accelerated hypertension --> encephalopathy, seizures (blood pressure

increases in 25% of patients)he
 bone acheshe
 skin rashes, urticaria, flu-like symptomshe
 pure red cell aplasia (due to antibodies against erythropoietin)he
 raised PCV increases risk of thrombosis (e.g. fistula)he
 iron deficiency 2nd to increased erythropoiesishe

he earaer aeraer asdsadas eerw dssdfsselleds

There are a number of reasons why patients may failure to respond to
erythropoietin therapy
 iron deficiencyhe
 inadequate dosehe
 concurrent infection/inflammationhe
 hyperparathyroid bone diseasehe
 aluminium toxicityhe

External links

Postgraduate Medical Journal

Review of erythropoietin

Correct

14- Which one of the following types of glomerulonephritis is most

characteristically associated with Goodpasture's syndrome?ia

A.A Diffuse proliferative glomerulonephritisia

B.A Mesangiocapillary glomerulonephritisia
C.A Membranous glomerulonephritisia
D.A Rapidly progressive glomerulonephritisia

E.A Focal segmental glomerulosclerosisia

(d)

13
Goodpasture's syndrome is rare condition associated with both pulmonary
haemorrhage and rapidly progressive glomerulonephritis. It is caused by anti-
glomerular basement membrane (anti-GBM) antibodies against type IV collagen

Glomerulonephritides
sqweqwesf erwrewfsdfs adasd dhe
Knowing a few key facts is the best way to approach the difficult subject of
glomerulonephritis:
he earaer aeraer asdsadas eerw dssdfsselleds
Membranous glomerulonephritis

 presentation: proteinuria / nephrotic syndrome / CRFhe

 cause: infections, rheumatoid drugs, malignancyhe
 1/3 resolve, 1/3 respond to cytotoxics, 1/3 develop CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

IgA nephropathy - aka Berger's disease, mesangioproliferative GN
 typically young adult with haematuria following an URTIhe

he earaer aeraer asdsadas eerw dssdfsselleds

Diffuse proliferative glomerulonephritis
 classical post-streptococcal glomerulonephritis in childhe
 presents as nephritic syndrome / ARFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Minimal change disease
 typically a child with nephrotic syndrome (accounts for 80%)he
 causes: Hodgkin's, NSAIDshe
 good response to steroidshe

he earaer aeraer asdsadas eerw dssdfsselleds

Focal segmental glomerulosclerosis
 may be idiopathic or secondary to HIV, heroinhe
 presentation: proteinuria / nephrotic syndrome / CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Rapidly progressive glomerulonephritis - aka crescentic glomerulonephritis
 rapid onset, often presenting as ARFhe
 causes include Goodpasture's, ANCA positive vasculitis, SLEhe

he earaer aeraer asdsadas eerw dssdfsselleds

Mesangiocapillary glomerulonephritis (membranoproliferative)
 type 1: cryoglobulinaemia, hepatitis Che
 type 2: partial lipodystrophyhe

Correct

15- Each of the following is a risk factor for renal stone formation, except:ia

A.A Renal tubular acidosisia

B.A Cadmiumia

14
 C.A Hyperparathyroidismia
D.A Dehydrationia
E.A Cystinosisia

(e)

Renal stones: risk factors

sqweqwesf erwrewfsdfs adasd dhe
Risk factors

 dehydrationhe
 hypercalciuria, hyperparathyroidism, hypercalcaemiahe
 cystinuriahe
 high dietary oxalatehe
 renal tubular acidosishe
 medullary sponge kidney, polycystic kidney diseasehe
 beryllium or cadmium exposurehe

he earaer aeraer asdsadas eerw dssdfsselleds

Risk factors for urate stones
 gouthe
 ileostomy: loss of bicarbonate and fluid results in acidic urine, causing
the precipitation of uric acidhe

he earaer aeraer asdsadas eerw dssdfsselleds

Drug causes
 drugs that promote calcium stones: loop diuretics, steroids,
acetazolamide, theophyllinehe
 thiazides can prevent calcium stones (increase distal tubular calcium
resorption)h

Correct

16- Which one of the following drugs may be safely continued at the same dose
in renal failure?ia

A.A Tetracyclineia
B.A Diclofenacia
C.A Warfarinia

D.A Nitrofurantoinia
E.A Lithiumia

(c)

Drugs in renal failure

sqweqwesf erwrewfsdfs adasd dhe
Questions regarding which drugs to avoid in renal failure are common in the
MRCP

15
he earaer aeraer asdsadas eerw dssdfsselleds
Drugs to avoid in renal failure

 antibiotics: tetracycline, nitrofurantoinhe

 NSAIDshe
 lithiumhe

he earaer aeraer asdsadas eerw dssdfsselleds

Drugs likely to accumulate in renal failure - need dose adjustment
 most antibiotics including penicillins, cephalosporins, vancomycin,
streptomycinhe
 digoxin, atenololhe
 methotrexatehe
 sulphonylureashe
 frusemidehe

he earaer aeraer asdsadas eerw dssdfsselleds

Drugs relatively safe - use in normal dose
 antibiotics: erythromycin, rifampicinhe
 diazepamhe
 warfarinhe

Correct

17- Which one of the following may be useful in the prevention of calcium renal
stones?ia

A.A Pyridoxineia
B.A Allopurinolia
C.A Lithiumia
D.A Ferrous sulphateia
E.A Thiazide diureticsia

(e)

Renal stones: management

sqweqwesf erwrewfsdfs adasd dhe
Calcium stones

 high fluid intakehe

 low animal protein, low salt diet (a low calcium diet has not been shown
to be superior to a normocalcaemic diet)he
 thiazide diuretics (reduce distal tubule calcium resorption)he
 stones < 5 mm will usually pass spontaneouslyhe
 lithotripsy, nephrolithotomy may be requiredhe

he earaer aeraer asdsadas eerw dssdfsselleds

Oxalate stones
 cholestyramine reduces urinary oxalate secretionhe
 pyridoxine reduces urinary oxalate secretionhe

16
he earaer aeraer asdsadas eerw dssdfsselleds
Uric acid stones
 allopurinolhe
 urinary alkalinization e.g. oral bicarbonatehe

Correct

18- Which one of the following is least recognised as a cause of membranous

glomerulonephritis?ia

A.A Malariaia
B.A Lymphomaia
C.A Hepatitis Bia
D.A Cryoglobulinaemiaia

E.A Goldia

(d)

Membranous glomerulonephritis
sqweqwesf erwrewfsdfs adasd dhe
Membranous glomerulonephritis is the commonest type of glomerulonephritis in
adults and is the third most common cause of end-stage renal failure (ESRF). It
usually presents as nephrotic syndrome or proteinuria
he earaer aeraer asdsadas eerw dssdfsselleds
Renal biopsy demonstrates:

 sub-epithelial immune complex (mainly IgG and C3) deposition in the

glomerulushe
 electron microscopy: the basement membrane is thickened with sub-
epithelial electron dense depositshe

he earaer aeraer asdsadas eerw dssdfsselleds

Causes
 idiopathiche
 infections: hepatitis B, malariahe
 malignancy: lung cancer, lymphoma, leukaemiahe
 drugs: gold, penicillamine, NSAIDshe
 systemic lupus erythematous (class V disease)he

he earaer aeraer asdsadas eerw dssdfsselleds

Prognosis - rule of thirds
 one-third: spontaneous remission he
 one-third: remain proteinuriche
 one-third: develop ESRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 immunosuppression: steroids, chlorambucil e.g. Ponticelli regimehe
 BP controlhe
 consider anticoagulationhe

17
 Incorrect

19- Which one of the following causes of glomerulonephritis is associated with

normal complement levels?ia

A.A Post-streptococcal glomerulonephritisia

B.A Mesangiocapillary glomerulonephritisia
C.A Subacute bacterial endocarditisia

D.A Goodpasture's syndromeia

E.A Systemic lupus erythematousia

Correct (D)

Goodpasture's syndrome is rare condition associated with both pulmonary

haemorrhage and rapidly progressive glomerulonephritis. It is caused by anti-
glomerular basement membrane (anti-GBM) antibodies against type IV
collagen. Complement levels are normal

Glomerulonephritis and low complement

sqweqwesf erwrewfsdfs adasd dhe
Disorders associated with glomerulonephritis and low serum complement levels

 post-streptococcal glomerulonephritishe
 subacute bacterial endocarditishe
 systemic lupus erythematoushe
 mesangiocapillary glomerulonephritishe

Correct

20- Which one of the following types of glomerulonephritis is associated with

fusion of podocytes on electron microscopy?ia

A.A Membranous glomerulonephritisia

B.A IgA nephropathyia
C.A Focal segmental glomerulosclerosisia
D.A Mesangiocapillary glomerulonephritisia
E.A Minimal change glomerulonephritisia

(e)

Minimal change glomerulonephritis

sqweqwesf erwrewfsdfs adasd dhe
Minimal change glomerulonephritis nearly always presents as nephrotic
syndrome, accounting for 75% of cases in children and 25% in adults
he earaer aeraer asdsadas eerw dssdfsselleds
The majority of cases are idiopathic, but in around 10-20% a cause is found:

 drugs: NSAIDs, rifampicinhe

18
  Hodgkin's lymphoma, thymomahe
 infectious mononucleosishe

he earaer aeraer asdsadas eerw dssdfsselleds

Features
 nephrotic syndromehe
 hypertensionhe
 highly selective proteinuriahe
 renal biopsy: electron microscopy shows fusion of podocytes he

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 majority of cases (80%) are steroid responsivehe
 cyclophosphamide is the next step for steroid resistant caseshe
 good prognosishe

Correct

21- Autosomal dominant polycystic kidney disease type 1 is associated with a

gene defect in:ia

A.A Chromosome 4ia

B.A Chromosome 8ia
C.A Chromosome 12ia
D.A Chromosome 16ia

E.A Chromosome 20ia

(d)

ADPKD type 1 = chromosome 16 = 85% of cases

ADPKD
sqweqwesf erwrewfsdfs adasd dhe
Autosomal dominant polycystic kidney disease (ADPKD) is the most common
inherited cause of kidney disease, affecting 1 in 1,000 Caucasians. Two disease
loci have been identified, PKD1 and PKD2, which code for polycystin-1 and
polycystin-2 respectively
he earaer aeraer asdsadas eerw dssdfsselleds

ADPKD type 1 ADPKD type 2

85% of cases 15% of cases
Chromosome 16 Chromosome 4
Presents with ESRF earlier

he earaer aeraer asdsadas eerw dssdfsselleds

The screening investigation for relatives is abdominal ultrasound:
he earaer aeraer asdsadas eerw dssdfsselleds
Ultrasound diagnostic criteria (in patients with positive family history)

19
  two cysts, unilateral or bilateral, if aged < 30 yearshe
 two cysts in both kidneys if aged 30-59 yearshe
 four cysts in both kidneys if aged > 60 yearshe

Correct

22- A patient with type 1 diabetes mellitus is reviewed in the nephrology

outpatient clinic. He is known to have stage 1 diabetic nephropathy. Which of
the following best describes his degree of renal involvement?ia

A.A Latent phaseia

B.A Hyperfiltrationia

C.A End-stage renal failureia

D.A Overt nephropathyia
E.A Microalbuminuriaia
(b)
For the purposes of the MRCP, increase in the glomerular filtration rate (GFR)
is most characteristic of stage 1 diabetic nephropathy. It is however known that
elevation of the GFR usually persists into stage 2

Diabetic nephropathy: stages

sqweqwesf erwrewfsdfs adasd dhe
Diabetic nephropathy may be classified as occurring in five stages*:
he earaer aeraer asdsadas eerw dssdfsselleds
Stage 1

 hyperfiltration: increase in GFRhe

 may be reversiblehe

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 2 (silent or latent phase)
 most patients do not develop microalbuminuria for 10 yearshe
 GFR remains elevatedhe

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 3 (incipient nephropathy)
 microalbuminuria (albumin excretion of 30 - 300 mg/day, dipstick
negative)he

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 4 (overt nephropathy)
 persistent proteinuria (albumin excretion > 300 mg/day, dipstick
positive)he
 hypertension is present in most patientshe
 histology shows diffuse glomerulosclerosis and focal glomerulosclerosis
(Kimmelstiel-Wilson nodules)he

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 5
 end-stage renal disease, GFR typically < 10ml/minhe

20
  renal replacement therapy neededhe

he earaer aeraer asdsadas eerw dssdfsselleds

The timeline given here is for type 1 diabetics. Patients with type 2 diabetes
mellitus (T2DM) progress through similar stages but in a different timescale -
some T2DM patients may progress quickly to the later stages
External links

SIGN
Diabetic nephropathy guidelines

Correct

23- A 73-year-old with a history of alcohol excess is admitted following a fall at

home. On admission the following blood results are obtained:
he earaer aeraer asdsadas eerw dssdfsselleds

Urea 3.5 mmol/l

Creatinine 110 µmol/l
Creatine kinase 180 u/l

he earaer aeraer asdsadas eerw dssdfsselleds

Three days later the blood results are as follows:
he earaer aeraer asdsadas eerw dssdfsselleds

Urea 14.5 mmol/l

Creatinine 248 µmol/l
Creatine kinase 4,400 u/l

he earaer aeraer asdsadas eerw dssdfsselleds

Which one of the following would have been most likely to prevent the
deterioration in renal function?ia

A.A Low dose dopamineia

B.A Urinary acidificationia
C.A Intravenous fluidsia

D.A Frusemideia
E.A Mannitolia

(c)

Collapse + ARF --> rhabdomyolysis - treat with IV fluids

Intravenous fluids are the most important management step in the prevent of
rhabdomyolysis in such patients

Rhabdomyolysis
sqweqwesf erwrewfsdfs adasd dhe

21
Rhabdomyolysis will typically feature in the exam as a patient who has had a
fall or prolonged epileptic seizure and is found to have acute renal failure on
admission
he earaer aeraer asdsadas eerw dssdfsselleds
Features

 acute renal failure with disproportionately raised creatininehe

 elevated CKhe
 myoglobinuriahe
 hypocalcaemia (myoglobin binds calcium)he
 elevated phosphate (released from myocytes)he

he earaer aeraer asdsadas eerw dssdfsselleds

Causes
 seizurehe
 collapse/coma (e.g. elderly patients collapses at home, found 8 hours
later)he
 ecstasyhe
 crush injuryhe
 McArdle's syndromehe
 drugs: statinshe

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 IV fluids to maintain good urine outputhe
 urinary alkalinization is sometimes usedhe

Correct

24- What percentage of cardiac output does renal blood flow accounts for:ia

A.A 5%ia
B.A 10%ia
C.A 15%ia
D.A 20-25%ia

E.A 30-35%ia

(d)

Renal physiology
sqweqwesf erwrewfsdfs adasd dhe
Renal blood flow is 20-25% of cardiac output
he earaer aeraer asdsadas eerw dssdfsselleds
Renal cortical blood flow > medullary blood flow (i.e. tubular cells more prone
to ischaemia

Correct

25- Which one of the following types of glomerulonephritis is most

characteristically associated with streptococcal infection in children?ia

22
 A.A Focal segmental glomerulosclerosisia
B.A Diffuse proliferative glomerulonephritisia

C.A Membranous glomerulonephritisia

D.A Mesangiocapillary glomerulonephritisia
E.A Rapidly progressive glomerulonephritisia

(b)

Glomerulonephritides
sqweqwesf erwrewfsdfs adasd dhe
Knowing a few key facts is the best way to approach the difficult subject of
glomerulonephritis:
he earaer aeraer asdsadas eerw dssdfsselleds
Membranous glomerulonephritis

 presentation: proteinuria / nephrotic syndrome / CRFhe

 cause: infections, rheumatoid drugs, malignancyhe
 1/3 resolve, 1/3 respond to cytotoxics, 1/3 develop CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

IgA nephropathy - aka Berger's disease, mesangioproliferative GN
 typically young adult with haematuria following an URTIhe

he earaer aeraer asdsadas eerw dssdfsselleds

Diffuse proliferative glomerulonephritis
 classical post-streptococcal glomerulonephritis in childhe
 presents as nephritic syndrome / ARFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Minimal change disease
 typically a child with nephrotic syndrome (accounts for 80%)he
 causes: Hodgkin's, NSAIDshe
 good response to steroidshe

he earaer aeraer asdsadas eerw dssdfsselleds

Focal segmental glomerulosclerosis
 may be idiopathic or secondary to HIV, heroinhe
 presentation: proteinuria / nephrotic syndrome / CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Rapidly progressive glomerulonephritis - aka crescentic glomerulonephritis
 rapid onset, often presenting as ARFhe
 causes include Goodpasture's, ANCA positive vasculitis, SLEhe

he earaer aeraer asdsadas eerw dssdfsselleds

Mesangiocapillary glomerulonephritis (membranoproliferative)
 type 1: cryoglobulinaemia, hepatitis Che
 type 2: partial lipodystrophyhe

Correct

23
26- A 26-year-old man with loin pain and haematuria is found to have
autosomal dominant polycystic kidney disease. A defect in which one of the
following genes is likely to be responsible?ia

A.A Fibrillin-2 geneia

B.A Polycystin geneia
C.A Fibrillin-1 geneia
D.A Von Hippel-Lindau geneia
E.A PKD1 geneia

(e)
Most cases of autosomal dominant polycystic kidney disease (ADPKD) are due
to a mutation in the PKD1 gene. The PKD1 gene encodes for a polycystin-1, a
large cell-surface glycoprotein of unknown function

ADPKD
sqweqwesf erwrewfsdfs adasd dhe
Autosomal dominant polycystic kidney disease (ADPKD) is the most common
inherited cause of kidney disease, affecting 1 in 1,000 Caucasians. Two disease
loci have been identified, PKD1 and PKD2, which code for polycystin-1 and
polycystin-2 respectively
he earaer aeraer asdsadas eerw dssdfsselleds

ADPKD type 1 ADPKD type 2

85% of cases 15% of cases
Chromosome 16 Chromosome 4
Presents with ESRF earlier

he earaer aeraer asdsadas eerw dssdfsselleds

The screening investigation for relatives is abdominal ultrasound:
he earaer aeraer asdsadas eerw dssdfsselleds
Ultrasound diagnostic criteria (in patients with positive family history)

 two cysts, unilateral or bilateral, if aged < 30 yearshe

 two cysts in both kidneys if aged 30-59 yearshe
 four cysts in both kidneys if aged > 60 yearshe

Correct

27- Each one of the following is associated with papillary necrosis, except:ia

A.A Acute pyelonephritisia

B.A Tuberculosisia
C.A Chronic analgesia useia
D.A Syphilisia

E.A Sickle cell diseaseia

(d)

24
Papillary necrosis
sqweqwesf erwrewfsdfs adasd dhe
Causes

 chronic analgesia usehe

 sickle cell diseasehe
 TBhe
 acute pyelonephritishe
 diabetes mellitushe

he earaer aeraer asdsadas eerw dssdfsselleds

Features
 fever, loin pain, haematuriahe
 IVU - papillary necrosis with renal scarring - 'cup & spill'he

Correct

28- Each one of the following is seen in renal osteodystrophy, except:ia

A.A Osteitis fibrosa cysticaia

B.A Primary hyperparathyroidismia

C.A High phosphateia

D.A Low calciumia
E.A Low vitamin Dia

(b)

Chronic kidney disease: bone disease

sqweqwesf erwrewfsdfs adasd dhe
Basic problems in chronic kidney disease

 low vitamin D (1-alpha hydroxylation normally occurs in the kidneys)he

 high phosphatehe
 low calcium: due to lack of vitamin D, high phosphatehe
 secondary hyperparathyroidism: due to low calcium, high phosphate and
low vitamin Dhe

he earaer aeraer asdsadas eerw dssdfsselleds

Several clinical manifestations may result:
he earaer aeraer asdsadas eerw dssdfsselleds
Osteitis fibrosa cystica
 aka hyperparathyroid bone diseasehe

he earaer aeraer asdsadas eerw dssdfsselleds

Adynamic
 may be due to over treatment with vitamin Dhe

he earaer aeraer asdsadas eerw dssdfsselleds

Osteomalacia
 due to low vitamin Dhe

25
he earaer aeraer asdsadas eerw dssdfsselleds
Osteosclerosis
he earaer aeraer asdsadas eerw dssdfsselleds
Osteoporosis

Correct

29- A 6-year-old boy presents is diagnosed as having nephrotic syndrome. A

presumptive diagnosis of minimal change glomerulonephritis is made. What is
the most appropriate treatment?ia

A.A Cyclophosphamideia
B.A Supportive treatment as an inpatientia
C.A Plasma exchangeia
D.A Renal biopsy followed by prednisoloneia
E.A Prednisoloneia

(e)

A renal biopsy is only indicated if response to steroids is poor

Minimal change glomerulonephritis

sqweqwesf erwrewfsdfs adasd dhe
Minimal change glomerulonephritis nearly always presents as nephrotic
syndrome, accounting for 75% of cases in children and 25% in adults
he earaer aeraer asdsadas eerw dssdfsselleds
The majority of cases are idiopathic, but in around 10-20% a cause is found:

 drugs: NSAIDs, rifampicinhe

 Hodgkin's lymphoma, thymomahe
 infectious mononucleosishe

he earaer aeraer asdsadas eerw dssdfsselleds

Features
 nephrotic syndromehe
 hypertensionhe
 highly selective proteinuriahe
 renal biopsy: electron microscopy shows fusion of podocytes he

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 majority of cases (80%) are steroid responsivehe
 cyclophosphamide is the next step for steroid resistant caseshe
 good prognosishe

Correct

30- Each one of the following is associated with Bartter's syndrome, except:ia

A.A Failure to thriveia

26
 B.A Hypertensionia

C.A Weaknessia
D.A Autosomal recessive inheritanceia
E.A Hypokalaemiaia

(b)

Bartter's syndrome is associated with normotension

Bartter's syndrome
sqweqwesf erwrewfsdfs adasd dhe
Bartter's syndrome is an inherited cause (usually autosomal recessive) of severe
hypokalaemia due to defective chloride absorption at the Na+ K+ 2Cl-
cotransporter in the ascending loop of Henle. It should be noted that is
associated with normotension (unlike other endocrine causes of hypokalaemia
such as Conn's, Cushing's and Liddle's syndrome which are associated with
hypertension)
he earaer aeraer asdsadas eerw dssdfsselleds
Features

 usually presents in childhood, e.g. failure to thrivehe

 hypokalaemiahe
 normotensionhe
 weaknessh

Correct

31- Which one of the following is least associated with retroperitoneal fibrosis?
ia

A.A Riedel's thyroiditisia

B.A Previous radiotherapyia
C.A Inflammatory abdominal aortic aneurysmia
D.A Methysergideia
E.A Sulphonamidesia

(e)

Retroperitoneal fibrosis
sqweqwesf erwrewfsdfs adasd dhe
Lower back pain is the most common presenting feature
he earaer aeraer asdsadas eerw dssdfsselleds
Associations

 Riedel's thyroiditishe
 previous radiotherapyhe
 sarcoidosishe
 inflammatory abdominal aortic aneurysmhe

27
  drugs: methysergideh

Correct

32- Which of the following factors would suggest that a patient has established
acute tubular necrosis rather than pre-renal uraemia?ia

A.A Urine sodium = 10 mmol/Lia

B.A Fractional urea excretion = 20%ia
C.A Increase in urine output following fluid challengeia
D.A Specific gravity = 1025ia
E.A Fractional sodium excretion = 1.5%ia

(e)

ATN or prerenal uraemia? In prerenal uraemia think of the kidneys holding on to

sodium to preserve volume

ARF: ATN vs. prerenal uraemia

sqweqwesf erwrewfsdfs adasd dhe
Prerenal uraemia - kidneys hold on to sodium to preserve volume
he earaer aeraer asdsadas eerw dssdfsselleds

Pre-renal uraemia Acute tubular necrosis

Urine sodium < 20 mmol/L > 30 mmol/L
Fractional sodium excretion* < 1% > 1%
Fractional urea excretion** < 35% >35%
Urine:plasma osmolality > 1.5 < 1.1
Urine:plasma urea > 10:1 < 8:1
Specific gravity > 1020 < 1010
Urine 'bland' sediment brown granular casts
Response to fluid challenge Yes No

he earaer aeraer asdsadas eerw dssdfsselleds

*fractional sodium excretion = (urine sodium/plasma sodium) / (urine
creatinine/plasma creatinine) x 100
he earaer aeraer asdsadas eerw dssdfsselleds
**fractional urea excretion = (urine urea /blood urea ) / (urine creatinine/plasma
creatinine) x 100

Correct

33- What is the most significant factor leading to the development of anaemia in
patients with chronic kidney disease?ia

28
 A.A Reduced absorption of ironia
B.A Increased erythropoietin resistanceia
C.A Reduced erythropoietin levelsia

D.A Reduced erythropoiesis due to toxic effects of uraemia on bone

marrowia
E.A Blood loss due to capillary fragility and poor platelet functionia

(c)

Chronic kidney disease: anaemia

sqweqwesf erwrewfsdfs adasd dhe
Patients with chronic kidney disease (CKD) may develop anaemia due to a
variety of factors, the most significant of which is reduced erythropoietin levels.
This is usually a normochromic normocytic anaemia and becomes apparent
when the GFR is less than 35 ml/min (other causes of anaemia should be
considered if the GFR is > 60 ml/min). Anaemia in CKD predisposes to the
development of left ventricular hypertrophy - associated with a three fold
increase in mortality in renal patients
he earaer aeraer asdsadas eerw dssdfsselleds
Causes of anaemia in renal failure

 reduced erythropoietin levels - the most significant factorhe

 reduced erythropoiesis due to toxic effects of uraemia on bone marrowhe
 reduced absorption of ironhe
 anorexia/nausea due to uraemiahe
 reduced red cell survival (especially in haemodialysis)he
 blood loss due to capillary fragility and poor platelet functionhe
 stress ulceration leading to chronic blood losshe

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 the 2006 NICE guidelines suggest a target haemoglobin of 10.5 - 12.5
g/dlhe
 determination and optimisation of iron status should be carried out prior
to the administration of erythropoiesis-stimulating agents (ESA). Many
patients, especially those on haemodialysis, will require IV ironhe
- ESAs such as erythropoietin and darbepoietin should be used in those
'who are likely to benefit in terms of quality of life and physical function'

Incorrect

34- What is the most common type of renal stone?ia

A.A Calcium phosphateia

B.A Cystine stonesia

C.A Triple phosphate stonesia
D.A Calcium oxalateia

E.A Xanthine stonesia

29
Correct (D)

Renal stones: imaging

sqweqwesf erwrewfsdfs adasd dhe
The table below summarises the appearance of different types of renal stone on
x-ray
he earaer aeraer asdsadas eerw dssdfsselleds

Type Frequency Radiograph appearance

Calcium oxalate 40% Opaque
Mixed calcium 25% Opaque
oxalate/phosphate stones
Triple phosphate stones 10% Opaque
Calcium phosphate 10% Opaque
Urate stones 5-10% Radio-lucent
Cystine stones 1% Semi-opaque, 'ground-glass'
appearance
Xanthine stones <1% Radio-lucent

Correct

35- Each of the following is a risk factor for renal stone formation, except:ia

A.A Cystinuriaia
B.A Berylliumia
C.A Hypoparathyroidismia

D.A Renal tubular acidosisia

E.A Dehydrationia

(c)

Renal stones: risk factors

sqweqwesf erwrewfsdfs adasd dhe
Risk factors

 dehydrationhe
 hypercalciuria, hyperparathyroidism, hypercalcaemiahe
 cystinuriahe
 high dietary oxalatehe
 renal tubular acidosishe
 medullary sponge kidney, polycystic kidney diseasehe
 beryllium or cadmium exposurehe

he earaer aeraer asdsadas eerw dssdfsselleds

Risk factors for urate stones
 gouthe

30
  ileostomy: loss of bicarbonate and fluid results in acidic urine, causing
the precipitation of uric acidhe

he earaer aeraer asdsadas eerw dssdfsselleds

Drug causes
 drugs that promote calcium stones: loop diuretics, steroids,
acetazolamide, theophyllinehe
 thiazides can prevent calcium stones (increase distal tubular calcium
resorption)he

Correct

36- Which one of the following is least associated with focal segmental
glomerulosclerosis?ia

A.A Alport's syndromeia

B.A Heroinia
C.A Sickle-cell anaemiaia
D.A Sarcoidosisia

E.A HIV infectionia

(d)

Focal segmental glomerulosclerosis

sqweqwesf erwrewfsdfs adasd dhe
Causes

 idiopathiche
 secondary to other renal pathology e.g. IgA nephropathy, reflux
nephropathyhe
 HIVhe
 heroinhe
 Alport's syndromehe
 sickle-cellhe

he earaer aeraer asdsadas eerw dssdfsselleds

Presentations
 nephrotic syndromehe

he earaer aeraer asdsadas eerw dssdfsselleds

Focal segmental glomerulosclerosis is noted for having a high recurrence rate in
renal transplants

Correct

37- What is the best way to differentiate between acute and chronic renal
failure?ia

A.A 24 hr creatinineia

31
 B.A Urinary albuminia
C.A Serum creatinineia
D.A Renal ultrasoundia

E.A Serum ureaia

(d)
Small kidneys is (usually) a sign of chronic renal failure

Acute vs. chronic renal failure

sqweqwesf erwrewfsdfs adasd dhe
Best way to differentiate is renal ultrasound - most patients with CRF have
bilateral small kidneys
he earaer aeraer asdsadas eerw dssdfsselleds
Exceptions

 autosomal dominant polycystic kidney diseasehe

 diabetic nephropathyhe
 amyloidosishe

he earaer aeraer asdsadas eerw dssdfsselleds

Other features suggesting CRF rather than ARF
 hypocalcaemia (due to lack of vitamin D)he

Correct

38- Which one of the following is least recognised as an indication for plasma
exchange?ia

A.A Guillain-Barre syndromeia

B.A Churg-Strauss syndromeia
C.A Myasthenia gravisia
D.A Cerebral malariaia

E.A Goodpasture's syndromeia

(d)

Cerebral malaria is not a standard indication for plasma exchange. Exchange

transfusions have been tried but it is generally only justified when peripheral
parasitemia is greater than 10% of circulating erythrocytes. The role of blood
transfusions remains controversial, as they are both expensive and potentially
dangerous in many malaria areas

Plasma exchange
sqweqwesf erwrewfsdfs adasd dhe
Indications for plasma exchange

 Guillain-Barre syndromehe
 myasthenia gravishe
 Goodpasture's syndromehe

32
  ANCA positive vasculitis e.g. Wegener's, Churg-Strausshe
 TTP/HUShe
 cryoglobulinaemiahe
 hyperviscosity syndrome e.g. secondary to myelomah

Correct

39- Which one of the following is not a recognised risk factor for the
development of diabetic nephropathy?ia

A.A Poor glycaemic controlia

B.A Smokingia
C.A Male sexia
D.A Low dietary proteinia

E.A Hypertensionia

(d)

Diabetic nephropathy
sqweqwesf erwrewfsdfs adasd dhe
Basics

 commonest cause of ESRF in western worldhe

 mechanism in type 1 and type 2 diabetes thought to be samehe
 T1DM: 33% of patients by 40 years have diabetic nephropathyhe
 some patients with T1DM seem immune from developing nephropathy,
if hasn't developed by 40 years then low chance of future developmenthe
 approximately 5-10% of patients with T2DM develop ESRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Pathological changes
 basement membrane thickeninghe
 capillary obliterationhe
 mesangial wideninghe

he earaer aeraer asdsadas eerw dssdfsselleds

Risk factors for developing diabetic nephropathy
 male sexhe
 poor glycaemic controlhe
 hypertension, hyperlipidaemia, smokerhe
 raised dietary proteinhe
 genetic predisposition (e.g. ACE gene polymorphisms)he

Correct

40- Which one of the following features is least likely to be seen in Henoch-
Schonlein purpura?ia

A.A Abdominal painia

33
 B.A Renal failureia
C.A Polyarthritisia
D.A Thrombocytopeniaia

E.A Purpuric rash over buttocksia

(d)

Henoch-Schonlein purpura
sqweqwesf erwrewfsdfs adasd dhe
Henoch-Schonlein purpura (HSP) is an IgA mediated small vessel vasculitis.
There is a degree of overlap with IgA nephropathy (Berger's disease). HSP is
usually seen in children following an infection
he earaer aeraer asdsadas eerw dssdfsselleds
Features

 palpable purpuric rash (with localized oedema) over buttocks and

extensor surfaces of arms and legshe
 abdominal painhe
 polyarthritishe
 features of IgA nephropathy may occur e.g. haematuria, renal failurehe

Correct

41- Renal cell carcinoma is least associated with which one of the following
hormones?ia

A.A Erythropoietinia
B.A Parathyroid hormoneia
C.A Growth hormoneia

D.A ACTHia
E.A Reninia

(c)

Renal cell cancer

sqweqwesf erwrewfsdfs adasd dhe
Overview

 also known as: hypernephromahe

 accounts for 85% of primary renal neoplasmshe
 arises from proximal renal tubular epitheliumhe

he earaer aeraer asdsadas eerw dssdfsselleds

Associations
 more common in middle-aged menhe
 smokinghe
 von Hippel-Lindau syndromehe
 autosomal dominant polycystic kidney diseasehe
 tuberose sclerosishe

34
he earaer aeraer asdsadas eerw dssdfsselleds
Features
 classical triad: haematuria, loin pain, abdominal masshe
 pyrexia of unknown originhe
 left varicocele (due to occlusion of left testicular vein)he
 endocrine effects: may secrete EPO (polycythaemia), PTH
(hypercalcaemia), renin, ACTHhe
 25% have metastases at presentationhe

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 radical nephrectomy for confined diseasehe
 in disseminated disease, recent studies have shown a survival advantage
for nephrectomy prior to interferon-alphahe

Correct

42- Microalbuminuria may be defined as an albumin excretion of:ia

A.A 0.1 - 1 mg/dayia

B.A 30 - 300 mg/dayia

C.A 1 - 10 mg/dayia
D.A 10 - 100 mg/dayia
E.A 3 - 30 mg/dayia

(b)

Proteinuria
sqweqwesf erwrewfsdfs adasd dhe
Microalbuminuria

 defined as an albumin excretion of 30 - 300 mg/dayhe

he earaer aeraer asdsadas eerw dssdfsselleds

Albumin:creatinine excretion ratio (ACR)
 used in clinical practice to quantify degree of proteinuriahe
 first morning urine samplehe
 urine albumin (mg) / creatinine (mmol)he
 normal ACR < 2.5he
 microalbuminuric range = 2.5 - 33he

Correct

43- A patient with type 1 diabetes mellitus is reviewed in the nephrology

outpatient clinic. He is known to have stage 4 diabetic nephropathy. Which of
the following best describes his degree of renal involvement?ia

A.A Microalbuminuriaia
B.A End-stage renal failureia

35
 C.A Latent phaseia
D.A Hyperfiltrationia
E.A Overt nephropathyia

(e)

Diabetic nephropathy: stages

sqweqwesf erwrewfsdfs adasd dhe
Diabetic nephropathy may be classified as occurring in five stages*:
he earaer aeraer asdsadas eerw dssdfsselleds
Stage 1

 hyperfiltration: increase in GFRhe

 may be reversiblehe

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 2 (silent or latent phase)
 most patients do not develop microalbuminuria for 10 yearshe
 GFR remains elevatedhe

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 3 (incipient nephropathy)
 microalbuminuria (albumin excretion of 30 - 300 mg/day, dipstick
negative)he

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 4 (overt nephropathy)
 persistent proteinuria (albumin excretion > 300 mg/day, dipstick
positive)he
 hypertension is present in most patientshe
 histology shows diffuse glomerulosclerosis and focal glomerulosclerosis
(Kimmelstiel-Wilson nodules)he

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 5
 end-stage renal disease, GFR typically < 10ml/minhe
 renal replacement therapy neededhe

he earaer aeraer asdsadas eerw dssdfsselleds

The timeline given here is for type 1 diabetics. Patients with type 2 diabetes
mellitus (T2DM) progress through similar stages but in a different timescale -
some T2DM patients may progress quickly to the later stages

Correct

44- A 45-year-old woman with nephrotic syndrome is noted to have marked loss
of subcutaneous tissue from the face. What is the most likely underlying cause
of her renal disease?ia

A.A Mesangiocapillary glomerulonephritis type IIia

36
 B.A Focal segmental glomerulosclerosisia
C.A Minimal change glomerulonephritisia
D.A Renal vein thrombosisia
E.A Membranous glomerulonephritisia

(a)

This patient has partial lipodystrophy which is associated with

mesangiocapillary glomerulonephritis type II

Mesangiocapillary glomerulonephritis
sqweqwesf erwrewfsdfs adasd dhe
Overview

 aka membranoproliferative glomerulonephritishe

 may present as nephrotic syndrome, haematuria or proteinuriahe
 poor prognosishe

he earaer aeraer asdsadas eerw dssdfsselleds

Type 1
 subendothelial immune depositshe
 cause: cryoglobulinaemia, hepatitis Che

he earaer aeraer asdsadas eerw dssdfsselleds

Type 2 - 'dense deposit disease'
 intramembranous deposits of electron dense materialhe
 causes: partial lipodystrophy, factor H deficiencyhe
 reduced serum complementhe
 C3b nephritic factor (an antibody against C3bBb) found in 70%he

he earaer aeraer asdsadas eerw dssdfsselleds

Type 3
 causes: hepatitis B and Che

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 steroids may be effectivehe

Correct

45- A 45-year-old female with nephrotic syndrome develops renal vein

thrombosis. What changes in patients with nephrotic syndrome predispose to the
development of venous thromboembolism?ia

A.A Reduced excretion of protein Sia

B.A Loss of antithrombin IIIia

C.A Reduced excretion of protein Cia

D.A Loss of fibrinogenia
E.A Reduced metabolism of vitamin Kia

37
(b)

Nephrotic syndrome
sqweqwesf erwrewfsdfs adasd dhe
Triad of
1. Proteinuria (> 3g/24hr) causing
2. Hypoalbuminaemia (< 30g/L) and
3. Oedema
he earaer aeraer asdsadas eerw dssdfsselleds
Loss of antithrombin-III, proteins C and S and a associated rise in fibrinogen
levels predispose to thrombosis. Loss of TBG lowers total, but not free
thyroxine levels

Correct

46- A 54-year-old man presents with nephrotic syndrome thought to be

secondary to amyloidosis. A renal biopsy is taken. Which one of the following
stains should be applied to the tissue?ia

A.A Rose Bengalia

B.A Pearl's stainia
C.A Congo redia

D.A Periodic acid Schiffia

E.A Cresyl blueia

(c)

Amyloidosis
sqweqwesf erwrewfsdfs adasd dhe
Overview

 amyloidosis is a term which describes the extracellular deposition of an

insoluble fibrillar protein termed amyloidhe
 amyloid is derived from many different precursor proteinshe
 in addition to the fibrillar component, amyloid also contains a non-
fibrillary protein called amyloid-P component, derived from the acute
phase protein serum amyloid Phe
 other non-fibrillary components include apolipoprotein E and heparan
sulphate proteoglycanshe
 the accumulation of amyloid fibrils leads to tissue/organ dysfunctionhe

he earaer aeraer asdsadas eerw dssdfsselleds

Classification
 systemic or localizedhe
 further characterised by precursor protein (e.g. AL in myeloma - A for
Amyloid, L for immunoglobulin Light chain fragments)he

he earaer aeraer asdsadas eerw dssdfsselleds

Diagnosis
 Congo red staininghe

38
  serum amyloid precursor (SAP) scanhe
 biopsy of rectal tissue

Correct

47- Fanconi syndrome is associated with each one of the following, except:ia

A.A Hydronephrosisia

B.A Osteomalaciaia
C.A Aminoaciduriaia
D.A Glycosuriaia
E.A Proximal renal tubular acidosisia

(a)

Fanconi syndrome
sqweqwesf erwrewfsdfs adasd dhe
A disorder of renal tubular function
he earaer aeraer asdsadas eerw dssdfsselleds
Features

 type 2 (proximal) renal tubular acidosishe

 aminoaciduriahe
 glycosuriahe
 phosphaturiahe
 osteomalaciahe

he earaer aeraer asdsadas eerw dssdfsselleds

Causes
 inherited: cystinosis, Wilson's diseasehe
 acquired: renal, Sjogren'she

Correct

48- A 61-year-old man with a history of hypertension presents with central chest
pain. Acute coronary syndrome is diagnosed and conventional management is
given. A few days later a diagnostic coronary angiogram is performed. The
following week a deteriorating of renal function is noted associated with a
purpuric rash on his legs. What is the most likely diagnosis?ia

A.A Aspirin-induced interstitial nephritisia

B.A Heparin-induced thrombocytopaeniaia
C.A Renal artery stenosisia
D.A Cholesterol embolisationia

E.A Antiphospholipid syndromeia

(d)
Cholesterol embolisation is a well-documented complication of coronary
angiography

39
Cholesterol embolisation
sqweqwesf erwrewfsdfs adasd dhe
Overview

 cholesterol emboli may break off causing renal diseasehe

 seen more commonly in arteriopaths, abdominal aortic aneurysmshe

he earaer aeraer asdsadas eerw dssdfsselleds

Features
 eosinophiliahe
 purpurahe
 renal failurehe
 livedo reticularishe

Correct

49- A 33-year-old man is admitted with bilateral leg oedema and heavy
proteinuria. He has a history of coeliac disease. What is the likely diagnosis?ia

A.A Diffuse proliferative glomerulonephritisia

B.A IgA nephropathyia

C.A Membranous glomerulonephritisia

D.A Minimal change diseaseia
E.A Rapidly progressive glomerulonephritisia
(b)
This man has nephrotic syndrome which is associated with coeliac disease

IgA nephropathy
sqweqwesf erwrewfsdfs adasd dhe
Basics

 also caused Berger's disease or mesangioproliferative

glomerulonephritishe
 commonest cause of glomerulonephritis worldwidehe
 pathogenesis unknown, ?mesangial deposition of IgA immune
complexeshe
 histology: mesangial hypercellularity, positive immunofluorescence for
IgA & C3he

he earaer aeraer asdsadas eerw dssdfsselleds

Differentiating between IgA nephropathy and post-streptococcal
glomerulonephritis
 post-streptococcal glomerulonephritis is associated with low
complement levelshe
 main symptom in post-streptococcal glomerulonephritis is proteinuria
(although haematuria can occur)he
 there is typically an interval between URTI and the onset of renal
problems in post-streptococcal glomerulonephritishe

40
he earaer aeraer asdsadas eerw dssdfsselleds
Presentations
 young male, recurrent episodes of macroscopic haematuriahe
 typically associated with mucosal infections e.g., URTIhe
 nephrotic syndromehe
 renal failurehe

he earaer aeraer asdsadas eerw dssdfsselleds

Associated conditions
 alcoholic cirrhosishe
 coeliac disease/dermatitis herpetiformishe

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 steroids/immunosuppressants not be shown to be usefulhe

he earaer aeraer asdsadas eerw dssdfsselleds

Prognosis

Correct

50- Which of the following types of renal stones are said to have a semi-opaque
appearance on x-ray?ia

A.A Calcium oxalateia

B.A Cystine stonesia

C.A Urate stonesia

D.A Xanthine stonesia
E.A Triple phosphate stonesia

(b)

Renal stones on x-ray

 cystine stones: semi-opaque

 urate + xanthine stones: radio-lucent

Renal stones: imaging

sqweqwesf erwrewfsdfs adasd dhe
The table below summarises the appearance of different types of renal stone on
x-ray
he earaer aeraer asdsadas eerw dssdfsselleds

Type Frequency Radiograph appearance

Calcium oxalate 40% Opaque
Mixed calcium 25% Opaque
oxalate/phosphate stones
Triple phosphate stones 10% Opaque

41
Calcium phosphate 10% Opaque
Urate stones 5-10% Radio-lucent
Cystine stones 1% Semi-opaque, 'ground-glass'
appearance
Xanthine stones <1% Radio-lucent

Correct

51- Each one of the following is a feature of renal cell cancer, except:ia

A.A Right-sided varicoceleia

B.A Pyrexia of unknown originia

C.A Loin painia
D.A Haematuriaia
E.A Polycythaemiaia

(a)

Renal cell cancer

sqweqwesf erwrewfsdfs adasd dhe
Overview

 also known as: hypernephromahe

 accounts for 85% of primary renal neoplasmshe
 arises from proximal renal tubular epitheliumhe

he earaer aeraer asdsadas eerw dssdfsselleds

Associations
 more common in middle-aged menhe
 smokinghe
 von Hippel-Lindau syndromehe
 autosomal dominant polycystic kidney diseasehe
 tuberose sclerosishe

he earaer aeraer asdsadas eerw dssdfsselleds

Features
 classical triad: haematuria, loin pain, abdominal masshe
 pyrexia of unknown originhe
 left varicocele (due to occlusion of left testicular vein)he
 endocrine effects: may secrete EPO (polycythaemia), PTH
(hypercalcaemia), renin, ACTHhe
 25% have metastases at presentationhe

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 radical nephrectomy for confined diseasehe
 in disseminated disease, recent studies have shown a survival advantage
for nephrectomy prior to interferon-alphahe

42
 Incorrect

52- You are asked to review a 75-year-old female on the surgical wards due to
hyperkalaemia. Results are as follows:
he earaer aeraer asdsadas eerw dssdfsselleds

Plasma Urine
+
Na (mmol/l) 129 5
+
K (mmol/l) 6.8
Urea (mmol/l) 26 350
Creatinine (µmol/l) 262
Osmolality (mosmol/kg) 296 470

he earaer aeraer asdsadas eerw dssdfsselleds

What is the most likely diagnosis?ia

A.A Acute tubular necrosisia

B.A Hyperosmolar non-ketotic comaia

C.A Hydronephrosisia
D.A Prerenal uraemiaia

E.A Pyelonephritisia

Correct (D)

ATN or prerenal uraemia? In prerenal uraemia think of the kidneys holding on to

sodium to preserve volume

The low urine sodium points towards prerenal uraemia, as does the urine:plasma
osmolality and urea ratio

ARF: ATN vs. prerenal uraemia

sqweqwesf erwrewfsdfs adasd dhe
Prerenal uraemia - kidneys hold on to sodium to preserve volume
he earaer aeraer asdsadas eerw dssdfsselleds

Pre-renal uraemia Acute tubular necrosis

Urine sodium < 20 mmol/L > 30 mmol/L
Fractional sodium excretion* < 1% > 1%
Fractional urea excretion** < 35% >35%
Urine:plasma osmolality > 1.5 < 1.1
Urine:plasma urea > 10:1 < 8:1
Specific gravity > 1020 < 1010
Urine 'bland' sediment brown granular casts
Response to fluid challenge Yes No

43
he earaer aeraer asdsadas eerw dssdfsselleds
*fractional sodium excretion = (urine sodium/plasma sodium) / (urine
creatinine/plasma creatinine) x 100
he earaer aeraer asdsadas eerw dssdfsselleds
**fractional urea excretion = (urine urea /blood urea ) / (urine creatinine/plasma
creatinine) x 100

Correct

53- In Goodpasture's syndrome anti-glomerular basement membrane (anti-

GBM) antibodies are directed against which type of collagen?ia

A.A Type I collagenia

B.A Type II collagenia
C.A Type III collagenia
D.A Type IV collagenia

E.A Type VI collagenia

(d)

Goodpasture's syndrome
sqweqwesf erwrewfsdfs adasd dhe
Goodpasture's syndrome is rare condition associated with both pulmonary
haemorrhage and rapidly progressive glomerulonephritis. It is caused by anti-
glomerular basement membrane (anti-GBM) antibodies against type IV
collagen. Goodpasture's syndrome is more common in men (sex ratio 2:1) and
has a bimodal age distribution (peaks in 20-30 and 60-70 age bracket). It is
associated with HLA DR2
he earaer aeraer asdsadas eerw dssdfsselleds
Features

 pulmonary haemorrhage he
 followed by rapidly progressive glomerulonephritishe

he earaer aeraer asdsadas eerw dssdfsselleds

Factors which increase likelihood of pulmonary haemorrhage
 young maleshe
 smokinghe
 lower respiratory tract infectionhe
 pulmonary oedemahe
 inhalation of hydrocarbonshe

he earaer aeraer asdsadas eerw dssdfsselleds

Investigations
 renal biopsy: linear IgG deposits along basement membranehe
 raised transfer factor secondary to pulmonary haemorrhageshe

44
he earaer aeraer asdsadas eerw dssdfsselleds
Management
 plasma exchangehe
 steroidshe
 cyclophosphamidehe

Incorrect

54- A 45-year-old presents to the Emergency Department with chest pain. An

ECG shows anterior ST elevation and he is thrombolysed with alteplase. His
chest pain settles and he is started on aspirin, atorvastatin, bisoprolol and
ramipril. Three days later his blood results are as follows:
he earaer aeraer asdsadas eerw dssdfsselleds

Urea 22 mmol/l
Creatinine 277 µmol/l

he earaer aeraer asdsadas eerw dssdfsselleds

What is the most likely cause for the deterioration in renal function?ia

A.A Renal artery stenosisia

B.A NSAID related nephropathyia

C.A Statin nephropathyia

D.A Dressler's syndromeia
E.A Haemorrhage into renal cystia

Correct (A)

Flash pulmonary oedema, U&Es worse on ACE inhibitor, asymmetrical kidneys

--> renal artery stenosis - do MR angiography

There is likely underlying renal artery stenosis revealed by the addition of an

ACE inhibitor

Renal vascular disease

sqweqwesf erwrewfsdfs adasd dhe
Renal vascular disease is most commonly due to atherosclerosis (> 95% of
patients). It is associated with risk factors such as smoking and hypertension that
cause atheroma elsewhere in the body. It may present as hypertension, chronic
renal failure or 'flash' pulmonary oedema. In younger patients however
fibromuscular dysplasia (FMD) needs to be considered. FMD is more common
in young women and characteristically has a 'string of beads' appearance on
angiography. Patients respond well to balloon angioplasty
he earaer aeraer asdsadas eerw dssdfsselleds
Investigation

45
  MR angiography is now the investigation of choicehe
 CT angiographyhe
 conventional renal angiography is less commonly performed used
nowadays, but may still have a role when planning surgery

Correct

55- What is the most likely outcome following the diagnosis of minimal change
nephropathy in a 20-year-old male?ia

A.A Chronic renal impairment requiring renal replacement therapyia

B.A Persistent proteinuriaia
C.A Full recoveryia

D.A Chronic renal impairment not requiring renal replacement

therapyia
E.A Relapsing-remitting courseia

(c)

Minimal change glomerulonephritis

sqweqwesf erwrewfsdfs adasd dhe
Minimal change glomerulonephritis nearly always presents as nephrotic
syndrome, accounting for 75% of cases in children and 25% in adults
he earaer aeraer asdsadas eerw dssdfsselleds
The majority of cases are idiopathic, but in around 10-20% a cause is found:

 drugs: NSAIDs, rifampicinhe

 Hodgkin's lymphoma, thymomahe
 infectious mononucleosishe

he earaer aeraer asdsadas eerw dssdfsselleds

Features
 nephrotic syndromehe
 hypertensionhe
 highly selective proteinuriahe
 renal biopsy: electron microscopy shows fusion of podocytes he

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 majority of cases (80%) are steroid responsivehe
 cyclophosphamide is the next step for steroid resistant caseshe
 good prognosis

Correct

56- Which one of the following types of glomerulonephritis is most

characteristically associated with Wegener's granulomatosis?ia

46
 A.A Mesangiocapillary glomerulonephritisia
B.A Membranous glomerulonephritisia
C.A Rapidly progressive glomerulonephritisia

D.A Focal segmental glomerulosclerosisia

E.A Diffuse proliferative glomerulonephritisia

(c)

Glomerulonephritides
sqweqwesf erwrewfsdfs adasd dhe
Knowing a few key facts is the best way to approach the difficult subject of
glomerulonephritis:
he earaer aeraer asdsadas eerw dssdfsselleds
Membranous glomerulonephritis

 presentation: proteinuria / nephrotic syndrome / CRFhe

 cause: infections, rheumatoid drugs, malignancyhe
 1/3 resolve, 1/3 respond to cytotoxics, 1/3 develop CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

IgA nephropathy - aka Berger's disease, mesangioproliferative GN
 typically young adult with haematuria following an URTIhe

he earaer aeraer asdsadas eerw dssdfsselleds

Diffuse proliferative glomerulonephritis
 classical post-streptococcal glomerulonephritis in childhe
 presents as nephritic syndrome / ARFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Minimal change disease
 typically a child with nephrotic syndrome (accounts for 80%)he
 causes: Hodgkin's, NSAIDshe
 good response to steroidshe

he earaer aeraer asdsadas eerw dssdfsselleds

Focal segmental glomerulosclerosis
 may be idiopathic or secondary to HIV, heroinhe
 presentation: proteinuria / nephrotic syndrome / CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Rapidly progressive glomerulonephritis - aka crescentic glomerulonephritis
 rapid onset, often presenting as ARFhe
 causes include Goodpasture's, ANCA positive vasculitis, SLEhe

he earaer aeraer asdsadas eerw dssdfsselleds

Mesangiocapillary glomerulonephritis (membranoproliferative)
 type 1: cryoglobulinaemia, hepatitis Che
 type 2: partial lipodystrophyhe

Correct

47
57- A two-year old boy presents with an abdominal mass. Which of the
following is associated with Wilm's tumour (nephroblastoma)?ia

A.A Deletion on short arm of chromosome 12ia

B.A Tuberose sclerosisia
C.A Beckwith-Wiedemann syndromeia

D.A Autosomal dominant polycystic kidney diseaseia

E.A Autosomal recessive polycystic kidney diseaseia
(c)
Beckwith-Wiedemann syndrome is a inherited condition associated with
organomegaly, macroglossia, abdominal wall defects, Wilm's tumour and
neonatal hypoglycemia.

Wilm's tumour
sqweqwesf erwrewfsdfs adasd dhe
Wilm's nephroblastoma

 occurs mostly < 3 years (80% < 5 years); 20% of all childhood
malignancieshe

he earaer aeraer asdsadas eerw dssdfsselleds

Features
 abdo mass in otherwise well childhe
 also: painless haematuria, abdo pain, anorexia, BP, feverhe

he earaer aeraer asdsadas eerw dssdfsselleds

Associations
 Beckwith-Wiedemann syndromehe
 AGR triad of Aniridia, Genitourinary, Retardationhe
 deletion on short arm of chromosome 11he

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 USS --> nephrectomy, chemohe
 prognosis: good, 80% cure ratehe

Correct

58- Which one of the following is the most common type of SLE associated
renal disease?ia

A.A Class II: mesangial glomerulonephritisia

B.A Class III: focal (and segmental) proliferative
glomerulonephritisia
C.A Class IV: diffuse proliferative glomerulonephritisia

D.A Class V: diffuse membranous glomerulonephritisia

E.A Class VI: sclerosing glomerulonephritisia

(c)

48
SLE: renal complications
sqweqwesf erwrewfsdfs adasd dhe
WHO classification

 class I: normal kidneyhe

 class II: mesangial glomerulonephritishe
 class III: focal (and segmental) proliferative glomerulonephritishe
 class IV: diffuse proliferative glomerulonephritishe
 class V: diffuse membranous glomerulonephritishe
 class VI: sclerosing glomerulonephritishe

he earaer aeraer asdsadas eerw dssdfsselleds

Class IV (diffuse proliferative glomerulonephritis) is the most common and
severe form
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Management
 treat hypertensionhe
 corticosteroids if clinical evidence of diseasehe
 immunosuppressants e.g. azathiopine/cyclophosphamidehe

Correct

59- Alport's syndrome is associated with each one of the following, except:ia

A.A Chronic renal failureia

B.A Presentation in childhoodia
C.A Microscopic haematuriaia
D.A Lenticonusia
E.A Anosmiaia

(e)

Alport's syndrome
sqweqwesf erwrewfsdfs adasd dhe
Alport's syndrome is a hereditary condition, usually X-linked dominant but may
be autosomal recessive or dominant. It is due to a defect in the gene which codes
for type IV collagen resulting in an abnormal glomerular-basement membrane
(GBM). The disease is more severe in males with females rarely developing
renal failure
he earaer aeraer asdsadas eerw dssdfsselleds
A favourite question in the MRCP is an Alport's patient with a failing renal
transplant. This may be caused by the presence of anti-GBM antibodies leading
to a Goodpasture's syndrome like picture
he earaer aeraer asdsadas eerw dssdfsselleds
Alport's syndrome usually presents in childhood. The following features may be
seen:

 microscopic haematuriahe
 progressive renal failurehe
 bilateral sensorineural deafnesshe

49
  retinitis pigmentosahe
 lenticonus: protrusion of the lens surface into the anterior chamberh

Correct

60-Which one of the following statements is true regarding autosomal recessive

polycystic kidney disease?ia

A.A Onset is typically in the third decadeia

B.A Liver involvement is rareia
C.A Is due to a defect on chromosome 16ia
D.A More common than autosomal dominant polycystic kidney
diseaseia
E.A May be diagnosed on prenatal ultrasoundia

(e)

ARPKD
sqweqwesf erwrewfsdfs adasd dhe
Autosomal recessive polycystic kidney disease (ARPKD) is much less common
than autosomal dominant disease (ADPKD). It is due to a defect in a gene
located on chromosome 6
he earaer aeraer asdsadas eerw dssdfsselleds
Diagnosis may be made on prenatal ultrasound or in early infancy with
abdominal masses and renal failure. End-stage renal failure develops in
childhood. Patients also typically have liver involvement, for example portal and
interlobular fibrosis

Correct

61- A 24-year-old man who has a sister with polycystic kidney diseases asks his
GP if he could be screened for the disease. What is the most appropriate
screening test?ia

A.A PKD1 gene testingia

B.A CT abdomenia
C.A Urine microscopyia
D.A Ultrasound abdomenia

E.A Anti-polycystin 1 antibodies levelsia

(d)

Ultrasound is the screening test for adult polycystic kidney disease

ADPKD
sqweqwesf erwrewfsdfs adasd dhe
Autosomal dominant polycystic kidney disease (ADPKD) is the most common

50
inherited cause of kidney disease, affecting 1 in 1,000 Caucasians. Two disease
loci have been identified, PKD1 and PKD2, which code for polycystin-1 and
polycystin-2 respectively
he earaer aeraer asdsadas eerw dssdfsselleds

ADPKD type 1 ADPKD type 2

85% of cases 15% of cases
Chromosome 16 Chromosome 4
Presents with ESRF earlier

he earaer aeraer asdsadas eerw dssdfsselleds

The screening investigation for relatives is abdominal ultrasound:
he earaer aeraer asdsadas eerw dssdfsselleds
Ultrasound diagnostic criteria (in patients with positive family history)

 two cysts, unilateral or bilateral, if aged < 30 yearshe

 two cysts in both kidneys if aged 30-59 yearshe
 four cysts in both kidneys if aged > 60 yearshe

Correct

62- Each one of the following is a recognised side-effect of erythropoietin,

except:ia

A.A Hypertensionia
B.A Flu-like symptomsia
C.A Encephalopathyia
D.A Pure red cell aplasiaia
E.A Thrombocytopeniaia

(e)

Erythropoietin
sqweqwesf erwrewfsdfs adasd dhe
Erythropoietin is a haematopoietic growth factor that stimulates the production
of erythrocytes. The main uses of erythropoietin are to treat the anaemia
associated with chronic renal failure and that associated with cytotoxic therapy
he earaer aeraer asdsadas eerw dssdfsselleds
Side-effects of erythropoietin

 accelerated hypertension --> encephalopathy, seizures (blood pressure

increases in 25% of patients)he
 bone acheshe
 skin rashes, urticaria, flu-like symptomshe
 pure red cell aplasia (due to antibodies against erythropoietin)he
 raised PCV increases risk of thrombosis (e.g. fistula)he
 iron deficiency 2nd to increased erythropoiesishe

51
he earaer aeraer asdsadas eerw dssdfsselleds
There are a number of reasons why patients may failure to respond to
erythropoietin therapy
 iron deficiencyhe
 inadequate dosehe
 concurrent infection/inflammationhe
 hyperparathyroid bone diseasehe
 aluminium toxicityhe

Correct

63- Each one of the following is a recognised complication of nephrotic

syndrome, except:ia

A.A Hyperlipidaemiaia
B.A Acute renal failureia
C.A Increased risk of infectionia
D.A Hypercalcaemiaia

E.A Increased risk of thromboembolismia

(d)

Nephrotic syndrome: complications

sqweqwesf erwrewfsdfs adasd dhe
Complications

 increased risk of infection due to urinary immunoglobulin losshe

 increased risk of thromboembolism related to loss of antithrombin III
and plasminogen in the urinehe
 hyperlipidaemiahe
 hypocalcaemia (vitamin D and binding protein lost in urine)he
 acute renal failurehe

Correct

64- Each one of the following is a cause of sterile pyuria, except:ia

A.A Renal stonesia

B.A Acute glomerulonephritisia

C.A Renal TBia

D.A Bladder/renal cell canceria
E.A Appendicitisia

(b)

52
Sterile pyuria
sqweqwesf erwrewfsdfs adasd dhe
Causes

 partially treated UTIhe

 renal TBhe
 appendicitishe
 bladder/renal cell cancerhe
 calculihe
 adult polycystic kidney diseasehe

Correct

65- Each one of the following is typically seen in patients with rhabdomyolysis,
except:ia

A.A Elevated ureaia

B.A Hypercalcaemiaia

C.A Elevated serum phosphateia

D.A Elevated creatinine kinaseia
E.A Myoglobinuriaia

(b)

Rhabdomyolysis
sqweqwesf erwrewfsdfs adasd dhe
Rhabdomyolysis will typically feature in the exam as a patient who has had a
fall or prolonged epileptic seizure and is found to have acute renal failure on
admission
he earaer aeraer asdsadas eerw dssdfsselleds
Features

 acute renal failure with disproportionately raised creatininehe

 elevated CKhe
 myoglobinuriahe
 hypocalcaemia (myoglobin binds calcium)he
 elevated phosphate (released from myocytes)he

he earaer aeraer asdsadas eerw dssdfsselleds

Causes
 seizurehe
 collapse/coma (e.g. elderly patients collapses at home, found 8 hours
later)he
 ecstasyhe
 crush injuryhe
 McArdle's syndromehe
 drugs: statinshe

he earaer aeraer asdsadas eerw dssdfsselleds

Management

53
  IV fluids to maintain good urine outputhe
 urinary alkalinization is sometimes usedhe

Correct

66- Which one of the following is not a feature of HIV-associated nephropathy?

ia

A.A Small kidneysia

B.A Normotensionia
C.A Elevated urea and creatinineia
D.A Proteinuriaia
E.A Focal segmental glomerulosclerosis on renal biopsyia

(a)

HIV: renal involvement

sqweqwesf erwrewfsdfs adasd dhe
Renal involvement in HIV patients may occur as a consequence of treatment or
the virus itself. Protease inhibitors such as indinavir can precipitate intratubular
crystal obstruction
he earaer aeraer asdsadas eerw dssdfsselleds
HIV-associated nephropathy (HIVAN) accounts for up to 10% of end-stage
renal failure cases in the United States. Antiretroviral therapy has been shown to
alter the course of the disease. There are five key features of HIVAN:

 massive proteinuriahe
 normal or large kidneyshe
 focal segmental glomerulosclerosis with focal or global capillary
collapse on renal biopsyhe
 elevated urea and creatininehe
 normotensionh

Correct

67- Which one of the following may be useful in the prevention of oxalate renal
stones?ia

A.A Ferrous sulphateia

B.A Thiazide diureticsia
C.A Lithiumia
D.A Pyridoxineia

E.A Allopurinolia

(d)

54
Renal stones: management
sqweqwesf erwrewfsdfs adasd dhe
Calcium stones

 high fluid intakehe

 low animal protein, low salt diet (a low calcium diet has not been shown
to be superior to a normocalcaemic diet)he
 thiazide diuretics (reduce distal tubule calcium resorption)he
 stones < 5 mm will usually pass spontaneouslyhe
 lithotripsy, nephrolithotomy may be requiredhe

he earaer aeraer asdsadas eerw dssdfsselleds

Oxalate stones
 cholestyramine reduces urinary oxalate secretionhe
 pyridoxine reduces urinary oxalate secretionhe

he earaer aeraer asdsadas eerw dssdfsselleds

Uric acid stones
 allopurinolhe
 urinary alkalinization e.g. oral bicarbonateh

Correct

68- A patient with type 1 diabetes mellitus is reviewed in the nephrology

outpatient clinic. He is known to have stage 3 diabetic nephropathy. Which of
the following best describes his degree of renal involvement?ia

A.A Overt nephropathyia

B.A Microalbuminuriaia

C.A Latent phaseia

D.A End-stage renal failureia
E.A Hyperfiltrationia

(b)

Diabetic nephropathy: stages

sqweqwesf erwrewfsdfs adasd dhe
Diabetic nephropathy may be classified as occurring in five stages*:
he earaer aeraer asdsadas eerw dssdfsselleds
Stage 1

 hyperfiltration: increase in GFRhe

 may be reversiblehe

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 2 (silent or latent phase)
 most patients do not develop microalbuminuria for 10 yearshe
 GFR remains elevatedhe

55
he earaer aeraer asdsadas eerw dssdfsselleds
Stage 3 (incipient nephropathy)
 microalbuminuria (albumin excretion of 30 - 300 mg/day, dipstick
negative)he

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 4 (overt nephropathy)
 persistent proteinuria (albumin excretion > 300 mg/day, dipstick
positive)he
 hypertension is present in most patientshe
 histology shows diffuse glomerulosclerosis and focal glomerulosclerosis
(Kimmelstiel-Wilson nodules)he

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 5
 end-stage renal disease, GFR typically < 10ml/minhe
 renal replacement therapy neededhe

he earaer aeraer asdsadas eerw dssdfsselleds

The timeline given here is for type 1 diabetics. Patients with type 2 diabetes
mellitus (T2DM) progress through similar stages but in a different timescale -
some T2DM patients may progress quickly to the later stages

Correct

69- Which one of the following is least recognised as a cause of membranous

glomerulonephritis?ia

A.A Streptococcal infectionia

B.A Penicillamineia
C.A Hepatitis Bia
D.A SLEia
E.A Lymphomaia

(a)

Membranous glomerulonephritis
sqweqwesf erwrewfsdfs adasd dhe
Membranous glomerulonephritis is the commonest type of glomerulonephritis in
adults and is the third most common cause of end-stage renal failure (ESRF). It
usually presents as nephrotic syndrome or proteinuria
he earaer aeraer asdsadas eerw dssdfsselleds
Renal biopsy demonstrates:

 sub-epithelial immune complex (mainly IgG and C3) deposition in the

glomerulushe
 electron microscopy: the basement membrane is thickened with sub-
epithelial electron dense depositshe

56
he earaer aeraer asdsadas eerw dssdfsselleds
Causes
 idiopathiche
 infections: hepatitis B, malariahe
 malignancy: lung cancer, lymphoma, leukaemiahe
 drugs: gold, penicillamine, NSAIDshe
 systemic lupus erythematous (class V disease)he

he earaer aeraer asdsadas eerw dssdfsselleds

Prognosis - rule of thirds
 one-third: spontaneous remission he
 one-third: remain proteinuriche
 one-third: develop ESRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 immunosuppression: steroids, chlorambucil e.g. Ponticelli regimehe
 BP controlhe
 consider anticoagulationhe

Correct

70- Which one of the following types of glomerulonephritis is most

characteristically associated with cryoglobulinaemia?ia

A.A Rapidly progressive glomerulonephritisia

B.A Mesangiocapillary glomerulonephritisia

C.A Focal segmental glomerulosclerosisia

D.A IgA nephropathyia
E.A Diffuse proliferative glomerulonephritisia

(b)

Glomerulonephritides
sqweqwesf erwrewfsdfs adasd dhe
Knowing a few key facts is the best way to approach the difficult subject of
glomerulonephritis:
he earaer aeraer asdsadas eerw dssdfsselleds
Membranous glomerulonephritis

 presentation: proteinuria / nephrotic syndrome / CRFhe

 cause: infections, rheumatoid drugs, malignancyhe
 1/3 resolve, 1/3 respond to cytotoxics, 1/3 develop CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

IgA nephropathy - aka Berger's disease, mesangioproliferative GN
 typically young adult with haematuria following an URTIhe

he earaer aeraer asdsadas eerw dssdfsselleds

Diffuse proliferative glomerulonephritis

57
  classical post-streptococcal glomerulonephritis in childhe
 presents as nephritic syndrome / ARFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Minimal change disease
 typically a child with nephrotic syndrome (accounts for 80%)he
 causes: Hodgkin's, NSAIDshe
 good response to steroidshe

he earaer aeraer asdsadas eerw dssdfsselleds

Focal segmental glomerulosclerosis
 may be idiopathic or secondary to HIV, heroinhe
 presentation: proteinuria / nephrotic syndrome / CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Rapidly progressive glomerulonephritis - aka crescentic glomerulonephritis
 rapid onset, often presenting as ARFhe
 causes include Goodpasture's, ANCA positive vasculitis, SLEhe

he earaer aeraer asdsadas eerw dssdfsselleds

Mesangiocapillary glomerulonephritis (membranoproliferative)
 type 1: cryoglobulinaemia, hepatitis Che
 type 2: partial lipodystrophyhe

Correct

71- Which of the following types of renal tubular acidosis is most likely to cause
renal stones?ia

A.A Type 1 renal tubular acidosisia

B.A Type 2 renal tubular acidosisia

C.A Type 3 renal tubular acidosisia
D.A Type 4 renal tubular acidosisia
E.A Type 5 renal tubular acidosisia

(a)

Renal tubular acidosis

sqweqwesf erwrewfsdfs adasd dhe
All three types of renal tubular acidosis (RTA) are associated with
hyperchloraemic metabolic acidosis (normal anion gap)
he earaer aeraer asdsadas eerw dssdfsselleds
Type 1 RTA (distal)

 inability to generate acid urine (secrete H+) in distal tubulehe

 causes hypokalaemiahe
 complications include nephrocalcinosis and renal stoneshe
 causes include idiopathic, RA, SLE, Sjogren'she

58
he earaer aeraer asdsadas eerw dssdfsselleds
Type 2 RTA (proximal)
 decreased HCO3- reabsorption in proximal tubulehe
 causes hypokalaemiahe
 complications include osteomalaciahe
 causes include idiopathic, as part of Fanconi syndrome, Wilson's disease,
cystinosis, outdated tetracyclineshe

he earaer aeraer asdsadas eerw dssdfsselleds

Type 4 RTA (hyperkalaemic)
 causes hyperkalaemiahe
 causes include hypoaldosteronism, diabetesh

Correct

72- Which one of the following is not a risk factor for the development of
calcium oxalate and calcium phosphate renal stones?ia

A.A Bendrofluazideia

B.A Aminophyllineia
C.A Acetazolamideia
D.A Frusemideia
E.A Prednisoloneia
(a)
Bendrofluazide may help prevent the formation of calcium based renal stones. It
may however theoretically increase the risk of urate based stones

Renal stones: risk factors

sqweqwesf erwrewfsdfs adasd dhe
Risk factors

 dehydrationhe
 hypercalciuria, hyperparathyroidism, hypercalcaemiahe
 cystinuriahe
 high dietary oxalatehe
 renal tubular acidosishe
 medullary sponge kidney, polycystic kidney diseasehe
 beryllium or cadmium exposurehe

he earaer aeraer asdsadas eerw dssdfsselleds

Risk factors for urate stones
 gouthe
 ileostomy: loss of bicarbonate and fluid results in acidic urine, causing
the precipitation of uric acidhe

he earaer aeraer asdsadas eerw dssdfsselleds

Drug causes
 drugs that promote calcium stones: loop diuretics, steroids,
acetazolamide, theophyllinehe

59
  thiazides can prevent calcium stones (increase distal tubular calcium
resorption)he

Correct

73- The albumin:creatinine excretion ratio (ACR) may be used to quantify the
degree of proteinuria in renal disease. A normal ACR may be defined as:ia

A.A 2.5 - 5ia

B.A < 0.25ia
C.A < 2.5ia

D.A 5 – 50ia
E.A < 25ia

(c)

Proteinuria
sqweqwesf erwrewfsdfs adasd dhe
Microalbuminuria

 defined as an albumin excretion of 30 - 300 mg/dayhe

he earaer aeraer asdsadas eerw dssdfsselleds

Albumin:creatinine excretion ratio (ACR)
 used in clinical practice to quantify degree of proteinuriahe
 first morning urine samplehe
 urine albumin (mg) / creatinine (mmol)he
 normal ACR < 2.5he
 microalbuminuric range = 2.5 - 33he

Correct

74- Which one of the following is least associated with minimal change
glomerulonephritis?ia

A.A Hodgkin's lymphomaia

B.A Goodpasture's syndromeia

C.A Thymomaia
D.A Non-steroidal anti-inflammatory drugsia
E.A Rifampicinia
(b)
Goodpasture's syndrome is associated with rapidly progressive
glomerulonephritis

Minimal change glomerulonephritis

sqweqwesf erwrewfsdfs adasd dhe
Minimal change glomerulonephritis nearly always presents as nephrotic
syndrome, accounting for 75% of cases in children and 25% in adults

60
he earaer aeraer asdsadas eerw dssdfsselleds
The majority of cases are idiopathic, but in around 10-20% a cause is found:

 drugs: NSAIDs, rifampicinhe

 Hodgkin's lymphoma, thymomahe
 infectious mononucleosishe

he earaer aeraer asdsadas eerw dssdfsselleds

Features
 nephrotic syndromehe
 hypertensionhe
 highly selective proteinuriahe
 renal biopsy: electron microscopy shows fusion of podocytes he

he earaer aeraer asdsadas eerw dssdfsselleds

Management
 majority of cases (80%) are steroid responsivehe
 cyclophosphamide is the next step for steroid resistant caseshe
 good prognosishe

Correct

75- Which one of the following causes of glomerulonephritis is associated with

low complement levels?ia

A.A IgA nephropathyia

B.A Membranous glomerulonephritisia
C.A Minimal change diseaseia
D.A Post-streptococcal glomerulonephritisia

E.A Focal segmental glomerulosclerosisia

(d)

Glomerulonephritis and low complement

sqweqwesf erwrewfsdfs adasd dhe
Disorders associated with glomerulonephritis and low serum complement levels

 post-streptococcal glomerulonephritishe
 subacute bacterial endocarditishe
 systemic lupus erythematoushe
 mesangiocapillary glomerulonephritishe

Correct

76- A 14-year-old boy develops haematuria following an upper respiratory tract

infection. What is the likely diagnosis?ia

A.A IgA nephropathyia

B.A Focal segmental glomerulosclerosisia

61
 C.A Diffuse proliferative glomerulonephritisia
D.A Rapidly progressive glomerulonephritisia
E.A Mesangiocapillary glomerulonephritisia

(a)

Glomerulonephritides
sqweqwesf erwrewfsdfs adasd dhe
Knowing a few key facts is the best way to approach the difficult subject of
glomerulonephritis:
he earaer aeraer asdsadas eerw dssdfsselleds
Membranous glomerulonephritis

 presentation: proteinuria / nephrotic syndrome / CRFhe

 cause: infections, rheumatoid drugs, malignancyhe
 1/3 resolve, 1/3 respond to cytotoxics, 1/3 develop CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

IgA nephropathy - aka Berger's disease, mesangioproliferative GN
 typically young adult with haematuria following an URTIhe

he earaer aeraer asdsadas eerw dssdfsselleds

Diffuse proliferative glomerulonephritis
 classical post-streptococcal glomerulonephritis in childhe
 presents as nephritic syndrome / ARFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Minimal change disease
 typically a child with nephrotic syndrome (accounts for 80%)he
 causes: Hodgkin's, NSAIDshe
 good response to steroidshe

he earaer aeraer asdsadas eerw dssdfsselleds

Focal segmental glomerulosclerosis
 may be idiopathic or secondary to HIV, heroinhe
 presentation: proteinuria / nephrotic syndrome / CRFhe

he earaer aeraer asdsadas eerw dssdfsselleds

Rapidly progressive glomerulonephritis - aka crescentic glomerulonephritis
 rapid onset, often presenting as ARFhe
 causes include Goodpasture's, ANCA positive vasculitis, SLEhe

he earaer aeraer asdsadas eerw dssdfsselleds

Mesangiocapillary glomerulonephritis (membranoproliferative)
 type 1: cryoglobulinaemia, hepatitis Che
 type 2: partial lipodystrophyhe

Correct

77- Which of the following types of renal tubular acidosis is most likely to cause
osteomalacia?ia

62
 A.A Type 1 renal tubular acidosisia
B.A Type 2 renal tubular acidosisia

C.A Type 3 renal tubular acidosisia

D.A Type 4 renal tubular acidosisia
E.A Type 5 renal tubular acidosisia

(b)

Renal tubular acidosis

sqweqwesf erwrewfsdfs adasd dhe
All three types of renal tubular acidosis (RTA) are associated with
hyperchloraemic metabolic acidosis (normal anion gap)
he earaer aeraer asdsadas eerw dssdfsselleds
Type 1 RTA (distal)

 inability to generate acid urine (secrete H+) in distal tubulehe

 causes hypokalaemiahe
 complications include nephrocalcinosis and renal stoneshe
 causes include idiopathic, RA, SLE, Sjogren'she

he earaer aeraer asdsadas eerw dssdfsselleds

Type 2 RTA (proximal)
 decreased HCO3- reabsorption in proximal tubulehe
 causes hypokalaemiahe
 complications include osteomalaciahe
 causes include idiopathic, as part of Fanconi syndrome, Wilson's disease,
cystinosis, outdated tetracyclineshe

he earaer aeraer asdsadas eerw dssdfsselleds

Type 4 RTA (hyperkalaemic)
 causes hyperkalaemiahe
 causes include hypoaldosteronism, diabeteshe

Incorrect

78- Autosomal dominant polycystic kidney disease type 2 is associated with a

gene defect in:ia

A.A Chromosome 4ia

B.A Chromosome 8ia

C.A Chromosome 12ia
D.A Chromosome 16ia

E.A Chromosome 20ia

Correct (A)

ADPKD type 2 = chromosome 4 = 15% of cases

63
ADPKD
sqweqwesf erwrewfsdfs adasd dhe
Autosomal dominant polycystic kidney disease (ADPKD) is the most common
inherited cause of kidney disease, affecting 1 in 1,000 Caucasians. Two disease
loci have been identified, PKD1 and PKD2, which code for polycystin-1 and
polycystin-2 respectively
he earaer aeraer asdsadas eerw dssdfsselleds

ADPKD type 1 ADPKD type 2

85% of cases 15% of cases
Chromosome 16 Chromosome 4
Presents with ESRF earlier

he earaer aeraer asdsadas eerw dssdfsselleds

The screening investigation for relatives is abdominal ultrasound:
he earaer aeraer asdsadas eerw dssdfsselleds
Ultrasound diagnostic criteria (in patients with positive family history)

 two cysts, unilateral or bilateral, if aged < 30 yearshe

 two cysts in both kidneys if aged 30-59 yearshe
 four cysts in both kidneys if aged > 60 yearshe

Correct

79- Which of the following types of renal stones are radio-lucent?ia

A.A Triple phosphate stonesia

B.A Cystine stonesia
C.A Calcium phosphateia
D.A Xanthine stonesia

E.A Calcium oxalateia

(d)

Renal stones on x-ray

 cystine stones: semi-opaque

 urate + xanthine stones: radio-lucent

Renal stones: imaging

sqweqwesf erwrewfsdfs adasd dhe
The table below summarises the appearance of different types of renal stone on
x-ray
he earaer aeraer asdsadas eerw dssdfsselleds

Type Frequency Radiograph appearance

Calcium oxalate 40% Opaque

64
Mixed calcium 25% Opaque
oxalate/phosphate stones
Triple phosphate stones 10% Opaque
Calcium phosphate 10% Opaque
Urate stones 5-10% Radio-lucent
Cystine stones 1% Semi-opaque, 'ground-glass'
appearance
Xanthine stones <1% Radio-lucent

Correct

80- A patient with type 1 diabetes mellitus is reviewed in the nephrology

outpatient clinic. He is known to have stage 2 diabetic nephropathy. Which of
the following best describes his degree of renal involvement?ia

A.A Microalbuminuriaia
B.A End-stage renal failureia
C.A Latent phaseia

D.A Hyperfiltrationia
E.A Overt nephropathyia

(c)

Diabetic nephropathy: stages

sqweqwesf erwrewfsdfs adasd dhe
Diabetic nephropathy may be classified as occurring in five stages*:
he earaer aeraer asdsadas eerw dssdfsselleds
Stage 1

 hyperfiltration: increase in GFRhe

 may be reversiblehe

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 2 (silent or latent phase)
 most patients do not develop microalbuminuria for 10 yearshe
 GFR remains elevatedhe

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 3 (incipient nephropathy)
 microalbuminuria (albumin excretion of 30 - 300 mg/day, dipstick
negative)he

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 4 (overt nephropathy)
 persistent proteinuria (albumin excretion > 300 mg/day, dipstick
positive)he

65
  hypertension is present in most patientshe
 histology shows diffuse glomerulosclerosis and focal glomerulosclerosis
(Kimmelstiel-Wilson nodules)he

he earaer aeraer asdsadas eerw dssdfsselleds

Stage 5
 end-stage renal disease, GFR typically < 10ml/minhe
 renal replacement therapy neededhe

he earaer aeraer asdsadas eerw dssdfsselleds

The timeline given here is for type 1 diabetics. Patients with type 2 diabetes
mellitus (T2DM) progress through similar stages but in a different timescale -
some T2DM patients may progress quickly to the later stages

Incorrect

81- Which of the following factors would suggest that a patient has pre-renal
uraemia rather than established acute tubular necrosis?ia

A.A Urine sodium = 70 mmol/Lia

B.A Fractional urea excretion = 20%ia

C.A No response to fluid challengeia

D.A Urine:plasma urea ratio 5:1ia
E.A Specific gravity = 1005ia

Correct (B)

End session

ATN or prerenal uraemia? In prerenal uraemia think of the kidneys holding on to

sodium to preserve volume

ARF: ATN vs. prerenal uraemia

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Prerenal uraemia - kidneys hold on to sodium to preserve volume
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Pre-renal uraemia Acute tubular necrosis

Urine sodium < 20 mmol/L > 30 mmol/L

Fractional sodium excretion* < 1% > 1%

Fractional urea excretion** < 35% >35%

Urine:plasma osmolality > 1.5 < 1.1

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Urine:plasma urea > 10:1 < 8:1
Specific gravity > 1020 < 1010
Urine 'bland' sediment brown granular casts
Response to fluid challenge Yes No

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*fractional sodium excretion = (urine sodium/plasma sodium) / (urine
creatinine/plasma creatinine) x 100
he earaer aeraer asdsadas eerw dssdfsselleds
**fractional urea excretion = (urine urea /blood urea ) / (urine creatinine/plasma
creatinine) x 100

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