nephrology PT 1 2016 modif

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Question 1 of 316

A week after an episode of infective diarrhoea, a 10-year-old child presents with fever,
hypertension and haematuria. You understand that he had been on a school visit to a model
farm a short period before he became unwell. Blood tests reveal anaemia with an Hb of 8.4
g/dl, and an elevated creatinine of 185 μmol/l.
A possible diagnosis could be:
A

Acute interstitial nephritis

B

Haemolytic–uraemic syndrome

C

Henoch–Schönlein purpura

D

Membranoproliferative glomerulonephritis

E

Post-infectious glomerulonephritis

Explanation



The answer is Haemolytic–uraemic syndrome –
Haemolytic–uraemic syndrome is characterised by microangiopathic haemolytic anaemia
and varying degrees of acute renal failure.
In many cases, an infectious or immune-complex mediated cause has been proposed.
Epidemics of bloody diarrhoea related to infection with a specific strain of
Escherichia coli (O157:H7) have been documented. The bacteria release a verotoxin
that damages endothelial cells, particularly those of the kidney.
The visit to the farm implies haemolytic-uraemic syndrome is the most likely cause
as opposed to other inherent or acquired pathology due to medications/infections
(the other options).

Acute interstitial nephritis (Option A) is incorrect. The farm visit makes HUS the most likely
cause.
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Henoch–Schönlein purpura (Option C) is incorrect. The farm visit makes HUS the most likely
cause.
Membranoproliferative glomerulonephritis (Option D) is incorrect. The farm visit makes HUS

the most likely cause.
Post-infectious glomerulonephritis (Option E) is incorrect. The farm visit makes HUS the
most likely cause.
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Question 2 of 316

A 55-year-old man presents with pain in his right flank, nephrotic syndrome, azotaemia,
collateral abdominal veins and gross haematuria. On examination, a mass is palpable in the
right lumbar area. Which one of the following is the most probable diagnosis?
A

Renal amyloidosis

B

Polyarteritis nodosa

C

Renal papillary necrosis

D

Renal cell carcinoma

E

Polycystic kidneys

Explanation
Renal-cell carcinoma (hypernephroma)
Diagnosis
The classic triad of haematuria, loin pain and abdominal mass, and the other clinical

features present in this case are suggestive of renal-cell carcinoma
Pathological consequences
Renal-cell carcinoma is a highly vascular tumour and can cause obstruction of the renal
veins
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Question 3 of 316

A 68-year-old man has a new-patient screen carried out by his GP. He is noted to have
microscopic haematuria. His GP also notes a raised ESR and a calcium concentration of 3.1
mmol/l. On examination he appears to have a varicocele. Which one of the following
diagnoses fits best with this clinical picture?
A

Transitional-cell carcinoma

B

Wilms’ tumour

C

Retroperitoneal sarcoma

D

Renal-cell adenocarcinoma

E

Urinary tract infection

Explanation
Renal-cell adenocarcinoma
Epidemiology
Renal-cell adenocarcinoma makes up 3% of all adult malignancies, with peak incidence
being 50–70 years
There is a male to female preponderance of 2:1
Clinical features
Abdominal mass a presenting feature in 25–45% of cases
Varicocele, resulting from obstruction of venous drainage, is found in 2–3% of cases
Laboratory features
Haematuria is the presenting feature in 50–60% of cases
Raised erythrocyte sedimentation rate (ESR) in found in 50–60% of cases
Hypercalcaemia occurs due to raised parathyroid-related protein in around 3% of cases
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Imaging studies
Ultrasound scan, iv pyelography (IVP), abdominal computed tomography (CT) with
contrast and magnetic resonance imaging (MRI)
Pattern of metastatic spread

Commonest sites of metastases are lung (50–60%) and bone (30–40%)
Treatment and prognosis
Treatment of choice is nephrectomy
Prognosis is related to tumour staging: the 5-year survival rate is around 80–100% in
those with TNM stage-1 lesions, but this falls to 5–10% in those with stage-4 lesions
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Question 4 of 316

Which one of the following statements is true regarding retroperitoneal fibrosis?
A

Bilateral swelling of the legs is often due to inferior vena cava obstruction

B

Hashimoto’s thyroiditis is a recognised association

C

Low back pain is the most common presenting symptom

D

Pizotifen (migraine treatment) is implicated in causing similar disease

E

Renal failure is due to fibrous tissue infiltrating the kidneys

Explanation



The answer is Low back pain is the most common presenting symptom –
Retroperitoneal fibrosis is one of the multifocal fibrosclerotic syndromes, which also
includes mediastinal fibrosis, sclerosing cholangitis and Riedel’s thyroiditis. It is more
common in males (2:1), with peak incidence in the fifth and sixth decades. The process
usually begins over the promontory of the sacrum and extends laterally across the
ureters and as high as the second or third lumbar vertebra. Hence low back is the most
common presenting symptom. This may be accompanied by fever and weight loss.
Diagnosis is suggested by the finding at intravenous pyelography of displacement of the
ureters toward the midline.
Thromboembolism and hypertension are recognised complications.
Methysergide, a semi-synthetic ergot alkaloid used to treat migraine headache, can cause
a similar syndrome. Other drugs such as ß-blockers (methyldopa and hydralazine) are
also implicated.

Bilateral swelling of the legs is often due to inferior vena cava obstruction (Option A) is
incorrect. The fibrosing process may surround the inferior vena cava, but obstruction of that
vessel is uncommon.

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Hashimoto’s thyroiditis is a recognised association (Option B) is incorrect. Systemic diseases
associated with retroperitoneal fibrosis include systemic lupus erythematosus (SLE),
scleroderma and carcinoid syndrome, not Hashimoto’s.
Pizotifen (migraine treatment) is implicated in causing similar disease (Option D) is incorrect.

Pizotifen is an antihistamine and serotonin antagonist structurally related to the tricyclic
antidepressants; its use is not associated with retroperitoneal fibrosis.
Renal failure is due to fibrous tissue infiltrating the kidneys (Option E) is incorrect. The
fibrous tissue does not infiltrate the kidneys or the ovaries.
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Question 5 of 316

A 55-year-old man presents with weakness, general malaise and ankle swelling. He has been
unwell for 4 months, the ankle swelling having developed quite rapidly over the previous 3
weeks. On examination he has marked peripheral oedema and 3+ proteinuria without
haematuria on dipstick urinalysis. His daily urine albumin excretion rate is measured at 6.9
g/24 h, creatinine is 130 μmol/l, serum albumin is 12 g/dl and Hb is 8.9 g/dl. Further
investigation reveals a paraprotein band in his serum with kappa light chains demonstrated
on immunofixation. Which one of the following is the most probable cause of the proteinuria?
A

Cast nephropathy

B

IgA nephropathy

C

Light-chain deposition disease

D

Membranous nephropathy

E

Minimal-change disease

Explanation
Renal disease in myeloma
The spectrum of renal disease in myeloma comprises a spectrum of presentations
including;
Cast nephropathy (presents as progressive renal insufficiency with Bence Jones
proteinuria – undetectable on urine dipstick – but little in the way of albuminuria)
Renal amyloidosis AL (nephrotic syndrome and mild renal insufficiency,
associated with glomerular lambda light-chain deposition)
Cryoglobulinaemic glomerulonephritis (mild to severe nephrotic syndrome,
haematuria is a clue) and light-chain deposition disease
Cryoglobulinaemic glomerulonephritis is almost invariably associated with kappa lightchain deposition and rarely coexists with renal amyloid
These two entities present with the nephrotic syndrome, thereby distinguishing them
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sharply from the much more common cast nephropathy, which is caused by
widespread precipitation of light chains in association with Tamm–Horsfall glycoprotein
in the tubular lumen
Light-chain deposition disease results in mild renal insufficiency (as in this question),
whereas cast nephropathy can cause severe renal failure, which is frequently irreversible
The principal clue to the diagnosis of cast nephropathy is the absence of significant
albuminuria (detected with urine dipstick) in the presence of significant proteinuria as
measured by formal 24-h collection (due to filtered light chains)
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Question 6 of 316

An 82-year-old man presents to his GP with increasing oedema and ascites. He is

hypertensive, for which he takes amlodipine. There is shortness of breath on exercise. His
alcohol history is two cans of stout per day. ECG is normal, and CXR reveals normal heart size
and no signs of cardiac failure. Serum albumin is 23 g/dl; urinary albumin excretion is 7 g/24
h, with no haematuria. He has mild anaemia with a normal MCV. Total cholesterol is elevated.
Which one of the following diagnoses fits best with this clinical picture?
A

Cardiac failure

B

Cirrhosis

C

Nephritic syndrome

D

Nephrotic syndrome

E

Polyarteritis nodosa

Explanation
Nephrotic syndrome
Diagnosis
Low albumin, abnormal cholesterol and increased urinary albumin excretion all point
towards nephrotic syndrome

Aetiology
In the older age group, membranous nephropathy is the commonest cause
Although it may be idiopathic, it can occur in association with Hodgkin’s lymphoma,
carcinoma, systemic lupus erythematosus (SLE) or gold therapy
Minimal-change nephropathy occurs more commonly in children
Treatment

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Treatment involves a low-salt diet and fluid restriction, with the use of high-dose
furosemide
Angiotensin-converting enzyme (ACE) inhibitors are the treatment of choice for
hypertension in this group
Prednisolone at a dose of 1-2 mg/kg per day may induce remission in those with
membranous glomerulonephritis, (maximum usually 80 mg/day)
The grossly abnormal cholesterol picture requires aggressive management with statin
therapy
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Question 7 of 316

A 36-year-old man with a history of intravenous drug use is found to have dipstick-positive
haematuria. His blood pressure is 170/90 mmHg, he appears clinically well and he has a trace
of peripheral oedema. Plasma creatinine is 140 μmol/l, bilirubin is 65 μmol/l, AST is 78 IU/l
and his 24-h urinary protein excretion rate is 4.1 g/24 h. Microscopy of the spun urine
sediment reveals the presence of red-cell casts. Complement C3 is 0.5 (0.7–1.3) and C4 is
0.09 (0.12–0.27). Which one of the following is the aetiology of the renal abnormalities?
A

Hepatorenal syndrome

B

Infection with hepatitis C

C

Infection with HIV

D

Infectious endocarditis

E

Renal emboli

Explanation
Mesangiocapillary glomerulonephritis
Aetiology
Hepatitis C is now considered the principal cause of ‘idiopathic’ mesangiocapillary
glomerulonephritis (MCGN), an immune-complex deposition disorder that presents with
a mixed nephritic–nephrotic picture associated with pan-hypocomplementaemia;
hepatitis C is endemic among the iv drug-using population. It is also closely associated
with the development of cryoglobulinaemia
Other chronic sources of immune complexes, such as hepatitis B, schistosomiasis,
chronic abscess, malaria and leprosy, may also be associated with MCGN
Differential diagnosis

Infectious endocarditis is the principal differential diagnosis and may present with
haematuria and hypocomplementaemia, although hypertension is unusual and other
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manifestations of infection, such as fever, would usually be present
HIV nephropathy is suggested by the presence of nephrotic syndrome, rapidly
progressive renal impairment and large kidneys on ultrasound
Renal emboli tend to present with flank pain and haematuria
Hepatorenal syndrome occurs in patients with advanced cirrhosis with marked
peripheral oedema and cirrhosis
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Question 8 of 316

A 7-year-old boy presents with generalised oedema. Urinalysis shows marked albuminuria.
Blood tests reveal hypoalbuminaemia and hyperlipidaemia. A renal biopsy appears normal on
light microscopy. Which one of the following would be the most characteristic finding on
electron microscopy?
A

Deposition of electron-dense material on the capillary basement membrane

B

Splitting of the capillary basement membrane

C

Fusion of foot processes of the glomerular epithelial cells

D

Thinning of the capillary basement membrane

E

Fibrils of amyloid protein in the mesangium

Explanation
Minimal-change nephropathy
Complications
Minimal-change nephropathy is the most common cause of nephrotic syndrome in
children
Electron microscopic diagnosis
There is loss of foot processes – these may also be seen in other proteinuric states such
as membranous glomerulonephritis and diabetic nephropathy, but light microscopic
changes would also be evident in these conditions
Splitting of the capillary basement membrane is seen in mesangiocapillary
glomerulonephritis, while thinning is noted in thin glomerular basement membrane
disease
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Question 9 of 316

A 35-year-old woman who has two children and has had one stillbirth and one miscarriage
now presents with 8 weeks’ amenorrhoea. There is a history of Raynaud’s phenomenon and
dysphagia. On examination, her blood pressure is 170/120 mmHg. Ulcers are noted in the
index and middle fingers of both hands. The doctor advises her not to continue with the
pregnancy. Which one of the following conditions is she most likely to be having?
A

Systemic lupus erythematosus

B

Autosomal-dominant polycystic kidney disease

C

Diffuse systemic sclerosis

D

Diabetic nephropathy

E

Antiphospholipid syndrome

Explanation
Pregnancy complications in systemic disease
Diffuse systemic sclerosis is associated with maternal deaths; reactivation of quiescent
scleroderma can occur during pregnancy and the puerperium and the fetal prognosis is
poor. Therapeutic abortion must be considered in these patients.

In diabetic nephropathy, pregnancy does not accelerate functional loss
The prognosis is most favourable in systemic lupus erythematosus (SLE) if the disease
has been in remission for at least 6 months before conception
Antiphospholipid syndrome may cause recurrent miscarriage but is not an indication
for termination of pregnancy
Autosomal-dominant polycystic kidney disease (ADPKD) is again a condition where
pregnancy is not contraindicated, but blood pressure and renal functions must be
carefully monitored throughout pregnancy
This woman, however, does not have the features of ADPKD
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Question 10 of 316

A middle-aged man with chronic renal failure is diagnosed as having pulmonary tuberculosis.
His creatinine clearance is 50 ml/min. Which one of the following drugs can be administered
with no change in the dosage?
A

Isoniazid

B

Streptomycin

C

Rifampicin

D

Ethambutol

E

Pyrazinamide

Explanation
Renal insufficiency and anti-tubercular treatment
If the excretion of anti-tubercular drugs is hampered because of renal insufficiency they
may cause serious side-effects, for example:
isoniazid may cause hepatitis and polyneuropathy
pyrazinamide may precipitate hyperuricaemic gout and cause hepatic toxicity
ethambutol can cause dose-related optic retrobulbar neuritis
streptomycin can cause irreversible damage to the vestibular nerve
However, rifampicin is only mildly hepatotoxic and so it is a safe and effective drug for
the treatment of tuberculosis in the presence of renal insufficiency. It should be
stopped only if the serum bilirubin becomes elevated or transferases are elevated more
than threefold, which is extremely rare
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Question 11 of 316

You are asked to review a man who is being considered for peritoneal dialysis for chronic
renal failure. He has a number of concerns before proceeding to surgery and placement of a
Tenckhoff catheter. Regarding pitfalls and complications of peritoneal dialysis, which of the
following is a key consideration?
A

Diabetes treatment may need to be adjusted once dialysis is commenced

B

Abdominal hernias do not need to be repaired before commencing dialysis

C

Stomas rarely present a problem when considering catheter placement

D

Staphylococcus epidermidis is a rare cause of CAPD peritonitis

E

Fungal infections commonly cause CAPD peritonitis

Explanation
Considerations in peritoneal dialysis
Peritoneal dialysis fluid contains a high concentration of glucose (standard fluid at a
concentration of 1.36%)
More concentrated dialysis fluid may contain glucose at concentrations of up to 6.36%,
this means that patients with diabetes may require significantly more diabetes

treatment to reduce their blood glucose once dialysis is commenced
Due to the large fluid volume expansion once fluid is introduced, abdominal hernias
may significantly worsen once dialysis treatment is commenced, and should be
surgically repaired
Stomas may be associated with significant adhesions and changes within the
abdominal cavity making catheter placement impossible
Staphylococcus epidermidis is the commonest cause of CAPD (continuous ambulatory
peritoneal dialysis) peritonitis (40–50% of cases); fungal infections such as candida are
responsible for only 2% of cases
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Question 12 of 316

You are asked by your surgical colleagues to review a 70-year-old man with acute
pancreatitis. He is clearly very unwell, and the surgeons have noticed deteriorating renal
function. His current urine output is around 5 ml/h, with a raised urine sodium concentration
of 55 mmol/l. The surgeons have been keeping him well filled, and a recent central venous
pressure was measured at 16 mmH2O. The latest serum creatinine that you have is 320 μmol/l
(60–110). Which one of the following diagnoses fits best with this clinical picture?
A

Acute tubular necrosis (ATN)

B

Pre-renal uraemia

C

Glomerulonephritis

D

Acute interstitial nephritis

E

Renal vein thrombosis

Explanation
Acute tubular necrosis
Aetiological associations
Acute tubular necrosis is associated with pancreatitis and a number of other conditions
associated with hypotension and shock such as haemorrhage, burns, diarrhoea and
vomiting and myocardial infarction
Aminoglycosides, non-steroidal anti-inflammatory drugs (NSAIDs), angiotensinconverting enzyme (ACE)-inhibitors and platinum-based chemotherapeutic agents may
also be associated
In the present case ATN is likely to have developed from a period of renal ischaemia
associated with hypotension and shock during the early period of his admission with
pancreatitis
In this case ATN is likely to have developed from a period of renal ischaemia associated
with hypotension and shock during the early period of his admission with pancreatitis
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Diagnostic findings
Helpful pointers towards a diagnosis of ATN include an urine osmolality less than 350
mosmol/kg, urine sodium > 40 mmol/l and a fractional sodium excretion of greater
than 1%
Urinary sodium measurement is useful in differentiating between pre-renal and intrinsic
renal dysfunction
Pre-renal failure is considered likely if urinary sodium excretion is less than 20 mmol/l,
whereas intrinsic renal failure (ATN) is considered if urinary sodium excretion is above
40 mmol/l
Prognosis
The prognosis of ATN varies according to the severity and duration of the renal insult,
but recovery of renal function typically occurs after 7–21 days, but may take up to 6
weeks in total
The mortality rate associated with ATN may be up to 50%, but this is largely dependent
on the precipitating illness
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Question 13 of 316

A 40-year-old patient with known chronic liver disease presents with exertional dyspnoea. On
examination his blood pressure is 130/80 mmHg and his JVP is slightly elevated. He has
bilateral pleural effusions, ascites and marked pitting oedema for which his GP has prescribed
furosemide. Investigations reveal: plasma sodium 136 mmol/l, potassium 3.5 mmol/l,
bicarbonate 30 mmol/l and chloride 99 mmol/l. Which one of the following is the most
characteristic physiological activity that retains sodium in the face of salt and water
overload?
A

Arterial underfilling

B

Activation of the parasympathetic system

C

Inhibition of ADH release

D

Decrease in pressure and volume receptors

E

Decreased atrial natriuretic peptide levels

Explanation
Mechanism of salt and water retention in chronic liver disease
In cirrhosis, arterial vasodilatation due to nitric oxide overactivity, coupled with
hypoalbuminaemia which drives low colloid osmotic pressure, leads to arterial
underfilling
This is perceived by the pressure and volume receptors as hypovolaemia
There is consequent activation of the sympathetic system, non-osmotic release of ADH
and activation of the renin–angiotensin–aldosterone system
These mediators lead to salt and water retention
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