|
5-year-old boy is seen in A&E due to lethargy and pallor. There is no recent history of diarrhoea. The following results are obtained:
-
Hb
|
8.4 g/dl
|
|
Platelets
|
30 * 109/l
|
|
Urea
|
24 mmol/l
|
Creatinine
|
164 µmol/l
|
Urinalysis reveals proteinuria and haematuria. What is the most appropriate management?
|
A.
|
IV cyclophosphamide
|
|
B.
|
Ciprofloxacin
|
|
C.
|
Oral prednisolone
|
|
D.
|
IV methylprednisolone followed by oral prednisolone
|
|
E.
|
Plasma exchange
|
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
Haemolytic uraemic syndrome is generally seen in young children Causes
post-dysentery - classically E coli 0157:H7 ('verotoxigenic',
'enterohaemorrhagic')
tumours
pregnancy
ciclosporin, the Pill
systemic lupus erythematous
HIV
Management
treatment is supportive e.g. fluids, blood transfusion and dialysis if required
there is no role for antibiotics, despite the preceding diarrhoeal illness in many patients
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 diarrhoea
5- Which of the following types of renal tubular acidosis is associated with hyperkalaemia?
|
A.
|
Type 1 renal tubular acidosis
|
|
B.
|
Type 2 renal tubular acidosis
|
|
C.
|
Type 3 renal tubular acidosis
|
|
D.
|
Type 4 renal tubular acidosis
|
|
E.
|
Type 5 renal tubular acidosis
|
Type 4 renal tubular acidosis is associated with hyperkalaemia
Renal tubular acidosis
All three types of renal tubular acidosis (RTA) are associated with hyperchloraemic metabolic acidosis (normal anion gap)
Type 1 RTA (distal)
inability to generate acid urine (secrete H+) in distal tubule
causes hypokalaemia
complications include nephrocalcinosis and renal stones
causes include idiopathic, RA, SLE, Sjogren's
Type 2 RTA (proximal)
decreased HCO3- reabsorption in proximal tubule
causes hypokalaemia
complications include osteomalacia
causes include idiopathic, as part of Fanconi syndrome, Wilson's disease, cystinosis, outdated tetracyclines
Type 4 RTA (hyperkalaemic)
causes hyperkalaemia
causes include hypoaldosteronism, diabetes
6- 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:
-
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 ++
|
What has likely caused the sudden deterioration in renal function?
|
A.
|
Exacerbation of SLE
|
|
B.
|
Renal vein thrombosis
|
|
C.
|
Bilateral hydronephrosis
|
|
D.
|
Acute interstitial nephritis
|
|
E.
|
Analgesic nephropathy
|
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
Complications
increased risk of infection due to urinary immunoglobulin loss
increased risk of thromboembolism related to loss of antithrombin III and plasminogen in the urine
hyperlipidaemia
hypocalcaemia (vitamin D and binding protein lost in urine)
acute renal failure
7- Which one of the following types of glomerulonephritis is most characteristically associated with partial lipodystrophy?
|
A.
|
Minimal change disease
|
|
B.
|
Diffuse proliferative glomerulonephritis
|
|
C.
|
Mesangiocapillary glomerulonephritis
|
|
D.
|
Membranous glomerulonephritis
|
|
E.
|
Rapidly progressive glomerulonephritis
|
Type 2 mesangiocapillary glomerulonephritis is associated with partial lipodystrophy. Type 1 is seen in association with hepatitis C and cryoglobulinaemia
Glomerulonephritides
Knowing a few key facts is the best way to approach the difficult subject of glomerulonephritis:
Membranous glomerulonephritis
presentation: proteinuria / nephrotic syndrome / CRF
cause: infections, rheumatoid drugs, malignancy
1/3 resolve, 1/3 respond to cytotoxics, 1/3 develop CRF
IgA nephropathy - aka Berger's disease, mesangioproliferative GN
typically young adult with haematuria following an URTI
Diffuse proliferative glomerulonephritis
classical post-streptococcal glomerulonephritis in child
presents as nephritic syndrome / ARF
Minimal change disease
typically a child with nephrotic syndrome (accounts for 80%)
causes: Hodgkin's, NSAIDs
good response to steroids
Focal segmental glomerulosclerosis
may be idiopathic or secondary to HIV, heroin
presentation: proteinuria / nephrotic syndrome / CRF
Rapidly progressive glomerulonephritis - aka crescentic glomerulonephritis
rapid onset, often presenting as ARF
causes include Goodpasture's, ANCA positive vasculitis, SLE
Mesangiocapillary glomerulonephritis (membranoproliferative)
type 1: cryoglobulinaemia, hepatitis C
type 2: partial lipodystrophy
8- What is the most common site for extra-renal cysts in a patient with autosomal dominant polycystic kidney disease (ADPKD)?
|
A.
|
Pancreas
|
|
B.
|
Brain
|
|
C.
|
Liver
|
|
D.
|
Spleen
|
|
E.
|
Thyroid
|
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
Features
hypertension
recurrent UTIs
abdominal pain
renal stones
haematuria
CRF
Extra-renal manifestations
liver cysts (70%)
berry aneurysms (8%)
CVS: mitral valve prolapse, mitral/tricuspid incompetence, aortic root dilation, aortic dissection
cysts in other organs: pancreas, spleen, thyroid
9- 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:
-
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
|
Which investigation is most likely to lead to the correct diagnosis?
-
|
A.
|
CT abdomen
|
|
B.
|
Plasma magnesium
|
|
C.
|
Intravenous urogram
|
|
D.
|
Rectal biopsy
|
|
E.
|
Renal angiogram
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
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.
Rheumatoid drugs such as gold may cause nephrotic syndrome but none of the other options point to this as an answer
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