A 14-year-old female presents with a two week history of deteriorating puffiness of the face.
She has been aware of tiredness and lethargy over the last two months since having acquired
a throat infection. Over this period she has gained at least 5 kg in weight over this time. She
had a similar throat infection aged 10 from which she made an uneventful recovery. There is
nothing else of note in the history and she takes no medication. Examination reveals
generalised puffiness with pitting oedema of the lower limbs. Her blood pressure is 133/86
mmHg with a pulse of 88 bpm. Chest, cardiovascular and abdominal examinations are normal.
Her urine dip stick produces +++ protein.
Other investigations reveal:
Haemoglobin 14.6g/dl
White cell count 6 x 109/l
Platelets 250 x 109/l
Sodium 136 mmol/l
Potassium 4.0 mmol/l
Chloride 103 mmol/l
Bicarbonate 24 mmol/l
Urea 4.2 mmol/l
Creatinine 93 micromol/l
Serum albumin 25 g/l
24 hour urine protein 4.3 g/l
Which is the best treatment for this patient?
Available marks are shown in brackets
1 ) Observe [0]
2 ) Salt poor albumin [0]
3 ) Intravenous frusemide [0]
4 ) High dose corticosteroids [100]
5 ) Cyclophosphamide [0]
Comments:
This patient has the nephrotic syndrome as defined by a triad of hypoalbuminaemia,
proteinuria > 3g/24hrs and oedema. The commonest cause of nephrotic syndrome in a child is
minimal change disease, which usually responds to a course of high dose corticosteroids.
Cyclophosphamide may hasten a remission, but given its cytotoxic profile, should be reserved
for steroid-resistant cases. If possible it should be avoided in childhood. Salt poor albumin and
iv frusemide may be useful adjuncts for managing oedema, but is not curative of proteinuria. If
the nephrotic syndrome is left unchecked, complications include Streptococcal sepsis, venous
thromboembolism and hypercholesterolaemia

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