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  1. Richard P Hull, specialist registrar,
  2. David J A Goldsmith, consultant nephrologist
  1. 1Guy’s Hospital Renal Unit, London SE1 9RT
  1. Correspondence to: DJA Goldsmith david.goldsmith{at}gstt.nhs.uk

    The nephrotic syndrome is one of the best known presentations of adult or paediatric kidney disease. The term describes the association of (heavy) proteinuria with peripheral oedema, hypoalbuminaemia, and hypercholesterolaemia (box 1). Protein in the urine (“coagulable urine”) was first described in 1821, 15 years before Richard Bright’s celebrated series of descriptions of “albuminous urine.”1

    Box 1 Diagnostic criteria for nephrotic syndrome

    • Proteinuria greater than 3-3.5 g/24 hour or spot urine protein:creatinine ratio of >300-350 mg/mmol

    • Serum albumin <25 g/l

    • Clinical evidence of peripheral oedema

    • Severe hyperlipidaemia (total cholesterol often >10 mmol/l) is often present

    Nephrotic syndrome has an incidence of three new cases per 100 000 each year in adults.2 It is a relatively rare way for kidney disease to manifest compared with reduced kidney function or microalbuminuria as a complication of systemic diseases, such as diabetes and raised blood pressure.3

    Summary points

    • Nephrotic syndrome is a relatively rare but important manifestation of kidney disease

    • It has serious complications and must be on the differential diagnosis for any patient presenting with new onset oedema

    • It can be caused by a wide range of primary (idiopathic) and secondary glomerular diseases

    • All patients should be referred to a nephrologist for further investigation, which (often) includes a renal biopsy

    • Initial management should focus on investigating the cause, identifying complications, and managing the symptoms of the disease

    Why should I read this article?

    Patients with nephrotic syndrome can present to primary or secondary care with diverse symptoms that reflect the primary process or with one of the many systemic complications of the syndrome.4 Although nephrotic syndrome is relatively common in renal practice, it is seen only rarely in primary or secondary care. This can result in a delayed or overlooked diagnosis, especially as many other conditions have similar symptoms. For example, severe peripheral (leg) oedema is seen in congestive cardiac failure, hypoalbuminaemia can be caused …

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