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Nephrotic Syndrome

Background Knowledge ๐Ÿง 

Definition

  • Nephrotic syndrome is a clinical syndrome characterised by significant proteinuria (>3.5 g/day), hypoalbuminemia, hyperlipidaemia, and oedema.
  • Results from increased permeability of the glomerular filtration barrier.

Epidemiology

  • Affects both adults and children.
  • More common in males in childhood, equal prevalence in adults.
  • Incidence in the UK: 3 per 100,000 adults per year.
  • Higher incidence in certain ethnic groups (e.g. South Asians).

Aetiology and Pathophysiology

  • Primary causes: Minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy.
  • Secondary causes: Diabetes mellitus, systemic lupus erythematosus, amyloidosis, infections (e.g., hepatitis B/C, HIV), drugs (e.g., NSAIDs, penicillamine).
  • Pathophysiology: Glomerular damage leads to increased permeability to proteins.

Types

  • Primary (idiopathic) nephrotic syndrome.
  • Secondary nephrotic syndrome.

Clinical Features ๐ŸŒก๏ธ

Symptoms

  • Generalized oedema (e.g., periorbital, lower limb)
  • Frothy urine
  • Fatigue
  • Loss of appetite
  • Abdominal pain (due to ascites)
  • Weight gain (due to fluid retention)

Signs

  • Pitting oedema
  • Ascites
  • Pleural effusion
  • Hypertension (in some cases)
  • Signs of underlying cause (e.g., diabetic retinopathy)
  • Pale skin (due to hypoalbuminemia)

Investigations ๐Ÿงช

Tests

  • Urinalysis: Proteinuria
  • Urine protein-creatinine ratio (PCR)
  • Blood tests: Serum albumin, cholesterol, creatinine, urea, electrolytes, renal screen (e.g. ANA, ANCA, anti-GBM etc.)
  • Renal biopsy to determine underlying cause
  • Imaging: Ultrasound of kidneys

Management ๐Ÿฅผ

Management

  • General measures: Low salt diet, fluid restriction.
  • Diuretics for oedema management.
  • ACE inhibitors or ARBs to reduce proteinuria.
  • Statins for hyperlipidaemia.
  • Anticoagulation if high risk of thrombosis.
  • Specific treatment for underlying cause (e.g. immunosuppression for minimal change disease).

Complications

  • Infections (due to loss of immunoglobulins)
  • Thrombosis (due to hypercoagulability)
  • Acute kidney injury
  • Chronic kidney disease
  • Malnutrition

Prognosis

  • Varies depending on underlying cause.
  • Primary nephrotic syndrome often responds well to steroids.
  • Secondary nephrotic syndrome prognosis depends on control of underlying disease.
  • Regular follow-up and monitoring required.

Key Points

  • Nephrotic syndrome is a serious condition requiring thorough investigation.
  • Identify and treat underlying cause.
  • Manage complications effectively.
  • Patient education on dietary and lifestyle modifications.
  • Multidisciplinary approach often needed.

References

  • NICE Guidelines: Nephrotic Syndrome (https://www.nice.org.uk/guidance/ng203) NICE Guidelines
  • Renal Association Guidelines: Nephrotic Syndrome (https://renal.org/guidelines) Renal Association Guidelines
  • Patient.info: Nephrotic Syndrome (https://patient.info/doctor/nephrotic-syndrome) Patient.info

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