Minimal change disease Flashcards
what is the definition of MCD?
Minimal change disease (MCD) is the most common form of nephrotic syndrome (a clinical condition characterised by heavy proteinuria, oedema, hypoalbuminemia, and hyperlipidaemia) affecting children.
what is the epidemiology of MCD?
Children and older age
More common in males
what is the aetiology of MCD?
Although typically idiopathic, MCD may be secondary to certain conditions, such as Hodgkin’s lymphoma, leukaemia, and, rarely, hepatitis B or C infection. In addition, it is important to consider the role of MCD in relation to nephrotic syndrome (NS).
what are the risk factors for MCD?
Between 1 and 8 yrs
Hodgkin lymphoma
Leukaemia
Recent viral illness
what is the pathophysiology of MCD?
In NS, an increase in glomerular permeability to proteins is the main pathological process resulting in heavy proteinuria. Although the pathogenesis of primary NS is unclear, some evidence suggests that dysregulation of the immune system plays a role in development. The observation that a viral illness (with the exception of measles infection) can precede the initial presentation or, in many cases, a relapse in known nephrotic patients, and the association of NS with primary immunological disorders such as lymphoma and leukaemia, support this hypothesis. In contrast, measles infection is known to suppress cell-mediated immunity and, in so doing, has been seen to induce remission of NS. In addition, giving immunosuppressive agents such as corticosteroids and calcineurin inhibitors can result in remission.
In MCD, no structural changes are seen in the filtration unit on light microscopy. However, with electron microscopy, effacement of the epithelial foot processes (podocytes) is seen.
what are the key presentations of MCD?
Facial or generalised oedema
1 to 8 yrs
what are the signs of MCD?
Normal BP Absence of haematuria Recent viral illness Lymphoma or leukaemia Facial or generalised oedema 1 to 8 yrs
what are the symptoms of MCD?
Dyspnoea
what are the first line and gold standard investigations for MCD?
Urinalysis - protein: 3+ (30 g/L [3 g/dL]) to 4+ (200 g/L or more [20 mg/dL or more]); no significant haematuria
24hour urine protein - >50 mg/kg/day or >40 mg/square metre of body surface area/hour
Urine protein: creatinine ratio - more than 2
Serum albumin level - typically <30 g/L (<3 g/dL)
Serum lipid profile - elevated triglyceride and cholesterol
Serum complement profile - normal C3 and C4
Serum electrolyte panel - normal or slightly elevated urea, decreased sodium level
FBC - increased haemoglobin and haematocrit, increase platelet count
GFR - normal
Serum LFTs - normal
Renal ultrasound - normal
what are the differential diagnoses for MCD?
Acute glomerulonephritis, focal segmental glomerulosclerosis, congestive heart failure
what other tests could be done for MCD?
Kidney biopsy - not in children (most likely is minimal change so no point)
how is MCD managed?
Corticosteroids, fluid restriction with albumin and furosemide
Immunosuppression if ongoing
how is MCD monitored?
Patients should undergo routine testing for serum urea and creatinine, and urinalysis at least every 6 months. Those taking immunosuppressants and corticosteroids should be regularly evaluated for complications of treatment.
what are the complications of MCD?
Spontaneous peritonitis
Thrombosis
Replace of MCD in adulthood
what is the prognosis of MCD?
Death in the developed world is rare but well documented, and mainly occurs from infectious and thrombotic complications. In the developing world, despite the limited therapeutic facilities, half of nephrotic syndrome (NS) patients benefit from corticosteroids, but corticosteroid resistance has been shown to result in high mortality. Lack of resources, late referrals, and the high cost of renal replacement therapy in these countries leads to poor outcomes in chronic renal disease due to all causes, including NS and MCD.
