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🌱 來自: Huppert’s Notes
Glomerular Pathologies Commonly Associated with Nephrotic-Range Proteinuria🚧 施工中
Glomerular Pathologies Commonly Associated with Nephrotic-Range Proteinuria
Minimal change glomerulopathy (MCG, “minimal change disease”)
• Epidemiology: Most common cause of nephrotic syndrome in children, less common in adults
• Pathophysiology:
- Defined by nephrotic-range proteinuria and kidney biopsy with little or no change to the glomerular structure on light microscopy
- Primary MCD is idiopathic
- Secondary MCD may be associated with atopic disease, mononucleosis, Hodgkin’s lymphoma, medications (e.g., NSAIDS, interferon, lithium)
• Treatment: Steroids 4–8 weeks, good prognosis
Focal segmental glomerulosclerosis (FSGS)
• Epidemiology: Most common cause of nephrotic syndrome in black adults
• Pathophysiology:
- Defined by proteinuria and scarring in scattered glomeruli on biopsy
- Primary FSGS may result from genetic mutations in podocyte proteins
- Secondary FSGS is associated with HIV (“collapsing” type), obesity, heroin use
• Treatment:
- Steroids or calcineurin inhibitors: 40–60% achieve remission, others have refractory disease and often advance to ESRD.
- Can recur in the transplanted kidney and is thought to be due to a soluble factor that has yet to be identified.
- Plasmapheresis can be utilized to remove this soluble factor to help prevent recurrence.
Membranous glomerulopathy (MGN, “membranous nephropathy)
• Epidemiology: Second most common cause of nephrotic syndrome in U.S. adults after diabetic nephropathy
• Pathophysiology:
- Primary MGN is associated with the anti-phospholipase A2 receptor (anti-PLA2R) antibody; majority of cases are primary
- Causes of secondary MGN: SLE, hepatitis B, solid tumor malignancy (lung, breast, and GI carcinomas), medications (penicillamine, NSAIDs, mercury, captopril)
• Diagnosis: Diagnosis requires a kidney biopsy, which shows deposits of IgG and C3
• Clinical features: Higher propensity for thromboembolic events (particularly renal vein thrombosis)
• Treatment: Up to one-third of patients will experience spontaneous remission; treatment with immunosuppression is considered for patients with a poor prognosis.
- <4 g protein/day: Good prognosis for remission
- 4–8 g protein/day: Possible chance of remission
- >8 g protein/day: Poor chance of remission, start treatment immediately