eMedicine Specialties > Nephrology > Glomerular Diseases
Minimal-Change Disease: Differential Diagnoses & Workup
Updated: Oct 15, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Differential Diagnoses
Other Problems to Be Considered
C1q nephropathy
Focal segmental glomerulosclerosis
Immunoglobulin M (IgM) nephropathy
Membranous nephropathy
Other variables
Workup
Laboratory Studies
- Urine analysis is benign, but profound proteinuria and oval fat bodies may be observed.
- In children, the critical level for diagnosis is more than 40 mg/h/m2.
- In adults, the threshold is more than 3.5 g/d/1.73 m2.
- A random albumin-to-creatinine concentration ratio is in excess of 5.
- Urine specific gravity is high because of proteinuria.
- A 24-hour urine measurement is obtained for protein and creatinine clearance.
- Hypoalbuminemia is an important marker of nephrotic syndrome. The level at which edema occurs varies, but it tends to be lower in children than in adults. Nephrotic syndrome in children is defined by a serum albumin of less than 2.5 g/dL.
- Hyperlipidemia also is a feature of a nephrotic state.
- Renal function usually is normal except in cases of undiagnosed FSGS or in those cases that progress to acute renal failure.
- Serologic workup (including antinuclear antibodies, complements, and cryoglobulins) is normal.
- Hyponatremia often is observed, which is, in part, a spurious finding secondary to the hyperlipidemic state. This condition also occurs from water retention caused by hypovolemia and antidiuretic hormone release.
- Elevated hemoglobin and hematocrit are consequences of plasma volume contraction.
Imaging Studies
- Renal sonogram is normal.
Procedures
- Because of the high prevalence of MCD in children with nephrotic syndrome, an empiric trial of corticosteroids commonly is the first step in therapy. Renal biopsy typically is performed only in resistant cases. Generally, if proteinuria remains after 2 relapses or courses of steroids, a tissue diagnosis should be made before starting cytotoxic or immunosuppressive therapy.
Histologic Findings
Light microscopy: In patients with MCD, the glomerulus is, by definition, normal or nearly so when examined with the light microscope; however, the precise limits of normal are not clearly defined. This creates difficulty in differentiating the appearance of minimal change with mild mesangial proliferation from a mesangial proliferative glomerulonephritis. Diagnosis can be even more difficult because, at the peak age of onset (approximately 3 y), the mesangial and epithelial cells are more prominent. In adult patients, diagnosis is made more challenging by superimposed arterionephrosclerosis secondary to hypertension. In children with frequently relapsing MCD, some involuted glomeruli may be present. These lesions are small and sclerotic but retain their podocyte and parietal epithelial cell constituents. The presence of these glomeruli is related to the duration of the disease.
The most common tubular lesion is protein and lipid droplets in epithelial cells due to increased reabsorption. The presence of areas of tubular atrophy and interstitial fibrosis should raise the suspicion of FSGS.
Immunohistology: These studies usually do not demonstrate significant glomerular deposition of immunoglobulins or complement components in patients with MCD. Some biopsy specimens may be positive for low-level IgM deposits not accompanied by mesangial dense deposits.
Electron microscopy: Retraction of the epithelial foot processes is observed consistently in patients with MCD. This is, at times, erroneously described as foot-process fusion and results from disordered epithelial cell structure with withdrawal of the dendritic process. This finding is not unique to MCD, and the diagnosis is one of exclusion of other diseases based on lack of other processes on light microscopy, immunohistology, or electron microscopy.
More on Minimal-Change Disease |
| Overview: Minimal-Change Disease |
Differential Diagnoses & Workup: Minimal-Change Disease |
| Treatment & Medication: Minimal-Change Disease |
| Follow-up: Minimal-Change Disease |
| References |
| Further Reading |
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References
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Further Reading
Clinical trials:
Kidney Disease Biomarkers
Retinoids for Minimal Change Disease and Focal Segmental Glomerulosclerosis
Steroid Treatment for Kidney Disease
Keywords
minimal-change disease, nephrotic syndrome, focal segmental glomerulosclerosis, idiopathic nephrotic syndrome of childhood, lipoid nephrosis, minimal-change nephropathy, minimal-change nephrotic syndrome, nil disease, steroid-sensitive nephrotic syndrome
Differential Diagnoses & Workup: Minimal-Change Disease