Table 1.
Clinical and pathological findings in late-onset oligomeganephronia
| Case 1 | Case 2 | Case 3 | |
|---|---|---|---|
| Age/sex | 36/male | 19/female | 21/male |
| Past history | Taken care in NICU | ||
| Family history | Father, ESRD; sister, MPGN | ||
| Gestation (weeks)/birth weight (g) | 37/2700 (median) | 41/2410 (light for date) | 41/3405 (median) |
| Body mass index (kg/m2) | 20.9 | 21.8 | 19.1 |
| Initiation | Hypertension at medical check-up | Proteinuria at school urinalysis | Proteinuria at school urinalysis |
| Initial→recent blood pressure (mmHg) | 166/113→110/70 | 98/60→96/60 | 132/70→110/70 |
| Urinalysis | No proteinuria or haematuria | Proteinuria (1 +), RBC 1/1–4 HPF | Proteinuria (3 +), no haematuria |
| 24-h urine protein (g)/urine volume (mL/day) | 0.18/1800 | 0.53/1090 | 0.65/1810 |
| Initial/recent serum creatinine (mg/dL) | 2.65/2.89 | 1.14/1.20 | 1.20/1.12 |
| Echo findings (right; left mm) | Atrophic (89 × 41; 93 × 47), high echogenicity | Atrophic (87 × 29; 80 × 47), high echogenicity | Normal (106 × 45; 105 × 42) |
| Medication | ACEI, ARB, CCB, αβ-blocker, aspirin | ACEI, ARB, dilazep | ARB |
| Follow-up at our centre (years) | 3.5 | 3 | 1.5 |
| Pathologic findings | |||
| Glomerular numbera (/mm2) | 1.29 | 0.76 | 0.97 |
| Maximum of glomerular diameterb (μm) | 200 | 310 | 270 |
| Number of glomerular sclerosis (/total) | 2/10 | 1/4 | 0/6 |
| Focal segmental glomerulosclerosis | (perihilar type) | ||
| Tubular atrophy and fibrosis (% of cortex) | 2.96 | 9.36 | 10.1 |
| Fluorescence staining | Non-specific IgM deposition | Non-specific IgM deposition | Non-specific IgM deposition |
| Electron microscopic examination | Partial foot process fusion | Partial foot process fusion | Almost normal |
NICU, neonatal intensive care unit; ESRD, end-stage renal disease.
a
Normal glomerular number (mean ± SD): 3.2 ± 1.2/mm2.
b
Normal glomerular diameter (mean ± SD): 201 ± 28 μm.