explained the diagnostic criteria of IRGN, in which at least three of the following clinical and pathological findings were required: (1) clinical or laboratory evidence of infection preceding or in the onset of glomerulonephritis; (2) decreased serum match; (3) endocapillary proliferative and exudative glomerulonephritis; (4) C3-dominating or co-dominant deposition in glomeruli on immunofluorescence staining; and (5) hump-shaped subepithelial deposits on electron microscopy [5]

explained the diagnostic criteria of IRGN, in which at least three of the following clinical and pathological findings were required: (1) clinical or laboratory evidence of infection preceding or in the onset of glomerulonephritis; (2) decreased serum match; (3) endocapillary proliferative and exudative glomerulonephritis; (4) C3-dominating or co-dominant deposition in glomeruli on immunofluorescence staining; and (5) hump-shaped subepithelial deposits on electron microscopy [5]. with KM55, which is a specific Imperatorin monoclonal antibody for galactose-deficient IgA1 (Gd-IgA1). The intensity of the KM55 signal in the present individual was weaker than that in individuals with IgA nephropathy. To our knowledge, this is the 1st statement of IRGN associated with PVB19 that progressed to ESRD without any underlying diseases. Further investigations are needed to determine the significance of IgA and Gd-IgA1 deposition in IRGN associated with PVB19. risk alleles [4] progressed to end-stage renal disease (ESRD), whereas remission can occur spontaneously or by treatment with steroids and immunosuppressive therapies in individuals with no underlying diseases [1]. In this study, we report the case of a 5-year-old boy with no underlying diseases who exhibited endocapillary glomerulonephritis with IgA-dominant deposition associated with PVB19 that rapidly progressed to ESRD. Case statement A previously healthy 5-year-old son presented with macrohematuria followed by erythema on his cheeks and vomiting. He had no family history of kidney disease. Urinary analyses performed at the age of 3?years under the mass screening program in Japan revealed no abnormalities. Four days later, he was admitted due to pretibial edema, proteinuria, and prolonged macrohematuria. The initial laboratory evaluation revealed a normal hemoglobin level of 11.6?g/dL, a slightly decreased albumin level of 3.1?g/dL, increased levels of blood urea nitrogen, and creatinine at 74.9 and 1.94?mg/dL, respectively, a potassium level of 4.8?mmol/L, and normal serum complement levels. Antibodies against hepatic B computer virus and hepatic C computer virus, as well as antistreptolysin-O titers and antistreptokinase antibodies, were unfavorable. Serum PVB19 DNA PCR and anti-PVB19 IgM antibody were positive. Serum antinucleus, anti-ds-DNA, antineutrophil cytoplasmic, and anti-glomerular basement membrane antibodies were negative. Urinary analysis revealed heavy proteinuria (3+, urinary protein/creatinine ratio?=?9.05?g/gCr) and hematuria (occult blood 3+, red blood cells? ?100/high power field) with no active casts. Ultrasonography Imperatorin revealed increased echogenicity in both kidneys. Physique?1 shows the clinical course of the present case. The patient was treated with a course of intravenous methylprednisolone pulse therapy [500?mg (29.4?mg/kg)??3?days] followed by daily oral prednisolone (2?mg/kg body weight). On day 4 after admission, open kidney biopsy was performed. The specimen contained 118 glomeruli, 18 of which (15.3%) had cellular crescents. Light microscopy revealed prominent endocapillary proliferation and mesangiolysis with occasional tuft rupture (Fig.?2a, b), but tubulointerstitial changes were not observed. Immunofluorescence analysis revealed that signals for IgA (Fig.?2e) and C3 (Fig.?2f) were co-dominantly positive in the mesangial area and along the capillary walls. Signals for C1q were negative. Immunofluorescence analysis of IgA subclasses revealed IgA1-dominant deposition (Fig.?2g, h). Electron microscopy showed electron-dense deposits in the subendothelial and mesangial areas (Fig.?2i), but not in the subepithelial area. No humps were observed. Podocyte hypertrophy, foot process effacement, and microvilli degeneration were also observed, which were suggestive of podocyte injury (Fig.?2i). PVB19 DNA was detected in the kidney specimen via PCR. Diagnosis of IgA-dominant infection-related glomerulonephritis (IRGN) associated with PVB19 was made on the basis of the serological and pathological findings. After a course of intravenous methylprednisolone pulse therapy, the patients serum creatinine level decreased. Therefore, two additional courses of intravenous methylprednisolone pulse therapy were administered. Serum anti-PVB19 IgG antibody positivity was detected on day 25 after Rabbit Polyclonal to HDAC3 admission, and serum PVB19 DNA was unfavorable on day 27 after admission (Table?1). However, the patients serum creatinine level increased again, which prompted us to perform eight sessions of plasma exchange to remove circulating immune Imperatorin complexes. Consequently, the patients serum creatinine level decreased from 2.5 to 0.7?mg/dL, whereas macrohematuria and heavy proteinuria persisted and urinary protein to creatinine ratio was 8.1?g/gCr. We performed follow-up biopsy on day 54 after admission. The specimen contained 13 glomeruli, three (23%) of which exhibited fibrocellular crescent formation (Fig.?3a). Global sclerosis was observed in eight (62%) glomeruli. However, there were no findings suggestive of thrombotic microangiopathy, such as endothelial cell swelling and thrombosis in the capillary lumen. Tubulointerstitial infiltration and fibrosis were not observed. Immunofluorescence analysis revealed prolonged IgA (IgA1 dominant) and C3 co-dominant depositions in the mesangial area and along the capillary walls (Fig.?3bCd). Electron microscopy revealed prolonged electron-dense deposits in the subendothelial and mesangial.