Japan Renal Biopsy Registry and Japan Kidney Disease Registry: Committee Report for 2009 and 2010

Abstract

123Clin Exp Nephrol (2013) 17:155-173 DOI 10.1007 nephritic syndrome (55.4 % and 50.0 % in 2009 and 2010, respectively), followed by nephrotic syndrome (22.4 % and 27.0 %); the most frequent pathological diagnosis as classified by the pathogenesis was IgA nephropathy (31.6 % and 30.4 %), followed by primary glomerular diseases (except IgA nephropathy) (27.2 % and 28.1 %). Among the primary glomerular diseases (except IgA nephropathy) in the patients with nephrotic syndrome, membranous nephropathy was the most common histopathology in 2009 (40.3 %) and minor glomerular abnormalities (50.0 %) were the most common in 2010 in native kidneys in the J-RBR. Five new secondary and longitudinal research studies by the J-KDR were started in 2009 and one was started in 2010.

Membranous Nephropathy with an Enhanced Granular Expression of Thrombospondin Type-1 Domain-containing 7A in a Pregnant Woman

“…Because reports of patients under 40 year of age are extremely rare (13,14), we reviewed the characteristics of THSD7A-related MN. Interestingly, approximately 60% of women with THSD7A-related MN were diagnosed before they turned 40, whereas there were no such patients among men.…”

“…Additionally, the expression of PLA2R/ THSD7A was enhanced in renal tissue specimens of patients with anti-PLA 2 R / THSD 7 A antibodies in the serum (2,8,10,12). Most idiopathic MN patients are over 50 years old, while those under 40 are extremely rare (13,14). In several cases, idiopathic MN first occurred during pregnancy (15,16).…”

“…Because MN patients under 40 year of age are extremely rare (13,14), we reviewed the characteristics of THSD7A-related MN. A review of the published cases of THSD7A-related MN (3,17) revealed that approximately 60% (8 of 14) of women diagnosed with MN were under 40 years old (average age, 41.0±15.7 S.D.…”

Clinical and Histological Characteristics in Patients with Non-IgA Mesangioproliferative Glomerulonephritis

“…The Figure-1 shows the representative histological findings of IgAN and N-IgAN. In nationwide biopsy registry data from Japan, N-IgAN comprises 3.5% of all biopsy-proven renal diseases 5) . However, the prevalence of this disease varies country to country 4) 6)-9) .…”

ACE insertion/deletion polymorphism (rs1799752) modifies the renoprotective effect of renin-angiotensin system blockade in patients with IgA nephropathy

“…Immunoglobulin A (IgA) nephropathy (IgAN) is the most common glomerulonephritis worldwide, [1][2][3][4] progressing to end-stage renal disease (ESRD) within 10 years of diagnosis in approximately 15%−25% patients. 5 A series of randomized controlled trials demonstrated that renin-angiotensin system (RAS) blockade using angiotensin-converting enzyme inhibitors (ACEIs) and angiotensin-receptor blockers (ARBs) decreased urinary protein [6][7][8][9][10] and suppressed progression of IgAN.…”