Fibronectin Glomerulopathy Without Typical Renal Biopsy Features in a 4-Year-Old Girl with Incidentally Discovered Proteinuria and a G417V Gene Mutation.

Fibronectin glomerulopathy (FG) is caused by fibronectin 1 () gene mutations. A renal biopsy was performed on a 4-year-old girl with incidentally discovered proteinuria (150 mg/dL); her family history of renal disease was negative. Markedly enlarged glomeruli (mean glomerular diameter: 196 μm; age-matched controls: 140 μm), α-SMA-positive and Ki-67-positive mesangial cell proliferation (glomerular proliferation index 1.

The value of PLA2R antigen and IgG subclass staining relative to anti-PLA2R seropositivity in the differential diagnosis of membranous nephropathy.

Background: The diagnostic performance of PLA2R and IgG subclass staining of kidney biopsies relative to anti-PLA2R seropositivity in the differentiation of primary and secondary membranous nephropathy (pMN, sMN) was examined. Besides PLA2R staining - which has a lower specificity than anti-PLA2R antibody serology - there is insufficient knowledge to decide which IgG1-4 subtype immunohistological patterns (IgG4-dominance, IgG4-dominance/IgG1-IgG4-codominance or IgG4-dominance/IgG4-codominance with any IgG subtype) could be used to distinguish between pMN and sMN.

Methods: 87 consecutive Hungarian patients (84 Caucasians, 3 Romas) with the biopsy diagnosis of MN were classified clinically as pMN (n = 63) or sMN (n = 24).

Peritubular capillary basement membrane multilayering in early and advanced transplant glomerulopathy: quantitative parameters and diagnostic aspects.

The ultrastructural quantitative aspects of peritubular capillary basement membrane multilayering (PTCBML) were examined in 57 kidney transplant biopsies with transplant glomerulopathy (TG). The measurements included three cutoffs [permissive: 1 PTC with 5 basement membrane (BM) layers, intermediate: 3 PTCs with 5 layers or 1 PTC with 7 layers, strict: 1 PTC with 7 layers and 2 PTCs with 5 layers] and the mean number of BM layers (PTC). Two groups were assigned, namely patients with mild TG (Banff cg1a and cg1b) and those with moderate-to-severe TG (cg2 and cg3).

[Banff score changes in kidneys from marginal donors].

Introduction: Despite an increase in the number of cadaver donors and the number of overall organ transplantations, the dramatic increase in the waiting list makes it necessary to reconsider donor criteria.

Aim: The authors examined whether differences could exist in the function and/or morphology of transplanted kidneys originated from marginal and ideal donors one and five years after transplantation.

Method: Kidney function and histopathologic findings were analysed and compared one and 5 years after transplantation in 97 patients having marginal donor kidneys and 178 patients who received ideal donor kidneys.

Morphologic Features and Clinical Impact of Arteritis Concurrent with Transplant Glomerulopathy.

Little is known about the morphology and clinical relevance of arteritis in renal allograft biopsies with transplant glomerulopathy. We retrospectively reviewed the morphologic findings and clinical course of 59 patients with cg, 16 of which featured concurrent arteritis (fibrosing intimal arteritis with luminal narrowing in 15, and acute intimal arteritis in 1 case). Fifteen out of the 16 cases with arteritis fulfilled the morphological diagnostic criteria for chronic active antibody-mediated rejection, and 11 cases with arteritis showed morphological evidence of concurrent, ongoing T-cell-mediated alloimmune response (acute T-cell-mediated rejection in 5, borderline changes in 6 cases).

[Frequency of T-cell mediated rejection in new-onset diabetes after kidney transplantation].

Introduction: New-onset diabetes is one of the most common complications after kidney transplantation.

Aim: The aims of the authors were to examine the frequency of new-onset diabetes mellitus in kidney transplanted patients receiving cyclosporine A (n = 95) and tacrolimus (n = 102) and to analyze the occurrence of T-cell mediated rejection in these two groups of patients.

Method: Age, laboratory results, renal function and histological findings were evaluated one year after kidney transplantation.

Assessment of donor biopsies.

Purpose Of Review: To provide an up-to-date overview about the assessment of donor biopsies and to discuss the current problems and chances of preimplantation biopsies for transplant allocation with a focus on the technical work up and the histological variables scored.

Recent Findings: Preimplantation biopsy results are the major reason for discarding procured extended donor criteria kidneys in the USA. There is neither a consensus on the work up, nor the reporting of preimplantation donor biopsies, nor the importance of the biopsy findings in the process of allocation.

Peritubular capillary basement membrane changes in chronic renal allograft rejection: Comparison of light microscopic and ultrastructural observations.

Marked peritubular capillary basement membrane (PTCBM) multilayering, the ultrastructural feature of chronic antibody-mediated rejection (ABMR) of kidney allografts, was found to correspond histologically to PTCs with thickened BMs; such PTCs have been suggested as a novel histological marker of chronic rejection. We investigated whether scoring of PTCBM thickening can substitute the ultrastructural search for PTCBM multilayering. The thickening was graded in PAS- and Jones-stained sections in 110 biopsies from recipients with a late dysfunction, all examined ultrastructurally for transplant capillaropathy (≥3 PTCs with ≥5 BM layers).

Plasma cell infiltrates in polyomavirus nephropathy.

Polyomavirus (PV) associated nephropathy (PVAN) has become an important cause of allograft dysfunction. We studied plasma cells (PCs) - which have not yet been characterized - present in the cellular infiltrate of 20 PVAN cases using immunohistochemistry and morphometry. The results were correlated with morphological, clinical and anti-BK virus serological findings.

Primer: histopathology of polyomavirus-associated nephropathy in renal allografts.

The BK polyomavirus exhibits tropism for the renal tubular epithelium, where it establishes latent infection. Vigorous immunosuppression of renal allograft recipients can lead to reactivation of the infection and the development of polyomavirus-associated nephropathy (PVAN). Clinically, gradually decreasing renal function, viremia and viruria are observed several months after transplantation; allograft failure occurs in 1-10% of patients.

[Collagen type IV nephropathy: from thin basement membrane nephropathy to Alport syndrome].

Introduction: Collagen type IV nephropathy includes the Goodpasture syndrome, thin basement membrane nephropathy and the Alport syndrome. Mutations in the coding Col(IV)A3/A4 and Col(IV)A5 genes are probable causes of the latter two. Thin basement membrane nephropathy is mostly familial and has an autosomal dominant inheritance, at least 40% of the families have hematuria that co-segregates with the Col(IV)A3 and/or Col(IV)A4 loci.

[The incidence of renal diseases as diagnosed by biopsy in Hungary].

Aim And Methods: The authors analysed the incidence of renal diseases as diagnosed by biopsy in the population living on the southern Great Hungarian Plain. 798 biopsy specimens were examined between 1990 and 2002.

Results: The most common diseases in decreasing order of frequency were IgA nephropathy (15%), membranous nephropathy (12%), thin-basement-membrane nephropathy (8%), minimal change nephropathy (7%), lupus glomerulonephritis (7%), focal sclerosis (6%), hypertensive kidney disease and arteriolosclerosis (5%), diabetic nephropathy (5%), and crescentic glomerulonephritis (4%).