IgA nephropathy is the most common form of primary glomerulonephritis which mainly accounts for the development of end-stage renal diseases. It is characterized by deposits of IgA1 in mesangium. The pathogenesis of IgA nephropathy is complicated. Moreover, there is a wide range of clinical features and variable histomorphologies in the diagnosed cases of IgA nephropathy. It was demonstrated that the galactose-deficient of IgA1 O-glycan chains led IgA1 to self-aggregation and eventual deposition in mesangium. Abnormality of glycosyltransferases, genetic mutation and immunologic disorder were involved in the aberrant glycosylation of IgA1 which was recognized as the key etiopathogenisis of IgA nephropathy. However, the exact source and the pathogenic mechanism of aberrantly glycosylated IgA1 remain obscure. The further studies on aberrant O-glycosylation of IgA1 would contribute to the understanding of IgA nephropathy and provide new therapeutical strategy.
Animals ; Glomerulonephritis, IGA ; etiology ; metabolism ; Glycosylation ; Humans ; Immunoglobulin A ; metabolism

Adult ; Glomerulonephritis, IGA ; etiology ; Hepatitis C ; complications ; Humans ; Male

Adult ; Cadmium ; Female ; Glomerulonephritis, IGA ; etiology ; Humans ; Occupational Exposure

Adult ; Atypical Hemolytic Uremic Syndrome ; etiology ; Female ; Glomerulonephritis, IGA ; complications ; Humans ; Puerperal Disorders ; etiology

OBJECTIVETo understand the clinical and pathological characteristics of IgA nephropathy (IgAN) with crescentic formation in children.

METHODSClinicopathological data of 29 children with IgAN accompanied by crescents were analyzed. These patients were divided into two groups according to the percentage of glomeruli affected by crescents more or less than 50%, and their data were compared.

RESULTS(1) CLINICAL FEATURES: all the patients had hematuria and proteinuria, and macrohematuria (86%) and proteinuria were also common, protein excreted in urine was more than 1 g per day in 76% of the patients. The patients with edema, hypertension, and renal insufficiency were less than fifty percent. Nine patients in Group A (glomeruli affected by crescents > or = 50%) were crescentic IgAN. Significantly more cases in Group A had persistent macrohematuria, hypertension and renal failure than in Group B (glomeruli affected by crescents < 50%) (P < 0.05), with especially severe proteinuria (P < 0.01). It was easy to find nephritic syndrome in Group A, and asymptomatic hematuria combined with proteinuria in Group B. (2) Renal pathology: the glomeruli were affected by crescents from 5% to 85%. There were 52% to 85% in Group A, and 5% to 40% in Group B. Most crescents were cellular. All the cases had a diffuse mesangial proliferation and tubular-interstitial injury to different degree. Three cases had crescentic IgAN. Glomerulosclerosis was significantly more often seen in Group A (P < 0.05) and tuft adhesion was more frequently seen in Group B (P < 0.05). (3) Immunofluorescence: All the patients presented deposition of IgA, IgM and C3. There were 45% specimens combined with the deposition of IgG. Five cases showed 'full house' (17%), four of them were in Group A. None had IgA deposition alone.

CONCLUSIONThe main clinical feature of IgAN with crescentic formation were hematuria combined with proteinuria, especially persistent gross hematuria and severe proteinuria. All of them showed diffuse mesangial proliferation and tubular-interstitial injury in morphology of kidney. Most of them had tuft adhesion. The main type of immunofluorescence were IgA + IgM and IgA + IgM + IgG deposition. Some showed 'full house' phenomenon. The clinical manifestation and renal lesions of IgAN with diffuse crescentic formation were worse than IgAN with glomeruli affected by crescents < 50%.

Adolescent ; Biopsy ; Child ; Child, Preschool ; Female ; Glomerulonephritis, IGA ; complications ; pathology ; Hematuria ; etiology ; Humans ; Hypertension ; etiology ; Kidney ; pathology ; Kidney Function Tests ; Male ; Prognosis ; Proteinuria ; etiology ; Renal Insufficiency ; etiology

The combination of idiopathic thrombocytopenic purpura (ITP) and chronic renal failure (CRF) is uncommon. This report highlights a case of renal transplantation in a patient with ITP. A 35-year-old man with ITP was admitted with uremic symptoms. A renal transplant and splenectomy was simultaneously performed. A prophylactic pneumococcous vaccination was performed and intravenous immunoglobulin (1 g/kg) was administered before and after the operation. The patient's platelet count increased gradually after the splenectomy. During a two-year follow up period, the graft function was well maintained. Renal transplantation in a patient with ITP is recommended with a well-designed strategy to prevent potential complications.
Adult ; Glomerulonephritis, IGA/complications ; Humans ; Kidney Failure, Chronic/*complications/etiology/*surgery ; *Kidney Transplantation ; Male ; Purpura, Thrombocytopenic, Idiopathic/*complications

A retrospective study of 223 patients with IgA nephropathy (IgAN) was performed to clarify the prognostic factors and the renal survival rates of the disease. One hundred twenty-two patients were followed-up for more than 6 months after their renal biopsy (mean follow-up duration: 43.0 months), and 20 of them (16.4%) had progressed to end-stage renal disease (ESRD). Using univariate analysis, 8 risk factors (2 clinical and 6 histopathological findings) for developing ESRD were identified: renal insufficiency at initial presentation (serum creatinine > or = 1.5 mg/dl); heavy proteinuria(> or = 3.5 gm/day); moderate to severe histopathologic findings such as class IV/V lesions by W.H.O. classification, mesangial hypercellularity, glomerular sclerosis, interstitial infiltration, interstitial fibrosis, and tubular atrophy. In multivariate regression analysis, class IV/V lesions and renal insufficiency at initial presentation were the independent prognostic factors of IgAN. The renal survival rates were 100% at 1 year, 97.0% at 3 years, and 78.9% at 5 years. In conclusion, it seems that about 20% of IgAN patients have a risk to progress to ESRD within 5 years, and a careful follow-up is recommended especially in patients who have either renal insufficiency at the time of presentation or severe renal pathology (class IV/V lesions).
Adolescent ; Adult ; Female ; Glomerulonephritis, IGA/*complications/pathology ; Human ; Kidney Failure, Chronic/*epidemiology/*etiology/pathology ; Male ; Prognosis ; Retrospective Studies ; Risk Factors

Diffuse glomerular basement membrane (GBM) lamellation, reminiscent of Alport's syndrome, has rarely, and exclusively, been reported in renal allografts from pediatric donors to adult recipients. We report on a similar lesion, identified in a 42-year-old male, who received a kidney from an unrelated 21-year-old living male donor. The disease of the recipient was unknown. Renal allograft biopsies were performed 3.5 and 4.8 years after the renal transplantation, due to massive proteinuria and serum creatinine elevation. The histological features of both biopsies were similar, but more advanced in the second biopsy. Glomerular mesangium was widened and had an IgA deposit in the first biopsy. In addition to the presence of mesangial electron dense deposits, the GBM showed diffuse lamellation and splintering on the subepithelial side, but no definite deposits. In the second biopsy, IgA deposits were extended to the peripheral capillary walls, but electron microscopic examination was not available. Two months after the second biopsy, the patient returned for hemodialysis.
Adult ; Basement Membrane/*pathology ; Case Report ; Glomerulonephritis, IGA/*etiology/*pathology ; Human ; Kidney Glomerulus/*pathology ; *Kidney Transplantation/*adverse effects ; Male

Diffuse glomerular basement membrane (GBM) lamellation, reminiscent of Alport's syndrome, has rarely, and exclusively, been reported in renal allografts from pediatric donors to adult recipients. We report on a similar lesion, identified in a 42-year-old male, who received a kidney from an unrelated 21-year-old living male donor. The disease of the recipient was unknown. Renal allograft biopsies were performed 3.5 and 4.8 years after the renal transplantation, due to massive proteinuria and serum creatinine elevation. The histological features of both biopsies were similar, but more advanced in the second biopsy. Glomerular mesangium was widened and had an IgA deposit in the first biopsy. In addition to the presence of mesangial electron dense deposits, the GBM showed diffuse lamellation and splintering on the subepithelial side, but no definite deposits. In the second biopsy, IgA deposits were extended to the peripheral capillary walls, but electron microscopic examination was not available. Two months after the second biopsy, the patient returned for hemodialysis.
Adult ; Basement Membrane/*pathology ; Case Report ; Glomerulonephritis, IGA/*etiology/*pathology ; Human ; Kidney Glomerulus/*pathology ; *Kidney Transplantation/*adverse effects ; Male

Immunoglobulin A nephropathy (IgAN), which can develop into end-stage renal disease, is the most common primary glomerulonephritis. The pathogenesis of IgAN is not clear. Many studies have confirmed that genetic susceptibility is associated with IgAN, and it belongs to polygenic disease. Some studies have found that IgAN is associated with chromosome 6q22-23, 2q36 by linkage analysis, and several candidate genes have been confirmed to be associated with IgAN, such as angiotensin converting enzyme, Fc fragment of IgA receptor, human leukocyte antigen. In recent years, as the progression of molecular genetics and the Human Genome Project, more attention has been paid to the role of genetic factors in the pathogenesis of IgAN.
Animals ; Chromosomes, Human, Pair 2 ; genetics ; Chromosomes, Human, Pair 6 ; genetics ; Genetic Association Studies ; Genetic Linkage ; Genetic Predisposition to Disease ; genetics ; Glomerulonephritis ; complications ; Glomerulonephritis, IGA ; etiology ; genetics ; Humans