OBJECTIVETo study the clinical characteristics of children with an initial onset of IgA nephropathy with nephrotic syndrome and compare them with children with primary nephrotic syndrome, in order to provide a theoretical basis for the differential diagnosis of the two diseases.

METHODSFifty children diagnosed with an initial onset of IgA nephropathy with nephrotic syndrome were included in this study. Seventy-two children diagnosed with an initial onset of primary nephrotic syndrome served as the control group. The clinical and laboratory examination characteristics were compared between the two groups.

RESULTSThe IgA nephropathy group had significantly higher incidence rates of gross haematuria, microscopic haematuria, hypertension, acute kidney injury, low serum high-density lipoprotein cholesterol, anemia, low serum complement C4, steroid resistance, and nephritis-type nephrotic syndrome and a significantly lower incidence of elevated serum IgE compared with the control group (P<0.05). There were significant differences in serum creatinine, serum uric acid, serum total cholesterol, serum high-density lipoprotein cholesterol, serum IgE, serum complement C4, and hemoglobin levels between the IgA nephropathy and the control groups (P<0.05). The thresholds of serum IgE (<131.2 IU/mL) and high-density lipoprotein cholesterol (<1.35 mmol/L) were reference parameters in the differential diagnosis of IgA nephropathy with nephrotic syndrome and primary nephrotic syndrome.

CONCLUSIONSChildren with IgA nephropathy presenting nephrotic syndrome manifest mainly as nephritis type and steroid-resistant type in the clinical classification. Cinical manifestations accompanied by serum levels of high-density lipoprotein cholesterol and IgE are helpful for differential diagnosis of IgA nephropathy presenting nephrotic syndrome and primary nephrotic syndrome.

Adolescent ; Child ; Child, Preschool ; Cholesterol, HDL ; blood ; Complement C4 ; analysis ; Female ; Glomerulonephritis, IGA ; blood ; complications ; Hematuria ; etiology ; Humans ; Immunoglobulin E ; blood ; Male ; Nephrotic Syndrome ; blood ; complications

OBJECTIVETo explore the changes in the clinicopathological features of patients with IgA nephropathy with elevated uric acid level.

METHODSA total of 171 patients with IgA nephropathy diagnosed at biopsy were classified into 3 groups, namely normotensive group with normal level uric acid (group 1), normotensive group with elevated uric acid level (group 2), and hypertensive group with elevated uric acid level (group 3). The clinicopathological features were compared between the 3 groups.

RESULTSFrom group 1 to group 3, the disease duration became elongated, body weight increased, systolic and diastolic pressures elevated, blood urea nitrogen and serum creatinine increased, glomerular filtration rate decreased, and 24-h urine protein increased; the apolipoprotein A, high-density lipoprotein and albumin levels decreased, while apolipoprotein B100, triglyceride, cholesterol and low-density lipoprotein increased. The glomerular damage, tubulointerstitial lesions and arteriole hypertrophy worsened, and Lee's grade III changes were predominant in group 1, grade III or IV in group 2 and grades III-V in group 3. Mesangial proliferative glomerulonephritis was the major pathological type in groups 1 and 2, as compared with focal segmental glomerulonephritis or sclerosing glomerulonephritis in group 3.

CONCLUSIONPatients with IgA nephropathy and elevated uric acid level have greater clinicopathological damage than those with normal uric acid level, and hypertension further aggravates such damages.

Adolescent ; Adult ; Creatinine ; blood ; Female ; Glomerulonephritis, IGA ; complications ; pathology ; Humans ; Hypertension ; complications ; pathology ; Hyperuricemia ; etiology ; pathology ; Kidney ; pathology ; Male ; Middle Aged ; Young Adult

Oxidative stress plays various roles in the development and progression of IgA nephropathy, while bilirubin is known as a potent antioxidant. We therefore hypothesized that serum bilirubin would be associated with renal prognosis in IgA nephropathy. The study subjects comprised 1,458 adult patients with primary IgA nephropathy in Korea. We grouped patients according to the following quartile levels of bilirubin: <0.4 mg/dL (Q1), 0.4-0.5 mg/dL (Q2), 0.6-0.7 mg/dL (Q3), and >0.8 mg/dL (Q4). The outcome data were obtained from the Korean Registry of end-stage renal disease (ESRD). Eighty patients (5.5%) contracted ESRD during a mean follow-up period of 44.9 months. The ESRD incidences were 10.7% in Q1, 8.2% in Q2, 2.8% in Q3, and 2.8% in Q4 (p<0.001). The relative risk of ESRD compared to that in Q1 was 0.307 (95% confidence interval [CI], 0.126-0.751) in Q3 and 0.315 (95% CI, 0.130-0.765) in Q4. The differences of ESRD incidence were greater in subgroups of males and of patients aged 35 yr or more, with serum albumin 4.0 g/dL or more, with normotension, with eGFR 60 mL/min/1.73 m2 or more, and with proteinuria less then 3+ by dipstick test. In conclusion, higher bilirubin level was negatively associated with ESRD incidence in IgA nephropathy.
Adult ; Bilirubin/*blood ; Disease Progression ; Female ; Glomerular Filtration Rate ; Glomerulonephritis, IGA/*blood/complications ; Humans ; Hypertension/complications ; Incidence ; Kidney Failure, Chronic/*blood/complications ; Male ; Middle Aged ; Risk ; Risk Factors ; Treatment Outcome

OBJECTIVETo analyze the characteristics of proteinuria and glomerular lesions in Henoch-Schönlein purpura nephritis (HSPN) of adults.

METHODSThe pathologic results and clinical data of 103 HSPN patients were analyzed.

RESULTSPatients with mild or moderate proteinuria accounted for 40.8% and 37.9%, respectively. Patients with nephropathic proteinuria accounted for about 21.4%. Severer proteinuria resulted in higher blood pressure and increased cholesterol, and albumin decreased gradually (P < 0.01); meanwhile, total glomerular lesion, tubulointerstitial lesion, and vascular lesion also became severer (P < 0.01).

CONCLUSIONProteinuria may indirectly reflect the severity of glomerular lesions in HSPN of adults.

Adolescent ; Adult ; Aged ; Female ; Glomerulonephritis, IGA ; diagnosis ; etiology ; Humans ; Immunoglobulin A ; blood ; Immunoglobulin G ; blood ; Immunoglobulin M ; blood ; Kidney Glomerulus ; pathology ; Kidney Tubules ; pathology ; Male ; Middle Aged ; Nephritis ; diagnosis ; etiology ; Proteinuria ; etiology ; urine ; Purpura, Schoenlein-Henoch ; complications ; pathology ; Retrospective Studies

OBJECTIVEHenoch-Schonlein purpura nephritis (HSPN) and IgA nephropathy are very similar in immunopathological changes, and therefore some nephrologists considered that they are substantially one disease entity caused by IgA immune abnormalities, and IgA nephropathy is, in fact, a kind of HSPN without rashes. The present study aimed to characterize their relationship through clinico-pathological comparison between IgA nephropathy and HSPN.

METHODSThirty-one children with IgA nephropathy aged from 3 to 15 years and 120 children with HSPN aged between 4 to 15 years were enrolled in this study. Their clinical manifestations, blood biochemical test, serum immunology and follow-up data were collected and analyzed. Renal pathological findings in light microscopy, immunofluorescence and electron microscopy were analyzed and also compared between 31 children with IgA nephropathy and 32 children with HSPN.

RESULTSThe age of onset was over 12 years in 25.8% children with IgA nephropathy, but only in 10% with HSPN, and the difference was significant (P < 0.05). The clinical patterns of IgA nephropathy and HSPN were similar, but extra-renal manifestations were seen more often in HSPN, all of them had skin purpura, 59% had gastrointestinal symptoms and 47% suffered from arthralgia. While the abdominal pain occurred only in 3.2% children with IgA nephropathy. The renal pathological investigation showed global sclerosis in 35.5% of IgA nephropathy and 3.1% of HSPN, mesangial sclerosis in 41.9% of IgA nephropathy and 6.3% of HSPN, but endothelial proliferation in 65.6% of HSPN and 29% of IgA nephropathy. The differences were extremely significant (P < 0.01). Thin basement membrane nephropathy were only found in 6.5% children with IgA nephropathy, but in none with HSPN. The electronic dense deposits in HSPN were sparse, loose and widely spread in glomerular mesangium, subendothelial area and even intra basement membrane. While the deposits were dense, lumpy and mostly limited in mesangium and paramesangium in IgA nephropathy. IgG was found in glomerular immune deposits in 71.9% of HSPN, but only 19.4% of IgA nephropathy. No IgG deposit was observed in 81.6% of IgA nephropathy, among them most showed IgA and IgM and/or C(3) deposit. Predominant IgG deposits were found in 12.5% of HSPN with relatively weak IgA deposit, moreover 6.3% of HSPN showed linear IgG deposits in glomerular capillary wall, which couldn't be found in IgA nephropathy. The follow-up data of average 20 months showed complete remission in 72.5% of HSPN and 19.4% in IgA nephropathy after 34 months follow-up. Moreover, 64.5% of IgA nephropathy had consistent hematuria and proteinuria, 16.1% had active nephritides, the difference was significant (P < 0.05).

CONCLUSIONSignificant clinico-pathological differences were found between HSPN and IgA nephropathy, which does not support the one disease entity hypothesis. HSPN and IgA nephropathy are probably two diseases with similar immune abnormalities.

Adolescent ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Glomerulonephritis, IGA ; diagnosis ; Humans ; Immunoglobulin A ; blood ; Immunoglobulin G ; blood ; Immunoglobulin M ; blood ; Immunologic Tests ; Male ; Nephritis ; diagnosis ; etiology ; Prognosis ; Purpura, Schoenlein-Henoch ; complications

Glomerulonephritis occurs as a rare form of renal manifestation in Plasmodium falciparum malaria. Herein, we report a case of falciparum malaria-associated IgA nephropathy for the first time. A 49-yr old male who had been to East Africa was diagnosed with Plasmodium falciparum malaria. Microhematuria and proteinuria along with acute kidney injury developed during the course of the disease. Kidney biopsy showed mesangial proliferation and IgA deposits with tubulointerstitial inflammation. Laboratory tests after recovery from malaria showed disappearance of urinary abnormalities and normalization of kidney function. Our findings suggest that malaria infection might be associated with IgA nephropathy.
Acute Kidney Injury/etiology/pathology ; Antimalarials/therapeutic use ; Creatinine/blood ; Glomerulonephritis, IGA/*diagnosis/*etiology ; Hematuria/etiology ; Humans ; Immunoglobulin A/*metabolism ; Malaria/*complications/drug therapy/*pathology ; Male ; Middle Aged ; Plasmodium falciparum/*isolation & purification ; Proteinuria/etiology ; Quinine/therapeutic use

OBJECTIVETo study the long-term outcome of idiopathic IgA nephropathy and analyze its clinical and histological prognostic factors.

METHODSWe conducted a retrospective analysis of 495 cases of IgA nephropathy patients in our hospital from 1986 to 2002. Renal survival was analyzed by Kaplan-Meier method, univariate analysis and multivariate Cox regression analysis.

RESULTSThe cumulative renal survival rate was 85% at 10 years and 70.9% at 15 years. Univariate analysis found the following parameters at the time of biopsy to be significantly correlated with poor renal survival: serum creatinine > or = 133 micromol/L (P < 0.001), hypertension (P < 0.05), 24-hour urine protein excretion > or = 3 g (P < 0.001), histological subclass V (P < 0.001), and without the history of gross hematuria (P < 0.05). The Cox regression analysis showed that only the presence of hypertension (HR=7.75, 95%CI 1.02 to approximately 2.19) and serum creatinine level (HR=1.49, 95%CI 1.54 to approximately 38.90) were independent prognostic indicators.

CONCLUSIONSThe findings in Chinese IgA nephropathy patients are in agreement with the results of many other studies worldwide.

Adolescent ; Adult ; Child ; Creatinine ; blood ; Female ; Glomerulonephritis, IGA ; complications ; pathology ; physiopathology ; Humans ; Kidney ; pathology ; physiopathology ; Male ; Middle Aged ; Prognosis ; Regression Analysis ; Renal Insufficiency ; etiology ; pathology ; physiopathology ; Retrospective Studies ; Treatment Outcome

BACKGROUND/AIMS: Tubulointerstitial injury plays an important role in the progression of immunoglobulin A nephropathy (IgAN), and neutrophil gelatinase-associated lipocalin (NGAL) is among the most sensitive tubular biomarkers. We investigated whether serum or urine NGAL predicts prognosis in patients with IgAN. METHODS: The present study enrolled patients with biopsy-proven IgAN from January 2005 to December 2010, whose serum and urine samples at the time of kidney biopsy were preserved by freezing. We retrospectively reviewed patient clinical data and followed patients until October 2012. Serum and urine NGAL levels were measured using an enzyme-linked immunosorbent assay kit. Renal progression was defined as an estimated glomerular filtration rate decline by > 50% or progression to end-stage renal disease. RESULTS: There were 121 patients enrolled in this study. During the median follow-up period of 41.49 months, renal progression was found in nine patients (7.4%). Serum or urine NGAL alone could not predict renal progression; however, when serum and urine NGAL levels were combined, belonging to the high NGAL group independently predicted renal progression (hazard ratio [HR], 5.56; 95% confidence interval [CI], 1.42 to 21.73; p = 0.014), along with tubular damage graded according to the Oxford classification as T2 (HR, 8.79; 95% CI, 2.01 to 38.51; p = 0.004). In addition, a Kaplan-Meier curve of renal survival showed significantly higher renal progression in patients in the high NGAL group (log rank, p = 0.004). CONCLUSIONS: In patients with IgAN, high serum and urine NGAL levels at the time of kidney biopsy predict renal progression.
Acute-Phase Proteins/*urine ; Adult ; Biomarkers/blood/urine ; Biopsy ; Chi-Square Distribution ; Disease Progression ; Enzyme-Linked Immunosorbent Assay ; Female ; Glomerular Filtration Rate ; Glomerulonephritis, IGA/*blood/complications/pathology/physiopathology/*urine ; Humans ; Kaplan-Meier Estimate ; Kidney/*metabolism/pathology/physiopathology ; Lipocalins/*blood/*urine ; Male ; Middle Aged ; Multivariate Analysis ; Predictive Value of Tests ; Prognosis ; Proportional Hazards Models ; Proto-Oncogene Proteins/*blood/*urine ; Retrospective Studies ; Risk Factors ; Young Adult

No abstract available.
Adult ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/*complications/diagnosis/drug therapy/immunology ; Antibodies, Antineutrophil Cytoplasmic/*blood ; Biomarkers/blood ; Biopsy ; Drug Therapy, Combination ; Fluorescent Antibody Technique ; Glomerulonephritis, IGA/*complications/diagnosis/drug therapy/immunology ; Humans ; Immunosuppressive Agents/therapeutic use ; Male ; Myeloblastin/*immunology ; Treatment Outcome

Anti-erythropoietin antibodies usually cross-react with all kinds of recombinant erythropoietins; therefore, erythropoiesis-stimulating agent (ESA)-induced pure red-cell aplasia (PRCA) is not rescued by different ESAs. Here, we present a case of ESA-induced PRCA in a 36-yr-old woman with chronic kidney disease, whose anemic condition improved following reintroduction of darbepoetin-alpha. The patient developed progressive, severe anemia after the use of erythropoietin-alpha. As the anemia did not improve after the administration of either other erythropoietin-alpha products or erythropoietin-beta, all ESAs were discontinued. Oxymetholone therapy failed to improve the transfusion-dependent anemia and a rechallenge with ESAs continuously failed to obtain a sustained response. However, her anemia improved following reintroduction of darbepoetin-alpha at 3 yr after the initial diagnosis. Interestingly, anti-erythropoietin antibodies were still detectable, although their concentration was too low for titration. In conclusion, darbepoetin-alpha can improve ESA-induced PRCA when the anti-erythropoietin antibody titer declines and its neutralizing capacity is lost.
Adult ; Anemia/drug therapy/etiology ; Antibodies/*blood/immunology ; Bone Marrow Cells/pathology ; Drug Hypersensitivity/immunology ; Erythropoietin/*analogs & derivatives/therapeutic use ; Erythropoietin, Recombinant/adverse effects/*immunology/therapeutic use ; Female ; Glomerulonephritis, IGA/complications ; Hematinics/adverse effects/immunology/*therapeutic use ; Humans ; Kidney Failure, Chronic/complications ; Oxymetholone/therapeutic use ; Red-Cell Aplasia, Pure/chemically induced/*drug therapy/immunology