Adolescent ; Child ; Female ; Glomerulonephritis, IGA ; diagnosis ; pathology ; Humans ; Male ; Prognosis

OBJECTIVETo analyze the pathological and clinical features of IgA nephropathy (IgAN) in west Guangdong province.

METHODSThe pathological type and clinical features of 120 patients with IgAN were retrospectively analyzed.

RESULTSMesangial proliferative glomerulonephritis and focal segmental glomerulosclerosis were the most frequent features of IgAN. IgM deposit could be found in half of the IgAN patients, especially in the IgAN patients with focal segmental glomerulosclerosis.

CONCLUSIONThe incidence of IgAN may vary between different regions. Clinically, misdiagnosis of other renal diseases as IgAN may often occur. The nature and severity of glomerular immunoglobulin deposition can be related to the pathogenesis and progression of IgAN.

Adult ; China ; Female ; Glomerulonephritis, IGA ; diagnosis ; pathology ; Glomerulonephritis, Membranoproliferative ; pathology ; Glomerulosclerosis, Focal Segmental ; pathology ; Humans ; Male ; Retrospective Studies ; Young Adult

A 27-year-old man presented at the emergency room with hemoptysis. His blood pressure was 180/100 mm Hg, and he had no history of hypertension. Chest radiographs showed bilateral infiltration, suggestive of alveolar hemorrhage. His laboratory data were consistent with acute kidney injury. His serum creatinine level increased abruptly; therefore, renal biopsy was performed. Steroid pulse therapy was administered because of a strong suspicion of immune-mediated pulmonary renal syndrome. Renal biopsy showed proliferative endarteritis, fibrinoid necrosis, and intraluminal thrombi in the vessels without crescent formation or necrotizing lesions. Steroid pulse therapy rapidly tapered and stopped. His serum creatinine level gradually decreased with strict blood pressure control. Ten months after discharge, his blood pressure was approximately 120/80 mm Hg with a serum creatinine level of 1.98 mg/dL. Pulmonary renal syndrome is generally caused by an immune-mediated mechanism. However, malignant hypertension accompanying renal insufficiency and heart dysfunction causing end-organ damage can create a pulmonary hemorrhage, similar to pulmonary renal syndrome caused by an immune-mediated mechanism. The present case shows that hypertension, a common disease, can possibly cause pulmonary renal syndrome, a rare condition.
Adult ; Glomerulonephritis/*diagnosis ; Hemoptysis/pathology ; Hemorrhage/*diagnosis ; Humans ; Hypertension, Malignant/*diagnosis ; Lung Diseases/*diagnosis ; Male

Child ; Early Diagnosis ; Evidence-Based Medicine ; Glomerulonephritis ; diagnosis ; therapy ; Glomerulonephritis, Membranous ; diagnosis ; therapy ; Humans ; Kidney Diseases ; diagnosis ; therapy ; Kidney Glomerulus ; pathology ; Nephrosis, Lipoid ; diagnosis ; therapy ; Renal Replacement Therapy

PURPOSE: There have been numerous researches on urine beta(2)-microglobulin (beta(2)-M) concerned with primary nephrotic syndrome and other glomerular diseases, but not much has been done in relation to pediatric age groups. Thus, our hospital decided to study the relations between the analysis of the test results we have conducted on pediatric patients and renal functions. METHODS: Retrospective data analysis was done to 102 patients of ages 0 to 4 with renal diseases with symptoms such as hematuria, edema, and proteinuria who were admitted to Chung-Ang Yongsan Hospital and who participated in 24-hour urine and urine beta(2)-M excretion test between January of 2003 and January of 2006. Each disease was differentiated as independent variables, and the statistical difference of the results of urine beta(2)-M excretion of several groups of renal diseases was analyzed with student T-test by using test results as dependent variables. RESULTS: Levels of urine beta(2)-M excretion of the 102 patients were as follows:52 had primary nephrotic syndrome [MCNS (n=45, 72+/-45 microgram/g creatinine, microgram/g-Cr), MPGN (n=3, 154+/-415 microgram/g-Cr), FSGS (n=4, 188+/-46 microgram/-Cr)], six had APSGN (93+/-404 microgram/g-Cr), seven had IgA nephropathy (3,414+/-106 microgram/g-Cr), 9 had APN (742+/-160 microgram/g-Cr), 16 had cystitis (179+/-168 microgram/g-Cr), and 12 had HSP nephritis (109+/-898 microgram/g-Cr). IgA nephropathy (P<0.05) and APN (P<0.05) were significantly higher than in other renal diseases. Among primary nephrotic syndrome, FSGS with higher results of beta(2)-microglobulin test had longer treatment period (P<0.01) when compared to the lower groups, but no significant differences in Ccr, BUN, or Cr were observed. CONCLUSIONS: IgA nephropathy and APN groups showed significantly higher level of beta(2)-M excretion value than other groups. Although beta(2)-microglobulin value is not appropriate as an indicator of general renal function and pathology, it seems to be sufficient in the differential diagnosis of the UTI and in the prediction of the treat-ment period of nephrotic syndrome patients.
Creatinine ; Cystitis ; Diagnosis, Differential ; Edema ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Hematuria ; Humans ; Nephritis ; Nephrotic Syndrome ; Pathology ; Proteinuria ; Retrospective Studies ; Statistics as Topic

OBJECTIVETo analysis the clinical and pathological characteristics of children with dense deposit disease (DDD).

METHODS12 Children diagnosed as DDD by electron microscope were enrolled in this study. The clinical and pathological data were analyzed.

RESULTSOf the 12 cases, 7 were males and 5 females, mean age 9.1 +/- 3.9 (5-13) years at onset, the duration from onset to renal biopsy was 1 month to 5 years and the follow-up period was 1-9 years. All cases had heavy proteinuria >50 mg/(kg x d), and persistent microscopic hematuria with recurrent gross hematuria during the course. Seven cases had hypertension (> or = 140/100 mm Hg, 1 mm Hg =0. 133 kPa), 5 cases had transient or recurrent abnormal renal function, and mild to severe anemia were observed in 8 cases respectively. All the cases had lower serum C3 (0.15-0.55 g/L). Clinically, 10 cases were diagnosed as nephritic syndrome (one case had partial lipodystrophy at the same time), and 2 cases were diagnosed as acute nephritic syndrome. Immunofluorescence study showed intense deposition of C3 along GBM, TBM and the wall of Bowman's capsule in a ribbon-like pattern and in the mesangial regions as coarse granules in all the cases. Under light microscopy, 9 cases showed the feature of membrane proliferative glomerulonephritis (MPGN), 1 case with focal segmental glomerulosclerosis (FSGS), 1 case with endocapillary proliferative glomerulonephritis (EnPGN) and 1 case with proliferative sclerosis (PSGN). Crescents were seen in 3 cases. Under electron microscopy, ribbon-like or linear electron-dense intramembranous deposits were identified in the lamina dense of GBM, and often along TBM and the wall of Bowman's capsule. All patients showed steroid resistance. After methylprednisone treatment, some patients showed transient remission. During the follow- up stage of 1-9 years, 3 cases showed normal urinalysis, 5 cases showed partial remission, 2 cases progressed to end stage renal disease (ESRD) and 2 cases were lost.

CONCLUSIONDDD is an in dependently rare disease with pathological-clinical varieties. Children with DDD presented with persistently lower C3, heavy proteinuria, recurrent gross hematuria and anemia. The characteristic immunopathologic finding is intense deposition of C3 along the GBM. Under electron microscopy, ribbon-like or linear electron-dense deposits in the lamina dense of the GBM, TBM and the wall of Bowman's capsule. Electron microscopic examination to demonstrate the intramembranous dense deposits is definitive diagnosis, regardless of the finding of light microscopy. All of them showed steroid resistant. Patients with steroid and CTX treatment showed some clinical improvement of their urinalysis.

Adolescent ; Child ; Child, Preschool ; Female ; Glomerular Basement Membrane ; pathology ; Glomerulonephritis, Membranoproliferative ; diagnosis ; pathology ; therapy ; Humans ; Male

A statistical analysis of the diagnostic value for 244 aspiration biopsy cytology(ABC) among a total 1,043 cases from various sites was performed. ABC, using diagnostic terminology similar to that of a surgical pathology reports, was compared to the final tissue diagnosis. For the entire series, a sensitivity of 91.8%, a specificity of 99.3%, a positive predictive value of 98.9%, a negative predictive value of 94.8%, and an efficacy of the test of 96.3% were shown. There were 8 false negative and 1 false positive diagnosis. The diagnostic accuracy was 89.8%. Those results indicate that the ABC is a considerably highly accurate procedure that should be routinely employed.
Biopsy, Needle ; Child* ; Diagnosis ; Glomerulonephritis, IGA* ; Humans ; Immunoglobulin A* ; Intranuclear Inclusion Bodies ; Meningioma ; Pathology, Surgical ; Sensitivity and Specificity

No abstract available.
*Asian Continental Ancestry Group ; Female ; Glomerulonephritis, IGA/*diagnosis ; Humans ; Kidney/*pathology ; Male

OBJECTIVETo investigate the value of serum IgA/C3 ratio in the diagnosis of IgA nephropathy and explore its relationship with the clinicopathological features of the patients.

METHODSSixty-six patients with IgA nephropathy, 111 with other glomerular diseases, and 40 healthy control subjects without kidney disease were tested for serum IgA and C3 levels using CRM470 adjusted standardized immune turbidimetric method, and the IgA/C3 ratio was calculated. According to Oxford and Lee's classification criteria, we analyzed the pathological grades of the renal biopsy samples from patients with IgA nephropathy. The ROC curve was used to assess the value of serum IgA and IgA/C3 ratio in predicting IgA nephropathy.

RESULTSPatients with IgA nephropathy had an elevated serum IgA/C3 ratio than those with other glomerular diseases and the control subjects, with an area under the ROC curve of 0.776. An elevated serum IgA/C3 ratio was not found to significantly correlate with the pathological grade of renal biopsy samples in patients with IgA nephropathy.

CONCLUSIONIn the absence of renal biopsy findings, serum IgA/C3 ratio can help in the diagnosis of IgA nephropathy.

Biopsy ; Case-Control Studies ; Complement C3 ; analysis ; Glomerulonephritis, IGA ; blood ; diagnosis ; Humans ; Immunoglobulin A ; blood ; Kidney ; pathology

OBJECTIVE: To explore the relationship between pathological features and clinical manifestations in children with nephropathy under 6 years old. METHODS: Renal biopsy by rapid percutaneous puncturation was performed on 313 children under 6 who were all diagnosed clinically as kidney diseases of 14 different kinds. The specimens were divided into 3 parts for microscope, electron microscope and immuno fluorescence examination respectively and processed by HE, PAS, PASM, and Masson staining. Immunofluorescence was used to detect the deposition of IgG, IgM, IgA, C3, C4, C1q, and Fb in the renal tissues. Additional examinations were done to detect HBs-Ag, HBeAg and HBcAg deposition in some cases with positive serum HBs-Ag. Altogether 290 of the specimens (290/313, 92.65%) were examined by electron microscope. RESULTS: All the renal biopsy performances were successful. The clinical manifestations comprised of persistent haematuria (32.92%, 103/313), idiopathic nephritic syndrome (26.1%, 82/313), acute nephritic syndrome (20.14%, 63/313), Henoch Schonlein purpura nephritis (8.32%, 26/313), HBV-nephritis (4.79%, 15/313), and isolated proteinuria (2.56%, 8/313). The main pathological patterns of glomerular disease were identified as mesangial proliferation (51.75%, 162/313), IgM nephropathy (8.31%,26/313), minor and minimal change (7.99%, 25/313), IgA nephropathy (7.35%, 23/313), endocapillary proliferative glomerulonephritis (5.11%, 16/313), focus segmental glomerulosclerosis (4.47%, 14/313), thin basement membrane nephropathy (4.47%, 14/313), and membrane nephropathy (4.47%, 14/313). Alport syndrome, congenital nephrotic syndrome, and thin basement membrane nephropathy can be diagnosed by electron microscope, white IgA nephropathy, IgM nephropathy and C1q nephropathy by immunopathology. CONCLUSION Similar clinical manifestations may differ in the pathology and the clinical features of one pathological diagnosis may vary greatly. Renal biopsy is of great help to the diagnosis, treatment and the prognosis evaluation for children with nephropathy under 6. Electron microscopes also play an important role in the diagnosis of nephropathy.
Biopsy, Needle ; Child ; Child, Preschool ; Glomerulonephritis ; diagnosis ; pathology ; Humans ; Infant ; Kidney ; pathology ; ultrastructure ; Kidney Diseases ; diagnosis ; pathology ; Nephrotic Syndrome ; diagnosis ; pathology