IgA nephropathy can occur rarely as a complication of D-penicillamine treatment, but it is exact pathogenesis remains unclear. If a patients has gross or microscopic hematuria during D-penicillamine treatment, D-penicillamine induced IgA nephropathy should be suspected as a cause of hematuria. In those cases, renal biopsy should be taken for diagnosis and proper management. We experienced a case of IgA nephropathy confirmed by renal biopsy in a 39-years-old female patient with scleroderma during D-penicillamine therapy and report this case with a review of literature.
Biopsy ; Diagnosis ; Female ; Glomerulonephritis, IGA* ; Hematuria ; Humans ; Immunoglobulin A* ; Penicillamine*

The authors report series of 360 cases of transthoracic fine-needle aspiration cytology (TFNA) from Oct. 1982, through Aug. 1986 at the Seoul National University Hospital. A diagnosis of neoplastic lesion was established in 50.3% of the cases. A non-neop- lastic diagnosis was made in 38.5%, nondiagnostic one in 6.5% and inadequate one in 4.7% of the total. Statistical findings on cytological diagnoses were as follows. Specificity was 100%: sensitivity, 92%; predictive value for positive, 1.0: predictive value for negative, 0.9; concordance rate, 84.2%: diagnostic accuracy in non-neoplastic lesion, 65.4%, and typing accuracy in malignant tumor, 0.77.
Biopsy, Fine-Needle ; Diagnosis ; Glomerulonephritis, IGA* ; Immunoglobulin A* ; Sensitivity and Specificity ; Seoul

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

Glomerulonephritis, IGA ; diagnosis ; therapy ; Humans ; Integrative Medicine ; Risk Assessment

BACKGROUND: Immunoglogulin A(IgA) nephropathy is the most common primary glomerular disease throughout the world. 30-50% of patients with IgA nephropathy(IgAN) have high serum IgA concentrations. However, we do not know if the degree of elevation in IgA level increases the likelihood of having IgAN. Neither do we know if the IgA level has any association with pathological findings of IgAN. METHODS: We analyzed the relationships between IgAN and the levels of serum IgA which has been a routine part of the study in all patients with glomerulonephritis in our institution for the last 4 years. We reviewed 270 patients in whom the pathological diagnosis and the results of their IgA levels were both available. RESULTS: Of 80 patients who were IgA nephropathy, 26 patients(32.5%) had higher than normal cut- off value of serum IgA(385 mg/dL). In contrast, 8.9 % of patients with other types of glomerulonephropathies showed the values above normal(p<0.0001). The risk ratio for an increase of one unit of the IgA level was 1.0025(logistic regression, p=0.0043), which was increased to 1.0079 when patients with low complement levels were excluded from the analysis. The data were also analyzed according to the immunofluorescence microscopic findings of IgAN, which were found to have no significant correlation with IgA concentrations. CONCLUSION: The IgA level is a risk factor for IgAN throughout the whole range. However, it does not correlate with the IgA deposition in the renal tissue. We believe that this study will help understanding the interpretation of IgA levels in patients with IgAN.
Complement System Proteins ; Diagnosis ; Fluorescent Antibody Technique ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Humans ; Immunoglobulin A* ; Odds Ratio ; Risk Factors

BACKGROUND: Since the introduction of cyclosporine, the short-term renal allograft survival has significantly improved. However, the long-term success is still limited by the development of chronic rejection and recurrent disease. Post-transplant glomerulonephritis (post-Tx GN) including recurrent disease is becoming an important cause of graft dysfunction. METHODS: From November 1988 to June 2004, a total of 629 renal transplants involving 588 patients were performed at our medical center. RESULTS: The prevalence rate of post-Tx GN was 11.9% in 629 renal transplant. Among 75 transplants diagnosed as post-Tx GN, IgA nephropathy (62.7%) was the most common histologic diagnosis, followed by focal segmental glomerulosclerosis (26.7 %), membranous glomerulonephritis (8.0%), membranoproliferative glomerulonephritis (1.3%) and diabetic nephropathy (1.3%). Documented histologic recurrence occurred in only 24.2% of patients with prior biopsy-proven glomerulonephritis of their native kidneys. The actuarial allograft survival at 5 and 10 years posttransplantation with post-Tx GN was 80.5 % and 27.9%, respectively; and the corresponding graft survival for patients without post-Tx GN was 74.9% and 52.3%, respectively (p<0.05). However, there was no significant difference in the graft survival according to type of post-Tx GN. The 5 and 10 year graft survival for patients with proteinuria over than 3.5 g/24 hr were 62.5% and 0%, which is significantly lower compared with 85.3% and 28.7% for patients with proteinuria less than 3.5 g/24 hr (p<0.01). CONCLUSION: In conclusion, post-Tx GN is associated with decreased long-term graft survival and nephrotic range proteinuria is most important prognostic factor for graft survival. A prospective study with rigorous efforts to make pretransplant diagnosis and standardized criteria for allograft biopsy will more accurately characterize the natural history of post-Tx GN and may provide insight regarding treatment.
Allografts* ; Biopsy ; Cyclosporine ; Diabetic Nephropathies ; Diagnosis ; Glomerulonephritis* ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Graft Survival ; Humans ; Kidney ; Natural History ; Prevalence ; Proteinuria ; Recurrence ; Transplants

Diagnosis, Differential ; Family Health ; Genetic Predisposition to Disease ; genetics ; Glomerulonephritis, IGA ; diagnosis ; genetics ; HLA Antigens ; genetics ; Humans

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

PURPOSE: To classify the results of renal biopsy in pediatric patients and to compare pathological findings with clinical features. METHODS: This study included data of 318 children who underwent renal biopsy at our hospital between December 1987 and November 2014. Biopsy specimens were examined histopathologically using light, immunofluorescence, and electron microscopy. RESULTS: Asymptomatic urinary abnormalities was the most common clinical diagnosis (35.9%), followed by nephrotic syndrome (29.3%), and acute glomerulonephritis (18.0%). Glomerular disease was identified in 98.1% of the renal biopsy specimens. The most common primary cause of glomerulonephritis was IgA nephropathy, with gross hematuria in 61.9% of the patients, hypertension in 14.2%, proteinuria >1.0 gm/24-hr in 33.3%, and impaired renal function in 3.6% patients. CONCLUSION: The most common clinical diagnosis was asymptomatic urinary abnormalities, with primary glomerular disease being the most common renal biopsy finding, and IgA nephropathy the most common histopathological lesion. This study provides a 27-year overview of pediatric renal disease at our center and underlines the importance of renal biopsy for accurate diagnosis and proper management.
Biopsy* ; Child* ; Diagnosis ; Fluorescent Antibody Technique ; Glomerulonephritis ; Glomerulonephritis, IGA ; Hematuria ; Humans ; Hypertension ; Microscopy, Electron ; Nephrotic Syndrome ; Proteinuria

BACKGROUND: Crescentic glomerulonephritis is expressed pathologically by crescent formation in Bowman's capsule and clinically by rapidly progressive loss of renal function. The pathologic experience of crescentic glomerulonephritis in one institution was analyzed here. METHODS: We classified 25 cases of crescentic glomerulonephritis patients into 4 categories and reviewed the cases pathologically and clinically. RESULTS: We found no case with group I (anti- GBM disease), 8 cases in group II (immune complex glomerulonephritis) including 3 patients with IgA nephropathy, 2 patients with Henoch-Sch nlein purpura and 3 patients with APSGN, 12 cases in group III (ANCA-associated glomerulonephritis) including 7 patients with microscopic polyangitis, 4 patients with Wegener's granulmatosis and 1 patient with ANCA GN, and 5 cases in group IV (idiopathic crescentic glomerulonephritis). The mean ages of patients with group II, III, and IV were 32.0, 59.3 and 39.0 years old, respectively, and mean serum creatinine levels at the time of biopsy were measured as 9.1, 5.2, 8.8 mg/dL in each group. On light microscopic findings, the frequency of crescents in glomeruli was 64.4% in group II, 43.7% in group III, and 51.2% in group IV. The score of infiltration into tubules of inflammatory cells was 0.8 in group II, 0.4 in group III, and 0.6 in group IV and the score of fibrosis in interstitium was 1.0 in group II, 0.8 in group III and 1.2 in group IV. The score of atherosclerosis in arteries was 1.4, 0.9 and 1.6 in each group. CONCLUSION: We conclude that the precise diagnosis and classification of crescentic glomerulonephritis by an early renal biopsy and clinical assessments are important in the management of rapidly progressive (crescentic) glomerulonephritis. Since the number of the cases was not so enough, we could not analyze the statistical significance between morphologic differences of each group of crescentic glomerulonephritis, but if more cases were collected, acknowledgements of differences and prognostic factors in pathologic findings could be possible.
Antibodies, Antineutrophil Cytoplasmic ; Arteries ; Atherosclerosis ; Biopsy ; Bowman Capsule ; Classification ; Creatinine ; Diagnosis ; Fibrosis ; Glomerulonephritis* ; Glomerulonephritis, IGA ; Humans ; Purpura