Adolescent ; Child ; Humans ; Male ; Nephritis, Interstitial ; pathology ; Uveitis ; pathology

Biopsy ; methods ; Glomerulonephritis, IGA ; pathology ; Humans ; Kidney ; pathology ; Kidney Diseases ; pathology ; Lupus Nephritis ; pathology

BACKGROUNDRelapses occur frequently in patients with lupus nephritis. Renal biopsy is the gold standard for assessing renal activity and hence guiding the treatment. Whether repeat renal biopsy is helpful during flares of lupus nephritis remains inconclusive. In the present study, we retrospectively reviewed the patients with lupus nephritis who had more than one renal biopsy with the hope to find the clinical value of repeat biopsy.

METHODSPatients who had a diagnosis of lupus nephritis and two or more renal biopsies were selected from the database of the patient pathology registration at this renal division. Renal biopsy was evaluated according to the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification of lupus nephritis. The pathological patterns and treatment regimens were analyzed after a repeat biopsy.

RESULTSWe identified 44 systemic lupus erythematosus patients with serial renal biopsies. In total, there were 94 renal biopsies. Overall, the pathological transition occurred in 64% instances according to the ISN/RPS class. When the transition was analyzed according to proliferative, membranous or mix lesions, it showed different profile: 35% in patients with proliferative lesion, 23.5% patients with mix lesions, 100% in patients with pure membranous lesion. The pathological transition could not be predicted by any clinical characteristics. After the repeat renal biopsy, 34% of patients had a change in their treatment regimens.

CONCLUSIONSThe pathological conversion was very prevalent in patients with lupus nephritis. However, the transitions became less prevalent when they were analyzed according to pure membranous, proliferative, and mix lesion. Repeat biopsy might be helpful to avoid unnecessary increased immunosuppression therapy.

Adult ; Aged ; Biopsy ; Female ; Humans ; Kidney ; pathology ; Lupus Nephritis ; diagnosis ; pathology ; Male

Child ; Collagen Type IV ; Female ; Humans ; Immunohistochemistry ; methods ; Kidney ; pathology ; Male ; Nephritis, Hereditary ; diagnosis ; pathology

OBJECTIVETo study the relationship of cystatin C (CysC), fibrinogen (Fbg), and 24-hour urinary protein with renal pathological grade in children with Henoch-Schönlein purpura nephritis (HSPN), and to explore their values.

METHODSThe clinical data of 48 children diagnosed with HSPN by renal biopsy from January 2011 to January 2015 were reviewed. According to renal pathological grading, in the 48 children with HSPN, 12 had stage IIa or lower, 12 stage IIb, 17 stage IIIa, and 7 stage IIIb or higher. The latex-enhanced immunoturbidimetric assay, turbidimetric measurement, and end-point method were used to determine the levels of serum CysC, Fbg, and 24-hour urinary protein, respectively. Pearson and Spearman correlation analyses were used to test the correlations between the indices and between the indices and renal pathological grade.

RESULTSThere were significant differences in the levels of serum CysC, Fbg, and 24-hour urinary protein between patients with different pathological grades (P<0.05). The level of each index increased with increasing pathological grade (P<0.05). In the 48 children with HSPN, the level of 24-hour urine protein was positively correlated with the levels of serum CysC (r=0.51, P<0.05) and Fbg (r=0.63, P<0.05). The level of Fbg was positively correlated with that of serum CysC (r=0.55, P<0.05). The levels of CysC, Fbg, and 24-hour urinary protein were all positively correlated with renal pathological grade (r=0.66, 0.64 and 0.68; respectively, P<0.05).

CONCLUSIONSThe levels of CysC, Fbg, and 24-hour urine protein can reflect the severity of renal injury, providing satisfactory prediction of the severity of renal injury in children with HSPN.

Child ; Cystatin C ; blood ; Female ; Fibrinogen ; analysis ; Humans ; Kidney ; pathology ; Male ; Nephritis ; pathology ; Proteinuria ; pathology ; Purpura, Schoenlein-Henoch ; blood ; pathology

OBJECTIVETo study feasibility of Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2000) and British Isles Lupus Assessment Group 2004 (BILAG-2004) scoring systems for assessing renal disease activity in children with lupus nephritis (LN).

METHODSThe clinical data of 159 children with systemic lupus erythematosus (SLE) and LN were collected, and disease activity was assessed by SLEDAI-2000 and BILAG-2004 scoring systems. The correlations between SLEDAI-2000 and BILAG-2004 scores and 24-hour urinary protein excretion and renal pathology index were analyzed. The SLEDAI-2000 and BILAG-2004 scoring systems were evaluated using ROC curve.

RESULTSApproximately one third (31.5%) of the 159 children had a moderate level of 24-hour urinary protein excretion. Among the 37 patients undergoing renal biopsy, 46.0% had diffuse LN (type Ⅳ). 24-hour urinary protein excretion was positively correlated with both SLEDAI-2000 (r=0.36, P<0.05) and BILAG-2004 scores (r= 0.39, P<0.05). Children with types Ⅰ, Ⅱ, Ⅲ, and Ⅳ LN had pathology activity index (AI) which positively correlated with SLEDAI-2000 scores (r=0.86, 0.88, 0.84, 0.77 respectively; P<0.05) and BILAG-2004 scores (r= 0.88, 0.98, 0.86, 0.89 respectively; P<0.05). SLEDAI-2000 score showed the best correlation with AI in patients with type Ⅱ LN, followed by those with type Ⅰ LN. BIILAG-2004 score showed the best correlation with AI in patients with type Ⅱ LN, followed by those with type Ⅳ LN. The BILAG-2004 scoring system had an area under the ROC curve (AUC) of 0.93, and the SLEDAI-2000 scoring system had an AUC of 0.88.

CONCLUSIONSBILAG-2004 and SLEDAI-2000 scoring systems can be used to assess renal disease activity of patients with LN. The BILAG-2004 scoring system can provide more reliable and comprehensive assessment.

Adolescent ; Child ; Feasibility Studies ; Female ; Humans ; Kidney ; pathology ; Lupus Erythematosus, Systemic ; pathology ; Lupus Nephritis ; pathology ; Male ; Proteinuria ; classification ; ROC Curve

OBJECTIVE: This study was designed to investigate the clinical outcomes of proliferative lupus nephritis and to identify the predictive factors of remission and relapse of proliferative lupus nephritis after intravenous cyclophosphamide (IVCYC) pulse therapy. MEHTODS: Seventy-four patients with proliferative lupus nephritis that had been diagnosed by renal biopsy and treated with IVCYC pulse therapy were studied. Their demographic data, clinical manifestations, laboratory findings, disease activity index, damage index, activity and chronicity indices of renal pathology, and treatment modalities were evaluated. Clinical outcomes of lupus nephritis were assessed by defined criteria. RESULTS: Remission or response were achieved in 79.7% of patients with proliferative lupus nephritis (remission in 32.4% and response in 47.3%, respectively), and 30.5% of those with remission or response experienced relapse or flare of lupus nephritis (relapse in 20.8% of those with remission and flare in 37.1% of those with response) after IVCYC pulse therapy. High creatinine clearance at diagnosis of lupus nephritis, short lag time from diagnosis of lupus nephritis to initiation of immunosuppressive treatment, and long-term cyclophosphamide pulse therapy were the independent predictive factors for remission or response. Long lag time from completion of immunosuppressive treatment to onset of remission or response, and incomplete cyclophosphamide were the independent risk factors for relapse or flare of lupus nephritis. CONCLUSION: Good renal function and early initiation of long-term IVCYC pulse therapy are important in induction of remission or response, while delayed remission or response and incomplete immunosuppressive treatment is strongly associated with poor outcome.
Biopsy ; Creatinine ; Cyclophosphamide* ; Diagnosis ; Humans ; Lupus Nephritis* ; Pathology ; Recurrence* ; Remission Induction ; Risk Factors

Alport syndrome is the most common type of hereditary nephritis, and acute poststreptococcal glomerulonephritis(APSGN) is a common disease in children. We experienced the clinical and pathologic findings of Alport syndrome and APSGN in brothers of one family. Both patients presented with heavy gross hematuria and proteinuria. ASO titer was elevated in both cases, and the C3 level was reduced in one of the cases. In renal pathology, both showed characteristics of Alport syndrome as well as the glomerular changes of APSGN with hump-like subepithelial deposits by electron microscopy. These clinical observation indicated that the patients had APSGN superimposed on Alport syndrome, and that the episode of APSGN might exacerbate the clinical course of Alport syndrome.
Child ; Glomerulonephritis* ; Hematuria ; Humans ; Microscopy, Electron ; Nephritis, Hereditary* ; Pathology ; Proteinuria ; Siblings*

PURPOSE: Henoch-Schonlein purpura(HSP) is usually a self-limited disease with a good eventual outcome. The prognosis of HSP is mainly determined by the renal involvement. In this study, We evaluated children with biopsy-proven Henoch-Schonlein purpura nephritis about the clinical outcome correlated with renal manifestation and morphologic findings. METHODS: The clinical features, initial laboratory and pathologic findings, and clinical outcome were evaluated in 60 children with biopsy-proven Henoch-Schonlein purpura nephritis at Yonsei University Severance Hospital during the period from Jan. 1990 to Dec. 2002. RESULTS: The ratio of male to female patients was 1.2:1. The interval between the onset of Henoch-Schonlein purpura and renal manifestation was less than 3 months in 81% of the patients. Initial renal manifestation was microscopic hematuria in 100% of patients, isolated hematuria in 15%, acute nephritic syndrome in 7%, nephrotic syndrome in 22% of patients. Renal manifestation correlated with clinical outcome. Grade II and III were the most common in histologic grades of ISKDC. Renal pathologic finding correlated with clinical outcome. CONCLUSION: Renal manifestation and pathologic findings correlated with the clinical outcome. It is necessary to evaluate the correlation between pathologic findings and treatment.
Child ; Female ; Hematuria ; Humans ; Male ; Nephritis* ; Nephrotic Syndrome ; Pathology ; Prognosis ; Purpura, Schoenlein-Henoch*

OBJECTIVETo analyze the clinical features and gene mutation of Chinese children with Alport syndrome(AS).

METHODFrom May 2011 to May 2014, clinical and pathological information gathered from 25 patients was retrospectively analyzed. COL4A5, COL4A4 and COL4A3 genes were analyzed using next-generation sequencing in these patients, and gene mutations of related family members were identified by Sanger method.

RESULTOf these 25 cases, 19(76%) had X-linked Alport syndromes (XL-AS), 6 had autosomal recessive Alport syndromes (AR-AS). Twenty five patients had an onset of hematuria and proteinuria and in 8 cases the disease was induced by upper respiratory tract infections. Hearing loss was present in 2 of 25 (8%) cases and ocular lesions in 1 of 25 (4%). Renal pathology showed that 16 of them had minimal change disease (MCD), 8 mesangial proliferative glomerulonephritis (MsPNG), 1 focal segmental glomerulo-sclerosis (FSGS). Extensive lamination and split of glomerular basement membrane (GBM) dense layers were found in 2 (8%) of 25 patients. Twenty one of 25 patients (84%) showed abnormal renal α-chain distribution. COL4A5, COL4A4 and COL4A3 genes of 25 patients (23 families) were analyzed and 24 pathogenic mutations were identified: 18 in COL4A5, 1 in COL4A3 and 5 in COL4A4. It was observed that 13 patients inherited the mutation from the mother, 3 patients inherited from the father, 2 patients inherited 1 mutation from the mother and another mutation from the father, and 7 patients carried the novel mutations.

CONCLUSIONXL is the main inherited type in AS. Most of patients showed MCD and MsPNG in renal biopsy. This research examined 24 mutations and 16 mutations were not reported previously.

Child ; Deafness ; Genes, Recessive ; Genotype ; Hematuria ; Humans ; Kidney ; Mutation ; Nephritis, Hereditary ; genetics ; pathology ; Pedigree ; Phenotype