OBJECTIVETo analyze the characteristics of repeated renal biopsy-proven primary focal segmental glomerulosclerosis (PFSGS) in 8 children, and to reveal the relationship between clinical features and pathology, between the two times of renal biopsy pathology, and the indications for repeated renal biopsy.

METHODThe records of cases who ever experienced renal biopsy in this hospital were reviewed, of whom 8 cases of repeated renal biopsy-proven PFSGS were enrolled. The clinical manifestations, the reason why they had renal biopsy again, the difference in renal pathological findings, between the two biopsies and their therapeutic response. The classification of focal segmental glomerulosclerosis (FSGS) was based on the new criteria suggested by D'Agati in 2004.

RESULTOf the 8 cases, age of onset ranged from 1 to 12 years, all were diagnosed as nephrotic syndrome (NS), the age of first biopsy ranged from 1.1 to 15.0 years, and the follow-up period was 10 months to 14 years. The reason for repeated biopsy was poor therapeutic response, continuous heavy proteinuria, or the progressive renal dysfunction. Four cases had the both biopsies in this hospital, and the first renal pathology showed minimal change disease (MCD), mesangial proliferation, FSGS CELL type and FSGS GTL type. After the second biopsy, they were additionally treated with immunosuppressive agents or switched to another one, 2 cases with FSGS COLL type presented renal dysfunction or end stage renal disease (ESRD), 1 case who developed the disease at 1.4 years of age, presented renal dysfunction at 10 months follow-up. The remaining 5 cases acquired complete remission.

CONCLUSIONFSGS is a clinicopathological syndrome, NS predominates clinically. It often indicates pathologic transformation when the patients show poor therapeutic response or continuous heavy proteinuria without remission. Mesangial proliferation can convert into FSGS, and the subtype of FSGS can shift. FSGS COLL type and onset at young age may suggest poor prognosis.

Biopsy ; Child ; Child, Preschool ; Female ; Glomerulosclerosis, Focal Segmental ; pathology ; Humans ; Infant ; Kidney ; pathology ; Male

OBJECTIVETo evaluate the correlation between clinico-pathological features and outcome of children with primary focal segmental glomerular sclerosis (FSGS).

METHODA total of 212 pediatric patients with D'Agati (2004) primary FSGS were included in this study between 1997 and 2008. According to FSGS histologic classification criteria, 5 pathologic variants were recognized: collapsing (COLL), cellular (CELL), glomerular tip lesion (GTL), perihilar, and not otherwise specified (NOS). Retrospective analysis of the therapeutic response, the relationship between the clinical efficacy and pathology and the outcome of the patients was made.

RESULTSOf the 212 patients, 178 (83.9%) had nephritic syndrome (NS), 97 (45.8%) had simple NS, 81 (38.2%) had nephritis-type NS, GTL variants were mostly appeared to be nephritic syndrome (n = 28) and COLL variants were the fewest (n = 11). The difference between the two variants had statistical significance (P < 0.05). Fourteen cases (6.6%) had nephrotic proteinuria, 20 cases (9.4%) had proteinuria with micro-hematuria. According to histologic classification, NOS (n = 86, 40.6%) was the most common type; perihilar type was seen in 25 cases (11.8%); CELL was seen in 58 cases (27.4%), COLL in 12 cases (5.6%), GTL in 31 cases (14.6%). Chronic tubular injury was present in most cases. CEL variants were mostly found in the early infancy. GTL and NOS variants initially appeared to be responsive to steroids, but subsequently became resistant or frequently recurrent; CELL and COLL appeared to be primarily steroid resistant, GTL and COLL variants had statistically significant differences (P < 0.05). The patients were followed-up for 5 months to 10 years. A response to therapy was observed in 50%, COLL FSGS had the highest rate of ESRD; 2 years renal survival rates were 67%, 3 years were 41%.

CONCLUSIONSFSGS is defined as a clinicopathologic syndrome manifesting proteinuria and focal and segmental glomerular sclerosis with foot process effacement. The location of the sclerosis within the glomeruli proved to have prognostic significance. Collapsing glomerulopathy is the most aggressive variant of FSGS. Compared with other variants, GTL variant may be the best type. Different histologic variants of FSGS have substantial differences in clinical features at the time of biopsy diagnosis and substantial differences in renal outcomes. Prolonged treatment of FSGS-NS with corticosteroids and immune suppressive agents may have some effects in achieving sustained remission and improve prognosis in children.

Adolescent ; Child ; Child, Preschool ; Female ; Glomerulosclerosis, Focal Segmental ; pathology ; Humans ; Infant ; Male ; Proteinuria ; pathology ; Retrospective Studies

Lessons learned over 25 years from treating focal segmental glomerulosclerosis (FSGS) in a regional pediatric nephrology program will be reviewed. In a cohort of children with biopsy-proven FSGS, consisting of a large percentage of African-American patients, the clinical characteristics and long-term outcomes are different from previously published data on mostly white children with the same condition. Because Asian and African children with nephrotic syndrome have the same higher incidence of FSGS, it is possible that Chinese children with FSGS have clinical characteristics, therapeutic responses, and long-term outcomes similar to African-American children as reviewed in this article.
African Americans ; Child ; Glomerulosclerosis, Focal Segmental ; diagnosis ; pathology ; Humans ; Nephrotic Syndrome ; diagnosis ; pathology ; Prognosis

OBJECTIVETo explore the correlation between pathological characteristics of target organs and excess evil syndrome in IgA nephropathy.

METHODSData were collected in multicenter cooperation. Totally 266 IgA nephropathy patients were typed into exogenous wind-heat affection syndrome (49 cases), lower energizer damp-heat syndrome (100 cases), damp-phlegm syndrome (43 cases), and blood stasis syndrome (74 cases). Meanwhile, percutaneous renal biopsy was performed in all patients for Hass classification, Oxford classification, Katafuchi integral, and Jiang's classification methods. The correlation between excess evil syndrome and pathological index was analyzed.

RESULTSFour syndrome types were correlated with their Hass levels (r = 0. 341, P <0. 01). Affection of exogenous wind-heat syndrome was correlated with segmental proliferation of endothelial cells and damaged active lesions of segmental capillary loops. Lower-energizer damp-heat syndrome was associated with Hass III level, destroying active lesions of capillary loops, segmental proliferation of endothelial cells, glomerular segmental lesions, focal interstitial infiltration of inflammatory cells, focal interstitial fibrosis and tubular atrophy. Blood stasis syndrome was associated with Hass IV level, glomerular sclerosis, segmental glomerulosclerosis (S)/adhesion, mesangial hypercellularity (M), angiohyalinosis, multi-foci interstitial infiltration of inflammatory cells, multi-foci interstitial fibrosis and tubular atrophy. Phlegm-damp syndrome had higher proportions of Hass I and III levels, but with no association with other pathological parameters.

CONCLUSIONSExcess evil syndrome was associated with partial pathological characteristics of IgA nephropathy. It could reflect pathological damage degree of target organs, activities, chronic lesions, and prognosis of IgA nephropathy to certain extent. Correlated pathological characteristics and its evolution could indicate excess evil syndrome types and their evolution rules.

Capillaries ; Fibrosis ; Glomerulonephritis, IGA ; pathology ; Glomerulosclerosis, Focal Segmental ; Humans ; Kidney Glomerulus ; Medicine, Chinese Traditional ; Prognosis ; Syndrome

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

To evaluate the distribution and changing patterns of renal diseases in Korea, a total of 4,514 cases of renal biopsy collected over a 23-year period between 1973 and 1995 were reviewed. Of 4,200 cases excluding 314 unsatisfactory biopsies, adult cases comprised 59.5% and pediatric cases, 40.5%. The male to female ratio was 1.5:1 in adults and 2.2:1 in children. Glomerulonephritis (GN) comprised 80.0% of the total. The most common primary GN in adults was minimal change disease (MCD) (26.6%), followed by IgA nephropathy (IgAN) (22.1%), membranous GN (MGN) (11.8%), and membranoproliferative GN (MPGN) (5.9%). In children, the primary GN incidence rates were MCD (24.8%), IgAN (10.3%), poststreptococcal (including postinfectious) GN (PSGN) (8.6%), and focal segmental glomerulosclerosis (FSGS) (4.0%). The most common secondary GN in adults was lupus nephritis and in children Henoch-Schonlein purpura nephritis. The most common cause of nephrotic syndrome was MCD in both adults and children, followed by MGN and FSGS. The elderly, aged sixty years and older, comprised 2.7% of cases and recorded equal numbers of MCD and MGN. The proportion of the biopsies found to be seropositive for HBs antigen was 27.9%, and these showed either MGN or MPGN pattern. Repeat biopsy was performed in 168 patients, due to previous biopsy failure in 15.5%. When the primary GN cases were analyzed at 5-year intervals, the prevalence of PSGN, which was greater than 25% during the 1973-1982 period, decreased abruptly in children thereafter, whereas the prevalence of FSGS increased slowly since the 1988-1992 period in both adults and children. The decrease of PSGN and the increase of FSGS suggest a role for socioeconomic and environmental factors in Korea.
Adult ; Age Distribution ; Biopsy ; Child ; Female ; Glomerulonephritis/pathology ; Glomerulonephritis/epidemiology ; Glomerulosclerosis, Focal/pathology ; Glomerulosclerosis, Focal/epidemiology ; Human ; Kidney/pathology* ; Kidney Diseases/pathology* ; Kidney Diseases/epidemiology* ; Korea ; Male ; Middle Age ; Prevalence ; Sex Distribution

Collapsing glomerulopathy (CG) is a recently described form of focal segmental glomerulosclerosis (FSGS) which was defined by renal pathology findings. CG is characterized by severe proteinuria and rapid progressive decline of renal function clinically. We experienced one case of CG in renal allograft. 24 year-old male recipient was admitted for evaluation of proteinuria (5.08 g/day) and increment of serum creatinine level at post-transplant 150 days. The graft biopsy was taken and the pathology specimen demonstrated a typical characteristics of CG such as glomerular capillary collapse, visceral epithelial hypercellularity, deposition of immunoglobulin/C3 and variable degree of tubulointerstitial injury. The patient was negative for HIV infection before transplantation and at the time of biopsy diagnosis. No specific treatment for CG was performed. The patient progressed to the graft failure and returned to hemodialysis 84 days after biopsy. In conclusion, recognition of CG by graft biopsy is important because it is a lesion with a high risk for rapid progression to graft failure.
Allografts* ; Biopsy ; Capillaries ; Creatinine ; Diagnosis ; Glomerulosclerosis, Focal Segmental ; HIV Infections ; Humans ; Male ; Pathology ; Proteinuria ; Renal Dialysis ; Transplants ; Young Adult

Focal segmental glomerulosclerosis (FSGS) is characterized by focal and segmental obliteration of glomerular capillary tufts with increased matrix. FSGS is classified as collapsing, tip, cellular, perihilar and not otherwise specified variants according to the location and character of the sclerotic lesion. Primary or idiopathic FSGS is considered to be related to podocyte injury, and the pathogenesis of podocyte injury has been actively investigated. Several circulating factors affecting podocyte permeability barrier have been proposed, but not proven to cause FSGS. FSGS may also be caused by genetic alterations. These genes are mainly those regulating slit diaphragm structure, actin cytoskeleton of podocytes, and foot process structure. The mode of inheritance and age of onset are different according to the gene involved. Recently, the role of parietal epithelial cells (PECs) has been highlighted. Podocytes and PECs have common mesenchymal progenitors, therefore, PECs could be a source of podocyte repopulation after podocyte injury. Activated PECs migrate along adhesion to the glomerular tuft and may also contribute to the progression of sclerosis. Markers of activated PECs, including CD44, could be used to distinguish FSGS from minimal change disease. The pathogenesis of FSGS is very complex; however, understanding basic mechanisms of podocyte injury is important not only for basic research, but also for daily diagnostic pathology practice.
Actin Cytoskeleton ; Age of Onset ; Capillaries ; Diaphragm ; Epithelial Cells ; Foot ; Glomerulosclerosis, Focal Segmental* ; Nephrosis, Lipoid ; Pathology ; Permeability ; Podocytes ; Rabeprazole ; Sclerosis ; Wills

Female ; Glomerulonephritis, IGA ; metabolism ; pathology ; Glomerulonephritis, Membranous ; metabolism ; pathology ; Glomerulosclerosis, Focal Segmental ; metabolism ; pathology ; Humans ; Kidney ; metabolism ; pathology ; Male ; Renal Insufficiency, Chronic ; metabolism ; pathology

OBJECTIVE: To observe the expression of plasma thrombospondin-1(TSP-1) at different time in protein-overload rats and to analyze the relationship between plasma TSP-1 expression and renal interstitial fibrosis. METHODS: Forty-five male Sprague-Dawley rats were randomly divided into a bovine serum albumin (BSA) group and a control group after uninephrectomization. Rats with protein overload nephropathy induced by intraperitoneally injected BSA were used as a model (control group received saline). At the 1st, 5th, and 9th weekend, the level of 24 h proteinuria and renal function was assessed. Pathological changes were observed by electron and fluorescent microscopy. The expression of plasma TSP-1 was detected by Western blot. The relationship between plasma TSP-1 and tubulointerstitial lesions (TIL) score was analyzed. RESULTS: Twenty-four hour proteinuria and blood urea nitrogen (BUN) significantly increased in protein-overload rats compared with those in the control group. While protein-overload rats developed more severe fibrosis in the tubular and interstitium. Glomerulosclerosis index and TIL score were upregulated compared with those in the control group. The expression of TSP-1 increased significantly at the 5th and 9th weekend. The expression of TSP-1 was positively correlated with TIL score (r=0.836, P<0.01). CONCLUSION Plasma TSP-1 expression is positively correlated with renal interstitial fibrosis in protein-overload rats. Plasma TSP-1 may be used for an important biomarker of renal interstitial fibrosis.
Animals ; Fibrosis ; metabolism ; pathology ; Glomerulosclerosis, Focal Segmental ; pathology ; Kidney ; metabolism ; pathology ; Male ; Nephrectomy ; Nephritis, Interstitial ; etiology ; metabolism ; pathology ; Rats ; Rats, Sprague-Dawley ; Thrombospondin 1 ; blood