Progression of chronic renal failure in focal segmental glomerulosclerosis: consequence of podocyte damage or of tubulointerstitial fibrosis?

Persistent proteinuria, chronic renal failure, and focal segmental glomerulosclerosis developed in three children with solitary kidneys. Two of these children were born with unilateral kidneys. The third had bilateral reflux and underwent a unilateral nephrectomy and reimplantation of the remaining ureter; persistent proteinuria developed 7 years later. It is postulated that hyperperfusion of a critical number of glomeruli during childhood may be the mechanism responsible for the production of focal segmental glomerulosclerosis in these patients.

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Analysis of Mutations in Alpha-Actinin 4 and Podocin Genes of Patients with Chronic Renal Failure Due to Sporadic Focal Segmental Glomerulosclerosis

Renal Failure ◽

10.1081/jdi-120017471 ◽

2003 ◽

Vol 25 (1) ◽

pp. 87-93 ◽

Cited By ~ 14

Author(s):

Atsushi Komatsuda ◽

Hideki Wakui ◽

Nobuki Maki ◽

Akihiro Kigawa ◽

Hiroyuki Goto ◽

...

Keyword(s):

Renal Failure ◽

Chronic Renal Failure ◽

Focal Segmental Glomerulosclerosis ◽

Segmental Glomerulosclerosis

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Metanephric Adenoma with Embryonal Hyperplasia of Bowman's Capsular Epithelium: Previously Unreported Association

Pediatric and Developmental Pathology ◽

10.1007/s100240010093 ◽

2000 ◽

Vol 3 (5) ◽

pp. 472-478 ◽

Cited By ~ 10

Author(s):

Keshani de Silva ◽

Vivienne Tobias ◽

Gad Kainer ◽

Bruce Beckwith

Keyword(s):

Renal Failure ◽

Peritoneal Dialysis ◽

Renal Transplantation ◽

Chronic Renal Failure ◽

Focal Segmental Glomerulosclerosis ◽

Left Kidney ◽

Bilateral Nephrectomy ◽

Metanephric Adenoma ◽

Histological Features ◽

Segmental Glomerulosclerosis

We report a case of a 9-year-old boy with focal, segmental glomerulosclerosis who, following peritoneal dialysis, underwent renal transplantation and bilateral nephrectomy. The kidneys showed histological features of embryonal hyperplasia of Bowman's capsular epithelium, an uncommon lesion that is seen most often in patients with chronic renal failure who are being maintained on dialysis. In addition, a 1-cm tumor in the left kidney showed features of metanephric adenoma. Although both lesions are uncommon, they share many similarities on a morphological, immunohistochemical, and ultrastructural basis. This association has not been previously reported and may shed some light on the histogenesis of these recently described lesions.

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Preoperative Low-Density Lipoprotein Apheresis for Preventing Recurrence of Focal Segmental Glomerulosclerosis after Kidney Transplantation

Journal of Transplantation ◽

10.1155/2018/8926786 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5 ◽

Cited By ~ 2

Author(s):

Akihito Sannomiya ◽

Toru Murakami ◽

Ichiro Koyama ◽

Kosaku Nitta ◽

Ichiro Nakajima ◽

...

Keyword(s):

Renal Failure ◽

Kidney Transplantation ◽

Focal Segmental Glomerulosclerosis ◽

Low Density Lipoprotein ◽

Density Lipoprotein ◽

Low Density ◽

Ldl Apheresis ◽

Segmental Glomerulosclerosis ◽

Living Related ◽

Observation Period

Background. Focal segmental glomerulosclerosis (FSGS) often develops rapidly and frequently progresses to renal failure, while the recurrence rate after kidney transplantation is 20–50%. We performed low-density lipoprotein (LDL) apheresis before kidney transplantation in FSGS patients to prevent recurrence. Methods. Five adult patients with chronic renal failure due to FSGS undergoing living related donor kidney transplantation were investigated retrospectively. LDL apheresis was done 1-2 times before transplantation. Postoperative renal function and recurrence of FSGS were assessed. Results. The patients were two men and three women aged 24 to 41 years. The observation period ranged from 60 days to 22 months. Preoperative LDL apheresis was performed once in one patient and twice in four patients. Blood LDL cholesterol levels were normal before LDL apheresis and remained normal both after LDL apheresis and after kidney transplantation. Additional LDL apheresis was performed once in one patient with mild proteinuria after transplantation. The renal graft survived in all patients and there was no evidence of recurrent FSGS. Conclusions. Although the observation period was short, FSGS did not recur in all 5 patients receiving preoperative LDL apheresis. These results suggest that LDL apheresis can be effective in preventing recurrence of FSGS after kidney transplantation.

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