scholarly journals End‐stage renal disease in a child with focal segmental glomerulosclerosis associated with a homozygous NUP93 variant

2021 ◽  
Vol 9 (11) ◽  
Author(s):  
Ratna Acharya ◽  
Kiran Upadhyay
Keyword(s):  
Renal Disease ◽  
Focal Segmental Glomerulosclerosis ◽  
End Stage Renal Disease ◽  
Segmental Glomerulosclerosis ◽  
Stage Renal Disease ◽  
End Stage

scholarly journals Successful treatment of collapsing focal segmental glomerulosclerosis in a patient

2020 ◽  
Vol 10 (4) ◽  
pp. e43-e43
Author(s):  
Luísa Helena Pereira ◽  
Ana Cabrita ◽  
Mário Góis ◽  
Helena Viana ◽  
Sandra Sampaio ◽  
...  
Keyword(s):  
Renal Disease ◽  
Focal Segmental Glomerulosclerosis ◽  
End Stage Renal Disease ◽  
Oral Corticosteroid ◽  
Segmental Glomerulosclerosis ◽  
Number Of Patients ◽  
Collapsing Fsgs ◽  
Stage Renal Disease ◽  
End Stage ◽  
High Index Of Suspicion

Focal segmental glomerulosclerosis (FSGS) is a recognized cause of renal disease worldwide. The collapsing variant is distinct from the others, characterized clinically by a more severe nephrotic syndrome generally resistant to immunosuppressive therapy. It is known that a great number of patients progress to end-stage renal disease. Recognizing this lesion in biopsy is frequently challenging owing to the focal nature of the process which highlights the need for keeping a high index of suspicion for the diagnosis. We report and discuss a case of a non-HIV collapsing FSGS, followed by a complete (unexpected) renal recovery after an oral corticosteroid course.

scholarly journals Focal Segmental Glomerulosclerosis

1999 ◽  
Vol 10 (9) ◽  
pp. 1900-1907
Author(s):  
MELVIN M. SCHWARTZ ◽  
JONI EVANS ◽  
RAY BAIN ◽  
STEPHEN M. KORBET
Keyword(s):  
Serum Creatinine ◽  
Focal Segmental Glomerulosclerosis ◽  
End Stage Renal Disease ◽  
Poor Response ◽  
Steroid Treatment ◽  
Response To Treatment ◽  
Therapeutic Trial ◽  
Segmental Glomerulosclerosis ◽  
Stage Renal Disease ◽  
End Stage

Abstract. The cellular lesion (CELL), seen in some patients with primary focal segmental glomerulosclerosis (FSGS), comprises proliferation, hypertrophy, and pathologic changes in the cells overlying the glomerular scar. The prognosis of the cellular lesion was retrospectively studied in 100 patients with FSGS (43 had FSGS-CELL and 57 had FSGS without the cellular lesion (classic segmental scar [CS]). Patients with the FSGS-CELL lesion were more often black and severely proteinuric and developed more end-stage renal disease (ESRD). Nephrotic patients with FSGS-CELL (n = 39) were more proteinuric at presentation than patients with FSGS-CS (n = 36). ESRD developed more frequently in patients with the FSGS-CELL (17 of 39, 44% versus 5 of 36, 14%, P = 0.005), and patients with extensive FSGS-CELL (≥ 20% glomeruli) were mainly black (94%), severely nephrotic (67%, >10 g/d), and had a poor response to treatment (23% remission). In nephrotic patients, initial serum creatinine, interstitial expansion ≥20%, and CELL independently predicted ESRD. However, the rates of remission in treated nephrotic patients with FSGS-CELL and FSGS-CS were the same (9 of 17, 53% versus 17 of 39, 52%), and patients in both groups who achieved a remission had a 5-yr survival of 100%. Steroid treatment was the only variable that predicted remission. Patients with the FSGS-CELL have an increased prevalence of ESRD, but the improved prognosis associated with remission is so significant that a therapeutic trial is warranted in all nephrotic FSGS patients, regardless of the presence of the cellular lesion.

scholarly journals Secondary Focal Segmental Glomerulosclerosis: From Podocyte Injury to Glomerulosclerosis

2016 ◽  
Vol 2016 ◽  
pp. 1-7 ◽  
Author(s):  
Jae Seok Kim ◽  
Byoung Geun Han ◽  
Seung Ok Choi ◽  
Seung-Kuy Cha
Keyword(s):  
Nephrotic Syndrome ◽  
Focal Segmental Glomerulosclerosis ◽  
End Stage Renal Disease ◽  
Renal Diseases ◽  
Podocyte Injury ◽  
Common Cause ◽  
Multiple Factors ◽  
Segmental Glomerulosclerosis ◽  
Stage Renal Disease ◽  
End Stage

Focal segmental glomerulosclerosis (FSGS) is a common cause of proteinuria and nephrotic syndrome leading to end stage renal disease (ESRD). There are two types of FSGS, primary (idiopathic) and secondary forms. Secondary FSGS shows less severe clinical features compared to those of the primary one. However, secondary FSGS has an important clinical significance because a variety of renal diseases progress to ESRD thorough the form of secondary FSGS. The defining feature of FSGS is proteinuria. The key event of FSGS is podocyte injury which is caused by multiple factors. Unanswered questions about how these factors act on podocytes to cause secondary FSGS are various and ill-defined. In this review, we provide brief overview and new insights into FSGS, podocyte injury, and their potential linkage suggesting clues to answer for treatment of the disease.

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