scholarly journals SP177MINIMAL CHANGE NEPHROTIC SYNDROME, WITH AND WITHOUT MESANGIAL IGA DEPOSITS: RESPONSE TO TREATMENT AND RELAPSING RATE

2015 ◽  
Vol 30 (suppl_3) ◽  
pp. iii437-iii437
Author(s):  
Ladislava Grchevska ◽  
Gordana Petrushevska
Keyword(s):  
Nephrotic Syndrome ◽  
Response To Treatment ◽  
Mesangial Iga Deposits

scholarly journals Clinical profile of patients with steroid sensitive nephrotic syndrome at tertiary care centre in Gujarat, India

2018 ◽  
Vol 5 (4) ◽  
pp. 1172 ◽  
Author(s):  
Nimisha K. Pandya ◽  
Kedar G. Mehta
Keyword(s):  
Nephrotic Syndrome ◽  
Chronic Renal Disease ◽  
Tertiary Care ◽  
Clinical Profile ◽  
Response To Treatment ◽  
Renal Diseases ◽  
Sex Distribution ◽  
Tertiary Care Centre ◽  
Steroid Dependence ◽  
Special Clinic

Background: Nephrotic syndrome is a significant cause of chronic renal disease in children. The objective of the study was to analyze demographic profile, response to steroids and associated complications, in children with Nephrotic syndrome.Methods: A retrospective study of all patients referred to Renal diseases clinic at GMERS Medical College Gotri Vadodara was done. Period of study was from year 2014 to June 2017. Variables assessed were sex distribution, age at presentation for first attack, occurrence of complications, steroid responsiveness and use of steroid sparing agents. Study was done from special clinic cards used for documentation of visits of patients in Nephrotic disease special clinic.Results: 59 patients were studied. Mean age at presentation of was 4.08 years. Sex distribution ratio was 1.18:1. 88% of cases were infrequent relapsers. Steroid dependence was observed in 8.4% of cases. Complications were noted in 38% children. UTI was the commonest complication 13.5%. Associated renal conditions were present in 5% of children.Conclusions: In the present study clinical profile of children with Nephrotic syndrome was concordant with typical nephrotic syndrome in children. Pattern of nephrotic syndrome and response to treatment did not differ significantly from other studies.

scholarly journals Nephrotic syndrome co-existing with type 1 diabetes in a 12-year-old boy: Case report and literature review

2019 ◽  
Vol 7 ◽  
pp. 2050313X1982773 ◽  
Author(s):  
Noor S Bawahab ◽  
Osama Y Safdar ◽  
Sarah A Nagadi ◽  
Asalh T Saeedi ◽  
Raghad W Mohammed Hussain
Keyword(s):  
Diabetes Mellitus ◽  
Type 1 Diabetes ◽  
Nephrotic Syndrome ◽  
Type 1 Diabetes Mellitus ◽  
Pediatric Nephrology ◽  
Regular Insulin ◽  
Response To Treatment ◽  
Low Protein ◽  
The Relationship

Occurrence of early nephrotic syndrome in type 1 diabetes mellitus patients is extremely rare. Herein, we report the case of a 12-year-old boy who presented to our pediatric nephrology clinic with generalized edema. He had been diagnosed with type 1 diabetes mellitus at age 9 and had been treated with regular insulin. Examinations revealed normal kidney function, hypoalbuminemia, proteinuria (4+), hyperlipidemia, and low protein-to-creatinine ratio. The patient was diagnosed with idiopathic nephrotic syndrome and was empirically administered prednisolone for 12 weeks. Subsequently, prednisolone was tapered over 10–12 weeks. The patient showed good response to treatment. In conclusion, co-existence of nephrotic syndrome and type 1 diabetes mellitus may suggest an immunological basis; therefore, further studies are needed to investigate the relationship between these two conditions.

Minimal-change nephropathy and focal segmental glomerulosclerosis

2020 ◽  
pp. 4919-4928
Author(s):  
Moin Saleem ◽  
Lisa Willcocks
Keyword(s):  
Nephrotic Syndrome ◽  
Focal Segmental Glomerulosclerosis ◽  
Parasitic Infection ◽  
Genetic Mutation ◽  
Minimal Change ◽  
Response To Treatment ◽  
Glomerular Sclerosis ◽  
Protein Factor ◽  
Segmental Glomerulosclerosis ◽  
Published Evidence

Minimal-change nephrotic syndrome (MCNS) is an immune-mediated condition, usually of unknown cause. On light microscopy the glomeruli appear normal, and on electron microscopy there is effacement of epithelial cell foot processes over the outer surface of the glomerular basement membrane. MCNS is the cause of about 75% of cases of nephrotic syndrome in children and 17% in adults. Management and prognosis—treatment in adults is with prednisolone at an initial dose of 80 mg/day, then tapering. This leads to complete remission in 90 to 95% of patients, but 50 to 75% of glucocorticoid-responsive adults will have a relapse. Progression to renal failure is not expected and would call the diagnosis of MCNS into question. Focal segmental glomerulosclerosis (FSGS) is not a specific disease entity but a histological lesion, often of unknown aetiology, which is characterized by segmental areas of glomerular sclerosis. It may be (1) primary—either due to genetic mutation, or associated with an unknown circulating protein factor that causes an increase in glomerular permeability; or (2) secondary—the end product of a variety of pathological processes including glomerular hyperfiltration, healed glomerulonephritis, viral (including HIV) infection, or parasitic infection. Management and prognosis—corticosteroid and immunosuppressive therapy should be considered only in patients with primary FSGS and nephrotic syndrome. The steroid regimen is as used for MCNS, but with lesser success. Steroid-resistant cases are treated with ciclosporin (for which there is most published evidence), mycophenolate mofetil, or cyclophosphamide. Prognosis depends on histology and response to treatment.

Steroid-Sensitive Nephrotic Syndrome with Mesangial IgA Deposits: a Separate Entity?

1986 ◽  
Vol 6 (2) ◽  
pp. 141-145 ◽  
Author(s):  
Barbiano di Belgiojoso ◽  
G. Mazzucco ◽  
S. Casanova ◽  
L. Radaelli ◽  
G. Monga ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Separate Entity ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive ◽  
Mesangial Iga Deposits

scholarly journals Childhood nephrotic syndrome: genetic basis in studying and treatment

2016 ◽  
Vol 14 (3) ◽  
pp. 393-404
Author(s):  
Nguyễn Thị Kim Liên ◽  
Nguyễn Huy Hoàng
Keyword(s):  
Nephrotic Syndrome ◽  
High Risk ◽  
Genetic Basis ◽  
Gene Mutations ◽  
Response To Treatment ◽  
Steroid Resistance ◽  
Childhood Nephrotic Syndrome ◽  
Filtration Barrier ◽  
Different Genetic Background ◽  
Serious Disease

Childhood nephrotic syndrome is a serious disease with the high risk of mortality. The prevalence worldwide is approximately 2 to 7 cases per 100,000 children. Hitherto, the patients are treatmed with steroid or immunosuppressive therapy and renal transplantation. However, many patients are steroid resistant and have a high risk of recurrence. The mutations in seven genes (NHPS1, NPHS2, CD2AP, PLCE1, ACTN4, TRPC6, INF2) have been implicated in different forms of nephrotic syndrome. Among them, two genes, NPHS1 and NPHS2, encoding for nephrin and podocin protein of the glomerular filtration barrier, have a particularly serious influence in almost of cases and steroid resistance in patients. So that, in the world there were a lot of researches conducted to identify gene mutations that related to disease manifestations and the response to treatment in the patients who have different genetic background. Mutations in NPHS1 and NPHS2 are considered the main cause of 75% cases with nephrotic syndrome. And NPHS2 mutations related to 40% cases with steroid resistance in patients. These results will help the doctors to have an effective treatment for the patients. However, in Vietnam no study has yet been done to detect gene mutations in patients with nephrotic syndrome. In this article, we summarize the research which have been published to give an overview of the genetic basis of causing and treatment for childhood nephrotic syndrome.

Focal and segmental glomerular sclerosis

2009 ◽  
pp. 215-260 ◽  
Author(s):  
Francesco Scolari ◽  
Claudio Ponticelli
Keyword(s):  
Nephrotic Syndrome ◽  
Signs And Symptoms ◽  
Response To Treatment ◽  
Glomerular Sclerosis ◽  
Term Outcome ◽  
Glomerular Lesion ◽  
Stage Renal Disease ◽  
End Stage ◽  
Segmental Glomerular Sclerosis

Focal and segmental glomerular sclerosis (FSGS) is a glomerular lesion which is associated with distinctive clinical features. Because it may be pathogenetically heterogeneous, it is not yet appropriate to call it a disease, yet its discovery in a renal biopsy in a patient with the nephrotic syndrome does have important connotations with respect to response to treatment and to long-term outcome. By light microscopy the characteristic finding is segmental areas of sclerosis (and hyalinosis) involving only some glomeruli. Patients with the FSGS lesion typically have proteinuria which is usually in the nephrotic range (〉3.5g/d in an adult), accompanied by the typical constellation of signs and symptoms of the nephrotic syndrome and arterial hypertension. A minority of patients may have only asymptomatic proteinuria and these patients usually do not progress to end-stage renal disease (ESRD) but the natural course of FSGS is ominous in most patients with nephrotic syndrome. However, numerous observational studies have shown that about 50–70% of patients may respond completely or partially to prolonged glucocorticoid therapy or other ‘immunosuppressive’ treatments and thus have a fair outcome in the long term.

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