Presentation
Depending on the cause it is broadly classified as: Primary, when no underlying cause is found; usually presents as nephrotic syndrome Secondary, when an underlying cause is identified; usually presents with kidney failure and proteinuria. [en.wikipedia.org]
The clinical presentation included proteinuria (mean 10.1 g/d; range 1.3 to 26.3 g/d) and renal insufficiency (mean serum creatinine 3.0 mg/dl; range 1.3 to 7.8 mg/dl); three (30%) patients presented with nephrotic syndrome. [doi.org]
The average age at presentation of FSGS patients was 24.28 19.3 years, ranging from 2 to 74 years. There were 38 females (32.76%) and 78 males (67.27%). The most common age-group at presentation was 11-30 years. [sjkdt.org]
In the current study, 63% of patients presented haematuria and 40% presented hypertension. El-Refaey et al. [14] showed that haematuria was present in 24% of cases and 21% patients presented hypertension. [latunisiemedicale.com]
These patients, predominately black adolescents, present either with nephrotic syndrome or asymptomatic proteinuria. [ncbi.nlm.nih.gov]
Psychiatrical
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Decreased Libido
He perceived himself as being too “skinny and weak” and complained of decreased libido. He wanted to resume bodybuilding but agreed to a reduced exercise regimen without supplements or hormones. [doi.org]
Workup
Figure 2: Pre-transplant workup and management of patients with FSGS [3]. [medcraveonline.com]
Urine
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Pyuria
Drug-induced Interstitial Nephritis Analgesic nephropathy Acute Drug-Induced Interstitial Nephritis Acute renal failure in 50% of patients May be clinically evident only as progressive increase in serum Cr Urine findings - hematuria, mild proteinuria, pyuria [sites.google.com]
Serum
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Creatinine Increased
Six weeks after restarting supplements and hormones, his serum creatinine increased to 2.3 mg/dl and proteinuria increased to 4.7 g/d. [doi.org]
Treatment
Antioxidant therapy may be a useful adjunct in the treatment of children with FSGS and proteinuria that is refractory to standard medical management. [ncbi.nlm.nih.gov]
The goal of treatment is to control the symptoms of nephrotic syndrome and prevent chronic kidney failure. [nlm.nih.gov]
The duration of vitamin E treatment, when no other therapy was introduced, was 2.9 0.4 months. Proteinuria was determined by measuring the protein:creatinine ratio (mg/mg) in an early morning urine specimen. [doi.org]
Treatment MCD is highly responsive to corticosteroids, which is first-line treatment. Almost all children respond within 2 months after initiating steroid treatment, whereas up to 95% of adults respond within 4 months. [renalandurologynews.com]
Product Code: 64100 Focal Segmental Glomerulosclerosis Treatment Market Overview The focal segmental glomerulosclerosis (FSGS) treatment market is expected to register a growth rate of about 7.5% over the forecast period, 2018-2023. [gii.co.jp]
Prognosis
The clinical course, prognosis and outcome of FSGS are heterogeneous. [sjkdt.org]
Prognosis is extremely variable and depends on the amount of proteinuria, the level of plasma creatinine, the morphological subtype, the degree of renal fibrosis, and the response to therapy. [bestpractice.bmj.com]
Prognosis of patients with effectiveness to prednisolone therapy was better than patients with ineffectiveness, and prognosis of patients with effectiveness to cyclophosphamide therapy was better than patients with ineffectiveness. [ci.nii.ac.jp]
[…] glomerulonephritis, in which the male:female ratio is 1.2:1. 3) The prognosis for females is no better than for males. 4) The severity of proteinuria at the time of biopsy has no influence on the prognosis. 5) The prognosis is particularly poor in cases [ncbi.nlm.nih.gov]
Prognosis: The natural history of FSGS varies a great deal. Treatment may cure the disease (remission) in some or at least slow down the progression of the disease. [glomerularcenter.org]
Etiology
[…] focal and segmental hyalinization of the glomeruli, often with immunostaining showing IgM and complement (C3) deposits in a nodular and coarse granular pattern. [4] In HIV-associated FSGS, an ultrasound generally reveals large echogenic kidneys. [3] Etiology [physio-pedia.com]
There is a complex interplay between etiologic and pathogenic factors, progression factors and intervention in the phenotypic expression of FSGS. [ncbi.nlm.nih.gov]
Etiologic agents or mechanisms that initiate glomerular injury which lead to glomerulosclerosis are largely unknown, except in certain animal models of viral-induced renal disease. [emedicine.medscape.com]
Epidemiology
Nevertheless, the available data suggest that large epidemiological case-control studies to examine the association between NPHS2 variants and nephrotic syndrome are warranted. [ncbi.nlm.nih.gov]
Dr Franceschini is supported by the Department of Epidemiology, University of North Carolina School of Public Health. [doi.org]
Epidemiology MCD is the most common cause of pediatric nephrotic syndrome but accounts for less than 15% of adult nephrotic syndrome. MCD may be idiopathic or develop in association with other pre-existing diseases or drugs. [renalandurologynews.com]
Focal segmental glomerulosclerosis – epidemiology aspects in chil- dren and adults. Pediatr Nephrol. 2007; 22 (2): 183-6. PMC1764601/ DOI: 10.1007/s00467-006-0370-5 10. Zamora I, Peña A, Mendizabal S, Bedoya R,Vilalta R, Torra R. [revistas.unilibre.edu.co]
Focal segmental glomerulosclerosis – epidemiology aspects in children and adults. Pediatr Nephrol. 2007; 22 (2): 183-6. DOI: 10.1007/s00467-006-0370-5 Zamora I, Peña A, Mendizabal S, Bedoya R,Vilalta R, Torra R. [revistasojs.unilibrecali.edu.co]
Pathophysiology
The primary pathophysiologic process in FSGS is an injury inherent within or directed to podocytes. [emedicine.medscape.com]
Prevention
Prevention of Recurrence Pretransplant plasmapheresis appears to prevent or delay recurrence in high-risk patients ( 28 ). [doi.org]
The goal of treatment is to control the symptoms of nephrotic syndrome and prevent chronic kidney failure. [nlm.nih.gov]
[…] study is to assess the efficacy of the bleselumab regimen (basiliximab induction, tacrolimus, steroids and bleselumab) compared with the Standard of Care (SOC) regimen (basiliximab induction, tacrolimus, steroids and mycophenolate mofetil [MMF]) in the prevention [clinicaltrials.ucsf.edu]
We hope that finding the genetic causes of FSGS will eventually lead to prevention and better treatment. [dmpi.duke.edu]
Nonspecific Treatment [3] “Treatment goals include maintenance of adequate nutrition, minimization or elimination of proteinuria, and prevention of complications resulting from edema. [physio-pedia.com]