Presentation
However, at present, GSEC is not routinely evaluated. We, in this study, present a case of glomerulosclerosis, in which the presence of GSECs should provide us one clue to understand the pathogenesis of its progressive decline of renal function. [ncbi.nlm.nih.gov]
We present the first report of immunoglobulin A nephropathy (IgAN) and associated focal segmental glomerulosclerosis in a patient with asymptomatic low grade B-cell lymphoma. [anzsnasm.com]
Genetic/familial forms of FSGS may present in childhood or early adulthood and can present abruptly or in a more indolent fashion. [renalandurologynews.com]
Only asymptomatic proteinuria was present at presentation in 25 patients, in whom nephrotic syndrome was absent at presentation and· during the clinical course in 11 patients. No patient in these had normal urine when last seen, ! [ci.nii.ac.jp]
Entire Body System
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Hypoxemia
The pathogenesis of this disorder is unclear and is known to occur in patients with hypertension, hypercholesterolemia, history of chronic smoking, chronic obstructive pulmonary disease with chronic hypoxemia, obesity, etc.[ 7, 8 ] Despite the growing [ncbi.nlm.nih.gov]
Musculoskeletal
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Myopathy
Although she had no symptoms of either myopathy or encephalopathy, no history of stroke-like episodes or difficulty in hearing, her serum concentrations of lactate and pyruvate were both elevated. [ncbi.nlm.nih.gov]
Urogenital
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Kidney Failure
Untreated, it can lead to kidney failure. In some cases, kidney failure can occur despite treatment. FSGS most frequently occurs without a cause. In such cases it is called idiopathic or primary FSGS. [webmd.com]
If kidney failure does occur, treatment options are available. [davita.com]
The goal of treatment is to control the symptoms of nephrotic syndrome and prevent chronic kidney failure. [mountsinai.org]
Kidney failure is a common outcome. glomerulosclerosis progressive hyalinization such that glomeruli become shrunken, eosinophilic and hypocellular masses. intercapillary glomerulosclerosis [medical-dictionary.thefreedictionary.com]
Workup
Autoimmune workup was negative. Workup for other causes of nodular glomerulosclerosis such as amyloidosis, LCDD, and immune deposition diseases was negative. [ncbi.nlm.nih.gov]
Other Pathologies
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Lymphocytic Infiltrate
In one cat, an islet showed a dense lymphocytic infiltration. — No lesions identical to human diabetic glomerulosclerosis were found in any of the dogs or cats. [glosbe.com]
Vessels and tubulointerstitium were unremarkable except for focal lymphocytic infiltrates. Immunofluorescence was negative for IgM, IgA, IgG, C3C, C1q, k, and λ. [ncbi.nlm.nih.gov]
Treatment
And the FDA has not approved any drugs specifically for its treatment. But several treatments can slow its progression and control symptoms. [webmd.com]
TGFβ treatment of podocytes does not alter cell morphology but increases connective tissue growth factor (CTGF) expression. [ncbi.nlm.nih.gov]
Prognosis
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]
Focal segmental glomerulosclerosis (FSGS) is a renal syndrome characterized by proteinuria (usually nephrotic range), limited response to conventional therapy, and a poor renal prognosis, with progression to end stage renal failure in at least 50% of [clinicaltrials.gov]
Prognosis The prognosis of MCD and FSGS is linked to the response to treatment. [renalandurologynews.com]
These treatments may include: Antibiotics to control infections Fluid restriction Low-fat diet Low- or moderate-protein diet Vitamin D supplements Dialysis Kidney transplant Outlook (Prognosis) A large portion of people with focal or segmental glomerulosclerosis [mountsinai.org]
Adult-onset minimal change disease among Taiwanese: clinical features, therapeutic response, and prognosis. Am J Nephrol. 2001;21(1):28–34. PubMed CrossRef 4. Philibert D, Cattran D. [springermedizin.at]
Etiology
The natural history of the condition varies, and although it may respond to treatment, FSGS is an important disease in the etiology of end-stage renal disease (ESRD). [ncbi.nlm.nih.gov]
As a syndrome, FSGS likely has many specific etiologies, only a few of which are well-defined. [clinicaltrials.gov]
[…] 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]
An unknown cause such as genetic, environmental or infections except drugs may be factors in the etiology of these two diseases. [revistanefrologia.com]
Segmental/*chemically induced — Humans — Infusions, Intravenous — Kidney Diseases/etiology/physiopathology — Kidney Failure, Chronic/etiology/therapy — Kidney Glomerulus/drug effects/pathology — Male — Multiple Myeloma/complications/drug therapy — Podocytes [archive-ouverte.unige.ch]
Epidemiology
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]
Deegens JK, Steenbergen EJ, Borm GF, Wetzels JF (2008) Pathological variants of focal segmental glomerulosclerosis in an adult Dutch population – epidemiology and outcome. Nephrol Dial Transplant 23:186–192 PubMed CrossRef Google Scholar 30. [link.springer.com]
Clinical and epidemiological assessment of steroid-resistant nephrotic syndrome associated with the NPHS2 R229Q variant. Kidney Int 2009; 75: 727¿735 [Pubmed] 75. Santín S, Bullich G, Tazón-Vega B, et al. [nefrologiaaldia.org]
Pathophysiology
Reidy K, Kaskel FJ (2007) Pathophysiology of focal segmental glomerulosclerosis. Pediatr Nephrol 22:350–354 PubMed CrossRef Google Scholar 26. [link.springer.com]
The primary pathophysiologic process in FSGS is an injury inherent within or directed to podocytes. [emedicine.medscape.com]
Focal segmental glomerulosclerosis: a case study with review of pathophysiology. Nephrology Nursing Journal. 2007 March; 34(2):176-183. Lim A, Lydia A, Rim H, Dowling J, Kerr P. [physio-pedia.com]
Prevention
BMP7 prevents TGFβ-induced GEC network regression, whereas TGFβ-induced MC nodule formation is prevented by SMAD3 siRNA knockdown or ALK5 inhibitors but not BMP7, and increased phospho-SMAD3 was observed in human glomerulosclerosis. [ncbi.nlm.nih.gov]
No prevention is known. [pennmedicine.org]
This prevents the body from making antibodies that may attack the glomerulus. Dialysis. A treatment to remove wastes and excess fluid from the blood after the kidneys have stopped working. Kidney transplant. [hopkinsmedicine.org]
Steroids such as prednisone or prednisolone and other immune system-suppressing drugs to reduce protein loss and improve kidney function Statins to control cholesterol Anticoagulants to prevent blood clots Diuretics, or water pills, to remove excess salt [webmd.com]