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Glomerulosclerosis

Glomerular Scarring %2F Sclerosis


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]
  • Moreover, patients with diabetic nephropathy have significantly more apoCI present in glomeruli compared to diabetic patients without nephropathy, suggesting that apoCI could be involved in the development of diabetic nephropathy.[ncbi.nlm.nih.gov]
  • Genetic/familial forms of FSGS may present in childhood or early adulthood and can present abruptly or in a more indolent fashion.[renalandurologynews.com]
  • In this study, we show that ILC populations are present in the healthy human kidney. A detailed characterization of kidney-residing ILC populations revealed that IL-33 receptor-positive ILC2s are a major ILC subtype in the kidney of humans and mice.[ncbi.nlm.nih.gov]
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]
Sleep Apnea
  • Some causes of secondary FSGS include: Kidney defects from birth (dysplasia) Urine backing up into kidneys (kidney reflux) Obesity Obstructive Sleep Apnea Viruses and blood disorders (such as HIV and sickle cell anemia) Autoimmune disorders (such as lupus[nephcure.org]
  • Glomerulomegaly and focal segmental glomerulosclerosis associated with obesity and sleep-apnea syndrome. Am J Kidney Dis. 1987 ;10:470-2. [Pubmed] 43. Fletcher EC. Obstructive sleep apnea and the kidney. J Am Soc Nephrol 1993; 4:1111-21 [Pubmed] 44.[nefrologiaaldia.org]
  • Kidney defects from birth Urine backing up into kidneys Obesity Obstructive Sleep Apnea Sickle Cell Anemia Viruses (such as HIV) Most people with the above conditions do not have FSGS.[unckidneycenter.org]
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]
Suggestibility
  • These results suggest that APOC1 expression induces glomerulosclerosis, potentially by increasing the cytokine response in macrophages. Furthermore, we detected apoCI in the kidneys of diabetic patients, but not in control kidneys.[ncbi.nlm.nih.gov]
  • This case suggests that the routine evaluation of GSECs can provide important clues to assess the etiopathogenesis of cryptogenic glomerulosclerosis.[ncbi.nlm.nih.gov]
  • These effects of TGF-β on HIF-1α were inhibited in Smad3-null mouse embryonic fibroblasts, suggesting a requirement for Smad3.[ncbi.nlm.nih.gov]
  • Recently, it has been suggested that some idiopathic FSGS patients have elevated circulating levels of a protein that induces glomerular permeability in vitro and in vivo.[clinicaltrials.gov]
  • The absence of widespread glomerular sclerosis and the rapid course suggest that unique renal hemodynamic mechanisms may be responsible for the progression. ( Arch Intern Med . 1990;150:287-292)[jamanetwork.com]
Psychomotor Retardation
  • According to previous reports of FSGS associated with psychomotor retardation and epilepsy, there are various types of syndromes or diseases that cause secondary FSGS.[ci.nii.ac.jp]
Spastic Paralysis
  • In this report, we discussed about an 8-year-old girl with mental retardation and spastic paralysis of the lower limbs who was diagnosed with focal segmental glomerulosclerosis.[ci.nii.ac.jp]
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]
  • The goal of treatment is to control the symptoms of nephrotic syndrome and prevent chronic kidney failure.[pennmedicine.org]
Microscopic Hematuria
  • Urine microscopy revealed bland urinary sediment without any microscopic hematuria. Ultrasound showed normal-sized kidneys with increased echotexture and maintained corticomedullary differentiation.[ncbi.nlm.nih.gov]
  • hematuria is occasionally present • Fatigue • Poor appetite • Headache • Itchy skin • Shortness of air • Nausea Associated Co-morbidities See etiology/causes for a full list of co-morbidities associated with primary and secondary FSGS.[physio-pedia.com]
  • hematuria (29%–94%), renal failure (48%–59%), and an increase in serum creatinine ( 1.3 mg/dL). 6 Definition and pathologic classification “Glomerulosclerosis” describes a lesion that leads to obliteration of the capillary lumina by the matrix component[dovepress.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]
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]
  • Short-term IL-33 treatment in mice led to sustained expansion of IL-33 receptor-positive kidney ILC2s and ameliorated adriamycin-induced glomerulosclerosis.[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]
  • 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

  • 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]
  • In this review, we summarize recent advances in the epidemiology, pathophysiology, and treatment of recurrent FSGS in pediatric patients who have received a kidney transplant.[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]
Sex distribution
Age distribution

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]
  • Cravedi P, Kopp JB, Remuzzi G (2013) Recent progress in the pathophysiology and treatment of FSGS recurrence. Am J Transplant 13:266–274 PubMed CrossRef Google Scholar 19.[link.springer.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]

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