Actualización de la glomerulopatía colapsante

Morales, Enrique; Alonso, Marina; Gutiérrez, Eduardo

doi:10.1016/j.medcli.2018.10.021

Cited by 9 publications

(4 citation statements)

References 49 publications

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“…It is not known if the patient will respond to high dose steroids. CG, on the other hand, is characterized by the absence of mature podocyte markers and patients with CG usually progress rapidly to end stage renal disease [22]. HIV-associated glomerulopathy is the other example of direct viral damage to the podocytes causing CG and is known to reverse and can be prevented by the use of retro-viral therapy [23].…”

Section: Discussionmentioning

confidence: 99%

Spectrum of podocytopathies in new-onset nephrotic syndrome following COVID-19 disease: a report of 2 cases

et al. 2020

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Background: Coronavirus disease-2019 (COVID-19) is an ongoing pandemic which has affected over 12 million people across the globe. Manifestations in different organs systems are being reported regularly. Renal biopsy findings in hospitalized COVID-19 patients presenting solely with acute kidney injury (AKI) have recently been described in published literature in few case reports. The findings include diffuse acute tubular injury (ATI) along with the glomerular lesion of collapsing glomerulopathy (CG). However, nephrotic syndrome as the presenting complaint of COVID-19 has not been reported widely, neither has any other glomerular lesion other than CG. Case presentation: We describe the kidney biopsy findings of two patients who had recent diagnoses of COVID-19 and presented with new-onset nephrotic syndrome. Renal biopsy in both patients showed ATI (as in previous reports) and distinct glomerular findings on light microscopythat of minimal change disease (MCD) initially in one patient followed by CG in a subsequent biopsy and CG at the outset in the other patient. The electron microscopic findings in both patients were that of severe podocytopathy (diffuse and severe podocyte foot process effacement). Conclusion: Our cases highlight a novel clinical presentation of COVID-19 renal disease, not described before, that of new-onset nephrotic syndrome. While all published case reports describe CG as the glomerular pathology, we describe a non-CG pathology (MCD) in one of our cases, thereby adding to the repertoire of renal pathology described in association with COVID-19 patients. However, the exact mechanism by which podocyte injury or podocytopathy occurs in all such cases is still unknown. Optimal treatment options for these patients also remains unknown at this time.

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Section: Discussionmentioning

confidence: 99%

Spectrum of podocytopathies in new-onset nephrotic syndrome following COVID-19 disease: a report of 2 cases

et al. 2020

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“…It is far more common in people with African ancestry and was initially associated with HIV infection. Nowadays, other causes are acknowledged, such as other infectious micro-organisms, autoimmune diseases, drugs or genetic disorders (3,12). Although it is increasingly considered a different entity due to its unique features, it is usually classified as a variant of FSGS (12).…”

Section: Discussionmentioning

confidence: 99%

“…Although it is increasingly considered a different entity due to its unique features, it is usually classified as a variant of FSGS (12). The pathogenesis of CN is still largely unknown, although it has been postulated that it is caused by podocyte dedifferentiation and gain of a proliferative phenotype (3).…”

Section: Discussionmentioning

confidence: 99%

“…Collapsing variant of focal segmental glomerulosclerosis (cFSGS) is diagnosed in light microscopy by the presence of at least one glomerulus tuff with segmental or global collapse and podocyte hypertrophy or hyperplasia (2). Although both entities present with NS, unlike MN, cFSGS has worse prognosis and usually fails to respond to therapy, evolves with rapid deterioration of kidney function and progresses to end stage kidney disease (3). Herein, we report a case of a patient with PLA 2 R-associated MN with collapse.…”

Section: Introductionmentioning

confidence: 99%

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Membranous nephropathy with collapse in a HIV negative patient; a case report with a 34-month follow-up

et al. 2022

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Membranous nephropathy (MN) is a common cause of nephrotic syndrome (NS) in nondiabetic adults. Collapsing nephropathy (CN) is a morphological pattern that is usually classified as a variant of focal segmental glomerulosclerosis (cFSGS). The simultaneous presence of both MN and CN is rare and their combination usually foresees an unfavorable outcome. Herein, we describe a case report of a patient with PLA2 R-associated MN with collapse, its treatment and clinical course.

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Responsiveness of sphingosine phosphate lyase insufficiency syndrome to vitamin B6 cofactor supplementation

Zhao

Liu

Hodgin

et al. 2020

J of Inher Metab Disea

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Sphingosine‐1‐phosphate (S1P) lyase is a vitamin B6‐dependent enzyme that degrades sphingosine‐1‐phosphate in the final step of sphingolipid metabolism. In 2017, a new inherited disorder was described caused by mutations in SGPL1, which encodes sphingosine phosphate lyase (SPL). This condition is referred to as SPL insufficiency syndrome (SPLIS) or alternatively as nephrotic syndrome type 14 (NPHS14). Patients with SPLIS exhibit lymphopenia, nephrosis, adrenal insufficiency, and/or neurological defects. No targeted therapy for SPLIS has been reported. Vitamin B6 supplementation has therapeutic activity in some genetic diseases involving B6‐dependent enzymes, a finding ascribed largely to the vitamin's chaperone function. We investigated whether B6 supplementation might have activity in SPLIS patients. We retrospectively monitored responses of disease biomarkers in patients supplemented with B6 and measured SPL activity and sphingolipids in B6‐treated patient‐derived fibroblasts. In two patients, disease biomarkers responded to B6 supplementation. S1P abundance and activity levels increased and sphingolipids decreased in response to B6. One responsive patient is homozygous for an SPL R222Q variant present in almost 30% of SPLIS patients. Molecular modeling suggests the variant distorts the dimer interface which could be overcome by cofactor supplementation. We demonstrate the first potential targeted therapy for SPLIS and suggest that 30% of SPLIS patients might respond to cofactor supplementation.

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Actualización de la glomerulopatía colapsante

Cited by 9 publications

References 49 publications

Spectrum of podocytopathies in new-onset nephrotic syndrome following COVID-19 disease: a report of 2 cases

Spectrum of podocytopathies in new-onset nephrotic syndrome following COVID-19 disease: a report of 2 cases

Membranous nephropathy with collapse in a HIV negative patient; a case report with a 34-month follow-up

Responsiveness of sphingosine phosphate lyase insufficiency syndrome to vitamin B6 cofactor supplementation

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