Glomerulopatía por invaginación podocítica; reporte del primer caso en Latinoamérica y revisión de la literatura.
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| Title: | Glomerulopatía por invaginación podocítica; reporte del primer caso en Latinoamérica y revisión de la literatura. |
|---|---|
| Alternate Title: | Podocyte infolding glomerulopathy; report of the first case in Latin America and review of the literature. |
| Authors: | Malvar, Ana1 avmperrin@yahoo.com.ar, Davila, Pedro1, Ferrari, Matías1, Delgado, Pamela1, Iscoff, Paula1, Lococo, Bruno1, Alberton, Valeria2 |
| Source: | Nefrologia. jul/ago2020, Vol. 40 Issue 4, p469-473. 5p. |
| Subjects: | BASAL lamina, GLOMERULOSCLEROSIS, IMMUNE complexes, ELECTRON microscopes, NEPHROTIC syndrome, LUPUS nephritis, FOCAL segmental glomerulosclerosis |
| Abstract (English): | Background: Podocyte infolding glomerulopathy (PIG) is a condition of uncertain origin, frequently associated with autoimmune diseases. Its specific treatment and clinical course are unknown. It is characterised by thickening of the capillary walls due to the presence of non-argyrophilic intramembranous bubbles similar to those found in membranous glomerulopathy, but without electron-dense deposits of immune complexes in the ultrastructure, where translucent microspheres generated by invagination of the podocyte cytoplasm into the basement membranes are observed. Objectives: Generally reported in young females patients. To date, few cases in Asian patients have been reported. Our case is the first to be reported in a Latin American Caucasian patient. Methods: A 38-year-old woman with SLE. In 2014 she presented with nephrotic syndrome empirically treated with corticosteroids (CO) and intravenous cyclophosphamide with good response. She had a relapse in April 2015 with normal renal function and no extrarenal lupus activity, so she was referred to our hospital to be biopsied. Results: The biopsy reported focal segmental glomerular sclerosis without deposits of immune complexes in the immunofluorescence. However, methenamine silver staining revealed clear spaces in the capillary walls accompanied by marked podocyte alterations. On electron microscope study, numerous aggregates of microvesicular and cylindrical ultrastructures bound to the membranes were observed, without evidence of dense deposits, and diffuse effacement of pedicel foot processes, confirming the suspected diagnosis. Conclusions: This is the first reported case of what can be considered a new pathological glomerular entity in a Latin American Caucasian patient, whose clinical course and therapy are still unknown. [ABSTRACT FROM AUTHOR] |
| Abstract (Spanish): | Antecedentes: La glomerulopatía por invaginación podocítica (GIP) es una enfermedad de origen incierto, frecuentemente asociada a enfermedades autoinmunes, de la que se desconoce el tratamiento específico y su evolución. Caracterizada por engrosamiento de paredes capilares por la presencia de burbujas no argirofílicas intramembanosas similares a las encontradas en la glomerulopatía membranosa, pero sin depósitos de inmunocomplejos electrodensos en la ultraestructura, donde se observan microesferas traslúcidas generadas por invaginación del citoplasma podocítico dentro de las membranas basales. Objetivos: Generalmente descrito en pacientes jóvenes de sexo femenino. Hasta la fecha, han sido reportados escasos casos en pacientes de origen asiático. Nuestro caso constituiría el primer reporte en paciente latinoamericano de raza blanca. Métodos: Mujer de 38 an? os con LES. En el an? o 2014 presentó síndrome nefrótico tratado empíricamente con corticoides (CO) y ciclofosfamida intravenosa (CF) con buena respuesta. Presenta recaída en abril del 2015 con función renal normal y sin actividad lúpica extrarrenal, por lo que es derivada a nuestro hospital para ser biopsiada. Resultados: La biopsia informó esclerosis glomerular focal y segmentaria sin depósitos de inmunocomplejos en la inmunofluorescencia, pero con técnica de metenamina plata se detectaron en las paredes capilares, espacios claros acompan? ados de marcadas alteraciones podocíticas. Al microscopio electrónico, se observaron agregados de ultraestructuras microvesiculares y cilíndricas unidas a las membranas sin evidencia de depósitos densos y borramiento difuso de pies pedicelares, confirmando el diagnóstico sospechado. Conclusiones: Reportamos el primer caso de lo que puede ser considerada, una nueva entidad patológica glomerular, en una paciente de raza blanca latinoamericana, cuya evolución y terapéutica aún se desconocen. [ABSTRACT FROM AUTHOR] |
| Copyright of Nefrologia is the property of Revista Nefrologia and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.) | |
| Database: | MedicLatina |
| FullText | Links: – Type: pdflink Text: Availability: 0 |
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| Header | DbId: lth DbLabel: MedicLatina An: 145180976 AccessLevel: 6 PubType: Academic Journal PubTypeId: academicJournal PreciseRelevancyScore: 0 |
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| Items | – Name: Title Label: Title Group: Ti Data: Glomerulopatía por invaginación podocítica; reporte del primer caso en Latinoamérica y revisión de la literatura. – Name: TitleAlt Label: Alternate Title Group: TiAlt Data: Podocyte infolding glomerulopathy; report of the first case in Latin America and review of the literature. – Name: Author Label: Authors Group: Au Data: <searchLink fieldCode="AR" term="%22Malvar%2C+Ana%22">Malvar, Ana</searchLink><relatesTo>1</relatesTo><i> avmperrin@yahoo.com.ar</i><br /><searchLink fieldCode="AR" term="%22Davila%2C+Pedro%22">Davila, Pedro</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Ferrari%2C+Matías%22">Ferrari, Matías</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Delgado%2C+Pamela%22">Delgado, Pamela</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Iscoff%2C+Paula%22">Iscoff, Paula</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Lococo%2C+Bruno%22">Lococo, Bruno</searchLink><relatesTo>1</relatesTo><br /><searchLink fieldCode="AR" term="%22Alberton%2C+Valeria%22">Alberton, Valeria</searchLink><relatesTo>2</relatesTo> – Name: TitleSource Label: Source Group: Src Data: <searchLink fieldCode="JN" term="%22Nefrologia%22">Nefrologia</searchLink>. jul/ago2020, Vol. 40 Issue 4, p469-473. 5p. – Name: Subject Label: Subjects Group: Su Data: <searchLink fieldCode="DE" term="%22BASAL+lamina%22">BASAL lamina</searchLink><br /><searchLink fieldCode="DE" term="%22GLOMERULOSCLEROSIS%22">GLOMERULOSCLEROSIS</searchLink><br /><searchLink fieldCode="DE" term="%22IMMUNE+complexes%22">IMMUNE complexes</searchLink><br /><searchLink fieldCode="DE" term="%22ELECTRON+microscopes%22">ELECTRON microscopes</searchLink><br /><searchLink fieldCode="DE" term="%22NEPHROTIC+syndrome%22">NEPHROTIC syndrome</searchLink><br /><searchLink fieldCode="DE" term="%22LUPUS+nephritis%22">LUPUS nephritis</searchLink><br /><searchLink fieldCode="DE" term="%22FOCAL+segmental+glomerulosclerosis%22">FOCAL segmental glomerulosclerosis</searchLink> – Name: Abstract Label: Abstract (English) Group: Ab Data: Background: Podocyte infolding glomerulopathy (PIG) is a condition of uncertain origin, frequently associated with autoimmune diseases. Its specific treatment and clinical course are unknown. It is characterised by thickening of the capillary walls due to the presence of non-argyrophilic intramembranous bubbles similar to those found in membranous glomerulopathy, but without electron-dense deposits of immune complexes in the ultrastructure, where translucent microspheres generated by invagination of the podocyte cytoplasm into the basement membranes are observed. Objectives: Generally reported in young females patients. To date, few cases in Asian patients have been reported. Our case is the first to be reported in a Latin American Caucasian patient. Methods: A 38-year-old woman with SLE. In 2014 she presented with nephrotic syndrome empirically treated with corticosteroids (CO) and intravenous cyclophosphamide with good response. She had a relapse in April 2015 with normal renal function and no extrarenal lupus activity, so she was referred to our hospital to be biopsied. Results: The biopsy reported focal segmental glomerular sclerosis without deposits of immune complexes in the immunofluorescence. However, methenamine silver staining revealed clear spaces in the capillary walls accompanied by marked podocyte alterations. On electron microscope study, numerous aggregates of microvesicular and cylindrical ultrastructures bound to the membranes were observed, without evidence of dense deposits, and diffuse effacement of pedicel foot processes, confirming the suspected diagnosis. Conclusions: This is the first reported case of what can be considered a new pathological glomerular entity in a Latin American Caucasian patient, whose clinical course and therapy are still unknown. [ABSTRACT FROM AUTHOR] – Name: Abstract Label: Abstract (Spanish) Group: Ab Data: Antecedentes: La glomerulopatía por invaginación podocítica (GIP) es una enfermedad de origen incierto, frecuentemente asociada a enfermedades autoinmunes, de la que se desconoce el tratamiento específico y su evolución. Caracterizada por engrosamiento de paredes capilares por la presencia de burbujas no argirofílicas intramembanosas similares a las encontradas en la glomerulopatía membranosa, pero sin depósitos de inmunocomplejos electrodensos en la ultraestructura, donde se observan microesferas traslúcidas generadas por invaginación del citoplasma podocítico dentro de las membranas basales. Objetivos: Generalmente descrito en pacientes jóvenes de sexo femenino. Hasta la fecha, han sido reportados escasos casos en pacientes de origen asiático. Nuestro caso constituiría el primer reporte en paciente latinoamericano de raza blanca. Métodos: Mujer de 38 an? os con LES. En el an? o 2014 presentó síndrome nefrótico tratado empíricamente con corticoides (CO) y ciclofosfamida intravenosa (CF) con buena respuesta. Presenta recaída en abril del 2015 con función renal normal y sin actividad lúpica extrarrenal, por lo que es derivada a nuestro hospital para ser biopsiada. Resultados: La biopsia informó esclerosis glomerular focal y segmentaria sin depósitos de inmunocomplejos en la inmunofluorescencia, pero con técnica de metenamina plata se detectaron en las paredes capilares, espacios claros acompan? ados de marcadas alteraciones podocíticas. Al microscopio electrónico, se observaron agregados de ultraestructuras microvesiculares y cilíndricas unidas a las membranas sin evidencia de depósitos densos y borramiento difuso de pies pedicelares, confirmando el diagnóstico sospechado. Conclusiones: Reportamos el primer caso de lo que puede ser considerada, una nueva entidad patológica glomerular, en una paciente de raza blanca latinoamericana, cuya evolución y terapéutica aún se desconocen. [ABSTRACT FROM AUTHOR] – Name: AbstractSuppliedCopyright Label: Group: Ab Data: <i>Copyright of Nefrologia is the property of Revista Nefrologia and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.) |
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| RecordInfo | BibRecord: BibEntity: Identifiers: – Type: doi Value: 10.1016/j.nefro.2019.09.010 Languages: – Code: spa Text: Spanish PhysicalDescription: Pagination: PageCount: 5 StartPage: 469 Subjects: – SubjectFull: BASAL lamina Type: general – SubjectFull: GLOMERULOSCLEROSIS Type: general – SubjectFull: IMMUNE complexes Type: general – SubjectFull: ELECTRON microscopes Type: general – SubjectFull: NEPHROTIC syndrome Type: general – SubjectFull: LUPUS nephritis Type: general – SubjectFull: FOCAL segmental glomerulosclerosis Type: general Titles: – TitleFull: Glomerulopatía por invaginación podocítica; reporte del primer caso en Latinoamérica y revisión de la literatura. Type: main BibRelationships: HasContributorRelationships: – PersonEntity: Name: NameFull: Malvar, Ana – PersonEntity: Name: NameFull: Davila, Pedro – PersonEntity: Name: NameFull: Ferrari, Matías – PersonEntity: Name: NameFull: Delgado, Pamela – PersonEntity: Name: NameFull: Iscoff, Paula – PersonEntity: Name: NameFull: Lococo, Bruno – PersonEntity: Name: NameFull: Alberton, Valeria IsPartOfRelationships: – BibEntity: Dates: – D: 01 M: 07 Text: jul/ago2020 Type: published Y: 2020 Identifiers: – Type: issn-print Value: 02116995 Numbering: – Type: volume Value: 40 – Type: issue Value: 4 Titles: – TitleFull: Nefrologia Type: main |
| ResultId | 1 |
