Glomerulopatía por invaginación podocítica; reporte del primer caso en Latinoamérica y revisión de la literatura

Malvar, Ana; Dávila, Pedro; Ferrari, Matías; Delgado, Pamela; Iscoff, Paula; Lococo, Bruno; Albertón, Valeria

doi:10.1016/j.nefro.2019.09.010

Cited by 7 publications

(4 citation statements)

References 20 publications

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“…Of note, the case we present showed segmental sclerosis. An association between PIG and segmental sclerosis has been reported in the largest case series and other case reports, 2 , 8 , 10 with 21.6% of cases (8/37) having segmental sclerosis as the primary diagnosis by light microscopy. It is possible that PIG is a manifestation of a form of podocytopathy that leads to altered podocyte/basement membrane interactions with incorporation of podocyte cell membranes into the glomerular basement membranes.…”

Section: Discussionmentioning

confidence: 73%

“… 2 Since then, there has been an additional 1 case identified from Japan, 5 3 cases from China, 6 - 8 1 case from Germany, 9 and 1 case from Argentina. 10 To our knowledge, we are the first to report a case of PIG in North America.…”

Section: Discussionmentioning

confidence: 89%

“…Proteinuria in PIG is in the subnephrotic range in the majority of cases, 2 , 7 with 28.6% (10/35) that have proteinuria in the nephrotic range, 2 , 8 , 9 and 27.7% (10/36) that are described as having nephrotic syndrome. 1 , 8 - 10 Of the case reports that documented patient outcomes, 45.2% (14/31) described complete remission of proteinuria, defined as a 24-hour urine protein of ≤0.3 g/day. 1 , 8 , 9 , 20 Another 45.2% (14/31) patients had partial improvement in proteinuria.…”

Section: Discussionmentioning

confidence: 99%

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Podocyte Infolding Glomerulopathy, First Case Report From North America

Ting

Hung

McRae

et al. 2021

Can J Kidney Health Dis

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Rationale: Podocyte infolding glomerulopathy (PIG) is a newly described condition with only 37 cases reported worldwide. Due to its rarity, the pathogenesis and evolution of this disease is unclear. This case report contributes to our collective knowledge about the clinical and histological progression of this disease. Presenting concerns of the patient: Over the course of a year, a 52-year-old Malaysian woman with no known prior medical history developed progressively worsening edema and other findings consistent with nephrotic syndrome. Diagnosis: Unlike most patients with PIG, this patient did not have any autoimmune disease. She was Hepatitis B core antibody positive with a Hepatitis B surface antibody >1000, suggesting prior Hepatitis B infection with immunity. A renal biopsy was performed which was consistent with PIG. A second renal biopsy was done 2 years later which again showed characteristic findings of PIG with worsened podocyte effacement but no interval change in chronicity. Interventions: The patient was treated with blood pressure control and renin-angiotensin-aldosterone system (RAAS) blockade with irbesartan and spironolactone. She was also treated with prednisone at 1 mg/kg for 2 months followed by a taper for a total of 7 months of prednisone treatment. Outcomes: The patient had a partial response to a course of prednisone. However, since stopping steroids, her proteinuria and renal function has been gradually worsening. Teaching points: PIG is mostly found in patients of East Asian descent. It presents as proteinuria and is often associated with autoimmune disease but can be idiopathic. It is characterized on renal biopsy by infolding or protrusion of podocyte cytoplasm into glomerular basement membrane, as well as intramembranous cytoplasmic microspherules or microtubules. Atypical membranous nephropathy should be ruled out prior to diagnosis. Unlike membranous nephropathy, PIG usually responds at least partially to steroid monotherapy. To our knowledge, this is the first reported case of PIG from North America. Furthermore, it is the first case of PIG with repeat biopsy showing interval worsening of PIG rather than either resolution of PIG or transformation of PIG to a different diagnosis.

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Section: Discussionmentioning

confidence: 73%

Section: Discussionmentioning

confidence: 89%

Section: Discussionmentioning

confidence: 99%

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Podocyte Infolding Glomerulopathy, First Case Report From North America

Ting

Hung

McRae

et al. 2021

Can J Kidney Health Dis

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“…A la fecha existen pocos casos reportados de esta entidad, la inmensa mayoría en Asia, predominantemente de Japón. Hay unos pocos casos en otras latitudes 2,4,5 . El actual caso es de origen peruano.…”

Section: Discussionunclassified

Glomerulopatía por invaginación podocitaria: reporte de un caso

Pimentel,

Mayo,

Sumire

et al. 2023

NEFRO

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La glomerulopatía por invaginación podocitaria (GIP) es una entidad patológica rara, que consiste en engrosamiento de capilares con presencia de burbujas no argirofílicas, que corresponden a microtúbulos y/o microesferas en la microscopia electrónica causadas por la invaginación podocitaria intramembranosa. Reportamos el caso de una mujer con síndrome nefrótico sin evidencia de enfermedad autoinmune. La biopsia renal mostró los hallazgos característicos de GIP, descritos líneas arriba. El presente es el primer caso de GIP en el Perú. Es necesario el reporte de más casos para una mejor comprensión del curso clínico y patogénesis.

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