Foamy podocytes

Fosfolipidosis Renal inducida por Cloroquina, reporte de un caso

“…El diagnóstico correspondió a una lipidosis renal, enfermedades caracterizadas por almacenamiento anormal de lípidos a nivel renal. Ellas se clasifican en primarias cuando hay déficits enzimáticos hereditarios específicos, o secundarias a otros trastornos como cirrosis hepática, síndrome nefrótico, síndrome de Alport, síndrome de Alagille e incluso por agentes tóxicos o medicamentos, lo que se conoce como fosfolipidosis inducida por medicamentos (FIM) (2)(3)(4)(5).…”

“…Compromiso de las células tubulares renal ocurre en la leucodistrofia metacromática, enfermedad de Refsum del adulto y la enfermedad de Sandhoff. Acumulaciones a nivel de células mesangiales y endoteliales glomerulares se identifica en la enfermedad por déficit de lecitin colesterol acetil transferasa, enfermedad de Gaucher, y enfermedad de Niemann Pick (2,3,4). Cuerpos de cebra o mieloides en los podocitos son más característicos de En la enfermedad de Fabry a pesar de ser una enfermedad ligada a X, las mujeres heterocigotas pueden desarrollar compromiso orgánico sistémico.…”

Familial globotriaosylceramide-associated cardiomyopathy mimicking Fabry disease

“…There were vacuoles with lamellar and/or granular bodies in the parietal epithelial, tubular and interstitial cells, but not in the podocytes. Lipid accumulation in the podocytes is an early sign of Fabry disease and some other lipidoses 24 25. The renal function of Patient 1 and Patient 2 remained normal until cardiac transplantation, but has decreased probably due to calcineurin inhibitor nephrotoxicity, now stable at a glomerular filtration rate (GFR) of 40–45 mL/min/1.73 m 2 .…”

Asymptomatic proteinuria in a child with recurrent urinary tract infections—answers

“…The differential diagnosis of this condition includes various primary lysosomal storage diseases, such as Fabry, Gaucher, Niemann-Pick, Hurler, Farber, Refsum, and Sandhoff disease [1]. Secondary lipidoses are more common than primary lipidosis, including those due to nephrotic syndrome, hepatic failure, and iatrogenic (medication related) phospholipidosis [2]. Primary renal lipidoses may be differentiated by cellular lipid distribution in renal and extrarenal tissues, histochemical staining features and the appearance of storage material, clinical presentation, and enzymatic studies [2].…”

“…Secondary lipidoses are more common than primary lipidosis, including those due to nephrotic syndrome, hepatic failure, and iatrogenic (medication related) phospholipidosis [2]. Primary renal lipidoses may be differentiated by cellular lipid distribution in renal and extrarenal tissues, histochemical staining features and the appearance of storage material, clinical presentation, and enzymatic studies [2]. The most useful feature in distinguishing Fabry disease from other forms of renal lipidosis is the ultrastructural appearance of accumulated lipid as electron-dense multilamellar myelin figures (zebra bodies), which are also present in our patient [1].…”