LAUSR

Question 1 The electron microscopy study revealed the presence of zebra bodies (lamellar lipid inclusion bodies) in the podocyte cytoplasm, which is pathognomonic for Fabry disease [1]. Since Fabry disease is very rarely encountered in pediatric nephrology practice, the symptoms of patients presenting with proteinuria and hearing loss are usually considered to be suggestive of Alport syndrome or of diseases affecting coenzyme Q10 metabolism. In our patient, the presence of proteinuria without hematuria should rule out Alport disease. Hematuria can also be observed in Fabry disease, although rarely, leading to confusion with glomerular diseases [2].