A Case of Congenital Nephrotic Syndrome with Crescents Caused by a Novel Compound Heterozygous Pairing of NPHS1 Genetic Variants

Figure 1

Histopathologic and immunohistochemical analysis of kidney sections from patient and controls. (a, b) Representative glomeruli showing FSGS: (a) H&E (400X) and (b) PAS (400X). (c–e) Representative glomeruli showing cellular crescent formation: (c) H&E (400X), (d) PAS (400X), and (e) Toluidine Blue (400X). (f) Representative section of the renal cortex illustrating severe interstitial fibrosis, trichrome (100X). (g, h) Representative images showing diffuse podocyte foot process effacement, electron microscopy (3000X and 8000X). (i) Immunofluorescence confocal microscopy comparing localization of nephrin and synaptopodin in the patient’s kidney to a control kidney. (j) Airyscan immunofluorescence microscopy comparing the patient’s kidney to MCD and control kidneys.