Clinicopathologic Spectrum of Lysozyme-Associated Nephropathy.

Introduction: Lysozyme-associated nephropathy (LyN), a rare cause of kidney injury in patients with chronic myelomonocytic leukemia (CMML), has not been well described to date. We report the clinicopathologic spectrum of LyN from a multi-institutional series.

Method: We identified 37 native kidney biopsies with LyN and retrospectively obtained clinicopathologic data.

LC-MS lipidomics of renal biopsies for the diagnosis of Fabry disease.

Introduction: Lipidomics analysis or lipid profiling is a system-based analysis of all lipids in a sample to provide a comprehensive understanding of lipids within a biological system. In the last few years, lipidomics has made it possible to better understand the metabolic processes associated with several rare disorders and proved to be a powerful tool for their clinical investigation. Fabry disease is a rare X-linked lysosomal storage disorder (LSD) caused by a deficiency in α-galactosidase A (α-GAL A).

Nipple adenoma in infancy.

We report the first patient with a nipple adenoma presenting in infancy. Nipple adenoma is a benign lesion typically affecting women between 45 and 55 years of age. This lesion can occur in the pediatric population and should be included in the differential diagnosis of an infantile breast lesion.

Ectopic prostate tissue within the processus vaginalis: insights into prostate embryogenesis.

We report an 11-year-old boy who presented with a scrotal mass superior to the epididymis in the processus vaginalis. The mass consisted of prostate tissue, including glands and fibromuscular stroma, communicating with a spermatocele. Strong immunostaining for prostate-specific antigen was seen in the glandular epithelium.