Abstract: PUB164
Nephrotic Syndrome and Microhematuria with a Twist: Adult Minimal Change Disease with Diffuse Mesangial Hypercellularity
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Bailey, Ronelle, New York University Grossman School of Medicine, New York, New York, United States
- Khan, Saila Azam, New York University Grossman School of Medicine, New York, New York, United States
- Drakakis, James, New York University Grossman School of Medicine, New York, New York, United States
Introduction
Minimal change disease (MCD) with diffuse mesangial hypercellularity (DMH) is a pattern of injnury that can be considered an unusual variant of minimal change disease, often described in the pediatric population. Patients with DMH are thought to have a higher risk of initial resistance to steroid therapy, although it is not clear if there is any difference in the long term prognosis. Also, this subset of patients is at higher risk of developing hypertension and hematuria over the course of the disease. Our case describes an adult male with nephrotic syndrome and persistent microscopic hematuria, found to have this entity on kidney biopsy.
Case Description
52 year old male with no past medical history presented with sudden onset of nephrotic syndrome, accompanied by microscopic hematuria. BP of 150/90 mm Hg (and even higher at times). UA revealed 4+ protein and 21-50 RBC (which was consistent on several checks). UPCR 8.5 g/g and UACR 5.75 g/g. Serum albumin 2.5 g/dL. Serum creatinine 1.5 - 1.7 mg/dL (from 1.2 mg/dL in 2025). Worth noting is that UA from two months prior was without protein or blood. Kidney biopsy was undertaken with sampling for light microscopy showing DMH and electron microscopy with diffuse podocyte effacement. Taken together, the findings were consistent with MCD with DMH. He was initiated on Prednisone 1 mg/kg (80 mg) daily and by eleven days later, UPCR dropped to 1.8 g/g with serum albumin 3.5 g/dL.
Discussion
DMH in its primary form is a rare histological finding, associated with idiopathic nephrotic syndrome. There is not much data in the literature to define the clinical course and prognosis in adults with this diagnosis. Different outcomes have been described despite similar appearance of initial biopsies. Most of the associations come from the pediatric populations, whereby there is often steroid reisstance or dependence. It is unclear whether DMH and MCD are distinct or related entities, or perhaps part of a continuum. Some suggest a more severe form of MCD, leading to slower rate of recovery. This case illustrates the course of an adult with MCD and DMH, with a rapid & favorable steroid response thus far. Our experience can perhaps redefine how this entitiy is viewed and treated in the adult population moving forward.