Introduction:
Glomerulocystic kidney disease (GCKD) is a rare condition characterized by the cystic enlargement of Bowman's space surrounding the glomeruli. Unlike other kidney diseases that affect the tubules, GCKD primarily involves the glomeruli. The formation of these cysts disrupts normal glomerular function, often leading to chronic kidney disease. GCKD can be inherited or may occur in association with other genetic or developmental disorders.
Methods:
We reviewed the clinical charts and histopathology slides of kidney biopsies from two cases of glomerulocystic kidney disease (GCKD) presented at our hospital between 2021 and 2024. Relevant clinical and histopathologic data were collected for analysis.
Results:
The first case involved a 17-year-old female who was referred to the kidney outpatient clinic due to renal impairment and worsening renal function. Her serum creatinine level was 105 µmol/L. There was no family history of similar conditions. A kidney core needle biopsy revealed cystic dilation of Bowman’s spaces in all sampled glomeruli (Figure 1). The Bowman’s spaces were enlarged by 2-3 times, containing rudimentary or shrunken glomerular tufts. The surrounding renal tubules did not exhibit cystic dilation but showed severe tubular atrophy. Her renal function continued to deteriorate, with serum creatinine now reaching 450 µmol/L. The patient was subsequently placed on hemodialysis and is currently awaiting renal transplantation.
The second case involved a 34-year-old male with end-stage renal disease (ESRD) of unknown cause, who underwent a kidney transplant 7 years ago. The transplant failed after 6 months, and the patient has remained on hemodialysis since then. He also had severe hypertension. A CT scan revealed bilateral small native kidneys, and the transplant multidisciplinary team (MDT) suspected them as a potential cause of his hypertension, leading to the recommendation for bilateral nephrectomy.
Microscopic examination of the nephrectomy specimens revealed cystic dilation of Bowman’s spaces involving all glomeruli in both kidneys. The Bowman’s spaces were enlarged by 2-3 times, containing rudimentary or shrunken glomerular tufts (Figure 1), or in some cases, an absence of the tuft, replaced by eosinophilic fluid. The surrounding renal tubules were not dilated, and there were no cystic changes in the medullary tubules, calyces, or renal pelvis. The cortex exhibited severe tubular atrophy and fibrosis. The intrarenal arteries showed moderate to severe fibrointimal proliferation, with some displaying pinpoint lumens.
Conclusions:
Glomerulocystic kidney disease (GCKD) is a rare disorder. We present two cases of GCKD that we believe are the first diagnosed in the United Arab Emirates. Both patients progressed to end-stage renal disease (ESRD) between the ages of 15 and 20 years and were managed by hemodialysis and transplantation. Genetic study and counseling are important to understand the mechanism of development, the pattern of inheritance and methods of prevention of this disease.
I have no potential conflict of interest to disclose.
I did not use generative AI and AI-assisted technologies in the writing process.