From the Guidelines
For patients with Dent disease approaching end-stage renal disease, renal transplantation is the preferred treatment option, with early evaluation recommended when the glomerular filtration rate falls below 20-30 ml/min/1.73m², as supported by the most recent study 1. The management approach for these patients should prioritize renal transplantation, considering both living and deceased donor options.
- Living donor transplantation is preferred when possible, but deceased donor transplantation is also effective.
- Pre-transplant evaluation should include genetic testing to confirm the diagnosis and rule out other conditions, as recommended by the American Society of Transplantation’s Living Donor Community of Practice 1.
- Post-transplant immunosuppression typically includes tacrolimus or cyclosporine, mycophenolate mofetil, and corticosteroids, with dosing individualized based on patient factors, as discussed in the American Journal of Transplantation 1. Unlike some other genetic kidney diseases, Dent disease does not recur in the transplanted kidney because the genetic defect affects the native kidneys specifically, making transplantation particularly effective with excellent long-term outcomes, as noted in the context of renal transplant dysfunction 1. While awaiting transplantation, conservative management should focus on:
- Controlling electrolyte imbalances
- Managing proteinuria
- Preventing complications of chronic kidney disease through appropriate medication and dietary adjustments Patients should be monitored for hypercalciuria, nephrocalcinosis, and bone disease both before and after transplantation, with consideration of the latest recommendations for the care and management of patients with failing kidney allografts 1.
From the Research
Renal Transplantation Management
As a patient approaches end-stage renal disease from Dent disease, the best management approach regarding renal transplantation involves several key considerations:
- Renal transplantation is the best therapy for patients with end-stage renal disease, as it offers the best patient outcomes 2, 3.
- To avoid graft rejection, adequate immunosuppressive therapy is crucial, with tacrolimus being a cornerstone of modern immunosuppressive therapy 2, 4.
- Tacrolimus has proven its efficacy in reducing the risk of acute rejection, refractory rejection, and improving graft function compared to cyclosporine 2.
- However, tacrolimus therapy can be associated with adverse effects such as nephrotoxicity and post-transplant diabetes mellitus, which need to be carefully managed 2, 4.
Pre-Transplantation Considerations
Before undergoing renal transplantation, patients with end-stage renal disease should:
- Be referred to nephrology to optimize disease management 3.
- Receive vaccinations against seasonal influenza, tetanus, hepatitis B, human papillomavirus, and Streptococcus pneumoniae 3.
- Have their blood pressure controlled through adequate dialysis and sodium restriction 3.
- Be monitored for signs of protein-energy wasting and malnutrition 3.
Dental Considerations
Patients with end-stage renal disease undergoing hemodialysis or awaiting kidney transplantation should also receive proper dental care, as oral diseases can be a potential cause of poor health outcomes:
- Dental operative protocols should be tailored to the patient's needs, taking into account the oral manifestations associated with end-stage renal disease and hemodialysis treatment 5, 6.
- Dentists should be aware of the oral and systemic manifestations of renal disease and manage afflicted patients accordingly 6.