Kidney trasplantation in a female infant with Down syndrome, Pseudo Prune Belly Syndrome, and a post-transplant lymphoproliferative disorder. First case report in Argentina

Abstract

Introduction: Patients with trisomy 21 have a higher risk for congenital anomalies including congenital anomalies of the kidney and urinary tract. The association between Prune Belly Syndrome and Down Syndrome has been described but the occurrence of both conditions would likely represent a coincidence. There are few published reports on renal transplantation in patients with this syndromes. Clinical Case: We reporte a 5-year-old female patient with antenal diagnosis of down syndrome. Post-natal abdominal ultrasound revealed megabladder and dysplastic kidneys. At five months of age, she was commenced on peritoneal dialysis. The patient underwent renal transplantation at age of 3. Acute thrombosis of transplanted renal artery was diagnosed, resulting in graft loss. Four months after second transplantation, the patient presented bilateral tonsillar enlargement and a post-transplant lymphoproliferative disorder was diagnosed. Conclusion: We describe the existence of these conditions in a single patient who underwent kidney transplantation, it’s clinical manement and follow up. This case has been reported with the hope of contributing to the existing knowledge which pertains to kidney transplantation patients with Down syndrome.