García-de León Gómez JM, Navarro-González A, Hernández-Valdéz MG, Aguirre-Ramírez P

Language: Spanish
References: 5
Page: 123-126
PDF size: 376.79 Kb.

ABSTRACT

Polycystic kidney disease is a pathology that affects both kidneys symmetrically.
It is usually diagnosed by obstetric ultrasound or at birth and is the result of a specific mutation of a gene on chromosome 16. This disease has a low incidence of 1 in 20 000 births and represents 4.24% of neonatal abdominal masses and 6.4% of all renal masses at this stage of life. It usually manifests as hard echogenic giant kidneys.
The case is presented of a neonate with antenatal detection of autosomal recessive polycystic disease and renal failure, treated in the first days of life with left nephrectomy and then, twelve months later, with right nephrectomy and kidney transplantation.
The neonatal patient was attended to at our hospital and had a past medical history of prenatal ultrasound diagnosis of large, echogenic kidneys and oligohydramnios, with no evidence of hydronephrosis. After multidisciplinary discussion (neonatology, nephrology, transplantation, and urology) the decision was made to perform left nephrectomy in order to improve respiratory failure and facilitate feeding. At one year of age, the patient weighed 8 kg and had 15% renal function and so it was decided in the Department of Nephrology and Transplantation to perform a living related kidney transplant. Right nephrectomy was carried out in the patient minutes before the transplantation to place the graft on the right side, with direct anastomosis to the vena cava and aorta.
One year after the transplantation, the two-year-old patient presents with excellent function of the kidney graft.
Autosomal recessive polycystic kidney disease has different degrees of severity. In the present case, early urologic treatment was possible and consisted of abdominal decompression through unilateral nephrectomy with subsequent successful kidney transplantation.

REFERENCES

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