[Unilateral neonatal cystic disease of the kidney as first manifestation of tuberous sclerosis].
OBJECTIVE
To report a case of unilateral cystic disease in a neonatal kidney as the first manifestation of tuberous sclerosis, with special reference to the diagnostic difficulties.


METHODS
The pathological and clinical features of unilateral cystic kidney disease, which was basically segmental, are described. This condition had been detected in a newborn at physical examination.


RESULTS
The pathological findings of the kidney cysts were characteristic of tuberous sclerosis, which allowed us to rule out a renal tumor. The clinical course confirmed the histological diagnosis.


CONCLUSIONS
The reported cases of bilateral cystic kidney disease as the first manifestation of tuberous sclerosis are few and only one case with unilateral involvement has been reported in the English literature. In the case described herein, biopsy proved to be very useful in the diagnosis of the disease since the kidney cysts had specific histological features.
Weil Lara
B
B
Departamento de Anatomía Patológica, Hospital Regional de Málaga, España.
Ibáñez Martínez
J
J
García González
I
I
Bedoya Belmonte
J J
JJ
D006801:Humans; D007231:Infant, Newborn; D008297:Male; D007690:Polycystic Kidney Diseases / Q000209:etiology* / Q000473:pathology; D014402:Tuberous Sclerosis / Q000150:complications*
Enfermedad quística renal neonatal unilateral como primera manifestación de esclerosis tuberosa.