Heminefrectomía en pacientes con doble sistema renal. Experiencia en el Hospital Garrahan / Heminephrectomy in patients with a duplex kidney. Experience at the Garrahan Hospital

La heminefrectomía puede constituir el tratamiento definitivo en el 85% de los pacientes con diagnóstico de doble sistema con uno de los mismos no funcionante. Actualmente el abordaje laparoscópico es de elección tanto por sus resultados estéticos como evolución postoperatoria. Materiales y métodos: se efectuó una revisión y análisis retrospectivo de todos los pacientes operados de heminefrectomía desde el año 2000 a 2014. Resultados: 44 pacientes fueron operados de forma convencional durante el período 2000-2010 y 30 de forma laparoscópica transperitoneal durante el período 2010-2014. La media de duración del procedimiento fue menor para el grupo convencional (89,6 vs 128 min, p = 0,000) mientras que la media de estadía hospitalaria fue menor para el grupo laparoscópico (4,2 vs 5,1, p = 0,19). La tasa de reoperación fue mayor en el grupo convencional (20,4% vs 6,7%, p = 0,18) y la principal causa fue por la presencia un muñón ureteral sintomático. En los controles por centellograma DMSA (en 19 pacientes del segundo grupo) no se objetivó la pérdida de función de la unidad remanente en ningún individuo. Conclusiones: La heminefrectomía es un procedimiento seguro con baja tasa de complicaciones cuya tendencia actual es realizar la cirugía de forma laparoscópica obteniendo resultados similares y menor tasa de complicaciones que en la forma convencional (AU)

Heminephrectomy may be the definitive treatment in 85% of patients diagnosed with a duplex kidney system of which one is not working. Currently, a laparoscopic approach is the treatment of choice because of both esthetic results and postoperative outcome. Material and methods: A retrospective analysis of all patients who underwent heminephrectomy between 2000 and 2014 was conducted. Results: 44 patients underwent conventional surgery in the period 2000-2010 and 30 underwent transperitoneal laparoscopy in the period 2010-2014. Mean duration of the procedure was less in the conventional group (89.6 vs 128 min, p = 0.000), while the mean hospital stay was less in the laparoscopic group (4.2 vs 5.1, p = 0.19). Reoperation rate was higher in the conventional group (20.4% vs 6.7%, p = 0.18) and the main cause was presence of a symptomatic ureteral stump. On DMSA whole body scan controls (in 19 patients in the second group) no loss of function of the remaining unit was observed in any of the patients. Conclusions: Heminephrectomy is a safe procedure with a low complication rate and a current trend to be performed using laparoscopy with similar results and a lower complication rate than in conventional surgery (AU)

Globotriaosylceramide (GL-3) Accumulation in the Renal Biopsy of a 1-year-old Patient with Fabry Disease and Ureteropelvic Junction Obstruction.

Introduction: Fabry disease is a x-chromosome hereditary disease with an incidence of 1/40000 newborns. Nowadays it presents as much in males as in females and its first clinical symptoms are seen in pediatric patients. Patients have reduced or no activity of alpha-galactosidase which leads to progressive accumulation of GL-3 in lysosomes of all types of cells. This early deposition disrupts lysosomal function, leading to cell death, metabolic problems, vascular lesions, endothelial dysfunction, oxidative stress, alterations in autophagy tissue ischemia, and finally producing fibrosis in different tissues. On the other hand, ureteropelvic junction obstruction (UPJO) is the most frequent congenital anomaly of the urinary tract; with an incidence of 1 in 1000-2000 newborns. A patient with antenatal diagnosis of Fabry disease and pre-natal diagnosis of UPJ is described. GL-3 deposits were found in all progeny of renal cells in the surgical biopsy. Patient Report: Prenatal diagnosis of FD and severe left hydronephrosis (left renal pelvis 23 mm) He was born at term with adequate weight. Enzymatic activity of Alpha-Gal A was low and the molecular analysis confirmed the family’s mutation. Pyeloplasty was performed when he was 17 months old and, having obtained informed consent, a small piece of kidney was studied, showing evidence of characteristic GL-3 deposits in all cell types and showed podocyte “effacement”, a marker of injury and stress. Conclusion: We demonstrate in this report that the deposits that lead to the sequence of a series of inflammation and fibrosis are present at a very early age. Based upon this finding, one can speculate about the prevention of late lesions with an early start of enzyme replacement therapy (ERT). Long term follow-up studies will be necessary to confirm this hypothesis.