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AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE (PCKD)
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Two genes, PKD1 and PKD2
Ten percent of cases of end-stage renal disease (ESRD)
No gender or race preference
Most frequent etiology of inherited renal failure.
Up to 50% of patients progress to ESRD by age 70
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Dysregulation of the renin–angiotensin–aldosterone system
Severe hypertension
Compression of surrounding viscera
Pain
Hemorrhage
Infections
Urosepsis
Potential risk of renal malignancies
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Midline laparotomy to access the native kidneys and iliac vessels
Native kidneys removed first
Transplant kidney implanted intraperitoneally after removal of native kidneys
Alternatively, the peritoneum at the midline incision can be separated from the abdominal wall laterally and dorsally up to the iliac vessels allowing for a retro-peritoneal implantation of the kidney
Allograft fixation is recommended to avoid graft torsion, given the intraperitoneal position and the vast amount of space after native nephrectomy
Usually performed in instances of live donor transplants
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With appropriate indications and informed consent, simultaneous bilateral nephrectomy and kidney transplantation is associated with resolution of symptoms and patient satisfaction in the setting of graft and recipient survival equivalent to ...