Autosomal dominant polycystic kidney disease in a kidney transplant population.

AIM To define specific manifestations of autosomal dominant polycystic kidney disease in kidney transplant patients. METHODS Of 874 consecutive first renal transplant patients 1985-1993, 114 (13%) had autosomal dominant polycystic kidney disease (ADPKD). Mean age was 53 +/- 8 years, 62% were men, and 83% received cadaveric kidneys. Control patients were matched for sex, age and donor type. Median follow-up time was 63 months. One patient was lost to follow-up. Medical records before and after transplantation were reviewed. RESULTS Survival of patients and grafts was similar in ADPKD patients and controls. Twenty-five ADPKD patients died, four of causes not seen in the controls; two aortic aneurysms, one urothelial cancer, one colon perforation. Four more ADPKD patients but no control had diverticulitis (P = 0.03), two with perforation. Cardiovascular morbidity was not increased. Eight patients had subarachnoidal haemorrhage before transplantation and two during follow-up. Nineteen patients had undergone nephrectomy before transplantation, 11 because of voluminous kidneys, five for infection, pain or bleeding, two for suspected malignancy, one for hypertension. After transplantation, seven patients underwent nephrectomy, only one related to kidney size. During the first year, need of phlebotomy occurred in 14% of patients versus 4% of controls, P = 0.02. Urinary tract infection rates were not increased. No morbidity was related to liver cysts. CONCLUSION The specific features of kidney transplantation to patients with ADPKD were few: enlarged kidneys, relevant only before transplantation, erythrocytosis, and as rare but serious events, diverticulitis with perforation.

[1]  G. Everson Hepatic cysts in autosomal dominant polycystic kidney disease. , 1993, Mayo Clinic proceedings.

[2]  M. Khokha,et al.  Impaired tubulogenesis of cyst-derived cells from autosomal dominant polycystic kidneys. , 1995, Kidney international.

[3]  W. Schievink,et al.  Neurovascular Manifestations of Heritable Connective Tissue Disorders: A Review , 1994, Stroke.

[4]  Ann M. Johnson,et al.  Causes of death in autosomal dominant polycystic kidney disease. , 1995, Journal of the American Society of Nephrology : JASN.

[5]  Charles E. Kahn,et al.  polycystic kidney disease , 1995 .

[6]  J. Goodlad Lesson to learn. , 1986, Nursing standard : official newspaper of the Royal College of Nursing.

[7]  Andrew J.W. Hilson,et al.  POLYCYSTIC KIDNEYS AND ABDOMINAL AORTIC ANEURYSMS , 1980, The Lancet.

[8]  X. Estivill,et al.  Abdominal aortic aneurysms and autosomal dominant polycystic kidney disease. , 1996, Journal of the American Society of Nephrology : JASN.

[9]  P. Gabow,et al.  Natural history of autosomal dominant polycystic kidney disease. , 1994, Annual review of medicine.

[10]  P. Gabow Autosomal dominant polycystic kidney disease. , 2010, The New England journal of medicine.

[11]  M. Rivera,et al.  Stroke in adult polycystic kidney disease. , 1992, Postgraduate medical journal.

[12]  E. Higashihara,et al.  Mediation of renal cyst formation by hepatocyte growth factor , 1994, The Lancet.

[13]  B. Oken Intracranial aneurysms in polycystic kidney disease. , 1983, The New England journal of medicine.

[14]  H J Van Peenen,et al.  Abdominal aortic aneurysms: the changing natural history. , 1984, Journal of vascular surgery.