Purpose
The aim of our study was to evaluate by MRI the effectiveness of rapamycin to reduce the volume of renal cysts in patients with autosomal dominant polycystic kidney disease (ADPKD),
type I.
[Fig.1]
In polycystic kidney disease,
a deficit of polycystin determines an "hyperproliferation " of the cells,
that causes the formation of cysts,
because of the lack of physiological inhibition of mTOR.
It was noted that the inappropriate stimulation of mTOR,
is the basis of the abnormal development of renal cysts.
Rapamycin (RAPA) is...
Methods and Materials
In a period of 12 months,
from June 2008 to June 2009,
we examined 29 patients affected by ADPKD in treatment with rapamycin.
The Mean age of patiens was 46 years old (range 25-68 years).
Inclusion Criteria:
Age between 18 and <70 years
Clinical,
genetic,
and instrumental diagnosis of Type I ADPKD
Creatinine clearance (MDRD)> 40 <80 ml / min
Women of childbearing age with negative pregnancy test before administration of rapamycin,
and willing to use contraception for up to three months after suspension of...
Results
After one year of treatment with rapamycin,
the volume of renal cysts was reduced in 17 patients (58.6%) [fig.1],
in 5 patients (17.2%) remained almost unchanged [fig.2]
while in the remaining 7 patients (24.2%) increased [fig.3].
Conclusion
Rapamycin seems to be efficient in reducing the volumetric progression of cysts in patients with ADPKD.
The clinical efficacy of treatment has yet to be demonstrated.
References
1.
Renal structure in early autosomal-dominant polycystic kidneydisease (ADPKD): The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) cohort
Kidney International,
Vol.
64 (2003),
pp.
1035–1045 [Fig.1]
2.
Arlene B.
Chapman
Autosomal Dominant Polycystic Kidney Disease: Time for a Change?
J Am Soc Nephrol 18: 1399–1407,2007.doi:10.1681/ASN.2007020155
[Fig.2]
3.
Magnetic Resonance Imaging Evaluation of Hepatic Cysts in Early Autosomal-Dominant Polycystic Kidney Disease: The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease Cohort
Clin J Am Soc Nephrol 1: 64 – 69,
2006.
doi:...
Personal Information
Christos Bristogiannis,
M.D.
Departmentof Surgical Sciences
Section of Diagnostic Imaging
Foggia University School of Medicine (http://www.unifg.it)
Azienda Ospedaliero-Universitaria "Ospedali Riuniti"
Viale L.
Pinto,1 - 71100 - Foggia
