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 the treatment
Exclusion Criteria:
- Systemic or localized infection at the inception of treatment with rapamycin
- Presence of consolidated,
cavity or infiltrating lesions during chest radiography screening
- Use of other experimental drugs in the month prior to enrollment in the study
- Hypersensitivity to rapamycin and its derivatives
- Treatment with terfenadine,
cisapride,
astemizole,
pimozide,
or ketoconazole
- Total white blood cell count <3000/mm3,
platelet count <100,000 / mm3;
- Severe fasting dyslipidemia (triglycerides> 300 mg / dL,
total cholesterol> 350 mg / dL)
- Proteinuria> 1 g/24 hours
- Psychiatric disorders and all conditions that affect the complete understanding of the proposals and the risks of the study
- Neoplasms,
even suspicious,
in the three years prior to enrollment in the study,
with the exception of treated cutaneous,
basal cell and squamous cell carcinoma
- HIV,
HCV and HBsAg positive patients
Patients underwent MRI,
before and after treatment,
to determine the cysts volume variation.
MRI was performed using a 1.5-T system (ACHIEVA®,
Philips Medical System,
Eindhoven,
The Netherlands) with a maximum gradient strength of 30 mT/m,
a slew rate of 150 mT/m/ms and a four-channel phased-array coil.
The imaging protocol of our study provides:
1. Localizer sequence.
2. AxialDUAL Fast Field Echo (FFE) T1–weighted sequences (TR=147 ms; double TE=4.6/2.3 ms in phase and out of phase; flip angle=80°; matrix=256×256; slice thickness=5mm; gap=0mm).
3. Axial Single – Shot (SSh) T2–weighted sequences (TR=338 ms; TE=80 ms; matrix=256×256; slice thickness=5 mm;gap=0mm).
4. Coronal BALANCE Fast Field Echo (FFE) T2–weighted sequences (TR=3.8ms; TE=1.9 ms; flip angle=60°; matrix=256×256; slice thickness=5mm; gap=0mm).
We avoid the use of gadolinium contrast enhanced sequences to these patients,
with compromised renal function,
because does not imply any vantage in volume calculation.
Both axial and coronal images were obtained in breath-hold (slice thickness 5mm,
gap 0mm) to minimize movement artifacts.
The FOV was suitable to patients stature (350-500).
The axial T1 images help us to characterize the cysts.
The Axial T2-weighted images to determine better the contours of the kidneys and the cysts.
Cyst volumes were measured in Coronal T2-Weighted images by the use of a semi-automated contour tracing software (ViewForum R5.1V1L1 2006).
The volumes were calculated by multiplying all outlined areas by the section thickness and summing the volume of each section.
[Fig.1]
Fig.: Cyst Volumetric Calculation