Glypicans are a family of heparan sulfate proteoglycans that are linked to the cell surface through a glycosyl–phosphatidylinositol anchor. One member of this family, glypican-3 (Gpc3), is mutated in patients with the Simpson-Golabi-Behmel syndrome (SGBS). These patients display pre- and postnatal overgrowth, and a varying range of dysmorphisms. The clinical features of SGBS are very similar to the more extensively studied Beckwith-Wiedemann syndrome (BWS). Since BWS has been associated with biallelic expression of insulin-like growth factor II (IGF-II), it has been proposed that GPC3 is a negative regulator of IGF-II. However, there is still no biochemical evidence indicating that GPC3 plays such a role.Here, we report that GPC3-deficient mice exhibit several of the clinical features observed in SGBS patients, including developmental overgrowth, perinatal death, cystic and dyplastic kidneys, and abnormal lung development. A proportion of the mutant mice also display mandibular hypoplasia and an imperforate vagina. In the particular case of the kidney, we demonstrate that there is an early and persistent developmental abnormality of the ureteric bud/collecting system due to increased proliferation of cells in this tissue element.The degree of developmental overgrowth of the GPC3-deficient mice is similar to that of mice deficient in IGF receptor type 2 (IGF2R), a well characterized negative regulator of IGF-II. Unlike the IGF2R-deficient mice, however, the levels of IGF-II in GPC3 knockouts are similar to those of the normal littermates.

Despite similar access difficulty between groups, access related complications were less and stone-free rates were improved during urologist acquired percutaneous access. Urologists instructed in percutaneous access may be able to provide improved stone-free rates during percutaneous nephrolithotomy while minimizing access related complications.

LSP offers advantages over OSP in terms of shorter catheter time, shorter hospital length of stay, and fewer urinary tract infections.

Purpose: To evaluate the long-term efficacy of prostate cancer control and complication rates, in the elderly, after focal therapy with high-intensity focused ultrasound (HIFU). Materials and Methods: Between June 1997 and March 2000, patients with localized prostate cancer were included into a focal therapy protocol. Inclusion criteria were: PSA ≤ 10 ng/mL, ≤ 3 positive biopsies with only 1 lobe involved, clinical stage ≤ T2a, Gleason score ≤ 7 (3+4), negative CT scan and bone scan. Hemi-ablation of the prostate was performed with the Ablatherm (R) device. Survival, complication rates and urinary continence were evaluated. Control biopsies were performed at 1 year. Treatment failure was defined as a positive biopsy or need for salvage therapy. Results: Twelve patients with a mean age 70 years were included. Median follow-up was 10 years. Control prostate biopsies were negative in 11/12 (91%) patients. Overall survival was 83% (10/12) and cancer specific survival was 100% at 10 years. Two patients died from other causes. Recurrence free survival was 90% (95% CI; 0.71-1) at 5 years, and 38% (95% CI; 0.04-0.73) at 10 years. Five patients had salvage therapy with repeat HIFU (n = 1) or hormonal therapy (n = 4) and all salvage patients were alive at 10 years. No patients developed lymph node or bone metastasis. No patients suffered from urinary incontinence. International Prostate Symptom Score was stable at 1 year. Complications included two urinary tract infections and one episode of acute urinary retention. Conclusions: Hemi-prostate ablation with HIFU can be safely performed in selected elderly patients with adequate longterm cancer control and low complication rates. Results from larger prospective studies using improved imaging techniques and extensive biopsy protocols are awaited.

We report oncological data on the largest prospective cohort of patients, with the longest followup, treated by prostate sparing cystectomy to date. Outcomes are comparable with the largest published series of cystoprosatectomies. With appropriate screening the risk of a clinically significant prostate cancer appears to be low. For certain selected patients this technique represents a valuable additional option for treatment.

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