Pharmacological chaperone therapy by active-site-specific chaperones in Fabry disease: in vitro and preclinical studies.

PURPOSE: To investigate the long-term effects of transurethral bladder neck incision (TUBNI) for female primary bladder neck obstruction (PBNO).

MATERIALS AND

METHODS

We retrospectively reviewed seventy women diagnosed with bladder neck obstruction by video-urodynamic study (VUDS). TUBNI was performed for each patient, with incisions made at 2 different sites on the bladder neck. Postoperatively, patients were assessed by international prostate symptom score (IPSS), quality of life (QOL) and uroflowmetry.

RESULTS

Follow-up data were available for 4-108 months (median 42 months) postoperatively. During follow-up, the IPSS, QOL, time to maximum uroflow rate, postvoid residual urine volume decreased significantly after TUBNI compared with preoperative [13.0 (10.0, 15.0) versus 3.0 (3.0, 8.0), P ＜ .001], [5.0 (5.0, 5.0) versus 2.0 (1.0, 3.0), P ＜ .001], [9.0 (5.0, 37.0) versus 6.1 (4.2, 8.7), P ＜ .001], [77.5 (23.5, 165.8) versus 0.0 (0.0, 30.0), P ＜ .001]. The maximum uroflow rate, average uroflow rate and the voided volume increased significantly compared with preoperative [7.0 (4.0, 10.3) versus 19.8 (12.8, 25.2), P ＜ .001], [3.0 (2.0, 5.0) versus 8.0 (4.9, 10.7), P ＜ .001] and [156.5 (85.0, 211.3) versus 261.3 (166.2, 345.6), P ＜ .001]. Several complications were identified after surgery, including bladder neck reobstruction, urethral stricture, and stress urinary incontinence, the corresponding number was 5 (7.1%), 7(10%) and 7(10%). Successful operation was achieved in 60/70 (85.7 %) patients.

CONCLUSION

PBNO is a very rare yet easily treatable condition. VUDS is the primary diagnostic tool for the diagnosis of bladder neck obstruction in women, while TUBNI can effectively relieve obstruction symptoms and improve the quality of life for patients.