Prevalence of cancer among 867 patients with Gaucher disease from the SZMC Gaucher unit

Molecular Genetics and Metabolism(2023)

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摘要
There are limited data in the literature that describe the management of Peyronie's disease (PD) with severe compound curvature, which often requires additional straightening procedures after plaque excision and grafting (PEG) to achieve functional penile straightening (<20 degrees).This study highlights the clinical distinction and our experience with men with PD and severe compound curvature treated with PEG and supplemental tunica albuginea plication (TAP).We performed a retrospective chart review of patients with PD and acute angulation who underwent PEG (group 1) and patients with compound curvature who underwent PEG with TAP (group 2) between 2007 and 2016.Primary post-operative outcomes of interest include change in penile curvature, change in measured stretched penile length, and subjective report on penile sensation and sexually induced penile rigidity.240 Men with PD were included in the study, of which 79 (33%) patients in group 1 underwent PEG and 161 (67%) in group 2 underwent PEG and TAP. There was no difference in associated PD co-morbidities including age, hypertension, hyperlipidemia, hypogonadism, diabetes, or tobacco use. After artificial induction of erection with intracorporal trimix injection, the average primary curvature was 73 (range, 20–120) degrees for group 1 compared to 79 (range, 35–140) degrees for group 2 (P = .01). Group 2 had an average secondary curvature of 36 (20–80 degrees). After completion of PEG, men in group 2 had an average residual curvature of 30 (range, 20–50) degrees which required 1–6 TAPs to achieve functional straightness (<20 degrees). At an average follow-up of 61 months, there was no difference for group 1 and group 2, respectively, for recurrent curvature (11.4% vs 12.4%, P = .33), change in penile length (+0.57 vs +0.36 cm, P = .27) or decreased penile sensation (6% vs 13%, P = .12). In all, 81% of group 1 and 79% of group 2 were able to engage in penetrative sex after penile straightening with or without pharmacotherapy (P = .73).Our review shows promising surgical outcomes for the use of PEG and supplemental TAP for this subtype of complex PD.This article reports the largest experience with treatment of PD with compound curvature to date. Limitations of this study include the retrospective nature of the analysis as well as the lack of a validated objective measurement of erectile function after penile straightening.Our study found no baseline difference in underlying co-morbidities in men with severe compound curvature compared with men with acute severe angulated curvature. Men with severe compound curvature represent a severe and under-recognized population of men with PD who can be surgically corrected with PEG and supplemental TAP(s) when needed without an increased risk of loss of penile length, recurrent curvature, decreased penile sensation, or erectile dysfunction when compared to men treated with PEG alone.Chow AK, Sidelsky SA, Levine LA. Surgical Outcomes of Plaque Excision and Grafting and Supplemental Tunica Albuginea Plication for Treatment of Peyronie's Disease With Severe Compound Curvature. J Sex Med 2018;15:1021–1029.
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关键词
gaucher disease,cancer,prevalence
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