Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) is a rare metabolic disorder that can lead to acute encephalopathy, liver disease, cardiomyopathy, rhabdomyolysis, and long-term complications involving the eye and peripheral nerves. LCHADD is a peroxisome biogenesis disorder (PBD). Except for the series presented by Tyni and colleagues (Ophthalmology 1998;105:810-824), which described visually insignificant lens opacities in association with LCHADD, previous ophthalmic papers have only reported retinal complications. We report on one case with progressive asymmetrical cataract. The more mildly affected eye had a similar morphology to that previously reported and the more severely affected eye had an unusual morphology we believe is unique to LCHADD. We discuss the range of ophthalmic presentations in our cases and in the literature. The variability of the severity of ocular complications, even between eyes in one individual, makes it difficult to test the effectiveness of therapeutic options upon the ophthalmic complications.
Introduction: Urethral stricture description is not standardized. This makes surgical decision-making less reproducible and increases the difficulty of objectively analyzing urethroplasty literature. Wiegand and Brandes 2 developed the UREThRAL stricture score to codify anterior strictures. The objectives of this study were to evaluate urethral stricture score in management of anterior urethral strictures.
Material and methods:This was a prospective observation study, which included all patients operated for stricture urethra between March 2015 to march 2017 after applying exclusion criteria. The UREThRAL STRICTURE SCORE for each patient was calculated. The obtained USS was related to the surgical procedure, intraoperative complexity, complications that patient underwent for any significant correlation.
Results:A total of 94 patients were included in study. A total of 26 (27.66%) patients underwent VIU while 26 (27.66%) and 42 (44.68%) of patients underwent anastomotic urethroplasty and BMG urethroplasty respectively with a overall success rate of 76.60%. Mean UREThRAL Stricture Score of 94 patients was 9.617 with a range of 5 -21.
Conclusion:This study confirms the validity of UREThRAL stricture score in predicting surgical complexity for anterior urethral strictures with higher USS score patient requiring more complex surgery.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.