Investigating Late-Onset Pompe Prevalence in Neuromuscular Medicine Academic Practices: The IPaNeMA Study.

Background And Objectives: We investigated the prevalence of late-onset Pompe disease (LOPD) in patients presenting to 13 academic, tertiary neuromuscular practices in the United States and Canada.

Methods: All successive patients presenting with proximal muscle weakness or isolated hyperCKemia and/or neck muscle weakness to these 13 centers were invited to participate in the study. Whole blood was tested for acid alpha-glucosidase (GAA) assay through the fluorometric method, and all cases with enzyme levels of ≤10 pmoL/punch/h were reflexed to molecular testing for mutations in the GAA gene.

Chemical activation of lipoplexes formed from DNA and a redox-active, ferrocene-containing cationic lipid.

We recently reported that the ferrocene-containing cationic lipid BFDMA [bis(11-ferrocenylundecyl)dimethylammonium bromide] can be used to mediate cell transfection, and that levels of transfection depend critically upon the oxidation state of the ferrocenyl groups of the lipid. Here, we report that the redox activity of BFDMA can be exploited to transform lipoplexes formed from oxidized BFDMA (which do not transfect cells) to lipoplexes that are "active" (and thus mediate high levels of transgene expression) by treatment with the chemical reducing agent glutathione (GSH). We demonstrate that GSH can be used to reduce the ferrocenium groups of oxidized BFDMA rapidly both (i) in solution and (ii) in lipoplexes formed by mixing oxidized BFDMA and DNA.

Diagnostic accuracy of an initial azoospermic reading compared with results of post-centrifugation semen analysis after vasectomy.

Purpose: Semen analysis is a common laboratory procedure but few data are available to support recommendations as to whether centrifugation is necessary in the post-vasectomy context. We evaluated the accuracy of a pre-centrifugation determination of azoospermia compared with post-centrifugation results.

Materials And Methods: We conducted a secondary analysis of data from 3,205 semen analyses performed during a randomized clinical trial of 2 vasectomy techniques--ligation and excision with fascial interposition vs ligation and excision without fascial interposition.

Characterization of the nanostructure of complexes formed by a redox-active cationic lipid and DNA.

We report characterization of the nanostructures of complexes formed between the redox-active lipid bis(n-ferrocenylundecyl)dimethylammonium bromide (BFDMA) and DNA using small-angle neutron scattering (SANS) and cryogenic transmission electron microscopy (cryo-TEM). A particular focus was directed to the influence of lipid oxidation state (where reduced BFDMA has a net charge of +1 and oxidized BFDMA has a charge of +3) on the nanostructures of the solution aggregates formed. Complexes were characterized over a range of charge ratios of reduced BFDMA to DNA (1.

Pharmacokinetic interactions between depot medroxyprogesterone acetate and combination antiretroviral therapy.

Objective: To evaluate the effect of an antiretroviral (ARV) therapy regimen containing zidovudine (AZT), lamivudine (3TC), and efavirenz (EFV) on the pharmacokinetics of depot medroxyprogesterone acetate (DMPA).

Design: Open-label, nonrandomized, clinical trial.

Setting: University hospital clinic.

Lipoplexes formed by DNA and ferrocenyl lipids: effect of lipid oxidation state on size, internal dynamics, and zeta-potential.

The effect of lipid oxidation state on the physical properties of complexes formed by plasmid DNA and the redox-active lipid bis-(11-ferrocenylundecyl)dimethylammonium bromide (BFDMA) is reported. With increasing concentration of BFDMA, the hydrodynamic sizes of complexes formed by BFDMA and DNA (in the presence of 1 mM Li(2)SO(4)) pass through a maximum and the zeta-potential changes monotonically from -40 mV to +40 mV. In contrast, complexes formed by oxidized BFDMA and DNA exhibit a minimum in size and maintain a negative zeta-potential with increasing concentration of BFDMA.

Reversible condensation of DNA using a redox-active surfactant.

We report characterization of aqueous solutions of dilute Lambda phage DNA containing the redox-active surfactant (11-ferrocenylundecyl)trimethylammonium bromide (FTMA) as a function of the oxidation state of the FTMA. FTMA undergoes a reversible one-electron oxidation from a reduced state that forms micelles in aqueous solution to an oxidized state (containing the ferrocenium cation) that does not self-associate in solution. This investigation sought to test the hypothesis that FTMA can be used to achieve reversible control over the conformation of DNA-surfactant complexes in solution.

Frequency and patterns of early recanalization after vasectomy.

Background: Our understanding of early post-vasectomy recanalization is limited to histopathological studies. The objective of this study was to estimate the frequency and to describe semen analysis patterns of early recanalization after vasectomy.

Methods: Charts displaying serial post-vasectomy semen analyses were created using the semen analysis results from 826 and 389 men participating in a randomized trial of fascial interposition (FI) and an observational study of cautery, respectively.

Bleeding patterns after use of levonorgestrel emergency contraceptive pills.

Objective: The objective of this study was to describe bleeding after use of an emergency contraceptive pill (ECP) regimen consisting of 1.5 mg of levonorgestrel in a single dose.

Methods: We asked 120 women who had been treated with the regimen to keep daily bleeding diaries for 9 weeks.

Ferrocene-containing cationic lipids for the delivery of DNA: oxidation state determines transfection activity.

The ability of two redox-active, ferrocene-containing cationic lipids [11-(ferrocenylundecyl)trimethylammonium bromide (FTMA) and bis(11-ferrocenylundecyl)dimethylammonium bromide (BFDMA)] to transfect mammalian cells was investigated. This study sought to determine the range of conditions over which these lipids were capable of transfecting cells and whether the oxidation state of the ferrocenyl groups in these materials could be used to influence the extent of transfection. Experiments conducted in the COS-7 cell line demonstrated that reduced and oxidized FTMA were substantially cytotoxic and did not transfect cells.

Electrochemical control of the interactions of polymers and redox-active surfactants.

We report the characterization of aqueous solutions (0.1 M Li(2)SO(4)) of dilute ethyl(hydroxyethyl) cellulose (EHEC) mixed with the redox-active surfactant (11-ferrocenylundecyl)trimethylammonium bromide (FTMA) by measurements of clouding temperatures and dynamic light scattering. The investigation sought to test the hypothesis that FTMA, which forms micelles in aqueous solution in its reduced state but not in its oxidized state, would permit reversible control over the formation of polymer-surfactant complexes in solution.

Ferrocene-containing cationic lipids: influence of redox state on cell transfection.

A ferrocene-containing, redox-active cationic lipid that can be transformed using electrochemical methods yields large differences in cell transfection depending on the oxidation state of the lipid. Expression of enhanced green fluorescent protein and firefly luciferase occurs at very high levels when DNA lipoplexes are formulated using the lipid in the reduced state. In contrast, transfection is negligible when oxidized lipid is used.

Vasectomy by ligation and excision, with or without fascial interposition: a randomized controlled trial [ISRCTN77781689].

Background: Randomized controlled trials comparing different vasectomy occlusion techniques are lacking. Thus, this multicenter randomized trial was conducted to compare the probability of the success of ligation and excision vasectomy with, versus without, fascial interposition (i.e.

Vasectomy effectiveness in Nepal: a retrospective study.

The main purpose of this retrospective, cross-sectional study was to evaluate the effectiveness of vasectomy in an ongoing public sector program in Nepal. We evaluated semen samples from men who had previously had a vasectomy, and asked about the occurrence of pregnancies in the men's partners. In addition, the surgeons who performed the vasectomies completed a questionnaire about their techniques.

Termination of a randomized controlled trial of two vasectomy techniques.

Family Health International (FHI) and EngenderHealth conducted a multicenter, randomized controlled trial to evaluate a fascial interposition (FI) component of a vas occlusion procedure for male sterilization. A data monitoring committee (DMC) was established to provide independent review of the interim report. The DMC met to review the interim report after enrollment of 552 men.