Urodynamic and clinical studies in patients with late-onset Pompe disease and lower urinary tract symptoms.

AIMS: In late-onset Pompe disease (LOPD), a lysosomal storage disorder with glycogen accumulation in several tissues, patients suffer from progressive skeletal muscle weakness. Lower urinary tract symptoms (LUTS) have rarely been reported. The aim of this study is to objectively assess LUTS in patients with LOPD for the first time using urodynamic studies and to determine differences between LOPD patients with and without LUTS. METHODS: Eighteen patients with LOPD were recruited, of whom seven patients (38.9%) reported LUTS (both voiding and storage symptoms). Six of these patients underwent urodynamic studies. Medical histories and motor function tests were compared between the 7 patients with LUTS and the 11 patients without LUTS. The Student t test was used to determine an association between the two cohorts. RESULTS: In the seven LOPD patients with LUTS urodynamics revealed neurogenic dysfunction, underactive detrusor, and bladder outlet obstruction. These patients had suffered from clinical symptoms for a longer period of time before starting enzyme replacement therapy (P = .017) than patients without LUTS. They also scored more poorly on muscle function tests. Urodynamic results point to neurogenic causes for LUTS in LOPD, that is, neurogenic reflex bladder or impaired filling sensation. This could be due to glycogen accumulation in the urothelium and central nervous system. Patients with LUTS also seem to be more severely affected by LOPD than patients without LUTS. CONCLUSION: LUTS in LOPD requires early and specific treatment to limit the development of severe health problems. Urodynamic studies should be considered in assessing LUTS.

Hydrogel-Based Matrix-Associated Autologous Chondrocyte Implantation Shows Greater Substantial Clinical Benefit at 24 Months Follow-Up than Microfracture: A Propensity Score Matched-Pair Analysis.

OBJECTIVE: To compare substantial clinical benefit (SCB) of a hydrogel-based, matrix-associated autologous chondrocyte implantation (M-ACI) method versus microfracture (MFx) in the treatment of knee cartilage defects. DESIGN: Propensity score matched-pair analysis, using the MFx control group of a phase III study as comparator for M-ACI treatment in a single-arm phase III study, resulting in 144 patients in the matched-pair set. RESULTS: Groups were comparable regarding baseline Knee Injury and Osteoarthritis Outcome Score (KOOS), sex, age, body mass index, symptom duration, smoking status, and previous knee surgeries. Defect sizes in the M-ACI group were significantly larger than in the MFx group (6.4 cm2 vs. 3.7 cm2). Other differences concerned location, number, and etiology of defects that were not considered to influence the interpretation of results. At 24 months, significantly more patients in the M-ACI group achieved SCB in KOOS pain (72.2% vs. 48.6%; P = 0.0108), symptoms (84.7% vs. 61.1%, P = 0.0039), sports/recreation (84.7% vs. 56.9%, P = 0.0008), and quality of life (QoL; 72.2% vs. 44.4%, P = 0.0014). The SCBs for KOOS activities in daily living and International Knee Documentation Committee score were higher for M-ACI but not significantly different from MFx. The SCB rates consistently favored M-ACI from 3 months onward. The highest improvements from baseline at 24 months in patients with SCB were observed for KOOS sports/rec. (M-ACI: 60.8 points, MFx: 55.9 points) and QoL (M-ACI: 58.1, MFx: 57.4). CONCLUSION: Hydrogel-based M-ACI demonstrated superior SCB in KOOS pain, symptoms, sports/rec., and QoL compared with MFx in patients with knee cartilage defects through 2 years follow-up.

[Female stress incontinence: aspects of pathogenesis and functional anatomy]. / Pathogenese, funktionelle und anatomische Aspekte der weiblichen Belastungsinkontinenz.

This article focuses on female stress incontinence in the form of pelvic floor dysfunction and urethral sphincter deficiency, although isolated stress incontinence accounts for less than half of all incontinence cases. Especially in women of old age and those with neurological comorbidities, the causes of incontinence are mostly multifactorial. Also it has to be considered that the female bladder, urethra, uterus and rectum as well as the muscular and ligamentous structures of the female pelvis minor are affected by phases of fertility, possible pregnancies, births and menopause in addition to the normal ageing process.

Input-dependent life-cycle inventory model of industrial wastewater-treatment processes in the chemical sector.

Industrial wastewater-treatment systems need to ensure a high level of protection for the environment as a whole. Life-cycle assessment (LCA) comprehensively evaluates the environmental impacts of complex treatment systems, taking into account impacts from auxiliaries and energy consumption as well as emissions. However, the application of LCA is limited by a scarcity of wastewater-specific life-cycle inventory (LCI) data. This study presents a modular gate-to-gate inventory model for industrial wastewater purification in the chemical and related sectors. It enables the calculation of inventory parameters as a function of the wastewater composition and the technologies applied. Forthis purpose, data on energy and auxiliaries' consumption, wastewater composition, and process parameters was collected from chemical industry. On this basis, causal relationships between wastewater input, emissions, and technical inputs were identified. These causal relationships were translated into a generic inventory model. Generic and site-specific data ranges for LCI parameters are provided for the following processes: mechanical-biological treatment, high-pressure wet-air oxidation, nanofiltration, and extraction. The input- and technology-dependent process inventories help to bridge data gaps where primary data are not available. Thus, they substantially help to perform an environmental assessment of industrial wastewater purification in the chemical and associated industries, which may be used, for instance, for technology choices.

Organic pollutant removal versus toxicity reduction in industrial wastewater treatment: the example of wastewater from fluorescent whitening agent production.

Industrial wastewater treatment in the chemical industry aims at eliminating organic contaminants, as these pollutants may be persistent and ecotoxic. In a case study performed in collaboration with the chemical industry, we investigated the removal of a fluorescent whitening agent and its side products in the wastewater-treatment system. Adsorption to activated carbon and biological treatment were simulated in laboratory tests. Algae toxicity tests were performed to quantify the toxicity of the wastewater mixture and of single components. The contaminants identified accounted for up to 82% of the wastewater's total organic carbon (TOC). Adsorption to activated carbon eliminated the TOC and the single contaminants only slightly. Nevertheless, the toxicity of the wastewater decreased by 40%. In contrast, biological treatment reduced the TOC by up to 80%, and the whole effluent toxicity increased. These results indicate that new ecotoxic metabolites were formed during the biological treatment. They also illustrate that mere reduction of the TOC in the wastewater-treatment system is not sufficient for ensuring a reduction of environmental impact. Therefore, simultaneously conducting TOC measurements and toxicity tests, as demonstrated in the current work, is recommended.