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Background

Musculoskeletal involvement in Morquio A syndrome (mucopolysaccharidosis IVA; MPS IVA) contributes significantly to morbidity and mortality. While the spinal manifestations of the disorder have received considerable attention in the literature, there have been few reported studies to date to guide the management of the orthopedic problems associated with the lower and upper extremities.

Purpose

The objective was to develop recommendations for the management of the extremities in patients with Morquio A syndrome.

Methods

A group of specialists in orthopedics, pediatrics and genetics with experience in the management of Morquio A patients convened to review and discuss current clinical practices and to develop preliminary recommendations. Evidence from the literature was retrieved. Recommendations were further refined until consensus was reached.

Results and conclusions

This present article provides a detailed review and discussion of the lower and upper extremity deformities in Morquio A syndrome and presents recommendations for the assessment and treatment of these complications. Key issues, including the importance of early diagnosis and the implications of medical therapy, are also addressed. The recommendations herein represent an attempt to develop a uniform and practical approach to managing patients with Morquio A syndrome and improving their outcomes.  相似文献   
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Unique performance characteristics of Novafil   总被引:1,自引:0,他引:1  
Polybutester is a unique copolymer that can be extruded as a flexible monofilament nonabsorbable suture. The mechanical performance of this new suture material was compared with that of polypropylene and nylon. The results indicate that polybutester sutures are as strong, have the same degree of total elongation at break and knot as the other monofilament sutures. In contrast with polypropylene and nylon sutures, the polybutester sutures have a perceptible stretch, are more elastic and flexible, and exhibit less creep. Polybutester sutures appear to be an acceptable alternative to polypropylene and nylon sutures and their unique mechanical properties may even prove to be superior in vivo.  相似文献   
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The soil receives enormous quantities of pesticides and PCBs as a waste product each year due to their wide application. The degradation and eventual fate of these chemicals in the soil largely determine ultimate environmental impact. A survey of an abandoned landfill site has been conducted in a fast developing industrial city Nagpur (India) to assess the degree of environmental contamination with PCBs. The purpose was to determine if any of the mixture of PCBs, Aroclor 1254 and Aroclor 1260 are retained in the compost amended soil of the site despite atmospheric losses possible due the peak summer (100-117 degrees F) in the city. The accuracy and precision for Aroclor 1254 and Aroclor 1260 determinations in the soil samples have been calculated as 76.5 and +/- 0.92 and 83.3 and +/- 1.13 respectively. The samples analysis showed the presence of Aroclor 1254 and Aroclor 1254 and Aroclor 1260 up to the levels of 0.55 and 0.80 mg/g respectively. The maximum total PCBs on dry weight basis are found as 1.2 mg/g.  相似文献   
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This report describes an innovative suture and needle clinical evaluation program jointly designed by hospital representatives of Consorta, Inc., a healthcare resource management and group purchasing organization, and United States Surgical/Davis & Geck Sutures (USS/D&G), manufacturer of surgical biomaterials. Nineteen Consorta shareholder hospitals enrolled 699 surgeons to participate in Phase I of this nonexperimental observational study of the clinical performance of surgical needles and sutures. Performance characteristics of the sutures and needles produced by USS/D&G, which were evaluated in 3407 surgical procedures, included packaging and ease of opening, needle strength and sharpness, tissue drag, knot security, tensile strength, and clinically acceptable and unacceptable determinations. In these 30-day studies, the surgeons concluded that the needles and sutures were clinically acceptable in 98.1% of the evaluations. The general, cardiothoracic, and orthopedic surgeons, who performed 73.8% of the product evaluations, reported that the suture and needle products were clinically acceptable in 97.2% of the evaluations. More than half (50.1%) of the evaluations involved the POLYSORB* braided synthetic sutures,which received a clinically acceptable rating in 98.4% of the evaluation. The next most frequently used sutures were the SOFSILK*, followed by the monofilament nylon suture. SOFSILK* was found to be clinically acceptable in 98.7% of the evaluations, whereas the monofilament nylon was noted to be clinically acceptable in 96.3% of the evaluations. Surgical needles made by USS/D&G had a 97.9% clinical acceptability rating.  相似文献   
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