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31.
Alkaptonuria is a rare hereditary metabolic disease of autosomal recessive inheritance, resulting from deficiency of the enzyme homogentisic acid oxidase. The term “alkaptonuria” was first used in 1859 by Boedeker to describe a patient’s urinary reducing compound, and in 1866, Virchow coined the term “ochronosis” due to typical yellow pigmentation. Deposition of this pigment in articular cartilage leads to ochronotic arthropathy, the most incapacitating complication of alkaptonuria. We report a rare case of shoulder ochronotic arthritis, treated with total shoulder arthroplasty, achieving a successful long-term clinical and radiological outcomes.  相似文献   
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Objective. We were aware of a small number of cases in our EMS system where patients in respiratory distress had a worsening of their condition after being removed from the home on a Reeves® stretcher (RS). We sought to determine if this prehospital lifting device causes additional respiratory effort used in normal subjects by describing changes in heart rate, pulse oximetry, tidal volume, minute ventilation, andrespiratory rate. Methods. Forty-nine subjects were entered into this study. Data were collected while the subject was supine on the floor in the RS andonce while suspended over the floor in the device. A randomized crossover design was used. Ten subjects were excluded because of inadvertent omission of a nose plug during spirometry. Data points were recorded in the final minute of a 3-minute exposure. Three minutes was chosen to simulate a prehospital transport time from the scene to the ambulance. Minute ventilation, tidal volume, heart rate, pulse oximetry, andrespiratory rate were recorded for each subject during each phase. Subjects were also asked to rate the difficulty of breathing using the modified Borg scale. Results. Data were obtained for 39 subjects. The mean respiratory rate while suspended was 9.9 ± 3.0 breaths per minute compared to 9.1 ± 2.5 breaths per minutes supine on the floor (p = 0.007). The mean minute ventilation while suspended in a RS was 8.17 ± 3.25 L/min versus 7.37 ± 2.37 while lying flat (p = 0.03). There was no difference in tidal volume, heart rate, pulse oximetry, or subjective modified Borg scale ratings. Conclusions. Subjects suspended in a RS for 3 minutes had statistically higher respiratory rates andminute ventilation than the same subjects lying flat. Although these modest changes are clinically insignificant in normal subjects, they could present a significant challenge to subjects in respiratory distress.  相似文献   
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Purpose. To review the complications and recovery of patients with Achilles tendon rupture managed by percutaneous repair, open repair, and non-operative means in a tertiary referral centre between 2001 and 2003.

Methods. The operating theatre register and logbooks of the Consultants were used to identify surgically managed patients, while plaster room records were used for conservatively managed patients. We collated demographic and management details.

Results. The number of plaster changes (p < 0.001), median length of time in cast (p < 0.001), and number of outpatient visits (p < 0.05) was greater in conservatively managed patients. We performed no formal statistical analysis given the small numbers. Conservative management was the least expensive and open surgery the most costly modality of management. Percutaneous surgery was approximately a third of the cost of open surgery when performed under local anaesthetic.

Conclusion. In our setting, percutaneous repair and conservative management are viable alternatives to open surgery, which carries higher complications rates and was the most costly of the three. Individual patients will have different needs due to their age, occupation, or level of sporting activity.  相似文献   
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Retinal pigment epithelial detachment, often asymptomatic is a common retinal finding. Multiple serous retinal pigment epithelial detachment especially in a female is very uncommon. We describe a case of multiple retinal pigment epithelial detachments throughout the fundus in a 51-year old female otherwise asymptomatic. Indocyanine green angiography and fundus auto fluorescence showed abnormalities with a normal systemic work-up. This is the first report of an asymptomatic patient correlating angiogram with auto fluorescence in multiple serous retinal pigment epithelial detachments.  相似文献   
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Purpose: The purpose of the study is to report the prognostic factors and outcomes of vitrectomy (PPV) with silicone oil tamponade in rhegmatogenous retinal detachment (RRD) secondary to acute retinal necrosis (ARN).

Methods: This retrospective, non-randomized, interventional comparative study included 38 eyes of 38 patients. All cases underwent PPV with silicone oil tamponade. The main outcome measure was improvement of final visual acuity relative to the presenting visual acuity and factors affecting the same Group A included eyes with favorable vision of 20/400 or better and Group B included the others.

Results: Group A included 16 eyes (42.10%), group B included 22 eyes (57.89%). In Group A 2 eyes out of 16 (12.5%) and in Group B 12 eyes out of 22 (54.54%) had RRD at presentation (p = 0.02, 95% CI for the difference 7.88–65.78%). The time interval between first presentation and development of RRD in Group A was 30.94 ± 38.8 days (median 30 days) whereas that in Group B was 10.81 ± 11.73 days (median 8 days) (p = 0.02). The odds of visual improvement post-vitrectomy when RRD occurred later was 8.4 (p = 0.01, 95% CI 1.53–46.1). The usage of systemic steroids (odds 5.2, p = 0.03, 95% CI 1.14–23.54) and oral valacyclovir (odds 4.33, p = 0.04, 95% CI 1.05–17.84) were associated with odds favoring a good visual outcome. Recurrent RRD was noted in 3/16 eyes (18.75%) in Group A and 13/22 eyes (59.09%) in Group B (p = 0.03).

Conclusion: Delayed occurrence of RRD after ARN is a good prognostic factor. Usage of systemic steroids and oral valacylocvir are associated with a favorable visual outcome when started before the onset of RRD.  相似文献   

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