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1.
Several mutations involving the fibroblast growth factor receptor (FGFR) gene family have been identified in association with phenotypically distinct forms of craniosynostosis. One such point mutation, resulting in the substitution of proline by arginine in a critical region of the linker region between the first and second immunoglobulin-like domains, is associated with highly specific phenotypic consequences in that mutation at this point in FGFR1 results in Pfeiffer syndrome and analogous mutation in FGFR2 results in Apert syndrome. We now show that a much more variable clinical presentation accompanies analogous mutation in the FGFR3 gene. Specifically, mental retardation, apparently unrelated to the management of the craniosynostosis, appears to be a variable clinical consequence of this FGFR3 mutation.  相似文献   

2.
AIMS/BACKGROUND: Recurrent peripheral retinal detachments may occur in eyes treated with vitrectomy and silicone oil for retinal detachments complicated by proliferative vitreoretinopathy (PVR). The aim of this study was to assess whether laser photocoagulation could be used in the presence of silicone oil to confine and stabilise recurrent PVR related peripheral retinal detachments enabling the timely removal of the oil. METHODS: 10 patients with recurrent peripheral retinal detachments after vitrectomy and silicone oil insertion were treated with posturing and subsequent focal argon laser to circumscribe the area of recurrent detachment. RESULTS: This technique alone was sufficient to limit the area of retinal detachment in seven of the cases. The remaining three cases required relieving retinotomies because of increasing retinal detachment despite the laser. In all 10 cases the silicone oil was later removed without progression of the detached areas. CONCLUSION: Silicone assisted argon laser 'confinement' can be effective in stabilising eyes with peripheral retinal detachments allowing the subsequent removal of silicone oil.  相似文献   

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BACKGROUND: Our purpose was to analyze the causes of persistent and recurrent sporadic primary hyperparathyroidism (PD and RD). METHODS: The histopathology, complications, and results of reoperation were studied. Five hundred sixty-eight patients with primary hyperparathyroidism were operated on initially by one surgeon and underwent follow-up examination for 3.7 +/- 3.8 years. During the operation, all parathyroids were sought and confirmed by biopsy. Enlarged glands were resected, and subtotal parathyroidectomy was done for multiglandular disease (hyperplasia). RESULTS: The cure rate after the initial surgical procedure was 96.4%, PD = 2.8% (16 of 568). At reoperation (10 of 16), nine of 10 were cured (90%) (two adenomas, six hyperplasias, one lung carcinoma). RD was documented (at years 4, 4, 10, 15, 16) in five (0.9%) patients, one with parathyroid carcinoma and four with hyperplasia. Thirty-five patients with PD and two patients with RD were referred for reoperation: 17 with adenomas (eight mediastinal) and 18 with hyperplasias (one mediastinal gland). Preoperative calcium level was higher for PD (12.57 mg/dl) and RD (13.89 mg/dl) versus all cases (12.19 mg/dl) (p < 0.03 and p < 0.0005, respectively). After reoperation, normocalcemia was achieved in 47 (92.%) of 51 patients with PD or RD. Transient hypocalcemia occurred in 22% of patients (permanent, 2.0%) and transient hoarseness in 2.0% of patients (no permanent nerve damage). Permanent hypocalcemia and nerve damage after 568 initial operations were 0% and 0%, respectively. Two perioperative deaths occurred. CONCLUSIONS: We conclude that inadequate neck exploration or resection of hyperplastic tissue accounts for most cases of PD and RD. Optimal results necessitate intraoperative identification of all parathyroids whenever possible, with minimal morbidity.  相似文献   

5.
G Woodman  MA Croce  TC Fabian 《Canadian Metallurgical Quarterly》1998,64(9):833-6; discussion 836-7
Risk factors for lower extremity ischemic complications (ICs) following iliac arterial injuries have not been addressed. Patients with penetrating iliac artery injuries over a 15-year period were reviewed. IC was defined as compartment syndrome with or without tissue loss. Patients with iliac artery repair who developed ICs were compared with those without ICs (excluding early deaths from hemorrhagic shock). Comparison included demographics, severity of shock, physical examination, and operative findings. There were 94 arterial injuries in 80 patients (34 common iliac, 23 internal iliac, and 37 external iliac). There were 26 deaths (33%), and 3 patients were excluded for technical reasons. Of the 51 who underwent arterial reconstruction, 34 had no ischemia, whereas 17 (33%) had ICs (9 with tissue loss and 8 with compartment syndrome only). Immediate fasciotomies were performed in 6 of the IC patients due to the early recognition of compartment syndrome; 1 required amputation from the profound ischemia. Delayed recognition of compartment syndrome in the remaining 11 IC patients resulted in eight amputations (P < 0.05). We conclude that ICs following iliac arterial injuries significantly correlate with shock as indicated by systemic pH, lactate and transfusion requirements, and a preoperative pulseless extremity. In these patients, close monitoring of compartment pressures is necessary, and immediate fasciotomies should be strongly considered.  相似文献   

6.
Seventy-five patients underwent a 90 percent distal gastric bypass for morbid obesity. The average weight was 121.4 kilogram, height 164 centimeters, and age 31.4 years in these 70 women and five men. There was a total of 20 surgical complications in the 75 patients, with wound infection being the most common. Sixteen chronic complications were noted and consisted of vomiting, diarrhea, reflux esophagitis, dysphagia, and vitamin deficiencies. None of the above complications were life-threatening or required dismantling of the bypass. Of 54 patients followed for 12 months or more after gastric bypass, there was a 24.5 percent average weight decrease at 6 months, and this progressed to 35.8 percent by 12 months. Fifty-two patients undergoing small bowel bypass previously at the same institution had a 25.4 percent weight reduction at 12 months. Of 54 patients, 83 percent followed for one or more years after gastric bypass have had an excellent or good clinical result, whereas only 42 percent of the 52 patients undergoing small bowel bypass have had an excellent or good clinical result with the same criteria. It is concluded that the Mason 90 percent distal gastric bypass is a suitable form of surgical treatment for the morbidity obese patient who cannot lose weight by dietary measures.  相似文献   

7.
We performed combined vitrectomy, lens removal and posterior chamber intraocular lens implantation for proliferative diabetic retinopathy in 120 eyes of 101 patients. Follow-up periods ranged from 3 to 63 months, with a mean of 17 months. Three lens removal methods were used: extracapsular cataract extraction (14 eyes), phacoemulsification and aspiration (49 eyes), and pars plana phacoemulsification (57 eyes). Preoperative rubeosis iridis or neovascular glaucoma was found in 21 eyes. Gas or temporary silicone oil tamponade was employed in 32 eyes. Surgical results were good, and the postoperative vision was finger counts or below only in 13 eyes. Thus the combined surgery proved to have no serious problems. Our results indicate two important points. (1) It is best to chose either of the following two methods for the lens surgery: phacoemulsification with continuous circular capsulorhexis, self sealing sclerocorneal incision, and in-the-bag fixation of the posterior chamber lens, or pars plana phacoemulsification leaving the anterior capsule, rub off and aspirating the lens epithelial cells, continuous circular capsulorhexis, and posterior chamber lens implantation in front of the anterior capsule from a self-sealing sclerocorneal wound. (2) It is mandatory to do complete vitrectomy and cut out the vitreous gels incarcerated in the sclerotomy site.  相似文献   

8.
OBJECTIVE: The authors performed a study to determine the effectiveness and safety of silicone oil as a substitute for gas to fill the vitreous cavity to treat macular holes. DESIGN: Multicenter, nonrandomized, interventional trial. PARTICIPANTS: Thirty-seven consecutive patients chose vitrectomy with silicone tamponade instead of gas to treat 40 eyes with stage-2 to stage-4 idiopathic age-related macular holes. Stage-2 holes constituted 40% of the holes, and stage-3 and stage-4 holes made up 60%. INTERVENTION: All eyes were treated with vitrectomy, manual detachment of the posterior vitreous face (not done for stage-4 holes), autologous serum instillation, and silicone fill of the vitreous cavity. After insertion of the oil, the patients resumed normal activity with no restriction of head or eye position except to avoid faceup position. The oil was removed after approximately 6 weeks. MAIN OUTCOME MEASURES: The authors considered the seal of the macular hole and the preoperative and postoperative logarithm of the minimum angle of resolution (logMAR) visions the most significant measures for comparison to other studies. RESULTS: Eighty percent of all holes and 86% of holes not treated previously were sealed with a single silicone tamponade of the vitreous cavity. The logMAR value of visual acuity improved an average of 0.26 (2.6 lines) to 0.61 (20/81) for all eyes and 0.34 (3.4 lines) to 0.52 (20/66) when the macular hole sealed. Completeness of fill of the vitreous cavity with silicone affected seal of the macular hole. Three of eight eyes in which open holes developed after oil removal had less than 90% fill of the vitreous cavity by silicone. Sixty-nine percent of lenses increased opacity one grade or were removed after silicone tamponade. There were no significant adverse effects arising from silicone tamponade. CONCLUSIONS: Silicone oil tamponade of macular holes is effective and safe. Silicone may be optimal for the treatment of macular holes in persons who must travel, who cannot maintain facedown positioning, or who have monocular vision. The most important factor in the successful closure of the macular hole was the completeness of fill of the vitreous cavity with silicone oil.  相似文献   

9.
Experience with the GUEPAR prosthesis in 292 cases of which 103 have been followed for more than 2 years, suggests that: implanting a hinge prosthesis is major surgery on elderly patients in whom severe complications have occurred and for this reason, the operations should be reserved for extremely damaged and unstable knees; the most important local complications have been deep sepsis for which we have noted a rate of 6.6 per cent; in the treatment of sepsis, everything must be done to preserve the prosthesis because arthrodesis is difficult to obtain; pain relief has been significant as a result of the operation. The prosthetic design allows flexion of more than 90 degrees in 85 per cent of the cases and 120 degrees in 26 per cent; after two years, the results seem relatively stable. We have not observed aseptic loosening after this period but a longer observation period is necessary to be reassured on this point; patellar pain remains a major concern because this arthroplasty has not solved the problem, and other solutions will have to be found.  相似文献   

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No quantitative, simple and non-radioactive method has been described for measuring the platelet content of experimental thrombi. The aim of the present study was to develop a simple method for quantifying platelets in thrombi formed on thrombogenic surfaces in flowing native human blood. To test the relevance of this new method, the effect of unfractionated heparin on arterial thrombus formation was investigated. Tissue factor (TF)- and collagen-coated coverslips were exposed to non-anticoagulated blood at an arterial wall shear rate (2,600 s(-1)) for 1 to 4 min. Platelet deposition was quantified by measuring the P-selectin (PS) and beta-thromboglobulin (betaTG) content of dissolved plasmin-digested thrombi using immunoenzymoassays; fibrin deposition was determined by measuring the D-dimer levels. These results were compared to those established by morphometrical analysis. Morphometric evaluation showed that fibrin deposition was maximum on TF by 1 min perfusion time. Platelets deposited subsequently and reached a maximum at 3 min. On collagen, platelets deposited directly on the collagen fibrils without detectable fibrin deposit. Platelet deposition increased from 1 to 4 min. Platelet deposition quantified by PS was correlated to the values obtained by morphometry (r = 0.72, r = 0.67, p <0.001, on TF and collagen, respectively). As compared to PS, betaTG measurements gave an underestimation of the size of the thrombus platelet number. Unfractionated heparin infused through a mixing device proximal to the perfusion chamber to obtain plasma concentrations of 0.5, 1 and 3 IU/ml, reduced fibrin deposition on TF-coated coverslips in a dose-dependent manner (77% reduction at 3 IU/ml, p <0.01), but had no significant effect on platelet deposition (33% at 3 IU/ml, p >0.05). In contrast, heparin had no effect on fibrin or platelet deposition on collagen-coated coverslips. Thus, a new quantitative and simple method for measuring platelet deposition in flowing blood has been developed and characterized. Utilizing this system, we have demonstrated that unfractionated heparin did not inhibit arterial thrombus formation either on procoagulant or on proaggregant surface.  相似文献   

12.
Psychodynamic clinical studies as well as empirical research have pointed out the importance and the specificity of object relations in eating disorders. Given this, the aim of this work was to identify the presence of significant differences in the object relations patterns, as described by the Parental Bonding Instrument (PBI), between anorectic and bulimic subjects. PBI was administered to 42 anorectic female and 26 bulimic female subjects, consecutively admitted to the Eating Disorders Outpatient Unit of H San Raffaele of Milan. Profile analysis revealed the presence of significant (T2 = 11.3453, d.f. 3,64, p = 0.017) qualitative differences in PBI profiles between anorectic and bulimic subjects. The most striking difference was represented by the bulimics' view of their parents as both caring and overwhelming, which contrasted with the anorectics' perception of their parents as absolutely caring.  相似文献   

13.
It has recently been suggested that patients with mania are often misdiagnosed as having schizophrenia. The authors report a favorable clinical response to lithium carbonate in a father and son with an apparent schizo-affective syndromes may respond favorably to lithium but caution that a favorable response in such cases does not absolutely confirm a diagnosis of mania.  相似文献   

14.
Over 2 months in 1995, 235 assault patients attended the accident and emergency department of the Royal Alexandra Hospital, Paisley (2.4% of total new attendances). 80% were male and their mean age was 28 years (range 6-64); men were the assailants in over 90% of attacks. Alcohol had been consumed by 69% of the victims and 9% admitted to taking illicit drugs. The commonest place of assault was the street (44%) but women were more likely to be assaulted in their homes. Penetrating weapons were used in 23% of assaults. 60% of all injuries were to the head and neck. 27% of the victims were admitted to hospital. Paisley has an assault rate similar to that of other UK centres but the use of penetrating weapons is much higher than elsewhere.  相似文献   

15.
Several series have documented the ability of the carbon dioxide laser to smooth facial rhytids; however, follow-up has been limited to several months. Since 1995, more than 600 full or partial facial resurfacings were performed with the pulsed CO2 laser. To assess the long-term efficacy and safety of this procedure, the results of 211 resurfacings were retrospectively reviewed using a custom-designed database. Variables that were input included patient demographics, Fitzpatrick skin type, smoking history, prior and concurrent facial procedures, laser pass data, and postoperative complications. Short and long-term aesthetic results were graded by a blinded panel of plastic surgery reviewers (none of whom performed the laser resurfacing) using a standardized photographic rhytid scale. For each facial region, this scale consisted of eight high-resolution photographs depicting increasingly severe wrinkling. Facial rhytids were almost completely ablated at the 3 and 6 month follow-up. Some relapse was seen at 1 year, but the overall aesthetic result remained very good. Regions with dynamic rhytids (e.g., the perioral region) showed more recurrence. The best and most durable results were seen in the cheeks. Infection and scleral show each occurred in 13 patients (6 percent). Forty-five patients (21 percent) developed postprocedure hyperpigmentation, but the overwhelming majority of this group were treated before our postoperative antipigment regimen. Hypopigmentation was noted in 17 patients (8 percent) in this early follow-up group. Two patients (1 percent) developed postoperative scarring. It is hoped that these data will serve to provide additional information on the long-term results of laserbrasion.  相似文献   

16.
OBJECTIVE: Cataracts are a frequent complication after silicone oil infusion for the repair of complicated retinal detachments, occurring in up to 100% of eyes retaining silicone oil for 6 months or more. The authors devised a combined procedure for cataract and silicone oil removal with intraocular lens (IOL) implantation through a single corneal incision and evaluated their results. DESIGN: A prospective, noncomparative case series. PARTICIPANTS: Thirty-four eyes of 34 consecutive patients with a history of retinal detachment repair requiring silicone oil placement in whom a clinically significant cataract subsequently developed were identified when removal of silicone oil was scheduled. INTERVENTION: All 34 eyes were prospectively entered into a study to evaluate the efficacy and potential complications of a combined procedure for cataract and silicone oil removal with posterior chamber lens implantation. All patients underwent uncomplicated phacoemulsification removal of cataract followed by removal of silicone oil and placement of an IOL through a single corneal incision. MAIN OUTCOME MEASURE: Recurrent retinal detachment and IOL-related complications were measured. RESULTS: Ten eyes had recurrent retinal detachments develop. Final visual acuity ranged from 6/12 to hand movements with 25 eyes (74%) showing stabilized or improved vision. Pre-existing macular pathology and recurrent retinal detachment generally were responsible for poor visual outcome. CONCLUSIONS: Combined phacoemulsification, IOL implant with silicone oil removal is a useful procedure in these complicated eyes. Visual outcome generally is good with improvement in visual acuity, even with recurrent retinal detachment or pre-existing macular pathology or both.  相似文献   

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19.
Previous studies showed that twice-daily application of emu oil lotion (mixture of emu oil/fat, vitamin E, and botanical oil) immediately after creation of full-thickness skin defects delayed wound healing 6 days later, perhaps owing to its antiinflammatory actions. If administration was delayed for 48 hours, a two-fold promotion of wound contraction, epithelialization, and infiltration of organized granulation tissue was observed. In the present study, emu oil lotion was applied to full-thickness skin defects in rodents 24 hours after surgery. Six days postoperatively, wound contraction and infiltration of fronts of epithelialized and granulation tissue were assessed. Results indicated a two-fold promotion of all of the above parameters with emu oil lotion. No such effects were exerted by pure emu oil, furasin, cortaid, or polysporin. Data obtained indicate promise for emu oil lotion as an aid in treating full-thickness skin defects if applied after the major postinflammatory stages of wound healing have transpired.  相似文献   

20.
OBJECTIVE: To develop a short, patient-based questionnaire for auditing the outcomes of treatment for benign prostatic hyperplasia (BPH) and to evaluate the acceptability, reliability and validity of the measure. PATIENTS AND METHODS: Data from a long research questionnaire used in the Oxford/North West Thames Prostatectomy Study were analysed to identify the subset of items which contained the most scientifically sound indicators of outcome. Items were selected on the basis of standard psychometric analyses to develop a short questionnaire, the Prostate Outcomes Questionnaire (POQ), a 27-item instrument covering urinary symptoms, complications after surgery, quality of life and patient satisfaction with outcome. The POQ was field tested for acceptability, reliability and validity in a postal survey of 125 men undergoing transurethral resection of the prostate for BPH from seven hospitals in south-east England. RESULTS: The POQ was highly acceptable to patients, as indicated by a 95% response rate, the absence of 'floor' and 'ceiling' effects and a low proportion of missing data. It showed excellent internal consistency reliability (Cronbach's alpha of 0.82-0.93 for the five summary scales and a mean item-total correlation of 0.56) and test-retest reliability (test-retest correlations for items were 0.40-1.00, with a mean test-retest correlation of 0.85, and for summary scales of 0.91-0.95). Construct validity was confirmed by: high intercorrelations between the five summary scales and the total score (0.88-0.95), with a pattern of intercorrelations among specific subscales that showed item-convergent and discriminant validity; higher scores for patients who reported an improvement after surgery than in those who were not improved (P < 0.001); the expected gradient of scores for patients reporting different levels of symptom distress (P < 0.001); high correlations with the longer parent questionnaire (0.75-0.88); and low to moderate correlations with the Nottingham Health Profile and Activities of Daily Living scores (0.15-0.67), with the pattern of correlations providing evidence of scale convergent and discriminant validity. Correlations with age and social class were low, suggesting that responses were not biased by sociodemographic factors. CONCLUSION: The POQ is a practical and scientifically sound patient-based measure of outcome after treatment for BPH which can be used for routine audit. It takes < 5 min to complete, is feasible for routine monitoring of large numbers of patients by postal survey, and is accompanied by a Users' Manual which provides practical help in conducting a local patient survey, and a computer program for scoring data. Most importantly, the POQ has been shown scientifically to perform well, having met standard psychometric criteria for reliability and validity.  相似文献   

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