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How many pathways to pheochromocytoma?   总被引:4,自引:0,他引:4  
Pheochromocytomas, like several other tumors, may be either sporadic or the manifestation of a familial cancer syndrome. Recently, major advances have occurred in both the understanding of diverse molecular mechanisms leading to pheochromocytoma and the diagnostic modalities available for detection of the disease. Familial pheochromocytoma may be a manifestation of multiple endocrine neoplasia type 2 (MEN-2), von Hippel-Lindau (VHL), or neurofibromatosis-1 (NF 1) disease. Tumor-suppressor genes responsible for the familial occurrence of extra-adrenal pheochromocytoma, called paraganglioma, have been identified. This wealth of genetic information, coupled with the availability of sensitive and specific biochemical tests as well as imaging studies, allows for genetic screening and early diagnosis of pheochromocytoma. In addition, genetic screening of relatives at risk is now feasible. In this article, we review recent clinical and molecular advances in our understanding of pheochromocytoma.  相似文献   

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BACKGROUND: Clinical studies assessing orthopaedic interventions often include data from two limbs or multiple joints within single individuals. Without appropriate design or statistical approaches to address within-individual correlations, this practice may contribute to false precision and possible bias in estimates of treatment effect. We conducted a systematic review of the orthopaedic literature to determine the frequency of inappropriate inclusion of nonindependent limb or joint observations in clinical studies. METHODS: We identified seven orthopaedic journals with high Science Citation Index impact factors and retrieved all clinical studies for 2003 for any intervention on any limb or joint. RESULTS: We identified 288 clinical studies, 143 of which involved two limbs or multiple joint observations from single individuals. These studies included nineteen randomized clinical trials (13%) fifty-eight two-group cohort studies (41%), and sixty-six one-group cohort studies (46%). Seventy-six (53%) of the 143 studies involved statistical comparisons between patient groups with use of tests of association, and an additional sixty studies (42%) presented estimates of proportions without statistical comparisons. Only sixteen of the seventy-six studies involving statistical comparisons involved the use of any technique or methodological approach to account for multiple, nonindependent observations. A median of approximately 13% of the patients in these studies contributed more than one observation. The median proportion of nonindependent observations to total observations (the unit of analysis) was approximately 23%. CONCLUSIONS: Our findings suggest that a high proportion (42%) of clinical studies in high-impact-factor orthopaedic journals involve the inappropriate use of multiple observations from single individuals, potentially biasing results. Orthopaedic researchers should attend to this issue when reporting results.  相似文献   

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OBJECTIVES: The most severe forms of acute heart failure have a dismal prognosis despite modern invasive treatment. For some of these patients, improved outcome must relay on early institution of ventricular assist devices (VAD). We aimed to estimate the potential VAD need in acute heart failure. DESIGN: All patients admitted to the ICU or CCU for acute heart failure (AHF) in 2003/04 (n=302) were reviewed. Non-survivors with severe acute heart failure, i.e. cardiogenic shock and postcardiotomy HF, were individually reviewed to assess eligibility for VAD-treatment. RESULTS: Cardiogenic shock and postcardiotomy HF was present in 23% (n=69) and 19% (n=57) of the AHF patients. Overall in hospital mortality in these groups was 38% (n=48). Of these, 15 were less than 75 years of age, without serious comorbidities and thus deemed to be potential candidates for VAD-treatment. CONCLUSION: This survey indicates that 12% of patients with severe acute heart failure are potential candidates for VAD-treatment. Extending these figures to a national level, indicate that approximately 70 patients per year could be candidates for short-term VAD-treatment in Norway.  相似文献   

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What's a CLIC doing in the podocyte?   总被引:1,自引:0,他引:1  
Chloride intracellular channel (CLIC) proteins are proposed to have numerous functions, including chloride channel activity, regulation of gene expression, and serving as a component of the cytoskeleton. Pierchala et al. have identified CLIC5A in podocytes, where it is complexed with ezrin, podocalyxin, and the actin cytoskeleton. Absence of CLIC5A results in altered podocyte morphology and proteinuria. Exactly how CLIC5A supports podocyte function is unclear. Potential mechanisms in which cytoskeletal interactions could regulate other functions of CLIC5A are discussed.  相似文献   

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BACKGROUND: Recently videolaparoscopy is considered to have a vaster use in surgery due to the undeniable benefits such as low operatory traumatism, quick recovery of canalization, a short stay in the hospital and minor scarring. METHODS: Forty patients were treated with peritoneal dialysis (PD); 15 videolaparoscopic procedures were performed on 13 patients before starting PD and two during the course of PD. The videolaparoscopy procedure was started by inducing pneumoperitoneum after initiation of general anaesthesia through endotracheal intubation. RESULTS: Peritoneal catheter placement was carried out in 11 ESRD patients showing abdominal scars due to previous laparotomies; their abdominal condition precluded safe PC placement using conventional non-laparoscopic procedures with local anaesthesia. Release of adhesions was performed only in two patients. Videolaparoscopy was also used in three patients for elective cholecystectomy; 2/3 underwent concomitant PC insertion. One patient was submitted to cholecystectomy during the course of CAPD; following the procedure we left the peritoneum dry overnight and then we started temporary IPD, using small volumes, avoiding haemodialysis (HD). Regular CAPD was resumed 6 days later. Finally, videolaparoscopy was also used for diagnostic purpose i.e. in one 59-year-old man patient who had a peritoneal catheter obstruction. Repeated rescue attempts using urokinase solution to irrigate the peritoneal catheter had been used in vain attempts prior to the procedure. CONCLUSIONS: Videolaparoscopy proves to be a useful tool in a PD programme. Firstly, it may be used as a technique for catheter implantation, not as a routine procedure but in patients with extensive abdominal scars due to previous laparotomy, i.e. at risk for accidental viscera perforation due to the possibility of adhesions between intestinal loops and parietal peritoneum. Secondly, videolaparoscopy used for abdominal surgery allows the resumption of PD immediately after surgical procedure and thus avoiding HD. Videolaparoscopy is fundamental for diagnosis and rescue of catheter dysfunction and has an integral role in the successful management of these patients in extending catheter function and permitting safe replacement of peritoneal catheter if it becomes necessary. Along with the undeniable advantages, remains the disadvantages that it must be carried out by an expert surgeon in an operating theatre while the patient is under general anaesthesia.  相似文献   

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Editor—In his editorial,1 Prof. Sneyd stresses the needfor large prospective studies addressing the effect of bispectralindex (BIS) monitoring on the risk of awareness. What may haveescaped him is that preliminary results from just  相似文献   

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Intraoperative awareness is a terrifying experience, which patientsfear and anaesthetists are anxious to avoid; any clinician whodoubts this should read a first hand account.1 Since the early1990s, the Bispectral Index (BIS) has been developed as a measureof hypnosis during anaesthesia and sedation. As BIS is a linearizedand monotonic measure, clinicians have become comfortable withthe idea that an increase in BIS may warn of actual or impendingawareness. Although  相似文献   

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《Injury》2022,53(8):2790-2794
PurposeInjury prevention is important. Injury as a result of an accident carries with it huge cost to the individual and society including health services costs. Understanding the mechanism of injury is important to identify those injuries that are preventable. The aim of this study was to assess how many acute orthopaedic injuries were preventable should reasonable human interventions have been takenMethodsThis prospective study was performed in a UK district general hospital that serves a population of 500,000 people. All patients referred to the Orthopaedic department over 4 weeks between 16th November and 14th December 2020 were reviewed. Data was collected about the mechanism of injury, patient demographics, injury type, injury location, treatment and length of stay in hospital. The mechanisms of injury were analysed thematically.ResultsWe assessed 605 patient attendances. 502 patients sustained 516 acute traumatic injuries. Preventable injuries were common, accounting for 23.9% of all referrals. The upper limb was most commonly injured with the hand and forearm being injured 33% of the time. Of the preventable injuries 35% were treated operatively. Thematic analysis identified common mechanisms of: occupational related, stairs, RTA, cycling, alcohol related, trampoline, DIY and assault.ConclusionThis study identifies that a large proportion of acute orthopaedic injuries are potentially preventable meaning this is an important area for further study. Occupation injuries were identified as an area in which there is the greatest scope to reduce the number of preventable accidents.  相似文献   

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