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Major Clinical (major + clinical)
Terms modified by Major Clinical Selected AbstractsThe value of early treatment in patients with haemophilia and inhibitorsHAEMOPHILIA, Issue 3 2010K. KAVAKLI Summary., Development of inhibitors to infused factor concentrates represents a major clinical and economic challenge in the treatment of haemophilic patients. It has been shown that a delay in initiation of treatment leads to requirement of a larger number of injections to stop the bleeding but this has never been formally linked to costs associated with the bleeding. The objectives of this study were to assess the relationship between time to initiation of NovoSeven® and total costs, number of doses administered and time to bleeding resolution in mild to moderate bleeding episodes. Data on time to treatment initiation, time to bleeding resolution and on all resource use related to the bleeding were extracted from medical records in Turkey for 129 bleeding episodes. Regression analysis was used to assess the impact of time to treatment on outcomes. Longer time to treatment initiation increased both total costs associated with the bleeding, the number of doses needed and the time to bleeding resolution. Treatment in hospital was associated with significantly longer time to treatment, higher costs and longer time to bleeding resolution as compared with home treatment or outpatient treatment. When controlling for other bleeding characteristics, the cost of bleedings treated in hospital was more than 150% higher. This study shows that treatment with NovoSeven® should be initiated as soon as possible after the onset of bleeding in order to minimize costs and optimize outcomes. Home treatment reduces time to treatment initiation and also reduces costs related to the bleeding. [source] European Study on Orthopaedic Status of haemophilia patients with inhibitorsHAEMOPHILIA, Issue 5 2007M. MORFINI Summary., ,Development of inhibitors against factor VIII (FVIII) or factor IX (FIX) in haemophilia patients is one of the most serious complications of repeated exposure to replacement therapy and has major clinical and economic consequences. To evaluate the relationship between inhibitor status of haemophilia patients and their quality of life (QoL) and degree of arthropathy and to compare the orthopaedic status of patients with/without inhibitors. An observational, cross-sectional, case control study enrolling: group A (n = 38), males aged 14,35 years, with severe congenital haemophilia A or B who had inhibitors against FVIII/FIX >5 years; group B (n = 41), as group A, but aged 36,65 years and group C (n = 49), as group A, but without inhibitors. Socio-demographics: medical history, clinical characteristics and QoL were assessed. In groups A and B, 16% and 27% were hospitalized for orthopaedic procedures vs. 4% in group C. Patient mobility was also severely reduced in groups A and B, with 24% and 22% using wheelchairs vs. 4% in group C, and 50% and 51% needing a walking aid vs. 29% in group C. Significantly more joint pain was reported by patients in group A vs. those in group C; clinical/radiological orthopaedic scores were also worse in group A vs. group C. Significantly more joint abnormality was reported by patients in group A vs. group C. The burden of orthopaedic complications and the impact on QoL are more severe in haemophilia patients who have developed inhibitors than in those without inhibitors. [source] Cell-based immunotherapy with mesenchymal stem cells cures bisphosphonate-related osteonecrosis of the jaw,like disease in miceJOURNAL OF BONE AND MINERAL RESEARCH, Issue 7 2010Takashi Kikuiri Abstract Patients on high-dose bisphosphonate and immunosuppressive therapy have an increased risk of bisphosphonate-related osteonecrosis of the jaw (BRONJ); despite the disease severity, its pathophysiology remains unknown, and appropriate therapy is not established. Here we have developed a mouse model of BRONJ-like disease that recapitulates major clinical and radiographic manifestations of the human disease, including characteristic features of an open alveolar socket, exposed necrotic bone or sequestra, increased inflammatory infiltrates, osseous sclerosis, and radiopaque alveolar bone. We show that administration of zoledronate, a potent aminobisphosphonate, and dexamethasone, an immunosuppressant drug, causes BRONJ-like disease in mice in part by suppressing the adaptive regulatory T cells, Tregs, and activating the inflammatory T-helper-producing interleukin 17 cells, Th17. Most interestingly, we demonstrate that systemic infusion with mesenchymal stem cells (MSCs) prevents and cures BRONJ-like disease possibly via induction of peripheral tolerance, shown as an inhibition of Th17 and increase in Treg cells. The suppressed Tregs/Th17 ratio in zoledronate- and dexamethasone-treated mice is restored in mice undergoing salvage therapy with Tregs. These findings provide evidence of an immunity-based mechanism of BRONJ-like disease and support the rationale for in vivo immunomodulatory therapy using Tregs or MSCs to treat BRONJ. © 2010 American Society for Bone and Mineral Research [source] SARS , a perspective from a school of nursing in Hong KongJOURNAL OF CLINICAL NURSING, Issue 2 2004David R. Thompson PhD Background., Severe acute respiratory syndrome (SARS) is a new infectious disease with significant morbidity and mortality that has had a major impact on health and health care services worldwide. Hong Kong has had a significant number of cases and deaths. Nurses, at the vanguard of the clinical health care team, have been particularly affected by it. The outbreak prompted the health authorities to implement a series of public health measures and hospital policies, including a guideline for the diagnosis and management of patients with SARS. Aims and objectives., This paper aims at providing an overview of what is known about SARS and the impact it has had in Hong Kong and to highlight from the perspective of a school of nursing the major clinical, educational and public health implications. Conclusions., The lack of understanding and uncertainty about the disease led to significant variation in the provision of information, contributing to the confusion and anxiety in the community. Therefore, there is a need to revise the nursing curriculum, to provide continuing education to all health care professionals, particularly with regard to infection control measures, and to revisit the range of public health policies to ensure the health of the community is protected by these policies. There also has been a reaffirmation of the importance of health promotion that highlights the importance of the partnership between nurses, health policy makers and public health personnel. It is evident that the organization and delivery of clinical practice, teaching and health promotion have to be flexible and responsive to a changing health scenario. Relevance to clinical practice., Nurses must play a crucial role in the prevention, detection and containment of SARS. They will need to implement and ensure strict adherence to infection control measures and, in some circumstances, isolation and quarantine may be warranted. Attention to the psychological state of patients and family members should not be overlooked. Paramount is education of patients, families and members of the public at large. [source] MDM2 SNP309 genotype influences survival of metastatic but not of localized neuroblastoma,PEDIATRIC BLOOD & CANCER, Issue 4 2009Chiara Perfumo PhD Abstract Background MDM2 is a major negative regulator of p53 function and is directly regulated by MYCN in neuroblastoma (NB) cells. MDM2 SNP309, a T-to-G substitution in the MDM2 promoter associated with higher gene expression compared to wild-type, may attenuate the p53 pathway in NB, in which p53 mutations are rare. We investigated its impact on NB development and survival in relation with major clinical and biological characteristics. Procedure A consecutive cohort of 497 NB children, diagnosed in Italy between 1995 and 2005, and a healthy control population of 471 adults were genotyped for MDM2 SNP309. NB patients were followed up until June 30, 2008. Results Patients and controls showed similar distribution of MDM2 SNP309 genotypes. In patients, the polymorphism was not associated with any characteristic at diagnosis. In localized stages no effect of the polymorphism on survival was evident. In stage 4 patients overall survival (OS), event free survival (EFS) and survival after relapse (SAR) were significantly poorer for TG/GG than for TT patients (P,=,0.008; P,=,0.013; P,=,0.046, respectively). In this group, such an effect was more evident in patients with MYCN amplification (OS: P,<,0.001; EFS: P,=,0.028; SAR: P,<,0.001). Conclusions While MDM2 SNP309 status does not affect the risk of developing NB nor disease outcome for localized cancer cases, it significantly correlates with survival in stage 4 NB patients, particularly in the presence of MYCN amplification. The impact of small molecule inhibitors of MDM2 activity in the management of such patients could be usefully considered. Pediatr Blood Cancer 2009;53:576,583. © 2009 Wiley-Liss, Inc. [source] HLA polymorphisms in African Americans with idiopathic inflammatory myopathy: Allelic profiles distinguish patients with different clinical phenotypes and myositis autoantibodiesARTHRITIS & RHEUMATISM, Issue 11 2006Terrance P. O'Hanlon Objective To investigate possible associations of HLA polymorphisms with idiopathic inflammatory myopathy (IIM) in African Americans, and to compare this with HLA associations in European American IIM patients with IIM. Methods Molecular genetic analyses of HLA,A, B, Cw, DRB1, and DQA1 polymorphisms were performed in a large population of African American patients with IIM (n = 262) in whom the major clinical and autoantibody subgroups were represented. These data were compared with similar information previously obtained from European American patients with IIM (n = 571). Results In contrast to European American patients with IIM, African American patients with IIM, in particular those with polymyositis, had no strong disease associations with HLA alleles of the 8.1 ancestral haplotype; however, African Americans with dermatomyositis or with anti,Jo-1 autoantibodies shared the risk factor HLA,DRB1*0301 with European Americans. We detected novel HLA risk factors in African American patients with myositis overlap (DRB1*08) and in African American patients producing anti,signal recognition particle (DQA1*0102) and anti,Mi-2 autoantibodies (DRB1*0302). DRB1*0302 and the European American,, anti,Mi-2,associated risk factor DRB1*0701 were found to share a 4,amino-acid sequence motif, which was predicted by comparative homology analyses to have identical 3-dimensional orientations within the peptide-binding groove. Conclusion These data demonstrate that North American IIM patients from different ethnic groups have both shared and distinct immunogenetic susceptibility factors, depending on the clinical phenotype. These findings, obtained from the largest cohort of North American minority patients with IIM studied to date, add additional support to the hypothesis that the myositis syndromes comprise multiple, distinct disease entities, perhaps arising from divergent pathogenic mechanisms and/or different gene,environment interactions. [source] Parosteal osteosarcoma: report of a case and review of the literatureAUSTRALIAN DENTAL JOURNAL, Issue 1 2010TC Huang Abstract Parosteal osteosarcoma is a rare malignancy of the bone that usually arises in the long bones. Involvement of the oral cavity is rare. Only 12 cases of intraoral parosteal osteosarcoma have been reported in the English language literature. This paper defines the major clinical, radiographic and histologic features of parosteal osteosarcoma and illustrates these with a case of a 33-year-old male presenting with a three-month history of a painless enlarging lump in the right maxilla. A critical and comprehensive review of the English language literature is also provided. [source] Multiple, large sialoliths of the submandibular gland duct: a case reportAUSTRALIAN DENTAL JOURNAL, Issue 1 2009TC Huang Abstract This paper reviews the major clinical and radiographic features of sialoliths and illustrates these with an unusual case of multiple sialoliths within the submandibular gland duct. The differential diagnosis of other calcific structures both within and outside the salivary gland that may mimic a sialolith is also presented. [source] Long-term Follow-up of Severely Atrophic Edentulous Mandibles Reconstructed with Short Branemark ImplantsCLINICAL IMPLANT DENTISTRY AND RELATED RESEARCH, Issue 4 2000Bertil Friberg DDS ABSTRACT Background: Oral implant treatment (Brånemark System) of edentulous mandibles has been presented in numerous studies. However, with regard to the severely atrophic lower jaw, no long-term follow-up studies with solely short implants are available. Purpose: The purpose of the present investigation was to retrospectively follow the long-term treatment outcome of patients with severely resorbed edentulous mandibles being subjected to oral implant placement with short (6,7 mm) Brånemark implants. Materials and Methods: A total of 247 standard (7 mm long, 3.75 mm) and 13 wide (6 mm long, 5 mm) implants were inserted in 49 patients, all of whom exhibited severe resorption of edentate mandibles. Fixed implant-supported prostheses were manufactured for 45 patients, whereas 4 patients received overdentures. The patients were followed for a mean period of 8 years (range, 1,14 yr). Results: Seventeen implants failed during the study period (cumulative implant survival rate 95.5% at 5-yr and 92.3% at 10-yr follow-up). Implant-supported constructions were worn continuously throughout the investigation by all study subjects. Marginal bone loss, measured after 1, 5, and 10 years of function, concurred with studies of Brånemark implants placed in more voluminous mandibles. No major clinical or construction complications occurred in the followed patients. Conclusions: The outcome of the present study showed that placement of short Brånemark implants without the use of bone grafting procedures for reconstruction of severely atrophic edentulous mandibles is a highly predictable treatment procedure. [source] | ||||||||||