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161.
162.
François Béïque Mohamed Ali Scott MacKenzie Mark Hynes 《Journal canadien d'anesthésie》2007,54(2):158-159
163.
164.
Elizabeth S Hart Marilyn H Kelly Beth Brillante Clara C Chen Navid Ziran Janice S Lee Penelope Feuillan Arabella I Leet Harvey Kushner Pamela G Robey Michael T Collins 《Journal of bone and mineral research》2007,22(9):1468-1474
Most lesions in FD and their attendant functional disability occur within the first decade; 90% of lesions are present by 15 years, and the median age when assistive devices are needed is 7 years. These findings have implications for prognosis and determining the timing and type of therapy. INTRODUCTION: Fibrous dysplasia of bone (FD) is an uncommon skeletal disorder in which normal bone is replaced by abnormal fibro-osseous tissue. Variable amounts of skeletal involvement and disability occur. The age at which lesions are established, the pace at which the disease progresses, if (or when) the disease plateaus, and how these parameters relate to the onset of disability are unknown. To answer these questions, we performed a retrospective analysis of a group of subjects with FD. MATERIALS AND METHODS: One hundred nine subjects with a spectrum of FD were studied for up to 32 years. Disease progression was assessed in serial (99)Tc-MDP bone scans by determining the location and extent of FD lesions using a validated bone scan scoring tool. Physical function and the need for ambulatory aids were assessed. RESULTS: Ninety percent of the total body disease skeletal burden was established by age 15. Disease was established in a region-specific pattern; in the craniofacial region, 90% of the lesions were present by 3.4 yr, in the extremities, 90% were present by 13.7 yr, and in the axial skeleton, 90% were present by 15.5 yr. Twenty-five of 103 subjects eventually needed ambulatory aids. The median age at which assistance was needed was 7 yr (range, 1-43 yr). The median bone scan score for subjects needing assistance was 64.3 (range, 18.6-75) compared with 23.1 (range, 0.5-63.5) in the unassisted subjects (p < 0.0001). Among subjects needing assistance with ambulation, 92% showed this need by 17 yr. CONCLUSIONS: The majority of skeletal lesions and the associated functional disability occur within the first decade of life. The implication is that the window of time for preventative therapies is narrow. Likewise, therapeutic interventions must be tailored to where the patient is in the natural history of the disease (i.e., progressive disease [young] versus established disease [older subjects]). These findings have implications for prognosis, the timing and type of therapy, and the development of trials of new therapies and their interpretation. 相似文献
165.
Aims and Objectives The significance of beaten copper appearance (BCA) on skull radiographs in children following surgery for isolated sagittal
craniosynostosis has not been studied. This study was designed to look for any correlation between BCA and symptoms suggestive
of intracranial hypertension in this group of patients.
Materials and Methods Forty-eight consecutive children, who were operated for isolated sagittal synostosis from1987 to 2000 and had postoperative
skull radiographs, were included. Patients were divided into: (a) BCA group (n = 20), consisting of children who had beaten copper appearance on skull radiographs at last follow up, and (b) Non-BCA group
(n = 28), consisting of children who did not have this finding. Records were reviewed to look for symptoms suggestive of intracranial
hypertension, such as headache, head banging, and irritability.
Results Median age at surgery was 4.8 months for BCA group and 4 months for the non-BCA group. Follow up ranged from 4 to 156 months
with a mean of 36.2 months. Total of 28.6% (n = 6) of the children with follow up radiographs done at ≤18 months of age had BCA. The incidence of BCA increased to 83.3%
in children with skull radiographs performed after 48 months of age. In 18 (90%) children, the BCA was ‘diffuse’ with 5 (25%)
children having the maximum possible score of 8. In the BCA group, 45% (n = 9) had symptoms compared to 10.7% (n = 3) in the control group (p = 0.0068).
Conclusions This study suggests a significant number of children with BCA on radiographs develop symptoms suggestive of raised ICP following
surgical treatment in infancy and prolonged follow up may be warranted in this group of patients. 相似文献
166.
167.
Luís Mariano Isabel Vila?a Jorge Almeida Mota Garcia Maria Júlia Maciel 《Revista portuguesa de cardiologia》2007,26(3):265-270
Apical ballooning is a novel clinical entity reported in different contexts of physical and psychological stress, which is more common in middle-aged women. Of unknown etiology, the syndrome is characterized by a sudden and transient dilatation of the left ventricular apex in the absence of obstructive atherosclerotic coronary disease or evidence of myocardial necrosis, with total late recovery of ventricular function. The authors report the case of a 53-year-old woman who was admitted to the emergency room with left arm ischemia and low cardiac output, requiring ventilatory support. Left catheterization showed typical medial and apical myocardial dysfunction, with normal coronary arteries. Transesophageal echocardiography revealed a thrombus attached to the lower face of the aortic arch, which probably explained the thromboembolism of the arm but was unlikely to be the cause of the left ventricular dysfunction since there were no enzymatic or electrocardiographic signs of myocardial necrosis and normal wall motion was fully recovered. 相似文献
168.
169.
170.
Maria A Hortis-Dzierzbicka Zofia Dudkiewicz 《The Cleft palate-craniofacial journal》2004,41(1):105; author reply 105-105; author reply 106