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21.
22.
Klippel-Feil syndrome (KFS) is a heterogeneous entity, characterized by specific congenital anomalies of segmentation of the cervical spine. We report a case of KFS plus atretic meningocele in one identical twin and anencephaly in the other. These identical twins were both male with no chromosome defect. One of them had anencephaly diagnosed at the gestational age of 17 weeks and died soon after birth, whereas the co-twin had KFS with multiple congenital anomalies including short neck with low hairline, occipital atretic meningocele, butterfly vertebrae, hemivertebrae, cervico-thoracic scoliosis, left thumb preaxial polydactyly, and right undescended testis. He received rehabilitation during infancy and could walk at the age of 15 months. Both of these infants had preaxial polydactyly, which is an uncommon anomaly associated with KFS. In conclusion, iniencephaly and anencephaly are probably the extreme manifestations of KFS rather than different dysraphisms. Prompt clinical suspicion with early diagnostic imaging for spine lesions and occult opening of neural tube is mandatory for further intervention and rehabilitation. 相似文献
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24.
Many people die in emergency departments (EDs) across the United States from sudden illnesses or injuries, an exacerbation of a chronic disease, or a terminal illness. Frequently, patients and families come to the ED seeking lifesaving or life-prolonging treatment. In addition, the ED is a place of transition-patients usually are transferred to an inpatient unit, transferred to another hospital, or discharged home. Rarely are patients supposed to remain in the ED. Currently, there is an increasing amount of literature related to end-of-life care. However, these end-of-life care models are based on chronic disease trajectories and have difficulty accommodating sudden-death trajectories common in the ED. There is very little information about end-of-life care in the ED. This article explores ED culture and characteristics, and examines the applicability of current end-of-life care models. 相似文献
25.
Lily Shum A B M Rabie Urban H?gg 《American journal of orthodontics and dentofacial orthopedics》2004,125(2):185-190
This study assessed the amount of vascular endothelial growth factor (VEGF) expression and related the findings to new bone formation in the posterior glenoid fossa during stepwise mandibular advancement. A total of 250 female Sprague-Dawley rats, 35 days old, were randomly divided into 10 groups, each including 5 control and 20 experimental rats. Within each group, 10 experimental rats were fitted with functional appliances with a 1-step advancement of 3.5 mm. Another 10 were fitted with stepwise appliances with an initial advancement of 2 mm and a subsequent increase to 3.5 mm on day 30. The rats in the experimental groups were killed on days 3, 7, 14, 21, 30, 33, 37, 44, 51, and 60, respectively. The matched controls were killed on the same time points. Sections (7 microm) were cut through the glenoid fossa sagittally and stained with anti-VEGF antibody. VEGF expression in the posterior glenoid fossa was evaluated with a computer-assisted image-analyzing system. Both VEGF expression and new bone formation were greater in the experimental rats than in the controls. During stepwise advancement, initial VEGF expression was less than that of 1-step advancement, but the second advancement elicited another peak on day 44. New bone formation was also less than that of 1-step advancement during early stages of stepwise advancement but then began to increase from day 37 onward. The maximum increase was observed on day 60. Stepwise advancement of the mandible delivers mechanical stimuli that produce a series of tissue responses that lead to increased vascularization and bone formation. 相似文献
26.
What factors influence survival in patients with unresected synchronous liver metastases after resection of colorectal cancer? 总被引:3,自引:0,他引:3
N. Chafai C. L. H. Chan E. L. Bokey O. F. Dent G. Sinclair P. H. Chapuis 《Colorectal disease》2005,7(2):176-181
OBJECTIVE: The aim of this study was to determine whether the survival of patients with untreated synchronous liver metastases after resection of a colorectal cancer was associated with any features of the primary tumour. METHODS: Information for 398 consecutive patients with unresected liver metastases in the period 1971-2001 was examined by multivariate survival analysis. RESULTS: Of 19 clinical and pathological variables considered, survival was independently associated only with residual tumour in a line of resection (hazard ratio (HR) 1.95), venous invasion (HR 1.87), right colonic tumour (HR 1.68), lymph node metastasis (HR 1.54), and extra-hepatic metastasis (HR 1.16); 8.3% of patients had none of these adverse features. Their 2-year overall survival rate was 39.2%, compared with only 16.5% (P < 0.001) in those with one or more adverse features. CONCLUSIONS: These findings may assist in selecting patients most likely to benefit from treatment of hepatic metastases and in counselling patients and their relatives. 相似文献
27.
The appearance of an acute effusion in a well-pneumatized temporal bone directs attention to the nasopharynx and skull base. Two patients are described in whom dehiscence of the temporomandibular joint allowed herniation of the contents of the joint posteromedically, where they obstructed the middle ear entrance of the eustachian tube, the protympanum. This is, to the authors' knowledge, a previously unreported cause of an acute middle ear and mastoid effusion. 相似文献
28.
Diagnosis of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes in a Chinese family by PCR/restriction enzyme analysis 总被引:1,自引:0,他引:1 下载免费PDF全文
C W Lam K Jain K Y Chan D K Silva Y W Chan L J C Wong 《Journal of clinical pathology》1995,48(5):M285-M288
The clinical presentation and the biochemical and molecular genetic findings are described in a 13 year old Chinese boy with MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes). The diagnosis was initially suspected because of the characteristic clinical features and the strong family history of convulsions. Using polymerase chain reaction—restriction enzyme analysis, the heteroplasmic nt3243 A→G mutation in mtDNA of peripheral blood leucocytes and a muscle sample was demonstrated. The oligosymptomatic relatives were then screened by this method and the degree of heteroplasmy was analysed. This appears to be the first report of a MELAS family in Hong Kong with this described mutation. Molecular genetic techniques are advantageous in the diagnosis of MELAS. 相似文献
29.
应用双光子及单光子吸收测定技术,检测了92名出生3d内的新生儿骨矿质含量,其中巨大儿30名,正常体重儿32名,低出生体重儿30名,其孕龄分别是40.1±0.7、39.1±1.5和37.4±1.2孕周。结果表明,巨大儿、正常体重儿、低出生体重儿的全身骨矿质含量分别是101.6±28.8、85.2±19.8、59.9±27.2g/cm2,巨大儿骨矿质含量最高,正常体重儿次之,低出生体重儿最低,差异有显著性(P<0.01)。颅骨、肱骨、股骨的骨矿质含量与全身的骨矿质含量有高度的相关性,相关系数分别是0.943、0.879和0.745(P<0.01)。全身的骨矿质含量与出生体重、孕龄及头围有高度相关性,相关系数分别是0.755、0.596和0.556(P<0.01)。提示颅骨、肱骨及股骨的任一部位均可代表全身的骨矿质含量,肱骨是确定全身骨矿质含量较好的部位,新生儿骨矿质含量受出生体重、孕龄及头围的影响较大。 相似文献
30.
Immunopathologic study of Vogt-Koyanagi-Harada syndrome 总被引:9,自引:0,他引:9
C C Chan A G Palestine T Kuwabara R B Nussenblatt 《American journal of ophthalmology》1988,105(6):607-611
We studied an enucleated eye from a patient with a 30-year history of Vogt-Koyanagi-Harada syndrome using both conventional and immunohistochemical techniques. Clinically, the eye was in the end stage of Vogt-Koyanagi-Harada syndrome, and was characterized by the absence of inflammation, large areas of chorioretinal scarring, and pigmentary changes. Histopathologic examination showed marked retinal gliosis, extensive chorioretinal adhesion and scar formation, migration of pigment into the retina, and severe retinal pigment epithelial changes. However, foci of mild to moderate nongranulomatous inflammation of the uvea were observed. These foci contained infiltrating cells that were mainly T lymphocytes with B lymphocyte aggregates at the center. Scattered macrophages were also noted in the uvea and retina. These findings suggest that both the cell-mediated and humoral immune arms may play roles in the pathogenesis of Vogt-Koyanagi-Harada syndrome. 相似文献