Fibrous dysplasia is a non‐neoplastic developmental process that affects the craniofacial bones, characterized by painless enlargement as a result of bone substitution by abnormal fibrous tissue. Postzygotic somatic activating mutations in the GNAS1 gene cause fibrous dysplasia and have been extensively investigated, as well as being helpful in the differential diagnosis of the disease. Fibrous dysplasia may involve one (monostotic) or multiple bones (polyostotic), sporadically or in association with McCune‐Albright syndrome, Jeffe‐Lichenstein syndrome, or Mazabreud syndrome. This review summarizes the current knowledge on fibrous dysplasia, emphasizing the value of integrating the understanding of its molecular pathogenesis with the clinical, radiological, and histopathological features. In addition, we address important aspects related to the differential diagnosis and patient management. 相似文献
Purpose: To study the prevalence and time of onset of ocular complications in intermediate uveitis (IU) and to assess their effects on visual outcome in short-term and long-term follow-up.
Methods: A retrospective cohort study of patients with IU who had a minimal follow-up of one year.
Results: 96 patients (174 eyes, 70% females) were included with a mean age at presentation of 30 years. Children constituted 38% of all patients. Mean follow- up was 64.9 months. Pars planitis was the most common form followed by sarcoidosis and multiple sclerosis. Cystoid macular edema (CME), cataract, epiretinal membrane and posterior synechiae were the most prevalent complications. Posterior synechiae, band keratopathy, cataract and papillitis at presentation were associated with worse presenting visual acuity (VA). Of the late-onset complications, glaucoma/ocular hypertension (OHT) was the most significantly associated with worse long-term VA. Most sight-threatening complications (namely CME and glaucoma) were diagnosed at presentation while late complications predominantly affected the posterior segment and included among others peripheral retinal elevations, vasoproliferative tumors, and vitreous hemorrhage. Median LogMAR VA improved at short-term and long-term follow-up, from 0.2 to 0.1 (p<0.001). Subgroup analysis revealed significant VA improvement for eyes with all the different complications except for eyes with glaucoma/OHT.
Conclusion: IU is a chronic low-grade uveitis that may be associated with protean early and late complications of the anterior or posterior segments or both. Optimal treatment regimens are imperative for the strict control of inflammation and proper management of complications thus allowing a favorable long-term prognosis. 相似文献
Anxiety disorders and anxiety symptoms are highly prevalent in the general population and more so in the medically ill. They
have a number of negative consequences for these patients and may worsen the outcome of the medical illness and increase health
care utilization. In the evaluation of these patients, it is of paramount importance to identify the etiology of the anxiety
and, in particular, to differentiate primary from secondary anxiety. Management includes medications (especially benzodiazepines
and selective serotonin reuptake inhibitors) and psychotherapy (particularly cognitive-behavioral therapy). 相似文献
Colorectal cancer, if detected early, has greater than 90% 5-year survival. However, survival has been shown to vary across
racial/ethnic groups in the United States, despite the availability of early detection methods. 相似文献
Between 4/1986 to 1/1989, 74 orthotopic liver transplantation were performed in 62 patients (62 first liver transplants, 10 as second graft and two as a third graft); 57 in adults and 17 in children. The main indication for the operation was liver cirrhosis (61.4%) (the most frequent etiology was alcoholic cirrhosis, 28.5%). Six cirrhotic patients had a hepatocarcinoma (9.6%). Two received a liver and kidney transplant due to terminal renal insufficiency and hemodialysis. The most frequent indication in children was biliary atresia (33.3%). Six patients had a fulminal liver failure (9.6%). AB0 blood group compatibility was identical in 87.5%, compatible in six and incompatible in three patients. Total orthotopic liver transplantation was performed in 67 patients, and size-reduced liver was indicated in 7 patients. Extracorporeal veno-venous bypass was used in adults but never in children. In 93.1% of the transplants a single hepatic artery was anastomosed to the recipient and in 6.9% a double anastomosis was performed. In 62.5% of the patients a end-to-end choledocho-choledochostomy was performed and in 34.8% hepatico-jejunostomy was indicated. Three months postoperative mortality rate was 12.9%. Arterial stenosis and thrombosis were the most frequent complication. 相似文献
Background and objectives: The aim of this study was to evaluate the practical value of the dermatoscopic classification of Clark nevi Patients and methods: Dermatoscopic images of 268 lesions clinically and dermatoscopically diagnosed as Clark nevi were presented to 2 dermatologists without knowledge of the histological diagnosis. The dermatologists evaluated the lesions according to a simplified version of the classification scheme for Clark nevi proposed by Hofmann‐Wellenhof and differentiated between 12 different types of Clark nevi. Results: The most common type of Clark nevus was the reticular‐homogenous type (n = 64, 23,9 %), followed by the globular‐homogeneous type (n = 32, 12 %) and by the homogenous type (n = 30, 11,2 %). The overall inter‐rater agreement between the examiners was moderate to good (kappa = 0,58). The highest level of agreement was found for the peripheral hyperpigmented type (kappa = 0,83). Histologically, 17 lesions (6,3 %) were diagnosed as melanomas. The frequency of melanoma was highest among the peripheral‐hyperpigmented type for one observer and among the homogenous type for the other observer. No melanoma was found among the globular, reticular‐globular, and the central‐hyperpigmented types. Conclusions: A dermatoscopic classification of Clark nevi is practically feasible and allows – to some extent – a risk stratification of Clark nevi, which could be useful for clinical management. 相似文献