The surgical treatment of transsphenoidal cephaloceles in children is controversial. Reduction and repair via a transcranial approach are associated with high postoperative rates of morbidity, mortality, and hypothalamic dysfunction. In this study, four patients, aged 3 to 35 months at surgery, underwent successful transpalatal repair of two encephaloceles and two meningoceles. Two patients presented with nasal obstruction in infancy, one presented with unexplained meningitis, and in one patient the lesion was found incidentally during evaluation for seizures. Two children had median cleft face syndrome, another had an associated Arnold-Chiari type I malformation, and the fourth had no other cranial abnormalities. All patients underwent preoperative evaluation including magnetic resonance (MR) imaging. Auditory, ophthalmological, genetic, endocrinological, or other evaluation was undertaken as indicated. Lesions were approached through the median raphe of the hard and soft palates. All cephaloceles were easily visualized and dissected after division of the nasal palatal mucosa. The dural sac and its contents were reduced by surface coagulation after division and dissection of the overlying mucosa. Once reduced, the bone defect was obliterated in three of four patients. The dura was not opened and anomalous neural elements were not resected. At follow-up evaluation, all patients demonstrated resolution of preoperative symptoms without evidence of infection or lasting morbidity. Follow-up MR imaging showed reduction in all cases. The authors conclude that this transpalatal approach is safe and reliable for the treatment of transsphenoidal cephaloceles in young children.
The first synthesis of ganodermanontriol, a bioactive lanostane triterpene from the medicinal mushroom Ganoderma lucidum, has been achieved in 15.3% yield over nine steps, along with its three stereoisomeric triols and ganoderol A. The key steps leading to this family of isomers involve the reconstruction of the trisubstituted alkene by stereoselective and chemoselective phosphonate reactions and the formation of the unusual Δ7,9(11)-diene core by the mild acidic opening of a lanosterone-derived epoxide. Ganodermanontriol showed promising activity on the inhibition and proliferation of breast cancer cells. The effect of ganodermanontriol and its isomers on cell proliferation was assayed; IC50 values of 5.8 and 9.7 μM on breast cancer cell lines MCF-7 and MDA-MB-231, respectively, were found for ganodermanontriol.
This article examines the challenges experienced by very old individuals and their consequences for well-being and mental health. In order to capture unique issues experienced in very old age, 75 participants of the population-based Fordham Centenarian Study answered open-ended questions on everyday challenges. Theme-based coding was then used to categorize and quantify responses. The challenges mentioned most often were challenges faced in the functional (e.g., physical health/activities of daily living restrictions, mobility, sensory impairment), psychological (e.g., loss of well-liked activity, dependency, negative emotions, death), and social (e.g., family loss) life domains. Functional challenges were negatively associated with aging satisfaction and positively associated with loneliness. Psychological challenges were positively linked to aging satisfaction. Social challenges were marginally related to loneliness. Notably, challenges were not related to depression. In conclusion, the challenges experienced in very old age are multidimensional and multifaceted, unique in nature, and have differential relations to mental health. Functional, psychological, and social challenges affect very old individuals' lives and therefore need to be better understood and addressed. Given their consequences, it is imperative for policy makers to develop an awareness for the different types of challenges faced by centenarians, as there may be unique policy implications related to each.
Providers support a reflex-testing program to improve the identification and management of suspected LS patients. However, how to support meaningful information provision to enable an opt-out without jeopardizing testing uptake and the anticipated public health benefits remains a policy challenge.Genet Med advance online publication 06 October 2016.
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