The first documented case of a symptomatic intrasellar hemangioblastoma is described, occurring in an 11-year-old girl with stigmata of von Hippel-Lindau disease who presented with headaches, progressive bitemporal hemianopsia, and adenohypophysial dysfunction. A subtotal resection of the lesion was achieved with two separate surgical procedures: a transsphenoidal approach and a subfrontal craniotomy. Subsequent growth of residual tumor was treated with combined conventional radiotherapy and stereotactic radiosurgery. Two years following completion of these adjuvant therapies, no residual tumor was evident on magnetic resonance imaging. Previous experience with hemangioblastoma in this region, as well as the rationale for radiotherapy in the treatment of incompletely resected lesions, is reviewed.
Dihydroxyacetone (DHA), a browning agent, protects photosensitive rats and humans against long ultraviolet radiation (UVA, 320–400 nm) and visible (blue) light. The photoprotective efficacy of DHA and structurally similar compounds was assessed as prevention of edema in the paws of psoralen-sensitized rats, after exposure to blacklight fluorescent lamps. Methylglyoxal produced a yellow-brown color and provided nearly the same protection as DHA, whereas monohydroxyacetone did not color the skin and afforded little or no protection. Glyceraldehyde provided a moderate amount of protection, which was enhanced by prior exposure of the agent to alkaline pH. A solution of 5-hydroxymethylfurfuraldehyde was yellow and provided minimal protection by staining the skin rather than browning it. We conclude that the ability to produce a brown color in skin is a useful criterion for screening compounds for photoprotective efficacy against UVA radiation.
Background/Objectives: Muscle biopsies are requested for the workup of myopathy when less invasive workup is inconclusive. We set forth to build a multivariate model of preoperative testing to increase the odds of diagnostic and therapeutic yield of a muslce biopsy. Methods:A 5-year retrospective chart review of 106 patients who underwent muscle biopsy was performed to evaluate preoperative workup including: magnetic resonance imaging, electromyography, muscle strength testing and laboratory evaluation including creatine kinase level, lactate level, urine myoglobin, acetylcholinesterase antibody testing, genetic testing or use of steroids in the preoperative period using Student's two-tailed t-test, two-tailed Fisher's exact test and multivariate regression model. Results:We found an overall biopsy diagnostic pathologic yield of 47% and therapeutic yield of 88%. None of the variables reached a level of significance of p-value < 0.05 in univariate testing. In the multivariate model preoperative use of steroids and creatine kinase were the closest predictors of diagnostic and therapeutic yield but failed to reach statistical significance. Conclusions:The therapeutic impact of muscle biopsies appears to be much greater than the diagnostic pathologic yield and the therapeutic impact larger than previously reported when rigorously defined. We were unable to create a statistically significant preoperative predictive model for muscle biopsy diagnostic and therapeutic yield.
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