Fronto-ethmoidal mucocoeles: a histopathological analysis.

From a series of 118 fronto-ethmoidal mucocoeles, 40 specimens have been submitted to detailed histological analysis. The frequency of each epithelial and sub-epithelial component is considered and reveals features which support a dynamic theory of bone resorption and formation rather than that of pressure erosion in the pathogenesis of mucocoeles.     

Clinical application of epiderm growth factor in patients with most severe eye burns

20 eyes of 11 patients suffering from most severe eye burns of grade 4 or worse were treated in addition to various other drugs with epiderm growth factor (EGF) or placebo in a double-blind test. Among 10 eyes treated with EGF, 6 achieved complete regeneration of the corneal epithelium, and 1 additional case, of the conjunctival epithelium. Among the eyes receiving placebo, only 3 out of 10 healed; all of them were also treated with fibronectin. Although in these very difficult cases EGF could not be investigated as a single therapy, and various other undefined factors may have been effective, in the extended course of the disease the period of EGF treatment was marked by a significant better regeneration of the epithelium.     

PADGEM (GMP140) is a component of Weibel-Palade bodies of human endothelial cells

PADGEM protein (PADGEM), also known as GMP140, is a platelet alpha- granule membrane protein that is translocated to the external membrane after platelet activation. Although the biosynthesis of this protein was originally thought to be confined to megakaryocytes, the synthesis of PADGEM in endothelial cells was recently demonstrated (McEver et al: Blood 70:1974a, 1987). We now describe the subcellular localization of this protein in endothelial cells. Immunofluorescence staining of permeabilized human umbilical vein endothelial cells with KC4, a well characterized monoclonal antibody to PADGEM, showed positively stained elongated structures similar in distribution and shape to Weibel-Palade bodies. Their identity as Weibel-Palade bodies was confirmed by double label immunofluorescence using KC4 and a polyclonal antiserum to von Willebrand factor (vWf), a protein known to be specifically stored in these organelles. All Weibel-Palade bodies were found to contain PADGEM. In contrast to strong perinuclear staining produced with anti- vWf antibodies, no significant perinuclear staining was obtained with KC4, indicating that relatively little PADGEM is present in the endoplasmic reticulum and in the Golgi apparatus. In endothelial cells treated with secretagogues that stimulate vWf release the elongated structures positive for PADGEM disappeared, further identifying these structures as Weibel-Palade bodies. This observation extends the parallels between Weibel-Palade bodies and alpha-granules and suggests a possible functional association between vWf and PADGEM.     

Usher syndrome: clinical findings and gene localization studies

The issue of genetic heterogeneity is a critical problem in the localization of the gene(s) for Usher syndrome. Based on the data obtained on families studied to date, the differences between type I and type II Usher syndrome appear quite distinct with regard to auditory and vestibular function. Although the majority of families can be confidently diagnosed as typical type I or type II, clinical investigations revealed four families with findings that did not fit into either of the two more common subtypes. These findings emphasize the critical importance of an in-depth clinical analysis concomitant with the linkage investigation to assure accurate subtyping of Usher syndrome. Based on an analysis of only those families with definite type I or type II Usher syndrome, approximately 17% of the genome can be excluded as a potential site of the gene for type I, and 14% can be excluded as the site for the type II gene. This study will continue until the Usher gene(s) is successfully localized.     

BUILDING THE FOUNDATION OF EXCELLENCE IN HEART FAILURE NURSING

This column contains the presidential address presented during the Third Annual Meeting of the American Association of Heart Failure Nurses on June 28, 2007, in San Diego, California, titled "Building the Foundation of Excellence in Heart Failure Nursing."     

MR imaging of the spine in children

MR imaging was reviewed in 66 pediatric spinal cord patients with diagnoses of posterior fossa tumor (n = 8), primary spinal cord tumor (n = 3), metastatic disease (n = 11), neuroectodermal disorder (n = 8), congenital malformation (n = 14), trauma (n = 2), and demyelinating, neurodegenerative, or metabolic disorders (n = 9). MR proved to be useful in delineating the extent of posterior fossa and cord tumor including metastasis to the cord. MR was of limited value in demyelinating and metabolic disorders. Arnold Chiari malformations, syringomyelia, tethered cord and meningoceles were all easily evaluated using MR.