Oral Everolimus for Treatment of a Giant Left Ventricular Rhabdomyoma in a Neonate—Rapid Tumor Regression Documented by Real Time 3D Echocardiography

The presented case reports on successful treatment with everolimus in a neonate with left ventricular giant rhabdomyoma. The authors used a different dosage regime compared to literature and documented rapid tumor regression by 3D echocardiography.     

A case of recurrent Miller Fisher syndrome mimicking botulism

Abstract Miller Fisher syndrome (MFS) is a rare and usually monophasic polyradiculoneuropathy characterised by ophthalmoplegia, decreased or absent tendon reflexes, and ataxia. The objective of this study was to report a case of recurrent MFS with a clinical presentation virtually indistinguishable from botulism. The patient was a young man with two episodes of increasing external ophthalmoplegia, ptosis, and ataxia with a long asymptomatic interval in between. The second episode occurred after consumption of rotten fish and was accompanied by gastrointestinal symptoms and an anticholinergic syndrome. Very rarely, MFS can present with a recurrent course. The importance of this case of recurrent MFS lies not only in its long asymptomatic period and identical clinical presentation, but also in its instructiveness regarding the differential diagnosis of MFS, particularly life-threatening botulism.     

Difficult-to-recruit respondents and their effect on prevalence estimates in an epidemiologic survey

Generous contact efforts were used to recruit 3,004 respondents into the first wave of the St. Louis Epidemiologic Catchment Area (ECA) project, a psychiatric epidemiologic study of the general population, conducted from 1981-1982. These efforts were analyzed to establish which sociodemographic characteristics or current psychiatric disorders were determinants of difficulty in recruitment. Being young, male, black, a nonrural resident, educated, and full-time employed were the demographic characteristics associated with increased contact efforts. Persons currently meeting criteria for an alcohol disorder required almost 20% more contact attempts than those without the disorder; this difficulty lay both in their being less available for an initial contact and refusing at a higher rate once contacted. An optimal recruitment effort cutoff point is provided, and a method is suggested for detecting that optimal point in the course of ongoing studies.     

Isolation of DNA from the centromere of human chromosome 7 by microdissection

Centromeres remain the least characterized regions of human chromosomes because they have a very high content of repetitive DNA. Here, we describe a micro-dissection library from the centromeric region of human chromosome 7 and its use for generating sequence tagged sites (STSs). The library contains about 1500 clones with an average insert size of 150 bp and only about 15% of the clones harbour repetitive human DNA. Seven clones hybridizing to alphoid DNA were found to correspond to a fragment of the D7Z2 alphoid array on chromosome 7, thus confirming the origin of the library. A number of clones not containing known repetitive DNA were used to generate STSs that identified yeast artificial chromosomes (YACs) and in turn allowed the STSs to be placed on the physical map. One STS is located between the two Genethon genetic markers closest to the centromere on the q side. Another STS was located 3–4 cM away in 7q11.2, while a third identified YACs containing both low-copy and alphoid sequences that are not yet mapped but are clearly centromeric. The library therefore comprises a collection of sequences from the centromeric region of chromosome 7 that can be used to generate STSs and to map the entire centromeric region.This revised version was published online in November 2005 with corrections to the Cover Date.     

Recommendations for the standardization of lead positions in surface electromyography

Summary Standardized lead positions for recording myoelectric signals are given for various muscles. The positions are given in terms of (1) the lead line connecting two anatomical landmarks and (2) the central lead point about which the electrodes are placed symmetrically on the lead line.This research was financially supported by the Sonderforschungsbereich 152 of the Deutsche Forschungsgemeinschaft     

Active detachment involves inhibition of cell-matrix contacts of malignant melanoma cells by secretion of melanoma inhibitory activity

Melanoma inhibitory activity (MIA) has been identified as a small protein secreted from malignant melanoma cells. Recent results revealed a direct interaction of MIA and epitopes within extracellular matrix proteins including fibronectin. The aim of this study was to analyze functional consequences mediated by this interaction. Here we show that MIA interferes specifically with attachment of melanoma cells to fibronectin, a phenomenon we refer to as active detachment. Antibodies inhibiting binding of alpha4beta1 and alpha5beta1 integrins to fibronectin cross-react specifically with MIA, suggesting that MIA shares significant structural homology with the binding pockets of these integrins and thereby masks the respective epitopes on extracellular matrix molecules. Several peptides derived from fibronectin and from a phage display screening were tested with respect to a potential MIA-inhibitory effect. In vitro tests identified two peptides affecting MIA function; both inhibited growth of melanoma metastases in vivo. In summary, we conclude that MIA may play a role in tumor progression and spread of malignant melanomas via mediating active detachment of cells from extracellular matrix molecules within their local milieu. Further, our results suggest that inhibiting MIA functions in vivo may provide a novel therapeutic strategy for metastatic melanoma disease.     

Self-organization of a liquid crystalline methacrylate-monofunctionalized crown-ether compound in low-shrinkage acrylate mixtures

Crystallization and supramolecular aggregation of 1,4,7,10,13-pentaoxacyclopentadecane-2-ylmethyl 3,4-bis[4-(dodecyl-1-oxy)benzyloxy]-5-(11-methacryloyloxyundecyl-1-oxy)benzoate ( 1 ) and its complexes with sodium triflate are described in solutions of the methacrylate monomers. Formation of a gel was observed covering a rather wide concentration range in the pseudo-binary phase diagram. In the low concentration regime and at low temperatures, gel formation by elongated crystals of 1 was observed. It was demonstrated that the formation of a crystalline network and the shape of the crystals strongly depend on the cooling rate.     

Synthesis and characterization of monomethacrylate-functionalized self-organizing crown ether compounds

Synthesis and characterization of methacrylate-monofunctionalized crown ether compounds based on derivatives of 2-hydroxymethyl-1,4,7,10,13-pentaoxacyclopentadecane and 4′-hydroxymethyl-1,4,7,10,13-pentaoxabenzocyclopentadecane is described. By differential scanning calorimetry (DSC) and thermooptical analysis (TOA) it is shown that the synthesized compounds and their sodium triflate complexes exhibit columnar mesophases.     

Orbital surgery – perioperative problems and results

Fragestellung: Orbitotomien nehmen innerhalb der Ophthalmochirurgie auf Grund der involvierten anatomischen Strukturen und des daraus resultierenden Spektrums m?glicher perioperativer Probleme eine Sonderstellung ein. Um einen überblick über die Art und H?ufigkeit intra- und postoperativer Probleme bei Orbitotomien zu erhalten, führten wir eine retrospektive Auswertung an unserer Klinik durchgeführter Orbitotomien durch. Patienten und Methode: Es wurden 48 Orbitotomien bei 46 Patienten berücksichtigt, die zwischen 08/1995 und 02/1998 operiert wurden. Ergebnisse: Schwerwiegende intraoperative Komplikationen waren mit zwei transfusionspflichtigen Blutungen und einer Liquorfistel selten. Sie wurden interdisziplin?r behandelt. Postoperativ traten vorübergehende funktionelle St?rungen mit guter Rückbildungstendenz wie Visusminderungen (35 %), Motilit?tsst?rungen mit oder ohne Doppelbildwahrnehmung (20 %) und Lidfehlstellungen auf. Ihnen liegt v. a. die postoperative ?dem- und H?matombildung zugrunde. Persistierende Funktionseinschr?nkungen waren dagegen selten (10 %). Schlu?folgerung: Unsere Untersuchung zeigt, da? perioperativ bei Orbitotomien v. a. vorübergehende funktionelle Einschr?nkungen auftreten, die sich rasch zurückbilden. Schwere Komplikationen sind dagegen selten, treten v. a. intraoperativ auf und k?nnen eine interdisziplin?re Therapie erfordern.