Cutaneous mucinosis of infancy: report of a rare case and review of the literature.

Cutaneous mucinosis of infancy (CMI) is a rare dermatologic condition, first reported in 1980 and currently classified within the complex group of papular mucinoses. We report a case of CMI and review the prior 13 cases in the literature. The patient was a 5-year-old girl who presented with asymptomatic dermal papules and plaques on her leg and back with no overlying color change. These lesions were first noticed during infancy and had become slightly more evident over time. The patient had a history of birthmarks and eczema. Her family history included eczema, allergies, photosensitivity, and Graves disease. Pre-biopsy clinical differential diagnosis included connective tissue nevus, granuloma annulare, myofibroma, lipofibroma, and lymphangioma. Biopsies revealed significant increase in interstitial mucin within the reticular and mid dermis, without significant sclerosis or fibroblastic proliferation. The relatively quiescent pattern of interstitial mucinosis with slight fibrocyte hyperplasia presenting as dermal papules-plaques on the trunk and extremities was most consistent with a diagnosis of CMI. We report another case of CMI in an otherwise healthy patient. Our patient is unique as she is the first CMI patient with a family history of Graves disease, although our patient appeared euthyroid. We will also review the literature on this rare entity.

Photodynamic therapy provides local control of cholangiocarcinoma in patients awaiting liver transplantation.

Many transplant centers use endoscopically directed brachytherapy to provide locoregional control in patients with otherwise incurable cholangiocarcinoma (CCA) who are awaiting liver transplantation (LT). The use of endoscopic retrograde cholangiopancreatography (ERCP)-directed photodynamic therapy (PDT) as an alternative to brachytherapy for providing locoregional control in this patient population has not been studied. The aim of this study was to report on our initial experience using ERCP-directed PDT to provide local control in patients with unresectable CCA who were awaiting LT. Patients with unresectable CCA who underwent protocol-driven neoadjuvant chemoradiation and ERCP-directed PDT with the intent of undergoing LT were reviewed. Four patients with confirmed or suspected CCA met the inclusion criteria for protocol LT. All four patients (100%) successfully underwent ERCP-directed PDT. All patients had chemoradiation dose delays, and two patients had recurrent cholangitis despite PDT. None of these patients had progressive locoregional disease or distant metastasis following PDT. All four patients (100%) underwent LT. Intention-to-treat disease-free survival was 75% at mean follow-up of 28.1 months. In summary, ERCP-directed PDT is a reasonably well tolerated and safe procedure that may have benefit by maintaining locoregional tumor control in patients with CCA who are awaiting LT.

Dermatological phenotype in Costello syndrome: consequences of Ras dysregulation in development.

BACKGROUND: The RASopathies are a class of human genetic syndromes caused by germline mutations in genes that encode protein components of the Ras/mitogen-activated protein kinase (MAPK) pathway. Costello syndrome (CS) is a RASopathy caused by mutations in the HRAS gene, a key regulator of signal transduction. OBJECTIVE: To quantify the specific cutaneous phenotype observed in 46 individuals with Costello syndrome with confirmed HRAS mutations. METHODS: This was a cross-sectional study. Dermatological surveys were designed by the authors and were completed by parents of mutation-positive individuals with CS at the Costello Syndrome Family Network (CSFN) conferences in 2007 and 2009. Dermatological examinations were performed by the authors at the CSFN conferences. RESULTS: Cutaneous papillomas were reported in 33 of the 46 (72%) participants, with age of onset ranging from infancy to 22years. Individuals with CS are more likely than patients with cardiofaciocutaneous syndrome (CFC) to present with cutaneous papillomas (72% vs. 5%, P<0·001) and palmoplantar keratoderma (76% vs. 36%, P<0·001). Individuals with CS are less likely than individuals with CFC to present with sparse or absent eyebrows (9% vs. 90%, P<0·001) or keratosis pilaris (33% vs. 80%, P=0·001). This study also identified that loose, redundant skin on the hands and feet, 'stippled' dermatoglyphs (pachydermatoglyphia) on the fingertips (eight of 26, 31%) and acanthosis nigricans (17 of 46, 37%) are frequent features of CS. CONCLUSIONS: While there is significant phenotypic overlap among syndromes of the Ras/MAPK pathway, individuals with CS are more likely than individuals with CFC syndrome to present with cutaneous papillomas, palmoplantar keratoderma and full eyebrows, and are less likely to present with ulerythema ophryogenes, keratosis pilaris or multiple naevi. The dermatological features of CS, a Ras dysregulation syndrome, share many features with cutaneous paraneoplastic syndromes. This may provide further insight into the role of Ras signalling in cutaneous paraneoplastic syndromes.

Estimation of the hamaker coefficient for a fuel-cell-supported catalyst system.

Hamaker coefficients are estimated for various nanoparticle-support systems commonly used in polymer electrolyte membrane fuel cells. The interaction energies, cohesion between nanoparticles, and adhesion of nanoparticles on the support are also estimated from the experimental data. Comparison between the bulk properties of platinum metal, calculated from the optical spectra available in the literature, and the nanoparticles are provided. Measurements to obtain the optical properties of the systems of interest are also reported. Implications of the van der Waals forces on the supported catalyst structure properties are discussed. The algorithm used in calculation of the Hamaker coefficient using Lifshitz theory was evaluated with known materials, using the spectral parameters available in the literature, and the results are presented.

Surface fluctuation spectroscopy by surface-light-scattering spectroscopy.

In recent years surface-light-scattering spectroscopy has been transformed from a complex optical experiment requiring substantial effort to operate effectively to a simpler instrument for which an accurate theory of operation has been developed. The accuracy and precision are sufficiently enhanced that refinement of the theory of spectral band shapes is justified to include more subtle effects such as bending moduli and thin-film forces associated with the van der Waals and the Casimir effects. We show how to develop extensions of the theory of interfacial fluctuations through the mass and the momentum balances of interfacial transport. We also show that free-energy functionals can be used to express curvature effects crisply. The results are detailed formulas that can be used to fit experimental spectra.

Advances in surface-light-scattering instrumentation and analysis: noninvasive measuring of surface tension, viscosity, and other interfacial parameters.

A new generation of vibration-mitigating surface-light-scattering instrumentation has been designed and built. The computational application of an instrument function derived by use of Fourier optics is presented. This instrument and its accompanying suite of analysis software allow us to easily make accurate and noninvasive measurements of the interfacial tension, volume viscosity, and other interfacial parameters of fluids. We derived the necessary surface response function algorithms to study both simple fluids and binary fluids at their wetting transition and near their critical points. These developments can be applied to study systems with liquid-vapor and liquid-liquid interfaces, including spread monolayers, whenever optical access for a laser beam is available.

Capillary waves on an asymmetric liquid film of pentane on water.

Surface light-scattering measurements have been carried out on thin asymmetric films of pentane on water. We vary the film thickness ? over a wide range (10(-9) m < ? < 10(-5) m). Compared with the ripplons wavelength 1/q, thick films of pentane (? ? 1/q) display the same power spectrum as a pure pentane-vapor interface: a single peak. When thinning the film down to ? approximately 1/q, hydrodynamic coupling between the layers of the interface is revealed by the appearance of a second peak beside the first one. We describe the thickness dependence of the two coupled modes through variations of the positions, the widths, and the amplitudes of both peaks.

Hybrid reflection-transmission surface light-scattering instrument with reduced sensitivity to surface sloshing.

A hybrid reflection-transmission surface light-scattering instrumental design is presented, examined theoretically, and tested experimentally. The purpose of the design is to reduce the sensitivity of the instrument to vibration in general and surface sloshing in particular while sacrificing little performance. Traditional optical arrangements and two new optical configurations with varying trade-offs between slosh resistance and instrumental simplicity and accuracy are examined by use of Fourier optics methods. The most promising design was constructed and tested with acetone, ethanol, and water as subject fluids. The test involved backcalculation of the wave number of the capillary wave examined with the known physical parameters for the test fluids. The agreement of the computed wave number was +/-1.4%.

Ankle arthrodesis using an arthroscopic method: long-term follow-up of 34 cases.

Thirty-four ankle arthrodeses performed using an arthroscopic technique were followed for an average of 8 years. The fusion rate was 97% and the average time to fusion was 9 weeks; 86% of patients had good or excellent functional results. There were no wound infections or neurological injuries. There was one malunion and one additional minor complication. The arthroscopic method uses an abrader to denude the joint surfaces of cartilage, followed by screw fixation. The average time to fusion is significantly less than other ankle arthrodesis techniques, which hastens the recovery period. The shorter time to fusion is likely a result of the minimal soft tissue stripping that is performed during the procedure. The low morbidity of this technique eliminates the need for hospitalization in most cases. There were no long-term adverse sequelae. This is the largest reported series of arthroscopic ankle arthrodeses.

Orthopedic soft-tissue tumors. Concepts for the primary care physician.

Mesenchymal neoplasia includes an extensive variety of tumors. It is important that the primary care physician understand the different qualities of benign and malignant processes in incidence, pathogenesis, history, physical findings, and diagnostic work-up and, as a result, be able to make the appropriate orthopedic consultation when necessary. It also is important that they understand basic concepts of definitive treatment so that they may educate their patients. Table 2 lists some of the more common benign and malignant soft-tissue tumors. It is by no means an attempt to be inclusive, but rather to serve as an introduction to a vast group of neoplasms.

Parasitism of house fly (Musca domestica) pupae by four species of Pteromalidae (Hymenoptera): effects of host--parasitoid densities and host distribution.

Parasitoid-induced mortality of house fly, Musca domestica L., pupae and parasitoid progeny emergence by four species of pteromalid parasitoids, Muscidifurax raptor Girault & Sanders, M.zaraptor Kogan & Legner, Spalangia cameroni Perkins and S.endius Walker, were determined for a 24 h exposure period using parasitoid: host ratios ranging from 1:2 to 1:50. When the number of parasitoids was held constant (n = 5) and the numbers of hosts varied, and when the number of hosts was held constant (n = 100) and the number of parasitoids varied, both the number of pupae killed per parasitoid and the number of parasitoid progeny per parasitoid increased with increasing parasitoid:host ratios to reach an upper limit asymptotically. Maximum values were, respectively: M.raptor (14.7, 11.1), M.zaraptor (12.3, 9.3), S.cameroni (16.9, 5.5), S.endius (14.8, 9.7) with no consistent effects attributed to parasitoid interference. For M.raptor and S.cameroni at parasitoid:host ratios of 1:10, the pupal mortality and progeny emergence were determined for a 24 h exposure period when hosts were distributed in poultry manure at four levels of aggregation ranging from clumped to uniform. Pupal mortality was least in clumped distributions, while parasitoid progeny emergence was not significantly different.

Temperature-dependent development and parasitism rates of four species of Pteromalidae (Hymenoptera) parasitoids of house fly (Musca domestica) pupae.

Parasitoid development, parasitoid-induced host mortality and parasitoid progeny emergence were determined at five constant temperatures for Muscidifurax raptor Girault and Sanders, Muscidifurax zaraptor Kogan and Legner, Spalangia cameroni Perkins and Spalangia endius Walker using pupae of the house fly, Musca domestica L., as hosts. At temperatures of 20, 25, 30 and 35 degrees C the median development times (days from oviposition to adult emergence), respectively, were M. raptor (28.4, 20.7, 14.3, 14.5), M. zaraptor (30.6, 22.8, 14.1, 14.2), S. cameroni (55.6, 35.2, 21.8, 25.0) and S. endius (52.4, 31.5, 16.3, 14.6). All species failed to emerge at 15 degrees C. Using densities of five parasitoids and 100 hosts and a 24 h exposure period, Muscidifurax species oviposited at a greater rate over a wider range of temperatures than Spalangia species. At 15, 20, 25, 30 and 35 degrees C the mean number of pupae killed per parasitoid were, respectively, M. raptor (1.4, 7.4, 10.5, 13.7, 14.1), M. zaraptor (0.0, 3.3, 8.9, 14.4, 15.0), S.cameroni (0.0, 7.8, 11.0, 11.9, 7.4), S.endius (0.6, 4.0, 7.5, 12.0, 11.7), and means of the number of parasitoid progeny per parasitoid were, respectively, M.raptor (0.2, 5.2, 7.9, 11.8, 11.6), M.zaraptor (1.3, 4.4, 8.2, 13.0, 13.7), S.cameroni (0.0, 2.4, 4.7, 5.1, 1.0), S.endius (0.0, 0.9, 3.4, 7.5, 4.9). Development and ovipositional activity in S.cameroni was strongly inhibited at 35 degrees C. The model by Sharpe & DeMichele (1977) was used to describe temperature-dependent development and the number of parasitoid progeny produced per parasitoid at temperatures of 15-30 degrees C in all species.

Comparative evaluation of gastrointestinal intolerance produced by plain and tri-buffered aspirin tablets.

Two multi-investigator, double-blind, randomized, placebo-controlled, crossover trials were conducted to determine whether tri-buffered formulations of both regular strength aspirin and extra strength aspirin would be less likely than plain aspirin to provoke subjective gastrointestinal (GI) intolerance. Each trial was divided into two phases, a qualification phase and a test phase. During the qualification phase, subjects with a history of gastrointestinal intolerance to aspirin were randomized to a double-blind crossover treatment with aspirin and placebo (325 mg aspirin per tablet in study 1 and 500 mg aspirin per tablet in study 2), two tablets four times a day for 3 days or until the occurrence of stomach upset. Subjects who reported gastrointestinal symptoms with aspirin and not with placebo qualified to participate in the test phase of the study. They were rerandomized to participate in a three-way crossover study of plain aspirin, tri-buffered aspirin, and placebo in the test phase. Tri-buffered aspirin was associated with an appreciable reduction in the incidence of gastrointestinal upset relative to plain aspirin, 34 percentage points in study 1 (p less than 0.001) and 33 percentage points in study 2 (p less than 0.001). Similar results were obtained in the evaluation of the reduction of the severity of gastrointestinal symptoms.

Lower esophageal ring: experiences in treatment of 88 patients.

The lower esophageal ring is an unusual clinical disorder of the esophagus and consists of a thin submucosal circumferential scar that forms in the lower esophagus. It is probably an acquired lesion resulting from repeated insults to the lower esophageal mucosa. The symptom of dysphagia results from esophageal obstruction, and the degree of obstruction is directly related to the internal diameter of the ring. Episodic aphagia results from impaction of food at the site of the ring. Since 1970, 88 patients have been seen with either dysphagia or episodic aphagia. Sixty-five with chronic limited reflux were treated primarily by oral dilation. Two of them required an antireflux procedure at a later date because of accentuation of reflux symptoms. Eighteen patients received surgical treatment initially because of severe reflux disease. Treatment consisted of interruption of the ring combined with an antireflux procedure. Five patients received no treatment. Lower esophageal ring may be managed satisfactorily through oral dilation, resulting in relief of dysphagia. If reflux disease is present or is accentuated by dilation and cannot be controlled medically, then the appropriate antireflux procedure should be done.

Surface fluctuation scattering using grating heterodyne spectroscopy.

Heterodyne photon spectroscopy is used for the study of the viscoelastic properties of the liquid interface by studying light scattered from thermally generated surface fluctuations. A theory of a heterodyne apparatus based on a grating is presented, and the heterodyne condition is given in terms of the parameters of the experimental setup. Particular attention is paid to those conditions of the experiment that can cause systematic errors. An instrument function for the apparatus relating the measured output to the experimental parameters of interest is derived. It is shown that the instrument function is generally not Gaussian but that its functional form can depend strongly on the parameters of the experimental setup. In particular, it is shown that significant systematic deviations are expected from previous published analysis of the problem. This is primarily due to the nonlinear relation of the surface wave number to the frequency. An algorithm is suggested to arrive at the frequency and width parameters of the spectrum from a typical noisy experimental data set and applied to data obtained from ethanol surfaces.

Double-blind multicenter comparison of 1,200 mg. and 1,000 mg. cimetidine in hospitalized and ambulatory duodenal ulcer patients.

A multicenter double-blind study was made to compare whether 1,000 mg dosage of cimetidine, the dose commonly employed in the United Kingdom, was as effective as the 1,200 mg. dose recommended in the United States for the treatment of duodenal ulcer. Our second goal was to test whether hospitalization would enhance the efficacy of cimetidine in the healing of duodenal ulcer. There was no difference in healing rate after either one (40%) or four (86%) weeks of therapy. In addition, hospitalization (one week) did not enhance the efficacy of cimetidine therapy.

Swine vesicular disease: pathways of infection.

The pathways of infection in swine vesicular disease have been studied by (i) an estimation of the amounts of virus required to produce infection by different artificial inoculation procedures; (ii) the distribution and amounts of virus in various tissues of pigs killed at intervals after contact infection; (iii) an investigation of the susceptibility to virus infection of pig tissue explants. The results show that pigs can be infected by a number of pathways and that the skin, as the most susceptible tissue, is probably the most frequent route of infection.