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.

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.

Neuropathology of experimental swine vesicular disease in pigs.

A total of 26 young pigs were inoculated intracerebrally, intravenously or intradermally with the UKG27/72 strain of SVD virus, grown in tissue culture, and killed two, four, eight or 16 days after exposure. Overt nervous symptoms were seen only in pigs inoculated intracerebrally, but all pigs of all groups showed a non-suppurative meningitis and panencephalomyelitis principally affecting the mid- and fore-brain. Ganglioneuritis with intranuclear inclusion bodies in perineuronal amphicytes were found to be the most consistently useful characters for distinguishing SVD from other virus encephalitides of pigs. Spinal radiculitis was a feature of the early stages of CNS involvement and lesions were found also in the optic nerve, retina and cornea.

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.

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.

Poikilotherm vertebrate cell lines and viruses: a current listing for fishes.

A survey of the literature and of work being done in vertebrate cell culture shows that there are currently in existence and available to investigators some 61 cell lines representing 17 families and 36 species of fish. The literature of fish virology shows that at least 17 fish viruses have been isolated and that at least 15 others have been visualized by electron microscopy. A minimum of four major virus groups--rhabdovirus, orthomyxovirus, iridovirus and herpesvirus--are known from fish. Original references, key reviews and sources of cell lines are given.

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.

Purification and properties of a bacteriophage-induced cell wall peptidase from Staphylococcus aureus.

A phage-induced cell wall solubilizing enzyme isolated from phage-infected Staphylococcus aureus phage type 80 was purified 588-fold. The pH optimal activity was 6.8 to 7.3, and pH optimal stability, 6.5 to 7.5. It was inhibited by p-hydroxymercuribenzoate, ethylenediaminetetraacetic acid, and specific rabbit antisera. The cell wall lytic reaction is a peptidase resulting in cleavage of the cell wall peptide at N-terminal alanine, glutamic acid, and glycine. Electron micrographs are shown of cell wall "ghosts" remaining after the enzymatic digestion of cell walls.

General pathology of experimental swine vesicular disease.

Twenty-four minimal disease pigs were inoculated intracerebrally, intravenously or intradermally with an English strain of swine vesicular disease virus. In the skin, snout, tongue and tonsil the main lesion was a full-thickness coagulative necrosis of the stratified squamous epithelium. In the renal pelvis, bladder, tonsillar crypts and the collecting ducts of salivary glands and pancreas, epithelial degeneration with the formation of periodic acid-Schiff-positive material were consistent features of this disease. Histopathological examination alone could not be relied upon to differentiate between well-established skin lesions caused by swine vesicular disease and foot and mouth disease. The relationship between vesicular disease and Coxsackie B5 is discussed briefly.

Attemps to infect pigs with Coxsackie virus type B5.

Despite the existence of a close serological relationship between the enteroviruses Swine Vesicular Disease (SVD) and Coxsackie type B5 (Cx B5), the administration of this Coxsackie virus type to susceptible pigs by various routes failed to produce clinical disease.Viraemia was not detected after exposure but virus was recovered intermittently from faeces and buccal swabs. A mixed virus population was demonstrated in faecal cultures from some pigs, including Coxsackie virus type B5 and other agents, presumably native pig enteroviruses. The Coxsackie virus persisted in faeces in declining amounts for up to 8 days after primary exposure.Serum neutralizing antibody showed a transient rise to Coxsackie virus, reaching a peak at 14 days and declining below demonstrable titres by 28 days after exposure. The antibody titres attained were proportional to the dose of virus administered and the degree of neutralization was very similar to both SVD and Cx B5 viruses.On cross challenge by exposure to SVD virus 28 days after exposure to Cx B5 virus, most animals (5/6) succumbed with typical vesicular lesions, although the serum neutralizing antibody titres showed a characteristically anamnestic response to both viruses.

Swine vesicular disease: comparative studies of viruses isolated from different countries.

Seven viruses isolated from outbreaks of swine vesicular disease in various countries between 1966 and 1973 were compared in pigs and infant mice. All produced a similar disease and virus excretion pattern in the pig, although the Italy/66 virus was considerably less virulent than the other viruses. The results of cross neutralization tests of convalescent pig sera and the response of 5-day-old mice to intraperitoneal inoculation indicated minor differences between some viruses. The Italy/66, Hong Kong/71 and France/73 viruses differed from each other and also from the Italy/72, England/72, Austria/73 and Poland/73 group of viruses.

Laser light-scattering investigations of the teleost swimbladder response to acoustic stimuli.

When a laser pencil is directed through the teleost swimbladder fringe patterns can be seen in the far-field that are (a) highly sensitive to the orientation and position of the swimbladder with respect to the incident pencil and (b) a representation of contributions from each membrane through which the light passes. The fringe pattern fluctuates in intensity, and to some extent in position, in response to driving forces that distort the swimbladder. The spectrum of these very small distortions can be measured by standard light scattering techniques. This method was used to study the response of in situ swimbladders to imposed acoustic fields and evidence for a sharp roll-off of the response at frequencies above 1,000 Hz was found. Models for these effects are discussed.

Swine vesicular disease: virological studies of experimental infections produced by the England-72 virus.

Pigs exposed to relatively small amounts of virus by intradermal inoculation of the feet or by skin sacrification developed clinical disease. Large amounts of virus were recovered from samples taken from the nose, mouth, pharynx, rectum and the prepuce or vagina during the first week of infection and smaller amounts during the second week. Virus was recovered from the faeces of most animals 16 days after infection and from one animal for 23 days. Pigs in contact with inoculated animals were killed at intervals before the appearance of clinical disease. The distribution and amounts of virus in various tissues indicated that infection has most likely gained entry through the skin or the epithelia and mucosae of the digestive tract. Some pigs acquired subclinical infections in which no virus excretion was detected and no transmission of infection to susceptible pigs took place over a period of 5 weeks.