Initial characterization of stiff skin-like syndrome in West Highland white terriers

BACKGROUND Stiff skin syndrome and systemic or localized scleroderma are cutaneous disorders characterized by dermal fibrosis and present clinically with induration of the skin, with or without joint, internal organ or vascular involvement. OBJECTIVES To provide clinical, histological and preliminary genetic analysis of two West Highland white terrier siblings presenting with indurated skin resembling stiff skin syndrome in humans. ANIMALS Two client owned full sibling West Highland white terriers from two different litters. METHODS Clinical examination, histopathological examination and whole genome sequencing analysis of affected and unaffected West Highland white terriers. RESULTS Affected dogs exhibited markedly indurated skin that was attached firmly to the underlying tissue and incomplete closure of the mouth and eyes. No abnormalities were found by neurological or orthopaedic examination, radiographs of the head or whole body computed tomography. Histologically, the dermis and pannicular septa were thickened by a marked increase in coarse collagen fibres and a mild to moderate increase in collagen fibre diameter. The syndrome most likely follows an autosomal recessive mode of inheritance. The sequence analysis did not reveal any obvious causative variant in the investigated candidate genes ADAMTSL2 and FBN1. CONCLUSION AND CLINICAL IMPORTANCE The clinical phenotype and histopathological features of two West Highland white terrier siblings resembled stiff skin syndrome in humans. Unlike in humans, or previously described beagles with stiff skin, there was no restriction of joint mobility. Genetic analysis did not detect a candidate causative variant and warrants further research.

Ichthyolith (fish tooth and denticle) counts from DSDP Site 91-596 and ODP Site 145-886

While the history of taxonomic diversification in open ocean lineages of ray-finned fish and elasmobranchs is increasingly known, the evolution of their roles within the open ocean ecosystem remains poorly understood. To assess the relative importance of these groups through time, we measured the accumulation rate of microfossil fish teeth and elasmobranch dermal denticles (ichthyoliths) in deep sea sediment cores from the North and South Pacific gyres over the past 85 million years. We find three distinct and stable open ocean ecosystem structures, each defined by the relative and absolute abundance of elasmobranch and ray-finned fish remains. The Cretaceous Ocean (pre-66 Ma), was characterized by abundant elasmobranch denticles, but low abundances of fish teeth. The Paleogene Ocean (66-20 Ma), initiated by the Cretaceous/Paleogene Mass Extinction, had nearly 4 times the abundance of fish teeth compared to elasmobranch denticles. This Paleogene Ocean structure remained stable during the Eocene greenhouse (50 Ma) and the Eocene-Oligocene glaciation (34 Ma), despite large changes in overall accumulation of both groups during those intervals, suggesting that climate change is not a primary driver of ecosystem structure. Dermal denticles virtually disappeared from open ocean ichthyolith assemblages about 20 Ma, while fish tooth accumulation increased dramatically in variability, marking the beginning of the Modern Ocean. Together, these results suggest that open ocean fish community structure is stable on long timescales, independent of total production and climate change. The timing of the abrupt transitions between these states suggests that the transitions may be due to interactions with other, non-preserved pelagic consumer groups.

Cost evaluation for nine Federal Motor Vehicle Safety Standards: Task VIII - FMVSS 301, fuel system integrity - determination of the model year when manufacturers made changes to meet Standard's 1976 requirements. Final report.

National Highway Traffic Safety Administration, Washington, D.C.

Final report of a 1991 Plymouth Acclaim rear impact CNG fuel tank integrity.

National Highway Traffic Safety Administration, Washington, D.C.

A 1992 Dodge Ram B250 van rear impact CNG fuel tank integrity. Final report.

National Highway Traffic Safety Administration, Washington, D.C.

An analysis of fires in passenger cars, light trucks, and vans.

National Highway Traffic Safety Administration, Office of Research and Development, Washington, D.C.

Automobile fuel tanks.

Mode of access: Internet.

Evaluation methodology for federal motor vehicle safety standards. Volume I: executive summary. Final report.

National Highway Traffic Safety Administration, Washington, D.C.

Evaluation methodology for federal motor vehicle safety standards. Volume II: technical findings. Final report.

National Highway Traffic Safety Administration, Washington, D.C.

Evaluation methodologies for four federal motor vehicle safety standards. FMVSS 214: side door strength. FMVSS 301: fuel system integrity. FMVSS 215: exterior protection. FMVSS 208: occupant crash protection. Final report.

National Highway Traffic Safety Administration, Washington, D.C.

Review of four federal motor vehicle safety standards: FMVSS 214, 215, 301, 208. Task #1 report.

National Highway Traffic Safety Administration, Washington, D.C.

Final design and implementation plan for evaluating the effectiveness of FMVSS 301: fuel system integrity. Tasks #4 & #5 report.

National Highway Traffic Safety Administration, Washington, D.C.

Rear end structural crashworthiness of unitized construction vehicles - summary report. Final report.

National Highway Traffic Safety Administration, Washington, D.C.

Multidisciplinary accident investigation - school bus locomotive-caboose collision, Lafayette, Oregon, September 8, 1976. MDAI team report.

National Highway Traffic Safety Administration, Washington, D.C.

Layered silicate nanocomposites based on various high-functionality epoxy resins. Part II: The influence of an organoclay on the rheological behavior of epoxy prepolymers

The effect of an organically surface modified layered silicate on the viscosity of various epoxy resins of different structures and different functionalities was investigated. Steady and dynamic shear viscosities of the epoxy resins containing 0-10 wt% of the organoclay were determined using parallel plate rheology. Viscosity results were compared with those achieved through addition of a commonly used micron-sized CaCO3 filler. It was found that changes in viscosities due to the different fillers were of the same order, since the layered silicate was only dispersed on a micron-sized scale in the monomer (prior to reaction), as indicated by X-ray diffraction measurements. Flow activation energies at a low frequency were determined and did not show any significant changes due to the addition of organoclay or CaCO3. Comparison between dynamic and steady shear experiments showed good agreement for low layered silicate concentrations below 7.5 wt%, i.e. the Cox-Merz rule can be applied. Deviations from the Cox-Merz rule appeared at and above 10 wt%, although such deviations were only slightly above experimental error. Most resin organoclay blends were well predicted by the Power Law model, only concentrations of 10 wt% and above requiring the Herschel-Buckley (yield stress) model to achieve better fits. Wide-angle X-ray measurements have shown that the epoxy resin swells the layered silicate with an increase in the interlayer distance of approximately 15 Angstrom, and that the rheology behavior is due to the lateral, micron-size of these swollen tactoids.