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Query: UMLS:C0004093 (asthenia)
 2,650 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cutaneous asthenia is a hereditary connective tissue disease, primarily of dogs and cats, resembling Ehlers-Danlos syndrome in man. Collagen dysplasia results in skin hyperextensibility, skin and vessel fragility, and poor wound healing. The purpose of this study was to describe the histological findings in a dog with a collagenopathy consistent with cutaneous asthenia. An 8-month-old crossbreed female dog presented with lacerations and numerous atrophic and irregular scars. The skin was hyperextensible and easily torn by the slightest trauma. Ultrastructurally, the dermis was comprised of elaunin and oxytalan microfibrils. These are immature fibres in which the fibrillar component is increased but elastin is reduced. Collagen fibres were profoundly disorganized. The fibrils had a highly irregular outline and a corroded appearance when viewed in cross-section, and were spiralled and fragmented in a longitudinal view. Dermal fibroblasts displayed a conspicuous thickening of the nuclear lamina. Nuclear lamins form a fibrous nucleoskeletal network of intermediate-sized filaments underlying the inner nuclear membrane. Mutations in lamins or lamin-associated proteins cause a myriad of genetic diseases collectively called laminopathies. Disruption of the nuclear lamina seems to affect chromatin organization and transcriptional regulation of gene expression. A common link among all laminopathies may be a failure of stem cells to regenerate mesenchymal tissue. This could contribute to the connective tissue dysplasia seen in cutaneous asthenia.
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PMID:Increased elastic microfibrils and thickening of fibroblastic nuclear lamina in canine cutaneous asthenia. 1919 63

Hereditary equine regional dermal asthenia (HERDA) is an autosomal recessive skin disorder that has yet to be fully characterized. HERDA is predominately expressed in Quarter Horses, with the majority of these disseminating from elite cutting horse bloodlines, leading to the increased incidence of HERDA in recent years. Affected horses have loose, hyper-extensible, fragile skin and are frequently euthanized due to poor wound healing and disfiguring scars. This study sought to better characterize HERDA by analysis of the biomechanical parameters of tensile strength, modulus of elasticity, energy to failure and thickness of skin from 10 affected and 6 unaffected horses using an Instron Universal Testing Instrument. In addition, total soluble collagen and glycosaminoglycan concentrations of skin were analysed from 13 affected and 12 unaffected horses using Sircol Soluble Collagen and Blyscan Sulfated Glycosaminoglycan assays respectively. Affected horses exhibited a two to threefold reduction in tensile strength versus unaffected horses with statistically significant differences at six of seven sample locations (P < or = 0.05). The modulus of elasticity proved to be significantly different at six of seven sample locations, energy to failure at six of seven sample locations, and skin thickness at one of seven sample locations (P < or = 0.05). Affected horses exhibited significantly higher amounts of total soluble collagen than unaffected horses (P < or = 0.05). No significant difference was demonstrated between groups for glycosaminoglycan concentration. Affected horses demonstrated uniformly weaker skin across sample locations, indicating the biomechanical properties of HERDA are not regionally confined to specific areas of the horses' skin.
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PMID:Biomechanical and molecular characteristics of hereditary equine regional dermal asthenia in Quarter Horses. 2017 99