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“That venerable and princely custom of long-lying abed”
Published in James Kennaway, Rina Knoeff, Lifestyle and Medicine in the Enlightenment, 2020
Over the course of the eighteenth century, the concern that dissolute living habits were ruinous to health developed into a concern that they were threatening the very foundation of masculine identity. This anxiety took form in the stereotype of the “fop.” Appearing in the last decade of the seventeenth century, the fop was a successor to the Jacobean gull or gallant, sharing his desire to be thought refined in upper-class urban society alongside his inability to please. He was obsessed with appearance, spending large amounts of money on clothes and much of his time at fashionable resorts. Whereas the gull described in Dekker’s guide had only St. Paul’s Walk (the central isle of St. Paul’s Cathedral) to show off in, by the late seventeenth century, the fop had a greater range of public spaces for polite display. The coffee house, the theatre (where he could appear in a side-box) and the public park had become haunts of the urban socialite. The antisocial nature of the fop’s conduct stemmed from his narcissism, which was based in an excessive self-consciousness about the rules of politeness and a trivial approach to civility. Failing to understand that the purpose of civility was to ease social interaction, his showiness betrayed a lack of awareness of the needs of others or of what he owed to wider society (Carter, 1997).
Burns
Published in Tor Wo Chiu, Stone’s Plastic Surgery Facts, 2018
By definition, heterotopic ossification (HO) is the formation of lamellar bone in soft tissues where it does not normally form. Myositis ossificans (MO) is HO in muscles and other soft tissues, and strictly it is a misnomer and should be termed fibrodysplasia ossificans progressiva (FOP). It can occur with or without previous injury. FOP is a rare AD disease that begins at age 5 on average. Non-hereditary MO is uncommon in children and occurs from direct muscle trauma with ossification confined to the muscle. Ectopic calcification is mineralisation but histologically is not bone.
Chordoid Glioma, Angiocentric Glioma, and Diffuse Midline Glioma
Published in Dongyou Liu, Tumors and Cancers, 2017
Diffuse midline glioma (and DIPG) is linked to chromosomal and genomic abnormalities, including (i) somatic mutation in the histone H3.1 (H3F3A) or H3.3 (HIST1H3B) genes encoding the histone H3 variants, H3.1 and H3.3; (ii) activating mutation in the activin A receptor, Type I (ACVR1) gene, which may cause the autosomal dominant syndrome fibrodysplasia ossificans progressiva (this mutation often occurs concurrently with H3.3 mutations); (iii) receptor tyrosine kinase (PDGFRA) amplification; and (iv) TP53 gene deletion on chromosome 17p. Interestingly, in diffuse midline glioma (and DIPG), histone H3-K27M mutation is mutually exclusive with IDH1 mutation and epidermal growth factor receptor (EGFR) amplification, as well as BRAF-V600E mutation. H3K27 alterations appear to be the founding event in DIPG and the mutations in the two main histone H3 variants drive two distinct oncogenic programs with potential specific therapeutic targets [1,2].
Druggable targets, clinical trial design and proposed pharmacological management in fibrodysplasia ossificans progressiva
Published in Expert Opinion on Orphan Drugs, 2020
Robert J. Pignolo, Frederick S. Kaplan
Fibrodysplasia ossificans progressiva (FOP) is an ultra-rare genetic disorder characterized by heterotopic ossification (HO), congenital skeletal abnormalities especially of the great toes, and several features of accelerated aging [1–5]. HO in FOP forms at extra-skeletal sites in soft connective tissues, including muscle, tendons, ligaments, fascia, and aponeuroses [1]. Progression of the condition is relentless and occurs clinically via episodic inflammatory exacerbations or flare-ups and/or spontaneously (without flare-ups). The onset of flare-ups is unpredictable, but usually occurs by age five and generally in anatomical patterns involving progression from axial to appendicular sites, dorsal to ventral areas, cranial to caudal regions and within a given site (e.g., arm or leg) proximally to distally [6]. The formation of HO results in partial or complete joint ankyloses, ultimately rendering movement impossible. Life expectancy in FOP is about 56 years of age and death is often the result of cardiopulmonary complications [7].
Induced degradation of protein kinases by bifunctional small molecules: a next-generation strategy
Published in Expert Opinion on Drug Discovery, 2019
Of note, Levi and coworkers showed that knockdown of any one of the three ALK-encoding genes did not inhibit trauma-induced HO (tHO), however, combined ablation of ALK2 and ALK3 produced near-total elimination [81]. Thus, for treatment of non-genetic tHO patients, broadly selective therapeutics targeting two or all three of the redundantly acting type I BMP receptors, or an ALK2-selective in combination with an ALK3- and/or ALK6-selective compound, would be necessary. Such a therapeutic might be tolerated for the treatment of acute cases, whereas for FOP, which is a life-long, chronic affliction, a highly selective ALK2-targeting drug would almost certainly be required. Ideally, the drug could be administered as a prophylactic against the stepwise accumulation of mobility-arresting heterotopic bone.
Anaesthetic considerations in a child with fibrodysplasia ossificans progressiva
Published in Southern African Journal of Anaesthesia and Analgesia, 2019
Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterised by extraskeletal ossification of connective tissue such as tendons, ligaments and the connective tissue in skeletal muscle.1–3 Affected individuals are often completely immobilised by their third decade of life.3 As the disease progresses, complications may arise secondary to thoracic insufficiency syndrome.4 However, in young children, the main anaesthetic challenge may prove to be the airway management.3,5,6 Only a few reports on the anaesthetic management of young children with FOP exist.6 This case report describes the anaesthetic management of a three-year-old girl with FOP.