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Strachan’s syndrome in the UK population
IntroductionStrachan’s syndrome was originally described in Jamaican plantation workers in 1887. Affected individuals exhibit sensory, optic and auditory neuropathy. A nutritional cause has been suggested, and supported given its similarities to other endemic neuropathies, including in Cuba, Tanzania and Somalia. It is commonly seen in British Black African and Caribbean individuals, including siblings, following dietary restriction or acute illness leading to catabolic stress.MethodsThis study aims to characterise the clinical features and genetic profiles of a cohort of UK patients with Strachan’s syndrome and to increase recognition of the syndrome amongst UK neurologists.ResultsThirty patients including two sets of unrelated siblings were identified with a clinical diagnosis of Strachan’s syndrome. Plasma acylcarnitine and urine organic acid profiles measured acutely revealed elevated short, medium and long chain acyl carnitines. Muscle biopsies revealed a reduction in mito- chondrial DNA copy number and ragged red fibres in some individuals. Perivascular inflammatory cell infiltrate was evident in sural nerve biopsies.ConclusionStrachan’s syndrome may result from riboflavin deficiency. The occurrence of the syndrome in siblings and high prevalence in the UK Jamaican community suggests a genetic predisposition. We propose a low index of suspicion and early treatment with high dose B vitamins and riboflavin.
Journal Article
On Brothers and Sisters: South Asian and Japanese Idea Systems and their Consequences
The role expectations of cross siblings varies across culture. Such expectations, while not rigidly prescribing actual behaviors nevertheless influences relations between brothers and sisters in observable ways. In South Asia, a cultural rhetoric of sororal sacrifice and support coupled with fraternal protection are commonplace. While such noble sibling roles are regularly transgressed they remain powerful idioms of the relationship and transgressions require appropriate cultural justification. In contrast, Japanese rhetorical roles lack such explicit sacrifice-protection expectations between cross sibling interactions and instead include more competitive and conflictual idealized models of cross sibling behaviors. Looking at narrative accounts of cross siblings in ancient texts in South Asia and Japan as well as contemporary rituals and observed sibling interactions, this paper argues that the cross sibling relationship must be understood as part of an assemblage of cultural idea systems which inform behaviors, beliefs and attitudes in individuals.
Journal Article
Rules
Twelve-year-old Catherine just wants a normal life. Which is near impossible when you have a brother with autism and a family that revolves around his disability. She's spent years trying to teach David the rules from \"a peach is not a funny-looking apple\" to \"keep your pants on in public\"--In order to head off David's embarrassing behaviours. But the summer Catherine meets Jason, a surprising, new sort-of-friend, and Kristi, the potential next-door friend she's always wished for, it's her own shocking behaviour that turns everything upside down and forces her to ask: What is normal?
Batsheva Sadan's tale of life after her parents' murder by terrorists
Through the book, as she describes her parents' path to a living Jewish faith, we are introduced to a couple, Dina and Eli Horowitz, who embody such joyous results in their search for Yiddishkeit. How is she able to express herself in a fashion which allows us to plunge into the depths of her soul? [...]we understand in a most significant description how she and her siblings feel such piercing sadness, but the presence of their parents is there in a most unusual deja vu moment.
eBook
The passenger
\"Pass Christian, Mississippi, 1980: It is three in the morning when Bobby Western zips up the jacket of his wet suit and plunges from a Coast Guard tender into darkness. His dive light illuminates the sunken jet, nine bodies still buckled in their seats, hair floating, eyes devoid of speculation. Missing from the crash site are the pilot's flight bag, the plane's black box, and the tenth passenger. But how? A collateral witness to machinations that can only bring him harm, Western is shadowed in body and spirit--by men with badges; by the ghost of his father, an inventor of the bomb that melted glass and flesh in Hiroshima; and by his sister, the love and ruin of his soul\"--Dust jacket flap.
Book
EL YO FABULADO. NUEVAS APROXIMACIONES CRÍTICAS A LA AUTOFICCIÓN
En la anglosajona, sin embargo, no tuvo éxito. La primera razón que aduce ya implica que según Alberca le ha llegado la hora: \"La autoficción simboliza, para bien y para mal, con basbastante exactitud el espíritu de una época, el de las cuatro posmodernas décadas (del post-68 a 2007), que acaban con la crisis económica de 2008, cuyo hito inaugural lo puso la quiebra de Lehman&Brothers\" (p. 152). Mientras que Alberca caracteriza la autoficción como frívola, Domingo Ródenas de Moya sale en su defensa: \"La autoficción no es un género \"poco serio\" (Darrieusecq 1996: 369-380), y menos una forma de escritura frívola sino todo lo contrario: es un modo de intensificar en el lector la sensación de que se enfrenta con los aspectos oscuros, perturbadores, conflictivos de la realidad [...]\" En la última contribución a la segunda parte, Daniel Mesa Gancedo presenta un corpus exhaustivo de lo que llama el \"diario autoficcional\" latinoamericano (con excepción de lo argentino).
Journal Article