Middle Sister Research Articles

Contrasting magmatic evolutions of the Three Sister Volcanoes reflect increased heat flow, crustal melting and silicic magmatism in the Central Oregon Cascade Arc

The Three Sisters stretch, one of the most magmatically active segments of the Oregon Cascade Arc, includes the Three Sisters volcanoes proper (North, Middle and South) as well as the shields and cinder cones of the Mount Bachelor Chain and McKenzie Pass. The Three Sisters record a magmatic transition from fundamentally basaltic andesitic activity in older North Sister products to more diverse activity at Middle Sister, and unusually silicic activity in a Cascade volcano, including rhyolite lava, at South Sister.North Sister magmas reflect magmatic evolution of basaltic andesite through crystal fractionation, mostly of olivine, and incorporation of crustal melts. Middle Sister, although mostly composed of basaltic andesite and andesite, produced dacitic magma through assimilation fractional crystallization processes involving incorporation of silicic melts. South Sister developed a subjacent magma chamber under the southern half of the volcano, where andesitic magmas evolved, dominantly through fractional crystallization, to dacite and rhyolite. A prominent compositional gap at South Sister between dacite and rhyolite and depletion of middle and heavy rare earth elements in rhyolite suggest that amphibole was prominently involved in fractionation processes involving separation of silicic magma from a gabbroic or amphibolitic crystal mush. Silicic magma eventually formed a stagnant cap in the system, where it evolved through small scale fractional crystallization. Rhyolite magma was finally heated to elevated temperatures prior to eruption, presumably through influx of less evolved magma into the base of the stagnant silicic cap and was erupted in late rhyolite lava of Rock Mesa and the Devil's Hill Chain.South Sister lies along strike of the Northwest Rift of Newberry Volcano, which forms the northwestern terminus of the High Lava Plains trend of south-central Oregon. The increased magmatic activity of the Three Sisters stretch and unusual abundance of silicic products, including rhyolite, of South Sister may reflect the arrival and intersection of the High Plains magmatic anomaly with the Cascade Arc.

Spatial violence and everyday borders in contested cities: Literary representations of walking in Anna Burns’s Milkman

This article examines the representation of walking and the narrativization of borders in Troubles-era Belfast in Anna Burns’s 2018 novel Milkman. I argue that the protagonist, Middle Sister, develops her own narrative form and walking method as ‘tactics’ to challenge the city’s imposed sectarian geographies and as a response to navigating a city of intense surveillance. In her narrativization, Middle Sister replaces place references with her own complex naming system and lexicon and negotiates urban space by ‘reading-while-walking’. As a result, Middle Sister attempts to dislocate the political nature of the conflict by mediating Belfast on her own terms and asserting her own spatial practices. However, as I demonstrate in the final section in conversation with trauma theory, there are limitations to Middle Sister’s walking practice, which is disrupted by the urban hauntings of her troubled city, triggering particular psychosomatic responses and traumatic memories.

From Sarah to Sydney: The Woman Behind All-of-a-Kind Family by June Cummins

Reviewed by: From Sarah to Sydney: The Woman Behind All-of-a-Kind Family by June Cummins Lois Rauch Gibson (bio) From Sarah to Sydney: The Woman Behind All-of-a-Kind Family. By June Cummins with Alexandra Dunietz. Yale University Press, 2021. The long-awaited biography of Sydney Taylor, by the late June Cummins, will be warmly received by all who have been waiting. Cummins's extensive research and access to family papers and interviews make From Sarah to Sydney: The Woman Behind All-of-a-Kind Family a definitive, scholarly biography. Despite the impressive forty pages of endnotes and seven-page bibliography, Cummins's book will also attract more general readers and fans of Taylor's books, like novelist Jennifer Weiner, who reviewed it for the New York Times (June 22, 2021 digital; July 4, 2021, print edition). Like Weiner, and like Cummins herself, I read and loved the All-of-a-Kind Family books as a child. Set in the early twentieth century, and published from the 1950s to the 1970s, the books appealed especially to readers of Wilder's Little House books and the Betsy-Tacy series by Maud Hart Lovelace. But Taylor's books are about the immigrant experience in New York, and the family is Jewish. As Cummins notes in her introduction, these were the first books by a major publisher "to feature Jewish children and reach a widespread audience" (22). Though the first few chapters of the first book focus on events many children might experience—a lost library book, Mama's clever way of getting the girls to do household chores, a visit to Papa's junk shop, and a trip to buy candies with their saved-up pennies—the fifth chapter is about celebrating the Jewish sabbath. Cummins notes at several points that Taylor's editor wanted the books to seem more "American" than specifically Jewish. She insisted on changes to make this so. But Cummins also notes in her introduction that "Understanding why the series had such an impact requires knowledge of the historical and cultural environment in which its author grew up and the circumstances that shaped its publication" (22). She promises, "This biography not only recounts the life of a daughter of immigrants who achieved artistic and cultural success, but also shows how" her life and the books are directly connected to "the history of Jewish identity and of ethnic identity in children's books" (22). Cummins masterfully delivers on this promise. In each chapter, Cummins provides an astounding amount of background information, starting with the family history in chapter 1, which takes us back to nineteenth-century Germany and the Austro-Hungarian Empire, where Taylor's parents grew up, met, [End Page 109] and married. In the chapter discussing All-of-a-Kind Family Downtown, we learn about Lillian Wald and the Henry Street Settlement. In the sections on Taylor leaving school, Cummins provides data on school attendance rates; and later, as Taylor selects schools for her daughter, Cummins enriches the section with the histories of the Bank Street College and Cooperative School for Teachers. Even the section on Cejwin, a summer camp where Taylor taught drama and dance for forty years, explores the history and background of the camp and its place in Jewish culture. This is indeed a biography that delves deeply into the life of Sydney Taylor as well as the world in which she lived. Many of Taylor's readers assume the stories are about her own family—as they are, to some extent. Taylor's parents, Cilly (Cecelia) Marowitz and Morris Brenner, were the prototypes for the parents; and the five sisters (Ella, Henny, Sarah, Charlotte, Gertie), who are all dressed alike (all of a kind) in immaculate outfits sewn by their German-born mother, were indeed based on Taylor and her sisters. Taylor was born Sarah Brenner and was the middle sister. But readers of the biography will discover that Taylor smoothed over the wrinkles in family life, just as her mother smoothed the wrinkles in the girls' dresses. While Mama in the books is firm but loving, and more elegant that the other mothers in their immigrant neighborhood, Cilly was...

This Essay is a Manipulation of Matter

This Essay is a Manipulation of Matter Jax Connelly (bio) POINT OF ORIGIN: The fuck yous are booming through my sun-dappled neighborhood, threatening to shake branches from trees. The man is doubling over with rage, halting every few feet to bend his imposing body in two, hold his phone in front of his face and fuck you into it as if it has taken something from him he'll never be able to recover. It was a compliment, he's bellowing, over and over, so many times the words become a bassline: dada-da-DA-da-da-da-da-da-DA-da-da. It was a compliment it was a compliment it was a compliment fuck you fuck you you fucking bitch. COMPONENT: Seated on a block of cement, she is exactly as tall as I am. Her thighs are pressed together, legs stiff, knees bent at ninety degrees, toes pointed toward the floor. Her lap, wooden and flat and skeleton-narrow—uninviting. She sports a bland checkered coat, black squares alternating with grey, some tan, some brown. She leans backward, away from herself, arching her back like bark splayed out from a droughtracked tree, and where her sternum should be, an explosion: strips fanning out from her chest like a child's interpretation of a lion's mane, or Medusa's snakes scared still and straight, or the flames of a paper sun. Margaret Wharton's sculpture Leopatra is not a crime scene, just dissonance—what the body remembers and what it presents, contradictions occupying the same space because they have nowhere else to go. Half neatly composed—the dainty feet, the polite posture—but before the gaze even reaches her neck, she's come undone. She's lost her head and she's lost her hands, so here is her heart, revealing all the things she can no longer speak, or hold. COMPONENT: I once watched a woman's head explode. In the arthouse sci-fi film High Life, a shuttle's trajectory is altered by a molecular cloud and subsequently dives into a black hole. The pilot, Boyse, dies by spaghettification—the vertical stretching and horizontal compression of objects into long, thin shapes in a non-homogeneous gravitational field. This does not happen in an instant. The scene is impossibly slow, Boyse's face beginning to crumple almost imperceptibly, her brain unspooling like it's reluctant to let go. Earlier, Boyse is violently raped. As part of a crew of death-row inmates sent on a mission to extract energy from the black hole, she sleeps with her limbs strapped to her bedframe. She wakes up and it's already happening, she wakes up and understands immediately, in that waterline strip between unconsciousness and consciousness, that there's nothing she can do to stop it. Google tells me spaghettification is "caused by extreme tidal forces." According to some dude on a Quora forum, the process isn't painful: "The black hole will pull you so fast that electrons transmitting [End Page 115] the pain signals will not be strong enough to escape the gravitational pull in order to make it to the brain." POINT OF ORIGIN: My dog and I are close enough, now, to observe how the wrath has colored the man's face slap-red, how it's screwed his features in tight like they're bolted to a table. Carefully, I avert my eyes. I hold my breath. I move my limbs stiffly, quietly, I don't want him to notice me, but I can't help flinching as he passes by, not noticing me at all: da-da-da-DA-da-da-da-da-da-DA-da-da da-dada-DA-da-da fuckyoufuckyoufuckyou. COMPONENT: In her novel Milkman, which won the Man Booker Prize in 2018, Anna Burns explores the consequences of a disconnect between language and experience—what happens when we don't have words to describe and transcend trauma. It's Northern Ireland in the 1970s, and the nameless narrator, middle sister, is stalked by a paramilitary known only as the milkman. He doesn't explicitly threaten violence but shows up unannounced and uninvited when she's alone and...

“Systemic, Transhistoric, Institutionalized, and Legitimized Antipathy”: Epistemic and Sexual Violence inA Girl Is a Half-formed ThingandMilkman

AbstractThis article concerns the interrelations of epistemic and physical gendered violence in two award-winning contemporary Irish novels, A Girl Is a Half-formed Thing (2013) and Milkman (2018) by Eimear McBride and Anna Burns, respectively. It argues that patriarchal naming power which defines women as sexual objects is inextricable from the physical violence featured in both texts. Using theories of violence and naming, it examines how, faced with a climate of constant and submerged sexual threat, “middle sister” (as the narrator is called) and “Girl” (the protagonist of A Girl Is a Half-formed Thing) attempt to appropriate dominant patriarchal narratives to retain subjectivity in the face of violence. While “middle sister” comes to recognize and articulate the violence done to her, Girl’s internalization of violence leads to the annihilation of both self and body.

Anna Burns’ Milkman

Anna Burns’ Milkman Daragh Downes There are, to misquote Oscar Wilde, two ways of dislikingAnna Burns’novel Milkman (2018). The first is to dislike it, the second is to praise it loudly for its bold experimentalism. Point one may be briskly disposed of with the banality that there is no accounting for taste. Point two requires a little more elucidation. That Milkman throws stylistic and narrative convention to the winds is beyond question. A novel that begins with the words, ‘The day Somebody McSomebody put a gun to my breast and called me a cat and threatened to shoot me was the same day the milkman died’, can hardly be said to scream ‘business as usual’. The word ‘experimental’, however, is pure kryptonite in the book trade, being a byword for ‘difficult’, ‘inaccessible’, ‘grist to the academic mill’. To dub Milkman’s antic style experimental, as so many did upon its publication and – especially – after its Man Booker Prize nomination and subsequent win, is thus about the most unhelpful way conceivable of welcoming it into the world. Whether used with good intent or ill, the word puts many potential readers off, depriving them of a remarkably refreshing and addictive reading experience. To apply this word to Milkman is to belie the wonderful democratic spirit behind Burns’ stylistic game. It is also to miss the fact that, in terms of her own literary development, Milkman does not in truth represent much by way of an experiment. Already in her superb first novel, No Bones (2001), we find the seeds of the signature Milkman style, especially in those parts where she ventures beyond the confines of traditional narrative realism (which latter mode, and it is useful to mention this when in conversation with a Burns sceptic, she masters with consummate facility). In her follow-up, Little Constructions (2007), a novel set in a small town and treating of a deranged crime family, we see her commit fully to what we now recognise as the Milkman style: that inimitable mixed economy of outré-absurdist storytelling; garrulous essayism lubricated by psychobabble, sociologese and bureaucratese; arch observational wit; sovereignly controlled irony; obsessive-compulsive repetition; and many a sentence that sounds like it has been written in a foreign language and run Studies • volume 110 • number 438 231 through a beta version of Google Translate. There is even a character called Mr McSomebody (proprietor of a local pharmacy known as ‘Almost Chemist of theYear’). To encounter this style for the first time in Little Constructions is exciting, but it still feels there like a style in search of the right story. One gets the same impression from Mostly Hero (2014), a novella published before Burns had finished work on Milkman and whose opening sentence – ‘The villains from downtown eastside put a magic spell on femme fatale so that she would kill superhero whilst under the influence of this magic spell’– tells us all we need to know about its commitment to soberly realist nomenclature and storytelling. No Bones and Milkman may be Burns’ two great works to date, but Little Constructions and Mostly Hero tell the fascinating story of how her pen was fully freed up in the way that was to make her famous. Milkman is in part a delightfully unfashionable love letter to fiction itself, and to the counter-cultural power of fiction to offer relief to the lonely psyche wounded from having to live in a ‘hair-trigger society’ in which politics has totalistically and micrologically invaded every action, gesture, word and even thought. (It should be prescribed reading for anyone who spends too much time on Twitter). The novel’s first-person narrator – named only as ‘middle sister’ – finds that she has become an unsettling figure in the community by mere dint of her idiosyncratic habit of reading-while-walking. This habit, as her paramilitary-connected best friend from schooldays coolly informs her over a quiet drink, has led the community to ‘pronounce[] its diagnosis’ on her as a freak whose public immersion in pre-twentieth-century novels is ‘disturbing… deviant… optical illusional’ and ‘[n]ot public-spirited’. No one, the friend points out, ‘should go around in a political scene with...

Surviving the Oppression in the Time of the Troubles: Anna Burns’s Milkman

Anna Burns’s Milkman (2018) recounts the story of an eighteen-year-old woman, named the middle sister, who struggles to survive the communal oppression and physical and psychological violence at the time of the Troubles in the Northern Ireland in the 1970s. The novel revolves around the middle sister’s tumultuous relationship with her family, boyfriend, and neighbours, and her attempts to avoid the stalking and sexual harassment of a Republican paramilitary, named Milkman. Accused of being different due to her habit of reading-while-walking, the middle sister is perceived as a threat to the community and stigmatised by the people. Demonstrating the operation of the communal oppression from the eyes of a young woman, Milkman also portrays in the background the violent atmosphere of the Troubles and its trauma experienced by the individuals. While the middle sister strives to protect herself from the neighbours’ gossips and calumny, she also tries to avoid Milkman’s threats and abuse. The middle sister, who struggles to survive in such an environment prevailed by oppression and abuse, also witnesses the general sense of paranoia and fear instilled in people’s minds due to the constant threat that the political conflicts and violence pose on the society. Therefore, this article aims to examine how the novel displays the working of violence and oppression in this tense and dreary atmosphere, and discuss how oppression is performed in every social layer, from a small neighbourhood to the whole country, both physically and psychologically.

“북아일랜드 분쟁” 쓰기: 애나 번즈의 밀크맨

This article aims to read Anna Burn’s Milkman, winner of 2018 Man Booker Prize, as a story of the Troubles in Northern Ireland. Even though the main event of the story occurs in Ulster in the late 1970s, it also covers “the Bloody Sunday” and many other tragic moments that happened during the period. To reflect on the meaning of the Troubles, the story employs experimental narrative techniques such as the odd naming practices; characters in this novel lack proper names; the protagonist is “middle sister”; feminists are “the women with issues.” Likewise, the political divisions and nationalities are not specified: the places are just indicated as “over the border,” “over the water,” and “over the road,” thereby making this story more universal. Milkman contains plenty of insights on sexuality and masculinity. The distorted view of sexuality and fixed gender ideals are inextricably connected with the brutal atmosphere of the community controlled by paramilitaries and their ideological causes. However, Milkman is also a Bildungsroman focusing on the way the protagonist resolves her difficulties and finally reunites with her family and the community. At this point, the women community, “traditional women” as well as “the issue women,” provides a crucial role to “middle sister,” helping her to start the next stage of her life. The narrative techniques are also worth special attention. The main plot, plus plenty of diverting episodes painstakingly analyze the internal social conditions and emotional states. It is the mordant humour, which can be associated with “women’s writing,” that makes it possible to resist and overcome the brutality and trauma during the Troubles.

City Folk and Country Folk by Sofia Khvoshchinskaya

Reviewed by: City Folk and Country Folk by Sofia Khvoshchinskaya Svetlana Grenier CITY FOLK AND COUNTRY FOLK, by Sofia Khvoshchinskaya, translater from Russian by Nora Seligman Favorov. Russian Library. New York: Columbia University Press, 2017. 272 pp. $30.00 cloth; $14.95 paper; $13.99 ebook. Nora Seligman Favorov, Hilde Hoogenboom, and Columbia University Press are to be commended for bringing us this delightful novel from 1860s Russia. Sofia Khvoshchinskaya's powers of observation, her light touch, ironic tone, and representations of down-to-earth human goodness are reminiscent of Jane Austen. Favorov's translation adroitly captures the sparkling humor and sophisticated irony of the original, and Hoogenboom's erudite introduction, providing biographical detail on the author and an extensive bibliography, firmly sets the novel in its historical and literary context. Sofia Khvoshchinskaya (1824-1865), who published under the masculine pen name Iv. Vesenev, was the middle sister in a trio that has [End Page 238] been considered a Russian counterpart to the Brontës. Her elder sister Nadezhda (1822-1889) was famous and sought-after in the second half of the nineteenth century as the author V. Krestovsky, and the less well-known Praskovia (1828-1916) printed her works also under a pseudonym, albeit as the feminine S. Zimarova. All three Khvoshchinskaya sisters contributed to the major journals of the time and were accomplished artists, who spent most of their life in the provincial capital of Ryazan. Before her death at the age of forty-one, Sofia instructed Nadezhda not to write about her or republish her work, which is why she has remained relatively unknown. Nadezhda's story of a young woman's quest for independence, The Boarding-School Girl (Pansīonerka, 1861), was published in an English translation by Karen Rosneck in 2000. The very different City Folk and Country Folk (Gorodskie i derevenskie, 1862) is a welcome addition to the still sparse list of translated works by nineteenth-century Russian women. It significantly adds to our idea of the diversity of their writing styles and world views, and it provides a new and refreshing perspective on the provincial Russia we know from Ivan Turgenev's novels such as Fathers and Sons (1867; Ottsy i deti, 1862), written almost simultaneously to City Folk and Country Folk. Khvoshchinskaya's novel radically breaks with several traditions prevalent in male-authored Russian literature and reinterprets a few others. First, the central character is not a man, nor even an exceptional young woman, but Nastasya Ivanovna Chulkova, a fifty-five-year-old widow and a small-time landowner. Second, although Nastasya has a seventeen-year-old daughter, Olenka, there is no romance plot to speak of in the novel. Third, Olenka, unlike a typical Russian heroine, is an average high-spirited girl—not a romantic dreamer and voracious reader, like Alexander Pushkin's Tatyana in Eugene Onegin (1936; Evgeniĭ Onegin, 1833), or an idealist looking for ways to make a difference in the world, in the mold of Turgenev's Elena Stakhova in On the Eve (1895; Nakanune, 1859). Olenka dislikes reading and studying; she is thoroughly average, intellectually speaking. At the same time, she is observant and quick to discover the ridiculous side of characters and situations. What is more, she does not let others order her and her mother around. One wonders if the author meant her as a polemical response to Pushkin's Olga, Tatyana's sister. The novel introduces Khvoshchinskaya's satirical take on the superfluous man, a perennial Russian literary archetype found in the novels of such authors as Alexander Herzen and Turgenev, who invariably considers himself far superior to all of the surrounding "undeveloped" Russians. In City Folk and Country Folk, Mr. Ovcharov, who graduated from Moscow University and regularly travels to Europe, thinks of himself as a progressive intellectual and is eager to share his ideas with supposedly backward provincials and readers in the capitals alike (he sends his stream-of-consciousness [End Page 239] notes to journals, and they sometimes get published). In a typical plot, the superfluous man arrives in the provinces where, in the process of spreading his novel ideas and enlightening the "natives," he manages to attract a young woman...

Sassafrass, Cypress & Indigo Revisited: The Intersections of Politics, Culture, and Self-Development

CLA JOURNAL 151 Sassafrass, Cypress & Indigo Revisited: The Intersections of Politics, Culture, and Self-Development Jacqueline Jones In an interview that appears in Claudia Tate’s Black Women Writers at Work, novelist, poet, and dramatist Ntozake Shange states that “if there is an audience for whom I write, it’s the little girls who are coming of age. I want them to know that they are not alone and that we adult women thought and continue to think about them” (162). Shange’s first novel Sassafrass, Cypress & Indigo (1982) does just that. Set in Charleston, South Carolina in the 1960s and 1970s, the bildungsroman charts the effects shifting cultural, political, and artistic values have on the coming-of-age experiences of sisters Sassafrass, Cypress, and Indigo. The product of several generations of women weavers, the sisters learn to weave, dye cloth, and cook from their mother Hilda Effania. Shange integrates poems, tonics, recipes, letters, and dance choreography into the narrative to provide additional context for the characters’ rich lives. Eldest sister Sassafrass follows in her mother’s footsteps and becomes a weaver; she also is a poet. Middle sister Cypress is a dancer. Youngest sister Indigo plays the fiddle, communicates with the spirits of slaves, and has magical powers. Drawing from black nationalist and black feminist ideologies of the period, Shange constructs the sisters as working toward dismantling various forms of oppression through self-development and redefinition. The sisters look toward African diasporic and women’s cultural practices, their connection to nature and the spirit world, as well as their artistic abilities to create and define individual identities. Sassafrass engages and extends the conversations of black women writers in the 1970s and early 1980s regarding the role black communities play in the lives and self-development processes of black girls and women. Using what we would now consider an intersectional approach, novelists of the period illustrate the effects patriarchy, sexism, violence, and internalized racism have on the development of black girl and women.1 As Barbara Christian asserts in “Trajectories of SelfDefinition ,” novels including Toni Morrison’s Sula (1973) and Alice Walker’s The Color Purple (1982) depict black communities as “a major threat to the survival and empowerment of women” (240). This was significant as black women writers articulated that not only do their experiences with sexual violence, patriarchy, and other issues within black communities require the same urgency as institutional racism in larger society but also being both black and women compounds their marginalization. In these works, then, protagonists challenge community norms as they work toward survival, autonomy, and self-definition. Characters in 152 CLA JOURNAL Jacqueline Jones 1970s novels were likely to find that their endeavors left them isolated from the community or with a limited sense of social inclusion. In several early 1980s novels, the addition of a “strong woman’s community” results in most protagonists surviving, with some experiencing “the possibility of wholeness” (Christian 243). Sassafrass, Cypress & Indigo has generally been linked to this latter category of early 1980s novels in which protagonists create “the possibility of wholeness” through female friendship and or engagement in communities of women. While the novel does depict problematic attitudes and behaviors related to gender and sexuality that pose a threat to black girls and women, it also incorporates instances where the sisters’ community of origin aids or empowers the protagonists’ self-development. Though most of the positive interactions that occur in the novel happen among women, there are also occasions, particularly during the sisters’ childhoods, where the protagonists are empowered by elder men in their community. Furthermore, Shange distinguishes the text from similar works of the time by providing her characters with more than the “possibility of wholeness” (Christian 243). With exposure to a broad array of spiritual, cultural, and political influences, Sassafrass, Cypress, and Indigo learn to internalize the values and lessons that work for them and discard or overcome the ones that do not. The sisters leave home on a quest for education and fulfillment, and they harmoniously return self-actualized and as meaningful participants in their community. As such, through Sassafrass, Shange offers a vision of black women establishing autonomy without sacrificing communal integration, which she...

MYOFIBRILLAR AND DISTAL MYOPATHIES: P.251Family with a new mutation in the DES gene of autosomal recessive transmission

The desminopathies manifest with different types of characteristics, ages of presentation and even with types of inheritance. The reason for this presentation is a family with a description that presents a phenotype and genotype not described. A 14-year-old patient who visited the clinic due to myalgia, exercise intolerance and serum creatine kinase (CK) levels maintained at around 1000 IU/L; the middle sister has the same symptomatology and the older brother is healthy, the great-grandmothers are sister cousins. A non-invasive hyperCKemia protocol is applied, including DBS Pompe and MLPA DYS that are normal. MRI presents a pattern of local involvement (soleus, gastronemius and semitendinosus (grade 1) and paravertebral (grade 2). Muscle biopsy presents a dystrophic profile with vacuoles. In successive years it presents mild palpebral ptosis and minimal facial, scapular and axial weakness (4-). A NGS genetic study with 40 detects a variant in homozygosis not described (c.1372-1G>A) in the DES gene. The variant segregates with a recessive pattern in the family study. Myofibrillar protein markers are abnormal, highlighting Desmin accumulations. Currently the brothers have similar manifestations at 22 and 28 years of age. We describe a new pathological variant in the DES gene (c.1372-1G>A) with AR transmission causing a desminopathy that presents as paucisintomatic HCK and without cardiac alterations.

Eruptive history of Middle Sister, Oregon Cascades, USA–Product of a late Pleistocene eruptive episode

Eruptive history of Middle Sister, Oregon Cascades, USA–Product of a late Pleistocene eruptive episode

Bruja Born by Zoraida Córdova

Reviewed by: Bruja Born by Zoraida Córdova Kate Quealy-Gainer, Assistant Editor Córdova, Zoraida Bruja Born. Sourcebooks Fire, 2018 [336p] ISBN 978-1-4926-5065-2 $17.99 Reviewed from galleys R Gr. 8–12 Surviving the hellish realm of Los Lagos has left seventeen-year-old Lula Mortiz (previously seen in Labyrinth Lost, BCCB 9/16, first title in the Brooklyn Brujas series) with a scarred face, a strained relationship with her family, and a desperate need for normalcy. Any chance at that, however, is lost when her school bus crashes, killing her classmates and her beloved boyfriend, Maks. She and her sisters use their bruja magic to bring Maks back from the dead, but defying Lady de la Muerte has its consequences, one of which is a horde of the zombie-like casimuertos—including Maks—descending upon Brooklyn. Córdova brings the wonder and horror she created in Los Lagos to the streets of New York, easily exchanging Bone Valle for Coney Island and a countryside of wandering souls for hipster-ridden cafes, and deftly expanding the story of the Mortiz family in both setting and character as the perspective shifts from middle sister Alex to older sister Lula. Flesh-eating zombies tend to heighten the excitement of any story, and this one is no different as the casimuertos mangle various characters, but there’s also a bit of philosophizing here, with Lula questioning the purpose of the Deos (the gods in bruja culture) and interrogating Lady de la Muerte. Although Lula seemingly gets off the hook for being the cause of a whole host of murders, the final page introduces another supernatural being and the next adventure for the Mortiz brujas. Copyright © 2018 The Board of Trustees of the University of Illinois

Book of the Generations

Book of the Generations Kelli Jo Ford (bio) Click for larger view View full resolution Beulah Springs, Oklahoma 1974 [End Page 145] 1 When Lula stepped into the yard, the stray cat Justine held took off so fast it scratched her and sent the porch swing sideways. Justine had been feeding the stray, hoping to find its litter of kittens in spite of her mother's disdain for extra mouths or creatures prone to parasites. She tried to smooth cat hair from her lap. She'd wanted everything to be perfect when she told her mom that she'd tracked down her father in Texas and used the neighbor's phone to call him. "That thing's going to give you worms." Lula dropped her purse onto the porch. She hadn't been able to catch a ride from work. With a deep sigh, she untucked her blouse and undid the long green polyester skirt she'd started sewing as soon as she'd seen the Help Wanted sign at the insurance office. She was a secretary now, and as she liked to tell Justine, people called her "Mrs." and complimented her handwriting. "I'll wash up," Justine said. She'd already decided today wasn't the day. Like yesterday. And the day before that. "At least let me say hi." Lula kicked off dusty pumps and let her weight drop into the swing beside Justine. The swing skittered haywire as Lula pulled bobby pins from her bun, scratching her scalp. Her long salt-and-pepper braid fell past her shoulder and curled under her breast. "Bless us, Lord," she said, the words nearly a song. She closed her eyes, and as she whispered an impromptu prayer, she touched the end of her braid to the mole on her lip that she still called her beauty mark. As a girl, Justine had pored over the pictures from Lula's time at Chilocco Indian School, trying to see her mother in the stone-cold fox who stared out from the old photographs. Lula's clothes hung loosely, even more faded than the other girls' in the pictures, but something about her gaze—framed by short black curls, of all things—made it seem as if she were the only one in the photo. If Marilyn Monroe had come of age in an Indian boarding school and had fierce brown eyes instead of scared blue ones, that would have been young Lula. Justine kept the old pictures in a box hidden in the top of the closet where she kept her Rolling Stones and a mood ring, other forbidden things. She hadn't thought of the pictures in ages, but she did now as she watched her mom in prayer. [End Page 146] Lula whispered "Amen," caught Justine staring at her. "Granny's out gathering wild onions with Aunt Celia," Justine said quickly. "Late in the year for it," Lula said. She unrolled her nylon stockings and wiggled her toes in the air. In the way of Cherokee women, Lula could still make you feel that she held down the Earth around her one moment and then seem almost like a girl the next. "Did you do your homework?" "I swept and did the rugs too." "My Teeny," Lula said, calling her the name that had stuck when Justine's middle sister hadn't been able to say her name. Together they pushed the swing back and let it fall forward. Justine closed her eyes. In the cool air that had come with the night's rain, her mother's warmth felt nice, which made the words she'd been practicing feel all the worse. "Evenings like this make me wonder how a body would want to set their bones anywhere other than these hills," Lula said. Justine opened her eyes. The two-bedroom house they rented with her Granny sat on the edge of Beulah Springs, the outer walls almost as much tar paper as asphalt shingle. She had her own room, now that her sisters had married themselves out of state, but her mother and Granny still split a room barely big enough for one. Hand-sewn curtains strung on...

The Doll Family

The Doll Family Rosaleen Bertolino Forbidden to wander their city neighborhood, where their parents said they might be beaten or kidnapped or chopped into tiny pieces, the three girls had no one to play with but each other. Brown-haired and plump, they were stuck together, day in, day out. Carmen, eight years old and the eldest, was bossy; the middle sister, Alice, placid; and the youngest, Bella, boisterous and loud—but those who didn't know the girls well might not be able to tell them apart. They were like bees in a hive, birds in a nest, a set of tea cups. The dollhouse was two levels, with a pitched roof, each room wallpapered, intricately furnished with rugs and framed pictures and candlesticks and miniature bowls of fruit. The childless neighbors who'd built and decorated the dollhouse, a man and woman, accepted small glasses of brandy from the father, observed the three girls as they tentatively touched the tiny beds, the teeny rocking chair that really rocked. Then, unsmiling, they stood and said they must be getting home. "Say thank you," said their mother to the girls. As soon as the grownups left, Carmen moved the dollhouse toilet into the dollhouse kitchen. Alice, who liked to climb, balanced the velveteen sofa and the dining room set on the roof, and Bella gnawed at the miniature bowl of red apples, which were shiny and looked delicious but tasted bitter. By the time the three sisters had finished rearranging the furniture, the dining chairs had snapped to pieces, and the braid rug in the living room, reluctant to give up its position, tore and left threads and a yellowish lump of glue behind. The girls were disappointed in the dolls that had come with the dollhouse—a family molded of rubbery plastic that included their hair and clothes, which meant the father doll had to sleep in his suit and the children to bathe in their school clothes. The mother's apron never came off, nor did her shoes. Imagine, never being naked or barefoot. And imagine their poo and pee. The girls laughed at how disgusting the dolls probably were under their plastic clothes. They tried putting other dolls in the dollhouse, but they were too large. Barbie lay on the floor, too tall to stand up, too big to fit on any of the furniture. She scared the dollhouse family, who ripped off her head and stewed it for dinner. The girls' parents, thin and glamorous with coarse, flyaway hair, had no idea what their children were up to—they were too busy arguing quietly behind their locked bedroom door. The doll parents fought, too, but differently, kicking, slapping, jumping up and down on one another, shouting, "I've had it up to here!" But sometimes they were almost the same as the real parents. The doll [End Page 74] mother would hum, like their own mother did, "I'm gonna wash that man right out of my hair." The doll father lit a pipe made from a stolen match and went, "hmm," as the flame flared up and melted his nose. One Sunday, the family went for a drive out of the city and along the coastal highway. Their father stopped the car at a lookout. "Don't go too close to the edge," he said. Their mother grabbed their arms, cried out, "Watch out! You'll kill yourselves!" None of them fell off the cliff that day, although later the dollhouse parents climbed to the roof of the dollhouse and jumped off. They were driven to it by the stress of having such awful children; couldn't wait to be dead. They jumped off the roof hundreds of times, always with the same result: dying, springing to life again. The dolls did other terrible exciting things, and had terrible exciting things done to them—drownings, car accidents, poisonings—every terrible thing their parents warned them about. How happy the three girls were playing with the dollhouse. Then the girls' father became ill. He went to the hospital, came home. He lived in his pajamas and bathrobe, only one outfit, just like the dolls. The...

A New and Extensive α0 Deletion in a Brazilian Patient with Hb H Disease

Alpha thalassemias are the commonest group of hereditary hemoglobin disorders in the world, the most common cause being deletions involving the α globin genes on 16p13.3. We found a new α0 deletionassociated with the -α3.7 deletion in a 3-month-old brown-skinned female Brazilian patient with Hb H disease. The deletion was identified by Multiplex Ligation-dependent Probe Amplification (MLPA) using the SALSA MLPA P140 C1 HBA kit (MRC Holland, Amsterdam, Holland). An approximately 360 kb-region of DNA extending from the telomeric region of the short arm of chromosome 16 to the DECR2 gene was analyzed, and the fragments generated were compared with Coffalyser.Net. In addition to the α3.7 deletion, an extensive trans deletion encompassing 70 to 105 kb of DNA and involving the α2and α1 genes and the contiguous LUC7L and ITFG3 genes was detected (Figure 1). The 5' breakpoint of the deletion is located between the ψα1and the α2 gene, while the 3' breakpoint region is between the ITFG3 and RGS11 genes. Familial analysis revealed that while the patient's father was heterozygous for the -α3.7 deletion, her mother and oldest sister were heterozygous for the α0 deletion and her middle sister also had Hb H disease. A polymorphism (C > T) in the 220 probe binding region in the hybrid gene (α2α1) (-α3.7 deletion) was also detected in the patient and her father, causing this probe to have a different pattern from that expected in MLPA. To our knowledge, this deletion does not have any similarities in terms of its breakpoint regions with other deletions previously described in the literature. It was named --ATB because the family came from the town of Atibaia in the state of São Paulo in southeast Brazil. Molecular characterization of α-thalassemia deletions is essential both for correct diagnosis of carriers of these deletions and for genetic counselling so that new cases of Hb H disease or Hb Bart's Hydrops Fetalis can be prevented. The hematologic and molecular data for the family studied here are shown in Table 1.Financial Support: FAPESP - 2014/00984-3, CNPq and CAPES/Brazil.Table 1Hematologic and molecular data for the family members.FamilyProband (3 months)MotherFatherSister (2 years)Sister (3 years)RBC RV: M:4,5-6,1/F: 4,2-5,44.555.815.395.505.07Hb RV: M: 14-18/F: 12-167.7512.114.87.610.4MCH RV: 27-321720.827.413.820.6MCV RV: 80-9954.164.279.641.561.4A2 (%) VR: 1,6-40.782.132.802.402.80F (%) RV: < 223.700.700.904.701Hb profileA2, F, A, Bart's + H (10%)A2, AA2, AA2, A, HA2, AGenotype-α3.7/--ATBαα/--ATBαα/-α3.7-α3.7/--ATBαα/--ATBRV: Reference values RBC: red blood cells (106/mm3); Hb: hemoglobin (g/dL); MCH: mean corpuscular hemoglobin (pg); MCV: mean corpuscular volume (fl). [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Fun Day

Fun Day Miriam Cohen (bio) Every year, there is one day when everyone loves the three sisters. Never mind the oldest’s going to therapy next door to the school bus-stop, or the youngest’s penchant for feeding her lunch to stray animals (sometimes to a cat, sometimes to a dog), which must by now surely have translated into rabies. Or the oddness of the middle sister, who twists her thin arms strangely when she doesn’t know the answer to a question and has too many rows of pencils, all lined up exactly on her desk, point to point. This day is Fun Day. Their father, William, is CEO of a company (that sells pants? belts?), and Fun Day is like office picnics they have seen on TV—the way real farms are like the ones for ants. It makes them gloat. There is cotton candy; there are games wherein stuffed animals can be won; and there are sideshows. The oldest, Elizabeth, and the middle, Lucille, decide to try their luck at a game. “Try their luck” is a phrase belonging to their father. They know they will win. They always win on Fun Day. They can fail to squirt water into the clown’s mouth every time—every time! What are the odds of that? And still, the man behind the counter will say, We have a winner! They are the daughters of the CEO. On Fun Day, as they are not on any other day, they are royalty. Elizabeth has her eyes set on a giant bear that will not, of course, prove soft. These kinds of bears, she knows from experience, are always stuffed with what feels like newspaper. Lucille would like the enormous frog. There will be no need to fight. After they lose wildly, each will get the prize of her choosing, the screaming of other children rising up delightfully around them. [End Page 23] But Sophie, who is the youngest, decides she would like to visit the sideshow. She visits the fortune teller because she wants to know the future. This is their last Fun Day, their mother, Anna, says because it’s true: she’s dying. The chemo is a troop of good soldiers attacking the bad cancer, the doctor has said. The idea of soldiers marching through her mother’s body, attacking cells who are not soldiers and who cannot fight back, horrifies Sophie. She prefers to think of it as medicine—a kind of viscous, cherry-flavored sludge—only it doesn’t work. Sophie is curious to find out when, exactly, her mother will die. She would like to be prepared, with a face most right for mourning, which she practices at home in the bathroom mirror. The face is devastated but dignified. She will hold her chin high and lower her eyes so the eyelashes almost touch her cheekbones. She will bite, just lightly, her lower lip. Oh! the mourners will say. What a trooper! And the sleeves of her black dress will be just a little bit too long, a reminder to all that she is now a daughter without a mother. The fortune teller is stationed inside a tent that smells like lavender. There are curtains made of beads Sophie must part in order to get to her. The fortune teller has no visitors. She tells Sophie she may sit down right away. Sophie holds out her hand to shake because she is practicing the kind of motherless daughter she will be, which is a polite one. But the fortune teller simply looks at Sophie’s hand, as though she has never seen one before. Sophie lines up her heels like she’s a dancer. “I’m Sophie,” she says. “Oh!” The fortune teller sounds just like Sophie’s mother already out the door, remembering she’s forgotten her wig. Her gums show when she smiles. “You can call me Madame Felicia.” “Do you have cards?” Sophie says. “Because I’m looking to find out about death.” [End Page 24] Madame Felicia nods. She takes out a pack of cards and flips over four. She sets them down on her table one at a time. There...

Mothers and Sisters

MCA focuses on central categories in talk-in-interaction. It involves enumerating the activities, attributes, and inferences (category-bound predicates) associated with a category, and, based on the category and its category-bound predicates, makes a connection with a social action. In this short paper, I will focus on the categories of ‘mother’ and ‘sister’ and the standardized relational pairs (SRPs) ‘mother-child’ (in this case, a young adult child) and ‘older sister-younger sister.’ I will now turn to a two-part excerpt which exemplifies what Stokoe (2012) calls “going categorical,” a term used when interlocutors explicitly name a category and, in some cases, its attributes. In the excerpt below, two categories are named: 1) ‘mother’ (also called ‘mom’); and 2) ‘older sister,’ a sub-category of ‘sister.’ Also, two attributes (obligations/rights) of ‘mother’ are named: 1) ‘caregiver,’ the person who does/takes care of everything for others; and 2) ‘order giver,’ the person who orders someone under her care to do something. The following excerpt is taken from a longer troubles-telling segment with two female interlocutors, C and N. In this spate, C is telling N about a dinner party she is giving that night, and about all the work yet to be done in preparation. C lives with her two younger sisters and she is complaining about the middle sister, who has invited several of the dinner guests, and who does not help around the house: Extract 1: Cleaning House

Geochemical and Visual Indicators of Hydrothermal Fluid Flow through a Sediment-Hosted Volcanic Ridge in the Central Bransfield Basin (Antarctica)

In the austral summer of 2011 we undertook an investigation of three volcanic highs in the Central Bransfield Basin, Antarctica, in search of hydrothermal activity and associated fauna to assess changes since previous surveys and to evaluate the extent of hydrothermalism in this basin. At Hook Ridge, a submarine volcanic edifice at the eastern end of the basin, anomalies in water column redox potential (Eh) were detected close to the seafloor, unaccompanied by temperature or turbidity anomalies, indicating low-temperature hydrothermal discharge. Seepage was manifested as shimmering water emanating from the sediment and from mineralised structures on the seafloor; recognisable vent endemic fauna were not observed. Pore fluids extracted from Hook Ridge sediment were depleted in chloride, sulfate and magnesium by up to 8% relative to seawater, enriched in lithium, boron and calcium, and had a distinct strontium isotope composition (87Sr/86Sr = 0.708776 at core base) compared with modern seawater (87Sr/86Sr ≈0.70918), indicating advection of hydrothermal fluid through sediment at this site. Biogeochemical zonation of redox active species implies significant moderation of the hydrothermal fluid with in situ diagenetic processes. At Middle Sister, the central ridge of the Three Sisters complex located about 100 km southwest of Hook Ridge, small water column Eh anomalies were detected but visual observations of the seafloor and pore fluid profiles provided no evidence of active hydrothermal circulation. At The Axe, located about 50 km southwest of Three Sisters, no water column anomalies in Eh, temperature or turbidity were detected. These observations demonstrate that the temperature anomalies observed in previous surveys are episodic features, and suggest that hydrothermal circulation in the Bransfield Strait is ephemeral in nature and therefore may not support vent biota.

Familial bilateral combined hamartoma of retina and retinal pigment epithelium associated with neurofibromatosis 1

We report a family of three siblings followed between 2005 and 2011 with bilateral combined hamartoma of the retina and retinal pigment epithelium, with the age of diagnosis ranging from 7 to 13years. The main reason for consultation was reduction of vision and squint. The diagnosis was determined based on the clinical findings on fundus examination: increased pigmentation at the macula with slightly elevated, gray–white macular lesion, tortuosity of perimacular blood vessels and glial epiretinal membrane. The elder brother was found to have left posterior subcapsular cataract. He was also confirmed to have neurofibromatosis type 1, the youngest sister fit in the diagnostic criteria for neurofibromatosis type 1, while the middle sister was presumed to have neurofibromatosis type 1. Follow-up showed stability of the retinal lesion in the three cases, with the progression to develop right posterior subcapsular cataract in the elder sister.This report is aimed to demonstrate that the occurrence of bilateral combined hamartoma of the retina and retinal pigment epithelium could raise the possibility of associated neurofibromatosis.