Analysis of Clinical Presentations of Bruton Disease: A Review of 20 Years of Accumulated Data from Pediatric Patients at Severance Hospital

PURPOSE: X-linked agammaglobulinemia (XLA) is a humoral immunodeficiency disease caused by a mutation in the Bruton tyrosine kinase (BTK) gene resulting in defective B cell differentiation. Because it is a relatively rare disorder, it is difficult for clinicians to have a comprehensive understanding of XLA due to a lack of exposure to the disease. Clinical presentations of patients with XLA were analyzed and discussed to improve care plans. MATERIALS AND METHODS: During a 20 year period, from January 1987 to June 2006, a total of 19 patients were diagnosed as XLA in the Department of Pediatrics at Severance Hospital, Seoul, Korea. A retrospective analysis of the clinical presentations of those patients was performed. RESULTS: The mean age of the XLA patients included in the study was 4.89 years, with a range of 6 months to 13 years. Twelve patients were diagnosed before age 5, while the other 7 patients were diagnosed after age 5. Recurrent infections observed in the patients included pneumonia, acute otitis media, septic arthritis, skin infection, sepsis, sinusitis, acute gastroenteritis, cervical lymphadenitis, epididymitis, meningitis, osteomyelitis, urinary tract infection and encephalitis. Frequency of admissions was variable from 0 to 12 times, depending on the time at which immunoglobulin therapy was started. Six cases had family histories positive for XLA. BTK gene mutations were found in 8 cases. CONCLUSION: The overall prognosis of XLA is good as long as patients are diagnosed and treated early with regular intra venous gamma globulin therapy before the sequelae of recurrent infections appear.ope

To formalize or not to formalize: women entrepreneurs’ sensemaking of business registration in the context of Nepal

Despite the depiction of decisions to formalize informal firms as rational and ethical, many entrepreneurs in developing countries continue to operate informally regardless of its perceived illicit status. While existing research on why entrepreneurs choose informality emphasizes the economic costs and benefits of such decisions, this often overlooks the realities of the informal economy and the constraints which marginal populations—particularly women—face. In this paper, we use institutional theory and sensemaking to understand the experiences of women in the informal economy and what formalization means to them. We use a qualitative approach to collect data from 90 women entrepreneurs in three different cities in Nepal. In our findings, we identify three groups of women with distinctive understandings of formalization—business sustainability, livelihood sufficiency and strategic alignment. Their interpretation of formalization reveals the complex, dynamic, and cyclical nature of formalization decisions. Decisions are also guided by the optimization of social and emotional logics, whereby formalization is conceived differently depending on different life stages, experiences within the informal economy and wider socio-cultural contexts. Our findings highlight the ethical implications of formalization where being a ‘good citizen’, rather than complying with formal rules and regulations, is about attuning to and fitting in with socially prescribed roles. Our research provides a nuanced view of formalization decisions, challenging idealized and ethical notions of formalization as a desired end state

Corporate governance and national institutions: A review and emerging research agenda

We present a critique of corporate governance research grounded in agency theory and propose that cross-national comparison of corporate governance should consider how the nature and extent of agency relationships differ across different institutional contexts. Building on prior governance studies grounded in sociology and organizational theory we argue that performance outcomes of boards of directors, ownership concentration, and executive incentives may differ depending on the legal system and institutional characteristics in a specific country. Institutions may also affect the extent of complimentarity/substitution among different firm-level governance practices producing patterned variations in firm-level governance mechanisms. Our discussion suggests that researchers need to develop more holistic, institutionally embedded governance framework to analyze organizational outcomes of various governance practices

The life and scientific work of William R. Evitt (1923-2009)

Occasionally (and fortunately), circumstances and timing combine to allow an individual, almost singlehandedly, to generate a paradigm shift in his or her chosen field of inquiry. William R. (‘Bill’) Evitt (1923-2009) was such a person. During his career as a palaeontologist, Bill Evitt made lasting and profound contributions to the study of both dinoflagellates and trilobites. He had a distinguished, long and varied career, researching first trilobites and techniques in palaeontology before moving on to marine palynomorphs. Bill is undoubtedly best known for his work on dinoflagellates, especially their resting cysts. He worked at three major US universities and spent a highly significant period in the oil industry. Bill's early profound interest in the natural sciences was actively encouraged both by his parents and at school. His alma mater was Johns Hopkins University where, commencing in 1940, he studied chemistry and geology as an undergraduate. He quickly developed a strong vocation in the earth sciences, and became fascinated by the fossiliferous Lower Palaeozoic strata of the northwestern United States. Bill commenced a PhD project on silicified Middle Ordovician trilobites from Virginia in 1943. His doctoral research was interrupted by military service during World War II; Bill served as an aerial photograph interpreter in China in 1944 and 1945, and received the Bronze Star for his excellent work. Upon demobilisation from the US Army Air Force, he resumed work on his PhD and was given significant teaching duties at Johns Hopkins, which he thoroughly enjoyed. He accepted his first professional position, as an instructor in sedimentary geology, at the University of Rochester in late 1948. Here Bill supervised his first two graduate students, and shared a great cameraderie with a highly motivated student body which largely comprised World War II veterans. At Rochester, Bill continued his trilobite research, and was the editor of the Journal of Paleontology between 1953 and 1956. Seeking a new challenge, he joined the Carter Oil Company in Tulsa, Oklahoma, during 1956. This brought about an irrevocable realignment of his research interests from trilobites to marine palynology. He undertook basic research on aquatic palynomorphs in a very well-resourced laboratory under the direction of one of his most influential mentors, William S. ‘Bill’ Hoffmeister. Bill Evitt visited the influential European palynologists Georges Deflandre and Alfred Eisenack during late 1959 and, while in Tulsa, first developed several groundbreaking hypotheses. He soon realised that the distinctive morphology of certain fossil dinoflagellates, notably the archaeopyle, meant that they represent the resting cyst stage of the life cycle. The archaeopyle clearly allows the excystment of the cell contents, and comprises one or more plate areas. Bill also concluded that spine-bearing palynomorphs, then called hystrichospheres, could be divided into two groups. The largely Palaeozoic spine-bearing palynomorphs are of uncertain biological affinity, and these were termed acritarchs. Moreover, he determined that unequivocal dinoflagellate cysts are all Mesozoic or younger, and that the fossil record of dinoflagellates is highly selective. Bill was always an academic at heart and he joined Stanford University in 1962, where he remained until retiring in 1988. Bill enjoyed getting back into teaching after his six years in industry. During his 26-year tenure at Stanford, Bill continued to revolutionise our understanding of dinoflagellate cysts. He produced many highly influential papers and two major textbooks. The highlights include defining the acritarchs and comprehensively documenting the archaeopyle, together with highly detailed work on the morphology of Nannoceratopsis and Palaeoperidinium pyrophorum using the scanning electron microscope. Bill supervised 11 graduate students while at Stanford University. He organised the Penrose Conference on Modern and Fossil Dinoflagellates in 1978, which was so successful that similar meetings have been held about every four years since that inaugural symposium. Bill also taught many short courses on dinoflagellate cysts aimed at the professional community. Unlike many eminent geologists, Bill actually retired from actively working in the earth sciences. His full retirement was in 1988; after this he worked on only a small number of dinoflagellate cyst projects, including an extensive paper on the genus Palaeoperidinium

"Walk slowly towards the light"

The underlying themes of this solo exhibition were the female performer, subjectivity, and the notion of spectacle within the context of abstract painting. The project mapped out, identified and theoretically analysed aspects of my own practice as ‘artist’, further exploring ideas of the spectacle within a public space through the activity of painting itself; exploring how a particular context informs our notions of performance and spectacle, and where the individual can be placed within this. Based in Las Vegas, I research and documented neon signs, architecture and Showgirl performances, also interviewing author Libby Lumpkin (‘Nine Little Art Histories’). Documentation, including video, photographic and digital processes, became a resource informing processes of layering within my painting practice. At Matt’s Gallery, lighting critically became an invitation and obstacle to viewing the paintings. Alluding to the ‘Show Girl’ I set out to make perceptible, a sense of performance using reflective materials. Florescent and neon light, and layers constructed within the physical painted surfaces of the works using layers of paint and glass beads aimed at drawing the viewer into the ‘act’ of looking, thereby on some level, performing the work. Other texts referenced: ‘Formations of Fantasy’ (Joan Rivierie); and ‘Womenliness and the Masquerade’, ‘The Showgirl’, ‘Deep Design’, (Libby Lumpkin). The context for my work engages with an emerging discourse around the female artist, aesthetics and the language of pleasure with painting practice; an area of research and practice challenging the existing canon of modernist art historical critique and analysis

Looking East, Looking West

'Looking east looking west' is a painting by Jo Bruton first exhibited in the Human Abstract in No4a Gallery in Worcestershire and then the Discerning Eye Exhibition at the Mall Galleries, London ‘Looking East, Looking West’ explores a dialogue between the spectacle of performance and abstract painting. Connections between painter and performer are reflected in gestures and activities that correspond with repetition and routine. Painted fragments of Costume and decoration are used to create an experience of form that evokes a narrative around a female presence. It also creates an awkward space for the subject to occupy and navigate. Archive material acts as a resource for new narratives to emerge where information is reduced and flattened to become visual motifs. By isolating, transferring, repeating and repositioning these physical and material fragments Bruton alludes to questions of stability, where elements appear moveable and subject to change. Costume and decoration reference popular entertainment such as the Circus and Music Hall. ‘Looking East, Looking West’, is one of a series of paintings that use references from the National Fairground Archive. Through costume and performance, individuals construct their own identities and transform the everyday through extraordinary acts of courage and daring. ‘Looking East, Looking West’ extends a dialogue with Bruton’s past works such as ‘Cosmic Candy’ and ‘Capitiane Can-Can’ where references to cheerleading and chorus girls are embedded within the work. The decorative as a subject has traditionally occupied the margins of mainstream fine art practice by using the chorus line up which usually frames the main event it becomes the focus and central theme for the audience to correspond with. ‘Cosmic Candy’ and ‘Capitaine Can-Can’ were included in group exhibitions ‘Variety’ at the De La Warr Pavilion, Bexhill on Sea in 2005 and ‘Working against the System’. At Gallery North, Newcastle and Transition Gallery, London. 2011 ‘Variety’ showcased artists using theatre in their work such as Mark Wallinger, Cindy Sherman and Susan Hiller. And ‘Working Against The System’, which challenged mainstream attitudes towards methods and materials within contemporary painting. Included DJ Simpson, Katie Pratt and Noel Forster. This formed part of the publication, ‘About Painting’

Wild

Wild is a series of drawings made into 3min film. The drawings were exhibited in a group show entitled Parallax at the Katowice Fine Art Academy in Poland 2015 then at Chelsea College of Arts, London 2016. This was part of a project and collaboration that acknowledged the art school’s function as a test site where ideas and approaches are shared, challenged and developed. Wild is a short film Bruton made in response to Jack Halberstam’s text 'Charming for the Revolution: A Gaga Manifesto’. Elements are drawn from a print by Jacques Le Moyne de Morgues; ‘A Young Daughter of the Picts’ 1533 and found images of mockingbirds; they are serenaded by Ronnie Ronaldo’s Mockingbird Hill. Artifice and mimicry challenge notions of fixed identities from a personal as well as cultural perspective. The film captures research material, images and stories, which Bruton has collected to inform her painting practice. Wild was initially made for the ‘The Performance Dinners’ a series of events hosted by the ‘Subjectivity and Feminisms’ Research Group. Wild is one of a series of film, animation and projection that Bruton has made in response to the groups' ongoing collaborations and examination of the inter-relationships between subjectivity and the artwork and underlying feminist theories

Hide-Out

‘Hide-Out’ is a painting by Jo Bruton first exhibited in the Human Abstract in No4a Gallery in Worcestershire and then the Discerning Eye Exhibition at the Mall galleries, London ‘Hide-Out’ explores a dialogue between the spectacle of performance and abstract painting. Connections between painter and performer are reflected in gestures and activities that correspond with repetition and routine. Painted fragments of Costume and decoration are used to create an experience of form that evokes a narrative around a female presence. It also creates an awkward space for the subject to occupy and navigate. Archive material acts as a resource for new narratives to emerge where information is reduced and flattened to become visual motifs. By isolating, transferring, repeating and repositioning these physical and material fragments Bruton alludes to questions of stability, where elements appear moveable and subject to change. Costume and decoration reference popular entertainment such as the Circus and Music Hall. ‘Hide-Out’, is one of a series of paintings that use references from the National Fairground Archive. Through costume and performance individuals construct their own identities and transform the everyday through extraordinary acts of courage and daring. ‘Hide-Out’ extends a dialogue with Bruton’s past works such as ‘Cosmic Candy’ and ‘Capitiane Can-Can’ where references to cheerleading and chorus girls are embedded within the work. The decorative as a subject has traditionally occupied the margins of mainstream fine art practice by using the chorus line up which usually frames the main event it becomes the focus and central theme for the audience to correspond with. ‘Cosmic Candy’ and ‘Capitaine Can-Can’ were included in group exhibitions ‘Variety’ at the De La Warr Pavilion, Bexhill on Sea in 2005 and ‘Working against the System’. At Gallery North, Newcastle and Transition Gallery, London. 2011 ‘Variety’ showcased artists using theatre in their work such as Mark Wallinger, Cindy Sherman and Susan Hiller. And ‘Working Against The System’, which challenged mainstream attitudes towards methods and materials within contemporary painting. Included DJ Simpson, Katie Pratt and Noel Forster. This formed part of the publication, ‘About Painting’

The Landing Girls

‘The Landing Girls’ is a permanent site-specific painting installation at Charing Cross Hospital, London by Jo Bruton and commissioned by Imperial Health Charity. Vinyl on 10’ x 30’ wall and etched free-standing glass panels 4 x 8’x10’. Framed original painting/template. 5’x4’. ‘The Landing Girls’ examines wallpaper as both a subject and a strategy to expand beyond the edges of the painting to work directly within a given site. Invited to develop this research as a permanent installation for Charing Cross Hospital Bruton has created an environment to navigate. Building on previous research and the experience of viewing she has visually adapted figures from aviation diagrams and manuals to play on the idea of communicating with and directing the viewer. Digital processes have been deployed to translate a painted template and repeat pattern onto vinyl where photographic detail and digital reworking maintain the physicality of the original painted surface. Her decorative motifs have also been etched onto glass panels, which creates a layered experience of form and space. Through the playful use of pattern, surface and material the work encourages movement. In this way, the installation animates both the space and an encounter with the viewer

Cactus Canyon

‘Cactus Canyon’, Acrylic on Canvas and Board 213x 78 cm is a painting by Jo Bruton Selected for the Marmite Prize for Painting. Block 336 London UK & Highlanes Gallery, Drogheda. Ireland 2016 Cactus Canyon examines how fragments of decoration can be understood as a different set of codes and signs for abstract painting. The space has become an environment for the female performer to navigate. Bruton continues to explore the archive as an expansive and collective record of personal and social histories. Found memorabilia from the circus and travelling shows provide open narratives around transformation and subjects on the move. There is an equation between the painter and performer where routine and gesture are repeated, isolated and repositioned in a process that is an inward as well as outward reality; an act of communication as well as constructed spectacle. ‘Cactus Canyon’ extends a dialogue and interrogation into this predominantly masculine territory of abstraction where Bruton's past paintings; Saloon Rosa and Cabaret, also open up a minimalist aesthetic to include personal and social histories. These earlier paintings were first exhibited in Warped Painting and the feminine Anglerow Gallery Nottingham alongside artists such as Valerie Jaudon and Shirley Kaneda. The publication recorded conversations between the artists and international debates around painting abstraction and otherness. ‘Cactus Canyon’ is a continuation of that debate and exploration into otherness and abstraction within works such as ‘Cosmic Candy’ and ‘Capitiane Can-Can’ where references to cheerleading and chorus girls are embedded within the work. The decorative as a subject has traditionally occupied the margins of mainstream fine art practice by using the chorus line-up which usually frames the main event it becomes the focus and central theme for the audience to correspond with. ‘Cosmic Candy’ and ‘Capitaine Can-Can’ were included in group exhibitions ‘Variety’ at the De La Warr Pavilion, Bexhill on Sea in 2005 and ‘Working against the System’. At Gallery North, Newcastle and Transition Gallery, London. 2011 ‘Variety’ showcased artists using theatre in their work such as Mark Wallinger, Cindy Sherman and Susan Hiller. And ‘Working Against The System’, which challenged mainstream attitudes towards methods and materials within contemporary painting. Included DJ Simpson, Katie Pratt and Noel Forster. This formed part of the publication, ‘About Painting’