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Mycophenolate mofetil therapy for children with steroid-resistant nephrotic syndrome. Short term efficacy of intravenous dexamethasone and methylprednisolone therapy in steroid resistant nephrotic syndrome. Mycophenolate mofetil in children with steroid/cyclophosphamide-resistant nephrotic syndrome. Cyclosporin A is superior to cyclophosphamide in children with steroid-resistant nephrotic syndrome-a randomized controlled multicentre trial by the Arbeitsgemeinschaft fur Padiatrische Nephrologie. Adult minimal change nephropathy: experience of the collaborative study of glomerular disease. Adult-onset minimal change disease among Taiwanese: clinical features, therapeutic response, and prognosis. High absolute risks and predictors of venous and arterial thromboembolic events in patients with nephrotic syndrome: results from a large retrospective cohort study. Idiopathic minimal change nephrotic syndrome in older adults: steroid responsiveness and pattern of relapses. Cyclophosphamide alone in the treatment of adult patients with minimal change glomerulonephritis. Favorable outcome of low-dose cyclosporine after pulse methylprednisolone in Japanese adult minimalchange nephrotic syndrome. Tacrolimus as a steroid-sparing agent for adults with steroid-dependent minimal change nephrotic syndrome. Treatment of adult idiopathic nephrotic syndrome with cyclosporin A: minimal-change disease and focal-segmental glomerulosclerosis. Combined cyclosporine and prednisolone therapy in adult patients with the first relapse of minimalchange nephrotic syndrome. Long-term renal tolerance of cyclosporin A treatment in adult idiopathic nephrotic syndrome. Mycophenolate mofetil therapy for childhood-onset steroid dependent nephrotic syndrome after long-term cyclosporine: extended experience in a single center. The use of enteric-coated mycophenolate sodium in the treatment of relapsing and steroiddependent minimal change disease. The risk of cardiovascular disease in adults who have had childhood nephrotic syndrome. Acute kidney injury complicating minimal change disease: the case for careful use of diuretics and angiotensin-converting enzyme inhibitors. The clinical effectiveness and costeffectiveness of treatments for children with idiopathic steroid-resistant nephrotic syndrome: a systematic review. Changing etiologies of unexplained adult nephrotic syndrome: a comparison of renal biopsy findings from 1976 to 1979 and 1995 to 1997. Podocyte foot process effacement as a diagnostic tool in focal segmental glomerulosclerosis. Podocin mutations in sporadic focalsegmental glomerulosclerosis occurring in adulthood. Nephrin mutations cause childhood- and adult-onset focal segmental glomerulosclerosis. Association of trypanolytic ApoL1 variants with kidney disease in African Americans. Primary focal segmental glomerulosclerosis: clinical course and response to therapy. The impact of prolonged immunosuppression on the outcome of idiopathic focal-segmental glomerulosclerosis with nephrotic syndrome in adults. Long-term outcome in children and adults with classic focal segmental glomerulosclerosis. Focal segmental glomerular sclerosis in adults: presentation, course, and response to treatment.

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  • Chromosome 8, monosomy 8p
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Arteritis in skeletal muscle and subcutaneous tissues causes tender erythematous nodules that can be identified on physical examination. Kawasaki disease almost always occurs in children younger than 6 years and, by definition, is accompanied by the mucocutaneous lymph node syndrome. A small-vessel vasculitis should be suspected if there is evidence for inflammation of vessels smaller than arteries, such as glomerular capillaries (hematuria and proteinuria), dermal venules (palpable purpura), or alveolar capillaries (hemoptysis). To discriminate among the small-vessel vasculitides, evaluation of serologic data, vessel immunohistology, or concurrent nonvasculitic disease. Evaluation of vessels in biopsy specimens, such as glomerular capillaries in kidney biopsies, alveolar capillaries in lung biopsies, or dermal venules in skin biopsies, can be helpful, especially if immunohistology is performed. Hypocomplementemia is common in patients with cryoglobulinemic vasculitis, lupus vasculitis, and hypocomplementemic urticarial vasculitis. The glomerulonephritis in all three of these vasculitides is characterized by capillary wall and mesangial immune complex deposition and a proliferative or membranoproliferative pattern of injury. The circles represent the major immunopathologic categories of vascular inflammation that affect the kidneys, and the shaded ovals represent the clinicopathologic expressions of the vascular inflammation. Patients with severe disease or steroid toxicity benefit from other immunosuppressive agents, including methotrexate, cyclophosphamide, mycophenolate mofetil, or azathioprine. After the inflammatory phase has passed and the sclerotic phase has developed, vascular surgery (stent or bypass) may be required to improve flow to ischemic tissues, especially in patients with Takayasu arteritis. Some patients with polyarteritis nodosa have a persistent viral infection, especially hepatitis B virus infection. In patients with no evidence for infection, management usually consists of corticosteroids with or without cytotoxic drugs. The preferred treatment for Kawasaki disease is a combination of aspirin (80-100 mg/kg/day) and high-dose intravenous gamma globulins (single 12-hour infusion of 2 g/kg). With appropriate treatment, more than 90% of patients with Kawasaki disease have complete resolution of the disease. Many patients with IgA vasculitis experience mild disease that requires only supportive care (see Chapter 20). IgA vasculitis causes decreased kidney function in approximately 20% of patients 20 years after onset, with a higher rate in patients with later onset disease. Arthralgias can be relieved by nonsteroidal antiinflammatory drugs, whereas corticosteroid treatment is beneficial for patients who experience severe abdominal pain caused by intestinal vasculitis. The treatment of severe glomerulonephritis in patients with IgA vasculitis is controversial. There is anecdotal evidence indicating that aggressive crescent glomerulonephritis should be treated with high-dose corticosteroids, cytotoxic agents, or plasmapheresis, but this has not been documented in controlled trials. Data from a large pediatric population suggest that corticosteroid treatment may decrease the risk for development of kidney involvement in those patients with severe abdominal pain and rash. Cryoglobulinemic vasculitis caused by hepatitis C infection may respond to pegylated interferon-alfa in combination with antiviral therapy. As many as 25% to 50% of patients experience either partial or complete response to therapy. In patients with severe vasculitis or glomerulonephritis, an induction phase of immunosuppression. Patients with pulmonary-renal vasculitic syndromes in whom hemoptysis is a major clinical feature require emergent therapy with plasma exchange. Iannuzzella F, Vaglio A, Garini G: Management of hepatitis C virusrelated mixed cryoglobulinemia, Am J Med 123:400-408, 2010. Jayne D, Rasmussen N, Andrassy K, et al: A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies, N Engl J Med 349:36-44, 2003. Rossi P, Bertani T, Baio P, et al: Hepatitis C virus-related cryoglobulinemic glomerulonephritis: Long-term remission after antiviral therapy, Kidney Int 63:2236-2241, 2003. Induction therapy includes pulse methylprednisolone at a dose of 7 mg/kg/day for 3 days, followed by daily oral prednisone or plasma exchange therapy for 7 to 14 days in addition to daily oral prednisone.

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Indeed, as Derrida has convincing argued, it is rather hermeneutics which risks a potential relativism. By contrast, deconstruction, for example, serves as an epistemological check against the appropriations of hermeneutics. Thus, far from promoting relativism, in some respects poststructuralism could be seen as a defence against relativism. Likewise, far from advocating negation, poststructuralism could be seen to be premised on affirmation, although the possibility for negation must always be left open. He has published extensively on the work of Martin Heidegger, Theodor Adorno, Walter Benjamin, Julia Kristeva and Jean-Franзois Lyotard. Here he recognizes the interdependency of philosophy and the visual arts, and architecture especially. It is at home in-and provides a home for-philosophy, aesthetics and those discourses which are thought to describe it. And yet it is precisely the generality as well as the singularity of these claims that makes such a description or location problematic. The assertion-even the argument-that architecture Rethinking Architecture 270 houses, fails in a concrete, philosophical and political sense to address housing. Equally the interplay between architecture and the home in which philosophy, aesthetics and discourse may be located, works with the assumption that the nature of what is housed is such that the act of housing it will not call into question the specificity of the act itself. In other words the unified nature of philosophy is assumed and thus is thought to have been provided either by the unity of tradition or the singularity of its object. What needs to be examined therefore are some of the elements at work within these assumptions; their premises and therefore that on which they are built. The impossibility of pure freedom, of pure positivity and thus of a radical and absolute break entails that what is at stake here is, as a consequence, precisely philosophy and architecture themselves. Tradition emerges as the site that occasions both an understanding of dominance-the categories and concepts which are handed down and which thus determine thinking within and as tradition-and the possibility of a thinking which, while it maintains (houses) the dominant, is neither reducible to nor explicable in terms of it. In addition the tension that marks the corresponding non-correspondence of a thinking (be it architectural or philosophical) that is situated within tradition, and a thinking that cannot be thus situated, provides a way to renew the concept of the avant-garde as well as providing access to an understanding of the accompanying mode of experience (that is sensibility) proper to the avant-garde. The context concerns the possibility of philosophy, of a new philosophical thinking, and thus whether tradition can be refurbished-what has been handed down by and as tradition-or, on the contrary, whether philosophy is constrained to start again. It is not difficult to see in the very formulation of the problem that Descartes thinks that tradition may be, in fact, left behind. Consequently, in discussing Descartes it will be essential to analyse the unfolding of the metaphor as well as to examine or assess the possibility of this purported complete departure. Thus we see that buildings undertaken and completed by a single architect are usually more attractive and better planned than those which several have tried to patch up by adapting old walls built for different purposes. Again, ancient towns which have gradually grown from mere villages into large towns are usually ill-proportioned, compared with those ordinary towns that planners lay out as the fancy (fantaisie) on level ground. Looking at the buildings of the former individually, you will often find as much art in them, if not more, than in those of the latter; but in view of their arrangement-a tall one here, a small one there-and the way they make the streets crooked and irregular, you would say it is chance (fortune) rather than the will of men using reason (raison) that placed them so. It should be remembered, for example, that the actuality of starting again is articulated within architectural terms in the First Meditation, where he describes his task in the following way: to start once again from the foundations commencer tout de nouveau dиs les fondements. Now, returning to the passage from the Discours de la mйthode, it is essential to note both the presentation of tradition, its interarticulation within the language of architecture, and thus its putting into play as well as demanding a specific conception of experience and thereby of the aesthetic. Inscribed therefore within the more general architectural metaphor is an additional trope. The relationship between self and other has become mapped onto the possibility of a departure from tradition. The self becomes that possibility that emerges within the break from that conception of the self/other relation that views both parts as inextricably linked. It is thus that Cartesian thought establishes the centrality, both within Rethinking Architecture 272 architecture and philosophy, of the subject, and thus of subjectivism. Cartesianism is the emergence of the centrality of the subject; the subject of epistemology as well as the subject of sentiment. Rather than pursuing this particular path, however, it is essential to move to a more detailed analysis of what is at play in the rejection of tradition.

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These older children acquiring a first language after delayed exposure exhibit aspects of a two-word stage of language development. Results from intelligence assessments, achievement tests, drawing tasks, and qualitative cognitive analyses show that Mei and Cal are at least of average intelligence and ability. However, results from language analyses clearly show differences from both age peers and younger native signers in the early twoword stage, providing new insights into the nature of this phase of language development. Article history: Accepted 10 February 2012 Available online 2 April 2012 Keywords: First language acquisition American Sign Language Two-word stage Language and cognition 1. Introduction One of the most robust findings in the study of first language acquisition in children is the existence of a two-word stage at about the age of 2 years. For many languages, the utterances of children in this stage include a predominance of nouns, and a lack of grammatical markers (DeVilliers & DeVilliers, 1979); for this Corresponding author. Lillo-Martin / Cognitive Psychology 65 (2012) 118­140 119 reason, these aspects have also become part of the linguistic characteristics typically associated with the two-word stage. Over the past five decades other criteria have been added to the definition of the two-word stage, based on what has been described as part of 2-year old language. Since the 1970s, some authors have emphasized the ties between linguistic and cognitive development with the two-word stage hypothesized to inextricably link maturation, linguistic, and/or cognitive development (Bloom, Lightbown, & Hood, 1975; Casasola, Bhagwat, & Ferguson, 2006; Clark, 1973; Ervin-Tripp, 1973; Papafragou, Cassidy, & Gleitman, 2007; Piaget, 1980; Schlesinger, 1971, among others). Other authors, however, point out the evidence from linguistically and/or cognitively delayed populations which indicates that dissociations between development of cognitive ability and linguistic ability are possible (Bellugi, Lai, & Wang, 1997; Bellugi, Marks, Bihrle, & Sabo, 1993; Capirici, Sabbadini, & Volterra, 1996; Coggins, 1979; Curtiss, 1982; Landau et al. While there is considerable debate over how closely cognitive and linguistic development are linked, one of the current, tempered views is that cognitive and linguistic development can occur at separate paces. The search for a way to dissociate the development of cognitive and linguistic ability in both the fields of Cognitive Psychology and Linguistics is an on-going goal, and has branched out in several directions. In addition to looking at special medical populations, social populations, and language isolates, various cognitive capacities of typically-developing prelinguistic infants are currently being studied. One of the aims of the above-mentioned line of study is to investigate cognitive development before the convergence of linguistic development. The concept of number as separate from language is currently under intense debate (Gelman & Butterworth, 2005; Gordon, 2004; Mix, Huttenlocher, & Levine, 2002; Spaepen, Coppola, Spelke, Carey, & Goldin-Meadow, 2011; Wynn, 1992; Zosh & Feigenson, 2009; among others; see Spelke, 2011 for review). Gelman and Butterworth (2005) suggest at least four arguments for a dissociation between numerical cognition and language. One is that children and adults can have problems, due to dementia or brain injury, with language but not with number calculation and vice versa. A second is that neuroimaging studies show different places of activation for numerical processing and for language processing. A third is that speakers of languages such as Munduruku and Pirahг, with limited, and inconsistent use of numbers, are still able to compare sets of up to as many as 80 dots, saying which set has more. Their fourth argument revolves around a study that analyzed the results of an experiment with children ages 5;0­8;6, examining what children think and say when asked about number relationships. From their evidence, Gelman and Butterworth (2005) propose that neural organization separates language from number, as reflected by cognitive development. Further, they suggest that while language most likely has an effect on numerical cognition, it does not cause its development. Spelke (2011) alternatively suggests that the development of abstract numerical and geometric concepts hinges on the role that acquisition and use of natural language plays in linking information from distinct systems of core knowledge, like natural number and natural geometry. As evidence for the important role of language, she presents a study by Spaepen et al. The participants lack a formal education but nonetheless hold jobs, and deal with money. However, despite having a good communication system, when these homesigners try to convey numerical information on their fingers, they do so inaccurately. They also perform non-symbolic matching tasks with number at less than accurate performance, suggesting a special role for language in the use of natural number. As more evidence, Spelke (2011) discusses studies of educated adults with language impairment, who show a dissociation of quantity and number words. Further, she presents evidence from bilingual adults suggesting that learning new number facts in one language elicits a cost when produced in the language opposed to the one the fact was originally learned in.