نتایج جستجو برای: urbach wiethe disease
تعداد نتایج: 1490423 فیلتر نتایج به سال:
Amorphous silicon thin films, fabricated by thermal conversion of neopentasilane, were used to passivate crystalline silicon surfaces. The conversion is investigated using X-ray and constant-final-state-yield photoelectron spectroscopy, and minority charge carrier lifetime spectroscopy. Liquid processed amorphous silicon exhibits high Urbach energies from 90 to 120 meV and 200 meV lower optical...
1. Calzavara-Pinton P, Ortel B, Carlino A, Honigsmann H, De Panfilis G. A reappraisal of the use of 5-methoxypsoralen in the therapy of psoriasis. Exp Dermatol 1992;1:46-51. 2. Honigsmann H, Szeimies RM, Knobler R. Photochemotherapy and photodynamic therapy. In: Wolff K, Goldsmith LA, Katz SI, Gilchrist AS, Leffell DJ, editors. Fitzpatricks dermatology in genereal medicine. 7th ed. New York: M...
In 2005, DeLong, Urbach, and Kutas took advantage of the a/an English indefinite article phonological alternation and the sensitivities of the N400 ERP component to show that readers can neurally preactivate individual words of a sentence (including nouns and their prenominal indefinite articles) in a graded fashion with a likelihood estimated from the words' offline probabilities as sentence c...
Exponential band edges have been observed in a variety of materials, both crystalline and amorphous. In this Letter, we infer the structural origins of these tails in amorphous and defective crystalline Si by direct calculation with current ab initio methods. We find that exponential tails appear in relaxed models of diamond silicon with suitable extended defects that emerge from relaxing point...
Lipoid CAH was first described in detail as an inherited endocrine disorder by Prader and colleagues (Prader & Gurtner 1955, Prader & Siebenmann 1957, Prader & Anders 1962), although at least four autopsy cases appeared earlier in the pathology literature (Tilp 1913, Brutschy 1920, Zahn 1948, Sandison 1955). Prader’s group described male pseudohermaphroditism, an apparent lack of adrenal steroi...
UNLABELLED Lipoid proteinosis is a rare autosomal recessive disease characterized by cutaneous and mucosal lesions and hoarseness appearing in early childhood that is caused by homozygous or compound heterozygous mutations in the ECM1 gene located on chromosome 1q21. The aim of the study was to investigate the molecular genetic defect underlying lipoid proteinosis in a consanguineous Pakistani ...
In the present study, the oxyfluoride glasses of SiO2-Al2O3-CaF2 system containing different amounts of Y3+ ions were prepared through the convenient melting method. The crystallization temperatures and size of the CaF2 nanocrystals were obtained from DTA curves and XRD patterns, respectively. As a consequence, the optimum amount...
The status of Bayesianism has considerably evolved since the beginning 1990s, when Howson and Urbach published Scientific Reasoning: Bayesian Approach (1989). At time, was a philosophical theory scientific reasoning rival to falsificationism. While it claimed account for important aspects in empirical science solve difficulties such as Duhem’s Problem or Hempel’s Raven Paradox, it hardly used b...
The interactions between the nephrogenic mesenchyme and the ureteric bud during kidney development are well documented. While recent studies have shed some light on the importance of the stroma during renal development, many of the signals generated in the stroma, the genetic pathways and interaction networks involving the stroma are yet to be identified. Our previous studies demonstrate that r...
Lipoid proteinosis is caused by loss-of-function mutations in the glycoprotein extracellular matrix protein 1 (ECM1). We report here mutation analysis of the ECM1 gene in a Chinese family with lipoid proteinosis. A 10year-old boy presented with a hoarse voice, acneiform scars and yellow skin nodules, as well as beaded eyelid papules and a thickened sublingual frenulum. Skin biopsy showed widesp...
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