195 research outputs found
Dynamical behavior of heat conduction in solid Argon
Background: Imunoglobulin A (IgA) deficiency (IgAD) is the most common form of primary immunodeficiency in Western countries. There have been several reports on IgAD complicated by glomerulonephritis in adults, but only very few cases of IgAD with nephropathy have been reported in children. We present two cases of IgAD with relapsing nephrotic syndrome in pediatric age. Case presentation: A 4-year-old boy and a 2-year-old boy presented with bilateral periorbital oedema and weight gain. The results of laboratory tests revealed IgAD (IgA < 7 mg/dL), normal creatinine, hypoprotidaemia, hypoalbuminaemia, and nephrotic proteinuria. A diagnosis of IgAD and idiopathic nephrotic syndrome was made, and steroid treatment (prednisone 60 mg/mq/day) was started. During steroid tapering, the children experienced several relapses and to obtain a positive outcome they required therapy with human monoclonal anti-CD20 antibodies (rituximab in the first child, ofatumumab in the second one). Conclusions: Our cases highlight that IgAD can be observed in nephrotic syndrome and nephropathy in children with IgAD appears to be complicated and difficult to treat with corticosteroids alone. Further research is needed to better describe the clinical manifestations and pathological pictures among subjects with IgAD and nephrotic syndrome to understand whether IgAD has a prognostic value in children with nephrotic syndrome and to let clinical physicians define a more personalized and appropriate approach for the management of these patients
Synthesis, Infra-red, Raman, NMR and structural characterization by X-ray Diffraction of [C12H17N2]2CdCl4 and [C6H10N2]2Cd3Cl10 compounds
The synthesis, infra-red, Raman and NMR spectra and crystal structure of 2,
4, 4- trimethyl-4, 5- dihydro-3H-benzo[b] [1, 4] diazepin-1-ium
tetrachlorocadmate, [C12H17N2]2CdCl4 and benzene-1,2-diaminium
decachlorotricadmate(II) [C6H10N2]2Cd3Cl10 are reported.
The [C12H17N2]2CdCl4 compound crystallizes in the triclinic system (P-1 space
group) with Z = 2 and the following unit cell dimensions: a = 9.6653(8)
angstrom, b = 9.9081(9) angstrom, c = 15.3737(2) angstrom, alpha =
79.486(1)degrees, beta = 88.610(8)degrees and gamma = 77.550(7)degrees. The
structure was solved by using 4439 independent reflections down to R value of
0.029. In crystal structure, the tetrachlorocadmiate anion is connected to two
organic cations through N-H...Cl hydrogen bonds and Van Der Waals interaction
as to build cation-anion-cation cohesion. The [C6H10N2]2Cd3Cl10 crystallizes in
the triclinic system (P-1 space group). The unit cell dimensions are a = 6.826
(5)angstrom, b = 9.861 (7)angstrom, c = 10.344 (3)angstrom, alpha = 103.50
(1)degrees, beta = 96.34 (4)degrees and gamma = 109.45 (3)degrees, Z=2. The
final R value is 0.053 (Rw=0.128). Its crystal structure consists of organic
cations and polymeric chains of [Cd3Cl10]4- anions running along the [011]
direction, In The [C6H10N2]2Cd3Cl10 compounds hydrogen bond interactions
between the inorganic chains and the organic cations, contribute to the crystal
packing.
PACS Codes: 61.10.Nz, 61.18.Fs, 78.30.-jComment: 19 pages, 10 figure
The Extracellular Matrix and Blood Vessel Formation: Not Just a Scaffold
The extracellular matrix plays a number of important roles, among them providing structural support and information to cellular structures such as blood vessels imbedded within it. As more complex organisms have evolved, the matrix ability to direct signalling towards the vasculature and remodel in response to signalling from the vasculature has assumed progressively greater importance. This review will focus on the molecules of the extracellular matrix, specifically relating to vessel formation and their ability to signal to the surrounding cells to initiate or terminate processes involved in blood vessel formation
Regulatory effect of a synthetic CRP recognition sequence placed downstream of a promoter.
A series of plasmids were constructed in which a promoter was introduced into a lac-based operon fusion vector. A perfectly symmetrical oligonucleotide of 22-bp corresponding to an idealized binding site for cAMP receptor protein (CRP) of E. coli was chemically synthesized. The synthetic CRP site was placed between the promoter and the lacZ structural gene with varying distances from the promoter. Specific binding of cAMP-CRP complex to the synthetic CRP site was shown by a gel retardation and a DNase I footprinting assays. Plasmid constructs were transformed into crp+ and crp- cells carrying a chromosomal deletion of the lac genes. The regulatory effect of the inserted CRP site was examined by comparing the beta-galactosidase activity and the levels of RNA transcript in two cells harboring the plasmids. We found a strong inhibitory effect of the CRP site in the presence of cAMP and CRP when it was placed close to the promoter. When the CRP site was placed far downstream of the promoter, a moderate repression of transcription was observed
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