Growth Pattern of Untreated Boys with Simple Virilizing Congenital Adrenal Hyperplasia Indicates Relative Androgen Insensitivity during the First Six Months of Life

Context: Mild forms of simple virilizing congenital adrenal hyperplasia (CAH) may be missed in newborn screening. In the pre-newborn-screening era, missed diagnosis of simple virilizing CAH was not infrequent in boys. Elevated adrenal androgens lead to accelerated growth and bone maturation. Traditional treatment of CA H consists of the suppression of ACTH through glucocorticoid replacement, in an attempt to reduce excessive androgen production. Objective: To retrospectively analyze early growth pattern and bone maturation in untreated boys with simple virilizing CAH. Patients: In the pre-newborn screening era, 13 boys had a late diagnosis of simple virilizing classical CAH. Diagnosis of 21-hydroxylase deficiency was confirmed by mutation analysis of the CYP21A2 gene in all patients. Growth data were retrospectively collected from standarized preventive medical checkups at the regular pediatrician until the time of diagnosis of CAH. Results: Length was 0.1 +/- 0.8 SDS (mean +/- SD) at birth, 0.2 +/- 1 SDS at 3 months, 0.2 +/- 0.9 SDS at 6 months, 0.7 +/- 1 SDS at 1 year, +1.1 +/- 0.9 SDS at 2 years and +1.8 +/- 1.2 SDS at 4 years. At diagnosis, mean chronological age was 4.4 +/- 1.6 years and height SDS was 2 +/- 1.7. Bone age was accelerated (9.4 +/- 4 years) at diagnosis. Signs that had led to diagnosis were pubic hair (n = 11), accelerated growth rate (n = 6) and birth of an affected sister (n = 3). Despite late start of hydrocortisone treatment, mean final height was 1 +/- 0.9 SDS. Seven of 18 patients had a final height within 1 SD of target height. Conclusion: Height velocity is not markedly increased in untreated boys with simple virilizing CAH in the first 6 months of life, indicating that infants are relatively androgen insensitive during that period. After the first 6 months of life, growth velocity increases significantly and elevated androgens lead to advanced skeletal maturation. This observation has implications for lower hydrocortisone doses to be used in CAH children during the first 6 months of life. In addition, staying alert for clinical symptoms and signs of simple virilizing CAH is still warranted, since mild forms may be missed in newborn screening. Copyright (C) 20105. Karger AG, Base

Growth Patterns in the First Three Years of Life in Children with Classical Congenital Adrenal Hyperplasia Diagnosed by Newborn Screening and Treated with Low Doses of Hydrocortisone

Background: Linear growth is the best clinical parameter for monitoring metabolic control in classical congenital adrenal hyperplasia (CAH). Objective: To analyze growth patterns in children with CAH diagnosed by newborn screening and treated with relatively low doses of hydrocortisone during the first year of life. Patients and Methods: 51 patients (27 females) were diagnosed with classical CAH by newborn screening. All patients were treated with relatively low doses of hydrocortisone (9-15 mg/m(2) body surface area). 47 patients were additionally treated with fludrocortisone. Results: At birth, height SDS (H-SDS) was 1.1 +/- 1 in girls and 0.9 +/- 1.5 in boys. After 3 months, H-SDS decreased to 0.4 +/- 0.9 in girls and to 0.1 +/- 1.3 in boys. Over the 3-year period, H-SDS further decreased to -0.4 +/- 1.8 in girls and to -0.8 +/- 1 in boys and approached the genetic height potential (target H-SDS of girls -0.5 +/- 0.3 and target H-SDS of boys -0.9 +/- 0.7). During the first 9 months of age, growth velocity was slightly decreased in girls (18.2 +/- 1.9 cm) and boys (17.3 +/- 1.6 cm) when compared to a healthy reference population (girls 19.0 +/- 3.9 cm and boys 18.7 +/- 4.7 cm). At the age of 3 years, bone age was appropriate for chronological age in both girls (2.7 +/- 0.5 years) and boys (2.9 +/- 0.5 years). Conclusion: Birth length is above average in children with classical CAH, which might be the result of untreated hyperandrogenism in utero. With relatively low doses of hydrocortisone treatment, growth velocity decreases slightly during the first 9 months and H-SDS then approaches the genetic height potential. Copyright (C) 2010 S. Karger AG, Base

Novel Symmetry of Non-Einsteinian Gravity in Two Dimensions

The integrability of $R^2$-gravity with torsion in two dimensions is traced to an ultralocal dynamical symmetry of constraints and momenta in Hamiltonian phase space. It may be interpreted as a quadratically deformed $iso(2,1)$-algebra with the deformation consisting of the Casimir operators of the undeformed algebra. The locally conserved quantity encountered in the explicit solution is identified as an element of the centre of this algebra. Specific contractions of the algebra are related to specific limits of the explicit solutions of this model.Comment: 17 pages, TUW-92-04 (LaTeX

Turbulent Interface Detector Using a Multiple Array of Single Hot Wires

Intermittency circuits can be used to experimentally determine some of the properties of the turbulent interface that occur in turbulent shear flows. The signal I(x,t) is unity if turbulence is present at x and zero otherwise. Using the signals obtained from detectors at one or more points, certain statistical measures of the interface position Y(x,t) can be determined. Also using a linear array of conventional single hot-wire probes, the position of the interface can be detected continuously to within some small error. The intent of this h-detector is similar to the wave-height detector used in studies of sea surfaces. The signal from the h-detector can be used directly to obtain a variety of important statistical measures of the shape of the interface. Measurements in a plane turbulent wall-jet are shown as an example of the application of an h-detector

Increased productivity of Clostridium acetobutylicum fermentation of acetone, butanol, and ethanol by pervaporation through supported ionic liquid membrane

Pervaporation proved to be one of the best methods to remove solvents out of a solvent producing Clostridium acetobutylicum culture. By using an ionic liquid (IL)-polydimethylsiloxane (PDMS) ultrafiltration membrane (pore size 60 nm), we could guarantee high stability and selectivity during all measurements carried out at 37C. Overall solvent productivity of fermentation connected with continuous product removal by pervaporation was 2.34 g l(-1) h(-1). The supported ionic liquid membrane (SILM) was impregnated with 15 wt% of a novel ionic liquid (tetrapropylammonium tetracyano-borate) and 85 wt% of polydimethylsiloxane. Pervaporation, accomplished with the optimized SILM, led to stable and efficient removal of the solvents butan-1-ol and acetone out of a C. acetobutylicum culture. By pervaporation through SILM, we removed more butan-1-ol than C. acetobutylicum was able to produce. Therefore, we added an extra dose of butan-1-ol to run fermentation on limiting values where the bacteria would still be able to survive its lethal concentration (15.82 g/l). After pervaporation was switched off, the bacteria died from high concentration of butan-1-ol, which they produced

Image interpolation using Shearlet based iterative refinement

This paper proposes an image interpolation algorithm exploiting sparse representation for natural images. It involves three main steps: (a) obtaining an initial estimate of the high resolution image using linear methods like FIR filtering, (b) promoting sparsity in a selected dictionary through iterative thresholding, and (c) extracting high frequency information from the approximation to refine the initial estimate. For the sparse modeling, a shearlet dictionary is chosen to yield a multiscale directional representation. The proposed algorithm is compared to several state-of-the-art methods to assess its objective as well as subjective performance. Compared to the cubic spline interpolation method, an average PSNR gain of around 0.8 dB is observed over a dataset of 200 images

Search for X-ray Afterglows from Gamma-Ray Bursts in the RASS

We report on a search for X-ray afterglows from gamma-ray bursts using the ROSAT all-sky survey (RASS) data. If the emission in the soft X-ray band is significantly less beamed than in the gamma-ray band, we expect to detect many afterglows in the RASS. Our search procedure generated 23 afterglow candidates, where about 4 detections are predicted. Follow-up spectroscopy of several counterpart candidates strongly suggests a flare star origin of the RASS events in many, if not all, cases. Given the small number of events we conclude that the data are consistent with comparable beaming angles in the X-ray and gamma-ray bands. Models predicting a large amount of energy emerging as a nearly isotropic X-ray component, and a so far undetected class of ``dirty fireballs'' and re-bursts are constrained.Comment: 5 pages, LATEX with aipproc.sty, incl. 1 ps-Fig., Proc. of the 5th Huntsville Gamma Ray Burst Symposium, Oct. 1999, ed. R.M. Kippen, AIP; also available at http://www.aip.de/~jcg/publis.htm

Relativistic J-matrix method

The relativistic version of the J-matrix method for a scattering problem on the potential vanishing faster than the Coulomb one is formulated. As in the non-relativistic case it leads to a finite algebraic eigenvalue problem. The derived expression for the tangent of phase shift is simply related to the non-relativistic case formula and gives the latter as a limit case. It is due to the fact that the used basis set satisfies the ``kinetic balance condition''.Comment: 21 pages, RevTeX, accepted for publication in Phys. Rev.

Polarizations and Nullcone of Representations of Reductive Groups

The paper starts with the following simple observation. Let V be a representation of a reductive group G, and let f_1,f_2,...,f_n be homogeneous invariant functions. Then the polarizations of f_1,f_2,...,f_n define the nullcone of k 0} h(t) x = 0 for all x in L. This is then applied to many examples. A surprising result is about the group SL(2,C) where almost all representations V have the property that all linear subspaces of the nullcone are annihilated. Again, this has interesting applications to the invariants on several copies. Another result concerns the n-qubits which appear in quantum computing. This is the representation of a product of n copies of $SL_2$ on the n-fold tensor product C^2 otimes C^2 otimes ... otimes C^2. Here we show just the opposite, namely that the polarizations never define the nullcone of several copies if n <= 3. (An earlier version of this paper, distributed in 2002, was split into two parts; the first part with the title ``On the nullcone of representations of reductive groups'' is published in Pacific J. Math. {bf 224} (2006), 119--140.