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

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

Quantum kk-core conduction on the Bethe lattice

Classical and quantum conduction on a bond-diluted Bethe lattice is considered. The bond dilution is subject to the constraint that every occupied bond must have at least $k-1$ neighboring occupied bonds, i.e. $k$-core diluted. In the classical case, we find the onset of conduction for $k=2$ is continuous, while for $k=3$, the onset of conduction is discontinuous with the geometric random first-order phase transition driving the conduction transition. In the quantum case, treating each occupied bond as a random scatterer, we find for $k=3$ that the random first-order phase transition in the geometry also drives the onset of quantum conduction giving rise to a new universality class of Anderson localization transitions.Comment: 12 pgs., 6 fig

Three-dimensional cell to tissue assembly process

The present invention relates a 3-dimensional cell to tissue and maintenance process, more particularly to methods of culturing cells in a culture environment, either in space or in a gravity field, with minimum fluid shear stress, freedom for 3-dimensional spatial orientation of the suspended particles and localization of particles with differing or similar sedimentation properties in a similar spatial region

Duality Symmetry in the Schwarz-Sen Model

The continuous extension of the discrete duality symmetry of the Schwarz-Sen model is studied. The corresponding infinitesimal generator $Q$ turns out to be local, gauge invariant and metric independent. Furthermore, $Q$ commutes with all the conformal group generators. We also show that $Q$ is equivalent to the non---local duality transformation generator found in the Hamiltonian formulation of Maxwell theory. We next consider the Batalin--Fradkin-Vilkovisky formalism for the Maxwell theory and demonstrate that requiring a local duality transformation lead us to the Schwarz--Sen formulation. The partition functions are shown to be the same which implies the quantum equivalence of the two approaches.Comment: 10 pages, latex, small changes, final version to appear in Phys. Rev.

Effect of Poisson ratio on cellular structure formation

Mechanically active cells in soft media act as force dipoles. The resulting elastic interactions are long-ranged and favor the formation of strings. We show analytically that due to screening, the effective interaction between strings decays exponentially, with a decay length determined only by geometry. Both for disordered and ordered arrangements of cells, we predict novel phase transitions from paraelastic to ferroelastic and anti-ferroelastic phases as a function of Poisson ratio.Comment: 4 pages, Revtex, 4 Postscript figures include

Kaluza-Klein electrically charged black branes in M-theory

We present a class of Kaluza-Klein electrically charged black p-brane solutions of ten-dimensional, type IIA superstring theory. Uplifting to eleven dimensions these solutions are studied in the context of M-theory. They can be interpreted either as a p+1 extended object trapped around the eleventh dimension along which momentum is flowing or as a boost of the following backgrounds: the Schwarzschild black (p+1)-brane or the product of the (10-p)-dimensional Euclidean Schwarzschild manifold with the (p+1)-dimensional Minkowski spacetime.Comment: 16 pages, uses latex and epsf macro, figures include