A New Case and Review of Chylothorax in Generalized Lymphatic Anomaly and Gorham-Stout Disease.

Generalized lymphatic anomaly (GLA) and Gorham-Stout disease (GSD) are related diseases involving the lymphatic vasculature. Patients with these diseases frequently develop chylothorax, which can cause respiratory distress, failure, and death. Unfortunately, the optimum treatment for GLA and GSD patients with chylothorax remains unknown. Here we review 64 previously reported cases of chylothorax in GLA and GSD and describe a GLA patient with bilateral chylothorax that was treated with a pleurovenous shunt after multiple other treatments failed. Unfortunately, this shunt was not able to control the patient's effusion, and she succumbed to her disease 3 years after the shunt was placed. Interestingly, our literature review revealed that patients with left-sided effusions had better outcomes than patients with either right-sided or bilateral effusions. Taken together, our report highlights the difficulty in managing chylothorax in patients with GLA or GSD and reveals that a better understanding of the cause of chylothorax is needed so that new therapies can be developed to treat this common complication of GLA and GSD.

High contrast x-ray speckle from atomic-scale order in liquids and glasses.

The availability of ultrafast pulses of coherent hard x rays from the Linac Coherent Light Source opens new opportunities for studies of atomic-scale dynamics in amorphous materials. Here, we show that single ultrafast coherent x-ray pulses can be used to observe the speckle contrast in the high-angle diffraction from liquid Ga and glassy Ni(2)Pd(2)P and B(2)O(3). We determine the thresholds above which the x-ray pulses disturb the atomic arrangements. Furthermore, high contrast speckle is observed in scattering patterns from the glasses integrated over many pulses, demonstrating that the source and optics are sufficiently stable for x-ray photon correlation spectroscopy studies of dynamics over a wide range of time scales.

Small-angle x-ray scattering study of kinetics of spinodal decomposition in N-isopropylacrylamide gels.

We present synchrotron-based time-resolved small-angle x-ray scattering (SAXS) measurements of spinodal decomposition in a covalently cross-linked N-isopropylacrylamide gel. The range of wave numbers examined is well beyond the position of the maximum in the structure factor S(q,t). The equilibrium structure factor is described by the sum of a Lorentzian and a Gaussian. Following a temperature jump into the two phase region, the scattered intensity increases with time and eventually saturates. For early times the linear Cahn-Hilliard-Cook (CHC) theory can be used to describe the time evolution of the scattered intensity. From this analysis we found that the growth rate R(q) is linearly dependent on q(2), in agreement with mean-field theoretical predictions. However the Onsager transport coefficient Lambda(q) approximately q(-4), which is stronger than the q dependence predicted by the mean-field theory. We found that the growth rate R(q)>0, even though the wave numbers q probed by SAXS are greater than sqrt[2]q(m) where q(m) is the position of the peak of S(q,t), also in agreement with the mean-field predictions for a deep quench. We have also examined the range of validity of the linear CHC theory, and found that its breakdown occurs earlier at higher wave numbers. At later times, a pinning of the structure was observed. The relaxation to a final, microphase-separated morphology is faster and occurs earlier at the highest wave numbers, which probe length scales comparable to the average distance between crosslinks.