Atypical onset of nephropathic infantile cystinosis in a Russian patient with rare CTNS mutation.

We report a Russian patient with atypical onset of infantile nephropathic cystinosis. The disease debuted with vomiting and loss of weight and motor skills. Nephropathic changes appeared 6 months after onset of disease. Exome sequencing can be useful for diagnosing cystinosis in patients with neurological abnormalities before onset of nephropathic symptoms.

Revisiting the Stability of Spatially Heterogeneous Predator-Prey Systems Under Eutrophication.

We employ partial integro-differential equations to model trophic interaction in a spatially extended heterogeneous environment. Compared to classical reaction-diffusion models, this framework allows us to more realistically describe the situation where movement of individuals occurs on a faster time scale than on the demographic (population) time scale, and we cannot determine population growth based on local density. However, most of the results reported so far for such systems have only been verified numerically and for a particular choice of model functions, which obviously casts doubts about these findings. In this paper, we analyse a class of integro-differential predator-prey models with a highly mobile predator in a heterogeneous environment, and we reveal the main factors stabilizing such systems. In particular, we explore an ecologically relevant case of interactions in a highly eutrophic environment, where the prey carrying capacity can be formally set to 'infinity'. We investigate two main scenarios: (1) the spatial gradient of the growth rate is due to abiotic factors only, and (2) the local growth rate depends on the global density distribution across the environment (e.g. due to non-local self-shading). For an arbitrary spatial gradient of the prey growth rate, we analytically investigate the possibility of the predator-prey equilibrium in such systems and we explore the conditions of stability of this equilibrium. In particular, we demonstrate that for a Holling type I (linear) functional response, the predator can stabilize the system at low prey density even for an 'unlimited' carrying capacity. We conclude that the interplay between spatial heterogeneity in the prey growth and fast displacement of the predator across the habitat works as an efficient stabilizing mechanism. These results highlight the generality of the stabilization mechanisms we find in spatially structured predator-prey ecological systems in a heterogeneous environment.

[Clinical variants of Churg-Strauss eosinophilic vasculitis].

The purpose of the study was to analyze clinical variants of eosinophilic vasculitis (EV). The authors observed 12 EV patients (five men and seven women; mean age 43 +/- 5.7) during a ten-year period. Two of the patients died of ventricular fibrillation due to myocarditis and of mesenterial thrombosis. Clinical variants of EV were diverse. The main syndromes in EV were: predominant lesion of the pulmonary system with polyneuropathy; lesion of the gastrointestinal tract (GIT) with eosinophilia; lesion of the cardiovascular system as well as association with other rheumatologic conditions (rheumatoid arthritis and diffuse eosinophilic fasciitis). The main therapeutic regimens in EV include steroidal therapy in medium to large doses, cyclophosphan intramuscularly, and intensive care--pulse therapy with metipred and plasmapheresis. Analysis of the results of the treatment of these patients demonstrated the efficacy of cyclophosphan administered in doses of approximately 2300 mg per course intramuscularly in combination with prednisolone (more than 40 mg a day), plasmapheresis (more than three sessions per a course), antiaggregants, heparin (more than 18000 units a day) and general hyperbaric oxygenation, as well as pulse therapy with metipred in a dose of 2200 mg per three days as intravenous infusion. The prognosis of EV was most poor in cases of lesion of the cardiovascular system and the GIT.