Plasmapheresis Combined with Continuous Plasma Filtration Adsorption Rescues Severe Acute Liver Failure in Wilson's Disease before Liver Transplantation.

Wilsonian crisis (hemolytic crisis and acute liver failure [ALF] in Wilson's disease) is fatal and almost all patients ultimately need a timely liver transplantation to save their lives. How to safely pass the critical period to liver transplantation is still a big challenge to doctors especially to pediatricians facing more difficult situations in blood purification. Here, we report about a 7-year-old child (weight 21 kg) presenting with severe hemolysis and impending ALF that made a rapid recovery with prompt initiation of plasmapheresis combined with continuous plasma filtration adsorption (CPFA) and chelation therapy. Rapidly efficient removal of copper, bilirubin, and albumin-binding toxins by hybrid blood purification alleviated hemolysis and liver injury and successfully bridged the patient over the critical period to late liver transplantation. Moreover, a review of the literature was performed examining the use of plasmapheresis, molecular adsorbent recirculation system, single-pass albumin dialysis, and continuous veno-venous hemodiafiltration in Wilson disease. The experience of our case points to that plasmapheresis combined with CPFA treatment can improve clinical symptoms and bridge children over through Wilsonian crisis to late live transplantation, and CPFA treatment is feasible and safe in children weighing more than 20 kg.

[Influence of nitrogen and phosphorus addition on the aboveground biomass in Inner Mongo- lia temperate steppe, China].

The plants in arid environment are constrained not only by water availability, but also by soil nutrient conditions. In order to clarify to what extent nutrient addition would facilitate the growth of plants in semi-arid region, we conducted a nitrogen (N) and phosphorus (P) addition experiment in Inner Mongolia temperate grassland in 2012 and 2013. In our experiment, N was added at 10 and 40 g N · m(-2) · a(-1) alone or in combination with P addition (10 g P · m(-2) · a(-1)). N addition significantly improved plant aboveground biomass (AGB) during the two study years. AGB in the treatments of 10 and 40 g · m2 · a(-1) was enhanced by 50.8% and 65.9% in 2012, and 71.6% and 93.3% in 2013, respectively. However, no significant difference in AGB enhancement was found between two N addition treatments. Compared with N addition treatments at the rates of 10 and 40 g · m(-2) · a(-1), N plus P addition improved AGB by 98.4% and 186.8% in 2012, and 111.7% and 141.4% in 2013, respectively. N addition generally increased all the three main functional types (i.e., Gramineae, Asteraceae and others) , and the three functional types contributed nearly equally to the increase of the community AGB. In comparison, Asteraceae contributed largest to the increments of AGB under the N plus P addition treatments. Our results also indicated that N and P addition remarkably increased the ground coverage, resulting in improved surface soil moisture condition, which might be one important reason that N and P addition could facilitate plant growth in arid environment.

A naturally-occurring mutation in Cacna1f in a rat model of congenital stationary night blindness.

PURPOSE: To identify the gene mutation responsible for a previously described rat model of X-linked congenital stationary night blindness (CSNB). METHODS: Rat orthologous genes for Nyx and Cacna1f were isolated from retina through rapid amplification the cDNA ends (RACE) and examined for mutations. Electroretinograms were used to identify affected animals. RESULTS: The rat Nyx cDNA spans 1,971 nucleotides and encodes a protein of 476 amino acids (GenBank: DQ393414). The rat Cacna1f cDNA spans 6,076 nucleotides and encodes a protein of 1,980 amino acids (GenBank: DQ393415). A c.2941C>T (p.R981Stop) mutation in Cacna1f was found in affected rats. Immunochemistry study showed labeling for rod bipolar and horizontal cells were reduced in affect retinas. For affected rats, b-wave and oscillatory potentials of scotopic ERG were absent, and b-wave of photopic ERG was clear but obviously reduced. CONCLUSIONS: The Cacna1f mutation identified in the rat model of CSNB was predicted to lead to a protein product that is shortened by 999 amino acids, indicating that this is a model for the incomplete subtype of human X-linked CSNB (CSNB2). This rat model will be useful for defining the pathophysiological properties of this human disorder.