Anisotropic N-Graphene-diffused Co3O4 nanocrystals with dense upper-zone top-on-plane exposure facets as effective ORR electrocatalysts.

We provide strong evidence of the effectiveness of homogenously self-propelled particle-in-particle diffusion, interaction and growth protocol. This technique was used for one-pot synthesis of novel nitrogen-graphene oxide (N-GO)/Co3O4 nanocrystals with cuboid rectangular prism-shaped nanorods (NRs) along {110}-plane and truncated polyhedrons with densely-exposed, multi-facet sites along {311} and {111} planes. These hierarchal nanocrystals create electrode catalyst patterns with vast-range accessibility to active Co2+ sites, a vascular system for the transport and retention of captured O2 molecule interiorly, and low adsorption energy and dense electron configuration surfaces during the oxygen reduction reaction (ORR). The superior electrocatalytic ORR activity of the N-GO/Co3O4 polyhedron nanocrystals in terms of electrochemical selectivity, durability and stability compared with NRs or commercial Pt/C catalysts confirms the synergetic contribution of multi-functional, dense-exposed, and actively topographic facets of polyhedrons to significantly activate the catalytic nature of the catalyst. Our findings show real evidence, for the first time that not only the large number of catalytically active Co2+ cations at the top surface layer but also the dense location of active Co2+ sites on the upper-zone top-on-plane exposure, and the electron density configuration and distribution around the Co2+ sites were important for effective ORR.

Hypermanganesemia with dystonia, polycythemia and cirrhosis in 10 patients: Six novel SLC30A10 mutations and further phenotype delineation.

Biallelic mutations in the SLC30A10 gene cause an inborn error of Mn metabolism characterized by hypermanganesemia, polycythemia, early-onset dystonia, and liver cirrhosis (HMDPC). To date, only 14 families from various ethnic groups have been reported. Here, we describe 10 patients from 7 unrelated Egyptian families with HMDPC. Markedly elevated blood Mn levels, the characteristic basal ganglia hyperintensity on T1-weighted images, and variable degrees of extrapyramidal manifestations with or without liver disease were cardinal features in all patients. Eight patients presented with striking early diseased onset (≤2 years). Unexpectedly, early hepatic involvement before the neurological regression was noted in 3 patients. Mutational analysis of SLC30A10 gene revealed 6 novel homozygous mutations (c.77T > C (p.Leu26Pro), c.90C > G (p.Tyr30*), c.119A > C (p.Asp40Ala), c.122_124delCCT (p.Ser41del), c.780_782delCAT (p.Iso260del) and c.957 + 1G > C). Treatment using 2,3 dimercaptosuccinic acid as a manganese chelating agent showed satisfactory results with improvement of biochemical markers, hepatic manifestations and relative amelioration of the neurological symptoms. Our findings present a large cohort of patients with HMDPC from same ethnic group. The majority of our patients showed severe and early presentation with clear phenotypic variability among sibship. Moreover, we extend the phenotypic and mutational spectrum and emphasize the importance of early diagnosis and treatment of this potentially fatal disorder.

Renal functional and structural integrity in infants with iron deficiency anemia: relation to oxidative stress and response to iron therapy.

BACKGROUND: Iron deficiency anemia (IDA) is the most common nutritional deficiency in the world. The aim of our study was to evaluate and compare renal functional and structural integrity in 50 infants with IDA and 50 healthy controls and to assess the relation between IDA and oxidative stress and response to iron therapy. METHODS: This was a prospective study in which peripheral blood samples were collected from all study subjects and the following laboratory investigations performed: serum iron profile, urinary microalbumin, urinary leucine aminopeptidase (LAP), fractional excretion of sodium (FeNa), serum total antioxidant capacity (TAC), serum malondialdehyde (MDA), serum and urinary trace elements (iron, copper, zinc, calcium and magnesium). All patients received oral iron therapy and were followed-up for 3 months. RESULTS: The levels of baseline urinary markers were higher among the patients with IDA than among the controls (p < 0.05). Patients had a lower pre-therapy TAC and lower serum zinc and magnesium levels than controls as well as higher MDA and serum copper levels (p < 0.05). MDA level was positively correlated to microalbumin and LAP level (p < 0.05). Urinary LAP concentration was positively correlated to urinary trace element concentrations (p < 0.05). A significant decrease in microalbumin, LAP, FeNa, and urinary trace elements was observed post-iron therapy while hemoglobin and ferritin levels were increased (p < 0.05). CONCLUSION: Among the study subjects, IDA had an adverse influence on renal functional and structural integrity which could be reversed with iron therapy. Oxidative stress played an important role in the pathogenesis of renal injury in IDA.