Orthopaedic management of Hurler’s disease after hematopoietic stem cell transplantation: a systematic review

The introduction of hematopoietic stem cell transplantation (HSCT) has significantly improved the life-span of Hurler patients (mucopolysaccharidosis type I-H, MPS I-H). Yet, the musculoskeletal manifestations seem largely unresponsive to HSCT. In order to facilitate evidence based management, the aim of the current study was to give a systematic overview of the orthopaedic complications and motor functioning of Hurler's patients after HSCT. A systematic review was conducted of the medical literature published from January 1981 to June 2010. Two reviewers independently assessed all eligible citations, as identified from the Pubmed and Embase databases. A pre-developed data extraction form was used to systematically collect information on the prevalence of radiological and clinical signs, and on the orthopaedic treatments and outcomes. A total of 32 studies, including 399 patient reports were identified. The most frequent musculoskeletal abnormalities were odontoid hypoplasia (72%), thoracolumbar kyphosis (81%), genu valgum (70%), hip dysplasia (90%) and carpal tunnel syndrome (63%), which were often treated surgically during the first decade of life. The overall complication rate of surgical interventions was 13.5%. Motor functioning was further hampered due to reduced joint mobility, hand dexterity, motor development and longitudinal growth. Stem cell transplantation does not halt the progression of a large range of disabling musculoskeletal abnormalities in Hurler's disease. Although prospective data on the quantification, progression and treatment of these deformities were very limited, early surgical intervention is often advocated. Prospective data collection will be mandatory to achieve better evidence on the effect of treatment strategies

Short-Lived Trace Gases in the Surface Ocean and the Atmosphere

The two-way exchange of trace gases between the ocean and the atmosphere is important for both the chemistry and physics of the atmosphere and the biogeochemistry of the oceans, including the global cycling of elements. Here we review these exchanges and their importance for a range of gases whose lifetimes are generally short compared to the main greenhouse gases and which are, in most cases, more reactive than them. Gases considered include sulphur and related compounds, organohalogens, non-methane hydrocarbons, ozone, ammonia and related compounds, hydrogen and carbon monoxide. Finally, we stress the interactivity of the system, the importance of process understanding for modeling, the need for more extensive field measurements and their better seasonal coverage, the importance of inter-calibration exercises and finally the need to show the importance of air-sea exchanges for global cycling and how the field fits into the broader context of Earth System Science

Nitrate formation during ozonation as a surrogate parameter for abatement of micropollutants and the N-nitrosodimethylamine (NDMA) formation potential

In this study, nitrate formation from ammonium and/or dissolved organic nitrogen (DON) was investigated as a novel surrogate parameter to evaluate the abatement of micropollutants during ozonation of synthetic waters containing natural organic matter (NOM) isolates, a natural water and secondary wastewater effluents. Nitrate formation during ozonation was compared to the changes in UV absorbance at 254 nm (UVA(254)) including the effect of pH. For low specific ozone doses UVA254 was abated more efficiently than nitrate was formed. This is due to a relatively slow rate-limiting step for nitrate formation from the reaction between ozone and a proposed nitrogen-containing intermediate. This reaction cannot compete with the fast reactions between ozone and UV-absorbing moieties (e.g., activated aromatic compounds). To further test the kinetics of nitrate formation, two possible intermediates formed during ozonation of DON were tested. At pH 7, nitrate was formed during ozonation of acetone oxime and methyl nitroacetate with second-order rate constants of 256.7 +/- 4.7 M-1 s(-1) and 149.5 +/- 5.8 M-1 s(-1), respectively. The abatement of the selected micropollutants (i.e., 17 alpha-ethinylestradiol (EE2), carbamazepine (CBZ), bezafibrate (BZF), ibuprofen (IBU), and p-chlorobenzoic acid (pCBA)) was investigated for specific ozone doses <= 1.53 mgO(3)/mgDOC and its efficiency depended strongly on the reactivity of the selected compounds with ozone. The relative abatement of micropollutants (i.e., EE2 and CBZ) with high ozone reactivity showed linear relationships with nitrate formation. The abatement of micropollutants with intermediate-low ozone reactivity (BZF, IBU, and pCBA) followed one- and twophase behaviors relative to nitrate formation during ozonation of water samples containing high and low concentrations of nitrate-forming DON, respectively. During ozonation of a wastewater sample, the Nnitrosodimethylamine formation potential (NDMA-FP) during chloramination decreased with increasing specific ozone doses. A good correlation was obtained between NDMA-FP abatement and nitrate formation. Therefore, nitrate formation after pre-ozonation may be a useful parameter to estimate the reduction of the NDMA-FP during post-chloramination. Overall, the results of this study suggest that nitrate formation (possibly in combination with UVA254 abatement) during ozonation of DON-containing waters may be a good surrogate for assessing the abatement of micropollutants and the NDMA-FP. (C) 2017 Elsevier Ltd. All rights reserved