We report an infant with intermittent urinary excretion of D-2-hydroxyglutaric (D-2-OHG) acid who died at the age of 10 months from cardiogenic shock due to cardiomyopathy. High urinary concentrations of D-2-OHG and succinic acid, as well as increased levels of lactic acid were detected on three different occasions, whereas a normal urinary profile of organic acids was found on one occasion. The clinical findings of our patient consisted of generalized hypotonia, irritability, developmental delay, generalized tonic seizures, lethargy, cardiomyopathy, and respiratory distress. Cerebral MRI revealed bilateral lesions in the substantia nigra, the periaqueductal area, the medial part of the thalamus, the hypothalamus, the caudate nucleus, putamen and globus pallidus. This pattern is suggestive of a mitochondriopathy. However, respiratory chain enzyme activities were normal in fibroblasts. Exogenous supplementation of D-2-OHG acid strongly inhibited cytochrome-c oxidase activity in fibroblasts from the patient and from normal controls in vitro. The results suggest that our patient has an unusual form of D-2-hydroxyglutaric aciduria (D-2-OHGA), different from the patients published so far, and that the increase of lactic acid and some citric acid cycle intermediates encountered in some patients with D-2-OHGA may be due to a functional defect of the respiratory chain caused by D-2-OHG acid.
Aims: To investigate the effect of different concentrations of ZnO nanoparticles on the compressive strength of type IV dental stone. Materials and methods: Total specimens of this research was 66 specimens, it was divided into 3 groups, the first one for wet strength , second group for dry strength and third groups for scanning electron microscope analysis. 0.2%, 0.5% , 1.0% ,1.5% and 2.0% weight of ZnO were incorporated into type IV dental stone. The study evaluated both wet and dry compressive strength and compared them with the control specimen and investigate it under SEM. The results of the study were analyzed statistically by (Mean ± standard deviation, ANOVA and Duncan's multiple range test) and the results were considered significant at p ≤ 0.05. Results: The compressive strength decreased in comparison to control specimens. This drop in strength value is not significant in wet strength, while significant in dry strength. SEM image exhibited the morphology of both stone crystals and zinc oxide NPs and exhibited the distribution of NPs inside the specimens Conclusions: The study showed that incorporation of zinc oxide nanoparticles into dental stone reduced the compressive strength property of dental stone and NPs evenly distributed into dental stone.
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