The spectrum of brain and spine MRI abnormalities in Hunter disease is extremely wide and requires a thorough evaluation. WMAs, atrophy/communicating hydrocephalus and spinal stenosis progress over time and might represent possible disease severity markers for new treatment efficacy assessment.
This study on serum ferritin levels in urinary iron excretion after 12h subcutaneous infusion of desferrioxamine in 10 thalassemia intermedia patients shows that even nontransfusion-dependent patients may have positive iron balance resulting in iron overload from 5 years of age. However, the iron overload found in these patients appears to be much lower than in age matched patients with transfusion-dependent thalassemia major. Iron overload increases with advancing age, as shown by increasing serum ferritin levels and desferrioxamine-induced urinary iron elimination. After a six month trial of 12h continuous subcutaneous desferrioxamine administration there was a significant decline in serum ferritin levels. From this study it seems that iron chelation is indicated in thalassemia intermedia patients over 5 years of age in order to prevent iron accumulation. However, the appropriate treatment schedule should be tailored to the individual needs of each patients, established by close monitoring of serum ferritin levels and desferrioxamine-induced urinary iron elimination.
The auditory function of 75 children affected by homozygous β°-thalassemia, managed with a low transfusion scheme and treated irregularly with low doses of desferrioxamine, and of 75 controls were examined. In 12 patients a mild bilateral conductive hearing impairment due to bony hypertrophy and/or adenoid hypertrophy was found. In 43 cases a moderate monolateral or bilateral sensory-neural hearing loss at high frequencies with recruitment phenomenon was observed. Ferritin levels were determined in a randomly chosen group of these patients with (14) and without hearing loss (11). In the subjects with sensory-neural hearing loss the mean ferritin levels were significantly higher than in those with no hearing defect. There was no obvious relation between sensory-neural damage on the one hand and Hb levels and unit of blood transfused on the other. The results of this study suggest that iron overload could be a cause of damage in the high frequency elements of the auditory mechanism. Intermittent hypoxia and slow 8th nerve compression due to bony hypertrophy as causes of auditory involvement are also discussed.
Dense point clouds acquired from Terrestrial Laser Scanners (TLS) have proved to be effective for structural deformation assessment. In the last decade, many researchers have defined methodology and workflow in order to compare different point clouds, with respect to each other or to a known model, assessing the potentialities and limits of this technique. Currently, dense point clouds can be obtained by Close-Range Photogrammetry (CRP) based on a Structure from Motion (SfM) algorithm. This work reports on a comparison between the TLS technique and the Close-Range Photogrammetry using the Structure from Motion algorithm. The analysis of two Reinforced Concrete (RC) beams tested under four-points bending loading is presented. In order to measure displacement distributions, point clouds at different beam loading states were acquired and compared. A description of the instrumentation used and the experimental environment, along with a comprehensive report on the calculations and results obtained is reported. Two kinds of point clouds comparison were investigated: Mesh to mesh and modeling with geometric primitives. The comparison between the mesh to mesh (m2m) approach and the modeling (m) one showed that the latter leads to significantly better results for both TLS and CRP. The results obtained with the TLS for both m2m and m methodologies present a Root Mean Square (RMS) levels below 1 mm, while the CRP method yields to an RMS level of a few millimeters for m2m, and of 1 mm for m.
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