"Soroka" University Hospital, Beer-Sheva.
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Biomedical subjects
Publications and source records attributed to Y Shapira.
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Retinitis pigmentosa (RP) and primary muscle disease are both relatively rare inherited disorders. We present a patient in whom both conditions coexisted. The possibility of a genetic association between RP and primary muscle disorder is discussed. It is our belief that this extremely rare association is merely coincidental.
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The development of biochemical parameters (cellular DNA and protein) in relation to birth weight was studied in the rabbit, a perinatal brain developer. To induce intrauterine growth retardation and to increase the number of low-birth-weight rabbits, experimental ischemia, in half the fetuses of each doe, was achieved by total ligation of approximately 30% of uteroplacental vessels during the last third of gestation. Following natural delivery, the rabbit pups were raised until 60 days of age, at which time the brains were removed and dissected into cerebral hemispheres, cerebellum and brain stem. The amount of DNA (representing cell number) and protein (suggesting cell size) was estimated in each brain region. A significant correlation was found between low birth weight and reduced DNA in the cerebellum and reduced protein in the cerebral hemispheres. These persistent deficiencies could be related to some lasting handicaps, especially motor incoordination, as an expression of cerebellar dysfunction.
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This article presents five cases of an unusual dental anomaly--transposition of mandibular canine and lateral incisor. While every one of the presented cases had its individual characteristics, there are certain common traits. For instance, deciduous canines are usually retained, and the anomaly is found concurrent with other dental problems, such as congenital absence of teeth or peg-shaped lateral incisors. Many transposition cases also have a severe arch length discrepancy, which in itself may be an etiologic factor. In two of our cases orthodontic management included extraction of the transposed lateral incisor, whereas in the remaining three cases teeth were left in their transposed positions. On the basis of the longitudinal records of one of our cases, we speculate that there are early signs of transposition in the making, signs which should be recognized and which could possibly make transposition preventable.
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The Denver Developmental Screening Test (DDST), standardized in the USA, is used for the early detection of developmental disabilities. For use in Israel, the DDST required translation into Hebrew and standardization for Israeli children in view of the test's known cultural bias. The sample included 2,248 children between the ages of 2 wk and 6.5 yr, and was stratified by demographic, ethnic and socioeconomic characteristics to approximate a representative cross-section of the Israeli population. The testers were not psychologists but they received special training. Our results revealed significant differences between the norms of the DDST and its Israeli version, mainly in the personal-social items. For 23 test items there was a difference of greater than 20% between the ages at which they were achieved by 50% of Israeli and Denver children, and for 35 items, there was a difference of greater than 20% in the 90th percentile ages in the two groups. Israeli children showed earlier development in language and personal-social items, while the Denver sample showed earlier development in fine and gross motor skills. The time range between the 25th and 90th percentile in many of the items in the Israeli sample was greater than in the Denver sample. There were sufficient differences between the findings in the two groups to justify using the Israeli version for the local population.
Blink reflex was studied in 12 neurologically intact infants and children aged 2 weeks to 3 years. A coaxial needle electrode inserted in the outer lower quadrant of the orbicularis oculi muscle was used for recording. Responses were elicited by a 50-100V current delivered through a stimulating electrode placed on the supraorbital groove. Absence of contralateral R2 response was a consistent finding. Ipsilateral R2 responses were elicited in 50% of the cases. R1 response was present in all instances and showed latency values similar to adults. The amplitudes of both R1 and R2 were lower than reported in adult population. The blink reflex may be an indication of the degree of myelination of the brain stem, facial and trigeminal pathways in children.
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Seven cases of ectopically erupting permanent mandibular lateral incisors are reported and their orthodontic management is described. In severely crowded cases the ectopic lateral incisor was extracted. When complete transposition occurred, the teeth were aligned in their transposed positions. If malposition is detected early, orthodontic correction may prevent development of a transposition. Panoramic survey of the developing dentition at the age of 6 to 8 years is a very useful tool in the early detection of this as well as many other dental developmental anomalies.
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An atypical clinical presentation, the diagnostic procedures and a novel surgical approach are described in a 67-year-old woman with a patent ductus arteriosus.