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The Chiari type II malformation is the leading cause of death in infants with myelomeningocele. The authors report 17 cases of symptomatic Chiari type II malformation occurring in two distinct age dependent population. In Group I, 13 neonates and infants in the first year of life presented with cranial nerve and brain stem dysfunction characterized by vocal cord paralysis, apnea, dysphagia and laryngeal stridor. In Group II, 4 patients developed signs and symptoms after the first year of life. In this group, the presentation was more insidious and included neck pain and cerebellar manifestations. The surgical treatment consisted initially in shunt implantation or revision and when there was no improvement, posterior fossa decompression was performed. The response to the surgical treatment differed considerably between the two groups: older patients improved promptly after surgery and there was no casualties; in newborn and infants, especially those under 6 months of age, the mortality rate was 46.1%. The authors stress that prompt diagnosis and surgical intervention should be performed in order to produce a favorable outcome.  相似文献   
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Combination therapies that include metronidazole (MTZ) are the most successful therapies used in eradicating Helicobacter pylori. In this study, the prevalence and the relevance of heterogeneity in susceptibility to MTZ among H. pylori populations of 156 patients were evaluated. The results of this study show that 37 patients (24%) were infected with MTZ-resistant H. pylori (MIC > or = 8 micrograms/ml). Furthermore, 33% (52 of 156) of the patients were found to be infected with H. pylori populations heterogeneous for their susceptibility to MTZ. The reassessment of the MICs of MTZ for these 52 H. pylori populations revealed MTZ resistance in 28 of them, increasing the number of MTZ-resistant H. pylori populations among the 156 patients to 65 (42%). Out of 20 isolates, 2 (10%) heterogeneous in their susceptibility to MTZ also appeared to be heterogeneous at the genome level as determined by randomly amplified polymorphic DNA fingerprinting. In conclusion, the results show the limitations and risk of possible misinterpretations when only a single colony, picked from the primary H. pylori populations isolated from patients, is analyzed for its susceptibility to MTZ.  相似文献   
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One hundred and twenty-four patients over the age of 75 years were assessed for the cause of their macrocytosis (MCV > 95 fl). A definitive diagnosis was reached in 75/124 (60%) by non-invasive techniques. The remainder underwent a bone marrow biopsy yielding a definitive diagnosis in a further six patients who had an identifiable myelodysplastic syndrome (MDS). A high proportion of the remainder had morphological abnormalities which fitted with no recognized pathological entity. It is suggested that these may represent MDS in evolution.  相似文献   
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In 4 experiments, chronometric evidence for keypress schemata in typing was sought by presenting stimuli to be typed in positions that were displaced from a central fixation point. Reaction times were shorter when stimulus positions corresponded to keyboard locations of the letters to be typed, suggesting that position was an important part of the internal representation of the response. Experiment 1 presented single letters left and right of fixation. Experiment 2 presented single letters above and below fixation. Experiment 3 presented words left and right of fixation and found evidence of parallel activation of keypress schemata. Experiment 4 found no effect of the eccentricity of the keyboard locations and responding fingers, suggesting that response-location codes are categorical, not metric. The results are consistent with D. E. Rumelhart and D. A. Norman's (1982) theory of typewriting. (PsycINFO Database Record (c) 2010 APA, all rights reserved)  相似文献   
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Buried heterostructure lasers with highly strained InGaAs-InGaAsP active regions, emitting at 2 μm have been fabricated and tested. The lasers exhibited threshold current densities of 500 A/cm2 for 1-mm-long cavities, an internal loss of 11 cm-1, and characteristic temperatures as high as 50°C. The gain characteristics were also investigated and a linewidth enhancement factor of 8 was determined  相似文献   
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Unstable expansion of the CTG repeats in the 3' untranslated region encoding a member of the protein kinase family in the q13.3 band on chromosome 19 is a mutation specific for myotonic dystrophy. To examine the correlation between clinical expression and CTG trinucleotide repeat length, we carried out Southern blot analysis in a family with myotonic dystrophy. In this pedigree, the expanded CTG repeats were transmitted maternally. The mother had three female children. The mother had about 200 CTG repeats, and the number of repeats for each child was about 800, 1500 and 1600 in birth order. The mother and the patient with 800 repeats were unaware of muscle weakness or myotonia. Symptoms were present from age 3 years in the patient with 1500 repeats and from birth in the one with 1600 repeats. Although the mother menstruated regularly, the patients with 800 and 1500 repeats both menstruated irregularly, and the one with 1600 repeats has never menstruated. The age of onset and severity of the disease were correlated with the size of the expanded repeats. Endocrinological studies revealed that the basal levels of the gonadotropins, PRL and E2 were within normal range, and a pituitary response to LHRH was observed. These data suggest that the amenorrhea and menstrual irregularities were caused by a suprahypophyseal dysfunction. When expanded CTG repeats are transmitted maternally, abnormal products resulting from the metabolic disturbance in the affected mother may harm the fetus in utero. A heterozygous fetus, who has more CTG repeats, may be unable to metabolize the pathologic products sufficiently and therefore may become more severely affected. This may explain the exclusive maternal transmission of congenital myotonic dystrophy.  相似文献   
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