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Neutropenia is an almost constant feature of Chediak-Higashi syndrome (CHS). There is evidence for a central mechanism of neutropenia. Ultrastructural studies of the bone marrow from a child with CHS showed marked autophagia phenomena within myeloid precursor cells and mature neutrophils. Autophagic vacuoles were randomly distributed in the cytoplasm of the cells from the granulocytic series and some of them contained giant granules which thus appeared particularly resistant to the autophagic process. The vital cellular damage through endophagocytosis suggests the possibility of intramedullary destruction as an explanation for neutropenia. 相似文献
104.
abstract – A material of 30 patients with metallic implants was used in a longitudinal study, based on lateral headplates, of the mandibular rotational pattern. The complexity of the rotational phenomenon in the mandibular rotation has necessitated the proposal of some operational definitions: morphogenetic and positional rotations of the mandible. A relation-ship has been established between the variation of the gonial angle and the variation of both morphogenetic and positional rotations of the mandible. A relationship has been established between the variation of the condylar growth direction and the variation of both morphogenetic and positional rotations of the mandible: this relationship is the strongest when the morphogenetic rotation of the corpus is considered. 相似文献
105.
PSORIASIS AND HIV INFECTION 总被引:3,自引:0,他引:3
ASHRAF MONTAZERI M.D. JEAN KANITAKIS M.D. JACQUES BAZEX M.D. 《International journal of dermatology》1996,35(7):475-479
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PARENTAL HISTORY OF ALCOHOLISM: A RISK FACTOR FOR ALCOHOL-RELATED PERIPHERAL NEUROPATHIES 总被引:6,自引:4,他引:2
PESSIONE FABIENNE; GERCHSTEIN JEAN LOUIS; RUEFF BERNARD 《Alcohol and alcoholism (Oxford, Oxfordshire)》1995,30(6):749-754
The aim of this study was to identify the risk factors of alcohol-relatedperipheral neuropathies. A case-control study was performedto compare two groups of alcoholic patients, one with peripheralneuropathy and the other without, but with alcohol-related cirrhosis,pancreatitis or cardiomyopathy. Ninety patients were recruitedin four in-patient units of a French hospital: 32 patients hada peripheral neuropathy and 58 patients did not. Univariateanalysis showed no differences between the two groups for sex,age, body mass index and duration of the alcoholic disease.Peripheral neuropathies were associated with a higher frequencyof parental history of alcoholism, severity of alcohol dependence,heavier alcohol consumption and more alcohol-related somaticdiseases. Multivariate analysis showed a strong relationshipbetween a parental history of alcoholism and the presence ofa neuropathy, when the severity of the alcoholic disease wastaken into account (adjusted OR = 6.8, IC95% [2.2-21.6], P <0.001). The hypothesis that neuropathy may be a marker of aninherited susceptibility to alcoholism is discussed. 相似文献
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Measurements of physiological deadspace/tidal volume ratio (VD/VTper cent) and alveolar-arterial oxygen tension difference (A-aPo2)were made during mechanical ventilation in patients undergoingopen-heart surgery. The inspiratory: expiratory time ratiosstudied were 0.5:2.5 and 1.5:1.5 before perfusion and 0.5:2.5,1:2 and 1.5:1.5 after perfusion. Pre-perfusion there were nosignificant differences in VD/VT or A-aPo2 between the two patternsof ventilation. Post-perfusion the A-aPo2 increased. Althoughthere were no significant differences in A-aPo2 between anyof the three ratios examined after perfusion, the VD/VT percent was significantly less when the inspiratory time was 1.5seconds than when it was 0.5 or 1.0 second. 相似文献
110.
The incidence of cystic fibrosis among the Australian born children of parents born in Italy was 1: 3 625 live births and among Australian born children of parents born in Greece 1: 3726. These incidences were significantly lower than the incidence for cystic fibrosis of 1: 2021 in children born to Australian born parents, about 90% of whom have ancestors born in the British Isles. The figures suggest there are significant variations in the incidence of cystic fibrosis in different European populations. 相似文献