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Crazy children, fantastical theories, and the many uses of metaphysics.

Woolley rightly challenges the incredible idea, held by some adults, that it is children who are peculiarly "fantastical" in their thinking. However, Woolley expresses little appreciation for "fantastical thinking" as it underlies the capacity for both grand delusions and amazing insights. In reducing "fantastical thinking" to conceptual error, she overlooks the mythical underpinnings of her own theorizing and neglects the many constructive roles of "fantastical thinking" in development.

Adult↗

Cartilage hair hypoplasia, metaphyseal chondrodysplasia type McKusick: description of seven patients and review of the literature.

We describe 7 cases of cartilage hair hypoplasia (CHH) with emphasis on the clinical and immunological aspects. The literature on CHH is reviewed and symptoms in 63 non-Amish cases are summarized. In this autosomal recessive disorder the immunodeficiency, hair abnormalities, and severity of skeletal involvement show extremely variable expressivity, between and within families. Two of the 3 sib-pairs among our cases demonstrate the great difference in expression within one family. At adult age roentgenological abnormalities can be very mild, or even absent. An impairment in cell-mediated immunity is present in all of our cases and seems a consistent manifestation in CHH; however, sometimes it is very subtle and without clinical symptoms.

Abnormalities, Multiple↗

Newly recognized syndrome of metaphyseal undermodeling, spondylar dysplasia, and overgrowth: report of two adolescents and a child.

We report on a previously undescribed syndrome characterized by generalized skeletal alterations and overgrowth in three unrelated individuals: a boy who died at age 16 years, a 16-year-old girl, and a 15-month-old boy. The skeletal changes included bony overgrowth of the skull base, spondylar dysplasia, and undermodeling of the tubular bones. Bone age was accelerated in early childhood. Overgrowth, which was independent of GH-IGF axis, was of prenatal onset in the two boys, but postnatal in the girl. In the two adolescents, growth rate did not decline with age, and high-dose estrogen therapy failed to induce physeal fusion. Their adolescent height reached +4 approximately +7 SD of the mean. Delayed puberty in the girl and cryptorchidism and hypospadias in the younger boy raised the possibility that hypogonadism is a syndromic constituent. Molecular analysis of IGF2, GPC3, and FGFR3 in the older boy yielded no abnormalities.

Adolescent↗

Detection of canine distemper virus in bone cells in the metaphyses of distemper-infected dogs.

In the light of recent evidence implicating canine distemper virus (CDV) as a possible etiologic agent in Paget's disease of bone, we thought that it would be of interest to examine distemper-infected bone in the natural host. Samples from the long bones, spleen, and bladder of four distemper-infected and three uninfected dogs were examined for the presence of CDV nucleocapsid and phosphoprotein genes and the measles virus (MV) nucleocapsid gene using the technique of in situ hybridization with radioactively labeled riboprobes. Two of the four distemper-infected dogs showed strongly positive hybridization with both of the CDV probes. The signal was present in marrow cells, in osteoblasts, in osteocytes, and particularly in osteoclasts. No hybridization was seen over the cartilage cells of the growth plate, and there was a clear line of demarcation at the point of invasion of osteoclasts and vascularization. The spleen and bladder samples from infected dogs also showed positive hybridization. There was no hybridization with the MV probe in any of the distemper-infected tissue. Samples from the uninfected dogs showed no evidence of hybridization with either the CDV or MV probes. These results show that CDV can infect bone cells of the natural host and provide further support for the theory that CDV may play a role in human Paget's disease of bone.

Animals↗

Infants' metaphysics: the case of numerical identity.

Adults conceptualize the world in terms of enduring physical objects. Sortal concepts provide conditions of individuation (establishing the boundaries of objects) and numerical identity (establishing whether an object is the same one as one encountered at some other time). In the adult conceptual system, there are two roughly hierarchical levels of object sortals. Most general is the sortal bounded physical object itself, for which spatiotemporal properties provide the criteria for individuation and identity. More specific sortals, such as dog or car, rely on additional types of properties to provide criteria for individuation and identity. We conjecture that young infants might represent only the general sortal, object, and construct more specific sortals later (the Object-first Hypothesis). This is closely related to Bower's (1974) conjecture that infants use spatiotemporal information to trace identity before they use property information. Five studies using the visual habituation paradigm were conducted to address the Object-first Hypothesis. In these studies, 10-month-old infants were able to use spatiotemporal information but failed to use property/kind information to set up representations of numerically distinct individuals, thus providing empirical evidence for the Object-first Hypothesis. Finally, infants succeed at object individuation in terms of more specific sortals by 12 months. The relation between success at our task and early noun comprehension is discussed.

Concept Formation↗