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[Radiological signs of type I primary hyperoxaluria].

The authors report the radiological signs observed in 14 patients between the ages of 3 months and 16 years, suffering from type I primary hyperoxaluria. Four of these patients were being treated conservatively for renal failure and ten had reached end-stage renal failure. Stones were demonstrated in 9 patients. Nephrocalcinosis was present on the plain abdominal x-ray in 6 patients before dialysis, and in all patients dialysed for more than 3 months. Bone signs characteristic of type I primary hyperoxaluria were only observed in children with end-stage renal failure. Metaphyseal dense bands were revealed in 5 patients. Metaphyseal clear bands, observed in 4 patients, occurred later and appeared to replace dense bands which moved towards the diaphysis. The other signs detected were osteosclerosis of the vertebrae and ilium (4 patients) and epiphyseal subchondral clear bands (one patient).

Adolescent↗

[Value of intramedullary locked nailing in distal fractures of the tibia].

PURPOSE OF THE STUDY: This study is a retrospective analysis of 38 extra-articular distal tibial fractures treated by intramedullary locked nailing. PATIENTS AND METHODS: 38 patients with a distal metaphyseal extra-articular fracture (43 A AO type) or with minimal ankle joint extension were managed. There was 26 men and 12 women with a mean age of 32.3 years, 10 fractures were open. The fractures were transverse or oblique in 13 cases, with torsional or flexion wedge in 12 cases and spiroïd in 13 cases. In only 2 cases was the fibula intact. AO classification was not useful because many fractures began more proximally than the limit described by Müller. All the fractures were fixed by closed locked intramedullary nailing : the nail was cut just after the distal hole and impacted close to the subchondral plate. In 7 cases the fibula was fixed too. RESULTS: There was no postoperative complication in 27 cases. Three patients had a transient nerve palsy (one tibial nerve and two common fibular nerve). In ten cases the nail was dynamized. One patient had a non union but healed with a new dynamic nail. Two patients had a delayed union and healed after dynamization and osteotomy of the fibula. The mean time to union was 5 months (2 to 8). 8 patients had a varus or a valgus deformity of 3 to 6 degrees. 11 patients suffered from anterior knee pain and in 5 patients the fracture site was painful. In 18 patients a CT scan was performed : 6 had a rotational deformity from 4 to 26 degrees, and 2 a tibial lengthening (discrepancy of 7 and 9 mm). CONCLUSION: Closed intramedullary nailing is a safe and effective method for the treatment of distal metaphyseal tibial fractures. The authors propose a new classification.

Adolescent↗

Failure mechanisms of anatomic porous replacement I cementless total hip replacement.

The APR-I Hip System was designed for metaphyseal bone attachment by proximal patch porous coating and the acetabular component was a hemisphere fixed with screws. The results of 100 consecutive primary total hip arthroplasties performed with the APR-I were studied in patients still alive after an average of 6.7 years. The selection of hips for implantation of this hip system was limited to those patients in whom a satisfactory intraoperative fit could be obtained. This resulted in a distribution of patients such that 75% were younger than age 65 years and only 15% had osteoporotic bone. The revision rate was 16% with a mechanical failure rate of 11%. Seventy percent of hips had progressive loss of fixation. loss of femoral component fixation was correlated with younger patient age, higher patient activity level, metaphyseal fill of less than 90%, and increased polyethylene wear and osteolysis. Eighty-nine percent of hips maintained femoral neck contact with the porous coated collar. The acetabular component was well fixed in 97% of hips. The failure rate of the APR-I stem is unacceptably high and this stem is no longer used by the authors.

Adult↗

Brace treatment of early infantile tibia vara.

We reviewed 24 children (42 extremities) who had a diagnosis of early infantile tibia vara treated by using a brace. The indication for bracing was either a varus deformity that was not improving by age 18-24 months, or a persistent varus deformity seen in a patient older than 24 months. We prescribed an above-the-knee brace with a free ankle, single medial upright with valgus-producing straps, and either no hinged joint or a locked hinge joint at the knee. The braces were worn during the day and were removed for bedtime. The patients were followed up for an average of 27.2 months (range, 12-72) from the initiation of brace treatment, and the outcome at latest follow-up was determined by using radiographic criteria. Before treatment, 29 extremities were Langenskiöld stage I, II were stage II, and two were stage III. Before treatment, the metaphyseal-diaphyseal angle averaged 16.4 degrees. Forty of the 42 extremities had metaphyseal-diaphyseal angles of > 11 degrees, and 20 were > 16 degrees. Based on our criteria, we rated 29 extremities good, nine fair, and four poor. We conclude that daytime, ambulatory brace treatment may favorably alter the natural history of tibia vara in patients who are younger than 3 years and who have Langenskiöld stage I or II deformity.

Bone Diseases, Developmental↗

[Medical and surgical therapy of familial hypophosphatemic vitamin D resistant rickets with ambulation disorders].

Three children with familial hypophosphatemic rickets showed, in the untreated state, active rickets of the metaphyseal areas of long bones. All were treated with combined phosphate /1-2 g daily by mouth in 4-5 divided doses) and vitamin D2 therapy for 2-5 years. The radiological appearance of the metaphyses became practically normal in two, whereas little change took place in the endosteal bone surface. Bone biopsies demonstrated osteomalacia. Therefore, two subjects were given phospate (1-2 g daily) and 1-alpha-hydroxyvitamin D (0.25-0.50 microgram daily) for 2 years; then phosphate (1 g daily) and 1.25 dihydroxyvitamin D (0.25-0.50 microgram daily) combined therapy for 5 years; the third patient had phosphate (1 g daily) and 1.25 dihydroxyvitamin D (0.25 microgram daily) combined therapy for 2 years. After therapy with low doses of 1.25 dihydroxyvitamin D, X-rays showed a nearly normal bone aspect, without nephrocalcinosis in all patients. All subjects were submitted to osteothomies with positive results.

Adolescent↗

Pacman dysplasia: report of two affected sibs.

We report on two sib fetuses with radiological and morphological findings similar to those of the recently described lethal skeletal dysplasia termed Pacman dysplasia (McKusick, 167220, Am J Med Genet 1993, 45:558-561). The first fetus, a male, was electively terminated after a routine ultrasound study at 20 weeks showed short-limb dwarfism. The second fetus, a female sib, was also electively terminated after similar, abnormal ultrasound findings were noted at 16 weeks of gestation. Similar to Pacman dysplasia, the radiographic appearance was characterized by under-mineralized bone, stippling, rhizomelic and mesomelic shortness, platyspondyly, and a short, broad pelvis. The metaphyses were dense, but the diaphyseal cortices were thin with undermodeled long bones, and there was a deficient trabecular pattern suggesting marrow replacement. Chondro-osseous structure was characterized by deficient trabecular bone formation, a fibrous marrow, and numerous, large, multinucleated osteoclasts lining the endosteal surfaces of the metaphyseal bone. The occurrence of this dysplasia in sibs of differing sex suggests autosomal recessive inheritance.

Female↗

[Treatment of complex bicondylar fractures of the tibial plateau by semi-circular anterior diaphyseal-epiphyseal plate].

PURPOSE: One third of all tibial plateau fractures are bicondylar, and some of them have posterior separation and metaphyseal comminution. In these cases, it may be difficult to reconstruct at the same time tibial axis, bone continuity and articular surface. The authors propose a new method for the treatment of these fractures. METHOD: Reduction is facilitated by permanent extension using a transcalcaneal pin. Knee approach involves elevation of the anterior tibial tuberosity (ATT). The new plate used is available for each side. It presents an epiphyseal surrounding anterior part applied on the epiphysis above the ATT and a vertical part applied medially. All the separated fragments are temporarily stabilized by pins which are used to guide the definitive plate. For this an aiming plate duplicating the epiphyseal part of the definitive plate is used to insert the pins. Then, the definitive plate is slided without removing the pins. The pins also guide cannulated screws. MATERIAL AND RESULTS: Out of 32 tibial fractures treated from January 1994 to June 1996, 3 cases were operated on using this method. All 3 cases have a frontal separation fracture, cartilage depression and metaphyseal complex fracture. Perfect articular reconstitution, fusion and axis were obtained. A fourth case was a proximal tibial fracture, very close to a total knee prosthesis. DISCUSSION: Complex bicondylar fractures are one of the most difficult fractures to treat. Identification of the lesions should be envisioned before operation and especially the separated posterior fragments. The peroperative extension combined with the anterior approach with ATT elevation provided a complete view of intra articular fracture and correction of the axis. The semi-circular anterior plate allowed the fixation of all the fragments, and furthermore, the original procedure using temporary pins to guide the definitive plate has avoided to loose the reduction.

Bone Plates↗

[Effect of ephedrine and support loads on development of osteopenia and growth of shin bones in suspended rats].

Effects of graded support loads and stimulators of the central nervous system (ephedrine and strychnine) on growth of bones and the volume of spongy bone tissue in the proximal metaphyses of the shin bones were studied in the 14-day experiment with rats suspended by tail in the orthostatic position. It is indicated that the support loads in combination with ephedrine injection prevent the suspension-induced inhibition of bone growth and development of osteopenia of spongiosa of metaphyses. The preventive effect of support loads and ephedrine relates with their stimulating influence on the functional activity of the STH-cells of adenohypophysis producing the growth hormone.

Animals↗

A heterodox model of healing.

A heterodox model of healing incorporating the basic dualism of Western philosophy--the world of concrete material substance versus that of thought, ideals, and spirit--is proposed. Such dualism is reflected in Western constructions of healing. An explanatory paradigm model with a horizontal axis representing paradigms of healing from positivism to metaphysics and a vertical axis representing activities of healing from the material to the spiritual was derived from ethnographic work with healers. A health practice model following the same philosophical patterns illustrates reflected American cultural values in the relative positioning of healing modalities from the orthodox biomedical to the most metaphysical or alternative. Examples of specific modalities of healing, many of which are resurfacing with increasing public interest in alternative therapies, are illustrated. This model places biomedicine in the same context as other philosophical approaches to healing as a standard for comparison. Implications for practice and research are discussed.

Culture↗

Space flight effects on skeletal bones of rats (light and electron microscopic examination).

In the light and electron microscopes, long tubular bones of Wistar rats that were flown for 22 d onboard the Cosmos-605 biosatellite and were exposed to a ground-based simulation experiment were examined. About half of the flight rats showed osteoporosis of metaphyses which was usually combined with a decrease of the mass of the primary spongiosa in the vicinity of the epiphyseal cartilaginous plate. This gives evidence that the growth of the bones could have been inhibited in flight. The light and electron microscopy of bones of flight rats revealed wide osteocyte lacunae which could have been produced by perilacunar osteolysis. In the simulation experiment, reduction in the metaphyseal spongiosa occurred only in one-third of the rats and was less pronounced than in flight rats; no decrease of the mass of the primary spongiosa near the cartilaginous plate was noted. Histological investigation of bones 27 d postflight demonstrated that that time period was not enough to eliminate all the changes in the bones tested.

Animals↗

Distinct, autosomal recessive form of spondyloepimetaphyseal dysplasia segregating in an inbred Pakistani kindred.

We describe a large inbred kindred from a remote area of Pakistan, comprising eight generations, with a distinct form of spondyloepimetaphyseal dysplasia (SEMD). We evaluated 16 affected individuals: 11 males and 5 females. Analysis of the pedigree strongly suggests autosomal recessive inheritance, and consanguineous loops could account for all the affected individuals being homozygous for the abnormal allele. The clinical findings included short stature evident at birth, short bowed lower limbs, mild brachydactyly, kyphoscoliosis, an abnormal gait, enlarged knee joints, precocious osteoarthropathy, and normal intelligence. Radiographs demonstrated delayed epiphyseal ossification at the hips and knees, platyspondyly with irregular end plates and narrowed joint spaces, diffuse, early osteoarthritic changes, primarily in the spine and hands, and mild brachydactyly. Mild metaphyseal abnormalities were seen predominantly at hips and knees. This distinctive phenotype is distinct from other autosomal recessive forms of SEMD because of the mild degree of metaphyseal involvement, the type of brachydactyly, and the absence of loose joints or other clinical findings.

Abnormalities, Multiple↗

Aristotle and headless clones.

Cloned organisms can be genetically altered so that they do not exhibit higher brain functioning. This form of therapeutic cloning allows for genetically identical organs and tissues to be harvested from the clone for the use of the organism that is cloned. "Spare parts" cloning promises many opportunities for future medical advances. What is the ontological and ethical status of spare parts, headless clones? This paper attempts to answer this question from the perspective of Aristotle's view of the soul. Aristotle's metaphysics as applied to his view of biological essences generates an ethic that can contribute to moral reasoning regarding the use of headless spare parts clones. The task of this paper is to show the implications that Aristotle's view of the soul, if it is true, would have on the ethics of headless, spare parts cloning.

Cloning, Organism↗

The ethics of choice in the struggle against industrial disease.

This article argues that broad recognition of the ethical basis for society's decisions about the reduction of the risks of workers, including notification, has been, is, and will be a more persuasive method of establishing programs implementing the rights to know and act in controlling the industrial vector in human disease than sheer power politics. If the argument is correct, then attention needs to be focused on the nature of the ethics of choice. The moral dialectic in the history of science and medicine is traced to the metaphysically assumed values of open and closed models of knowledge and action that determine how we weigh genetic and environmental factors in the process of choosing who shall live and who shall die of disease, with strong industrially generated vectors. The author suggests that we escape from the concepts of the closed systems of the past, whose grammar reflects values repugnant to the empowerment of those notified of risk, to open systems that enhance community-ecologic values of life and freedom.

Biomedical Research↗

Unifying psychology: epistemological act or disciplinary maneuver?

Two arguments with attempts to unify psychology are adumbrated in this commentary. First, the unification of psychology is largely a disciplinary maneuver and not primarily an epistemological act. Second, the discipline of psychology has been unified for some time around a series of methodological and functional categories that have served to support its institutional projects but hide metaphysical problems.

Animals↗

From ether theory to ether theology: Oliver Lodge and the physics of immortality.

This article follows the development of physicist Oliver Lodge's religio-scientific worldview, beginning with his reticent attraction to metaphysics in the early 1880s to the full formulation of his "ether theology" in the late 1890s. Lodge undertook the study of psychical phenomena such as telepathy, telekinesis, and "ectoplasm" to further his scientific investigations of the ether, speculating that electrical and psychical manifestations were linked phenomena that described the deeper underlying structures of the universe, beneath and beyond matter. For Lodge, to fully understand the ether was to force from the universe an ultimate Revelation, and psychical research, as the most modern and probatory science, was poised to replace religion as the means of that disclosure.

England↗

Catastrophe modelling in the biological sciences.

Catastrophe Theory was developed in an attempt to provide a form of Mathematics particularly apt for applications in the biological sciences. It was claimed that while it could be applied in the more conventional "physical" way, it could also be applied in a new "metaphysical" way, derived from the Structuralism of Saussure in Linguistics and Lévi-Strauss in Anthropology. Since those early beginnings there have been many attempts to apply Catastrophe Theory to Biology, but these hopes cannot be said to have been fully realised. This paper will document and classify the work that has been done. It will be argued that, like other applied Mathematics, applied Catastrophe Theory works best where the underlying laws are securely known and precisely quantified, requiring those same guarantees as does any other branch of Mathematics when it confronts a real-life situation.

Animals↗

Darwin's evolutionary philosophy: the laws of change.

The philosophical or metaphysical architecture of Darwin's theory of evolution by natural selection is analyzed and discussed. It is argued that natural selection was for Darwin a paradigmatic case of a natural law of change -- an exemplar of what Ghiselin (1969) has called selective retention laws. These selective retention laws lie at the basis of Darwin's revolutionary world view. In this essay special attention is paid to the consequences for Darwin's concept of species of his selective retention laws. Although Darwin himself explicity supported a variety of nominalism, implicit in the theory of natural selection is a solution to the dispute between nominalism and realism. It is argued that, although implicit, this view plays a very important role in Darwin's theory of natural selection as the means for the origin of species. It is in the context of these selective retention laws and their philosophical implications that Darwin's method is appraised in the light of recent criticisms, and the conclusion drawn that he successfully treated some philosophical problems by approaching them through natural history. Following this an outline of natural selection theory is presented in which all these philosophical issues are highlighted.

Biological Evolution↗

["We must relate to ourselves". Substance, causality, and narrative text].

Since Aristotle the concept of "substance" has described those features of an object which remain constant over time despite varying additional stochastic factors (coincidences). "Causality" places several or more chronological events into a direct relationship. The change of stochastic factors on a substance always has a causal justification. Based on these metaphysical considerations the present contemplations will apply the concept of substance to people. The causal explanation for changes during the lifetime is achieved by narrative sentences. These are, therefore, decisive for the maintenance of the identity of individuals and collectives despite all time-linked changes. The conclusion is reached that speech and thought processes based on speech patterns are essential for humans.

Communication↗