Major central nervous system malformation in "micromelic dwarfism with cone epiphyses, metaphyseal dysplasia and vertebral segmentation defects".
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Abnormalities, Multiple↗
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[Shwachman's syndrome: pancreatic exocrine insufficiency retarded growth, metaphyseal dysostosis and neutropenia (author's transl)].
A boy of 5 8/12 years of age affected with Shwachman's Syndrome is presented. He showed frequent respiratory infections that can be in order with a low account in T-lymphocytes found on immunological study. A cytoplasmic degenerative process was found after microscopical and ultrastructural vision of osteoid tissue, which is compatible with biochemical alterations.
Biopsy↗
[Experimental research on DNA content of the cell nuclei in the lengthening side physis after near metaphyseal osteotomy].
In order to study the influence of the diaphyseal distraction upon further growth of the lengthening side physis. We applied the modified Ilizarov-type external fixator to the left tibiae of thirty-five goats. Diaphyseal corticotomies were performed, and distraction was carried out by using the Ilizarov's method. A group of 5 goats each was sacrificed 7, 14, 21, 28, 35, 49 and 63 days postoperatively. Specimens of the lengthening side epiphysis for DNA quantitation VIDAS were decalcified with 5% EDTA and Feulgen stained. There was little influence subject to the Tension-Stress in lengthening side epiphysis as the mean DNA content corroborated its cell nuclei in 3C-4C region. The hypertrophic layer of physis on the lengthened side was thicker than that on the control side while the lines of epiphysis in roentgenography was still observable after removal of external fixator at 35 days post lengthening. Although lengthening by metaphyeal osteotomy was safe, the damage to the physis by Tension-Stress should be minimized.
Animals↗
[What is your diagnosis? Diaphyseal transverse fracture of the radius and ulna (distal third of the metaphyseal transition). Osteopetrosis].
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Animals↗
[Lytic epiphyseal-metaphyseal lesion].
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Biopsy↗
Role of endogenous regucalcin in bone metabolism: bone loss is induced in regucalcin transgenic rats.
The role of endogenous regucalcin in the regulation of bone metabolism was investigated by using regucalcin transgenic (TG) rats. The expression of regucalcin mRNA in the femoral-diaphyseal and -metaphyseal tissues of normal (wild-type) rats was shown by using reverse transcription-polymerase chain reaction (RT-PCR) with a specific primer of regucalcin cDNA. Regucalcin protein was detected in the femoral-diaphyseal and -metaphyseal tissues of normal (wild-type) rats using Western analysis. Regucalcin levels were significantly increased in the femoral-metaphyseal tissues of regucalcin TG male rats and in the diaphyseal and metaphyseal tissues of the TG female rats. The morphologic change in the femoral-diaphyseal and -metaphyseal tissues of regucalcin TG rats was demonstrated by using a peripheral quantitative computed tomography (pQCT); morphologic change was great in the femoral tissues of female rats as compared with that of male rats. Mineral content, mineral density and polar strength strain index in the femoral-diaphyseal and -metaphyseal tissues were markedly reduced in regucalcin TG female rats. A significant decrease in cortical thickness was seen in the femoral diaphysis of regucalcin TG female rats. Calcium content in the femoral-diaphyseal and -metaphyseal tissues was significantly decreased in regucalcin TG male and female rats; a remarkable decrease was seen in female rats. Femoral-metaphyseal alkaline phosphatase activity was significantly lowered in regucalcin TG female rats. The enzyme activity was not significantly changed in the femoral-diaphyseal tissues of the TG female rats. In the diaphyseal tissue of male rats, the enzyme activity was significantly decreased in the TG rats. A significant decrease in deoxyribonucleic acid (DNA) content was seen in the metaphyseal tissue of regucalcin TG male rats and in the diaphyseal and metaphyseal tissues of the TG female rats. This study demonstrates that bone loss is induced in the femoral tissue of regucalcin transgenic rats, and that a remarkable decrease in bone morphologic index and biochemical component was seen in the female rats. Regucalcin may be involved in the regulation of bone metabolism.
Animals↗
Pronation injuries of the ankle in children. Retrospective study of radiographical classification and treatment.
In a retrospective study in children aged 0-18 years, 457 ankle fractures in children were classified traumatologically according to Gerner-Smidt or Lauge-Hansen. Anatomically, ankle fractures with open growth plates were classified according to the Salter-Harris classification. Pronation injuries constituted 18% of the ankle injuries and showed different fracture patterns. In total 83 pronation injuries were found. Of these, 52 showed open growth plates: 25 pronation-abduction, 23 pronation-eversion, and 4 pronation-dorsal flexion injuries. The pronation-abduction injuries were classified into two groups. In 15, a detachment of the deltoid ligament at the medial malleolus, visible on radiographs as a minimal fragment or transverse fracture of the medial malleolus, was found; seven showed in addition a fracture through the growth plate (Salter-Harris type I or II) or a metaphyseal fracture of the distal fibula. In 10, a physeal fracture through the distal tibia (Salter-Harris type I) was found. Of these, seven had in addition a metaphyseal fibular fracture. Pronation-eversion injuries showed in 21 cases a physeal-metaphyseal fracture (Salter-Harris type II) with an antero-lateral metaphyseal fragment (Stage I-II); 17 had in addition a metaphyseal fibular fracture (Stage III). A minimal posterolateral metaphyseal fragment of the distal tibia represents the fourth stage but could not adequately be separated from the third, so Stages III and IV were combined. Pronation-dorsal flexion showed a physeal-metaphyseal fracture in four cases with an anteriorly situated metaphyseal fragment (Stages I-II); one case also had a metaphyseal fracture of the distal fibula (Stage III). Pronation-eversion injuries showed frequently displacement and were more commonly treated by reduction than pronation-abduction and supination injuries including supination-eversion injuries of intra-articular type. However, complete reduction of pronation-eversion injuries with closed methods often proved difficult.
Adolescent↗
[Roentgenographical evaluation of physiological bow-leg and the infantile type of Blount's disease in children].
Roentgenographical examinations were carried out in 41 joints of 22 cases with physiological bowleg and 7 joints of 5 cases with the infantile type of Blount's disease. The observation periods were from 1 year and 4 months to 8 years and 1 month with an average of 4 years and 2 months. The femorotibial angle, the proximal tibial metaphyseal diaphyseal angle, the distal tibial metaphyseal diaphyseal angle and the tibial metaphyseal metaphyseal angle were measured, and evaluated statistically. The measurement of the proximal tibial metaphyseal diaphyseal angle and the tibial metaphyseal metaphyseal angle were more significant than that of the femorotibial angle for early differentiation of the infantile type of Blount's disease from physiological bowleg. The degree and it's change of the distal tibial metaphyseal diaphyseal angle show no difference between physiological bowleg and the infantile type of Blount's disease.
Age Factors↗
What to say to a skeptical metaphysician: a defense manual for cognitive and behavioral scientists.
A wave of recent work in metaphysics seeks to undermine the anti-reductionist, functionalist consensus of the past few decades in cognitive science and philosophy of mind. That consensus apparently legitimated a focus on what systems do, without necessarily and always requiring attention to the details of how systems are constituted. The new metaphysical challenge contends that many states and processes referred to by functionalist cognitive scientists are epiphenomenal. It further contends that the problem lies in functionalism itself, and that, to save the causal significance of mind, it is necessary to re-embrace reductionism. We argue that the prescribed return to reductionism would be disastrous for the cognitive and behavioral sciences, requiring the dismantling of most existing achievements and placing intolerable restrictions on further work. However, this argument fails to answer the metaphysical challenge on its own terms. We meet that challenge by going on to argue that the new metaphysical skepticism about functionalist cognitive science depends on reifying two distinct notions of causality (one primarily scientific, the other metaphysical), then equivocating between them. When the different notions of causality are properly distinguished, it is clear that functionalism is in no serious philosophical trouble, and that we need not choose between reducing minds or finding them causally impotent. The metaphysical challenge to functionalism relies, in particular, on a naïve and inaccurate conception of the practice of physics, and the relationship between physics and metaphysics.
Animals↗
Effect of thyroid hormone and growth hormone on recovery from hypothyroidism of epiphyseal growth plate cartilage and its adjacent bone.
Hypothyroidism was induced in young female Sprague-Dawley rats by the addition of methimazole (0.67 mg/ml) to drinking water for a period of 7 weeks (7-14 weeks of age). The responses of the articular cartilage, epiphyseal growth plate cartilage, epiphyseal trabecular bone, and metaphyseal trabecular bone in the proximal tibia were assessed by structural parameters. In addition, replacement therapies were introduced for the last 2 weeks of the experimental period. These included 0.7 U/kg BW human GH (hGH), 15 micrograms/kg BW L-T4 (T4), and a combination of hGH and T4 at the same doses. In the hypothyroid rats, the width of epiphyseal growth plate cartilage decreased by 27%, that of articular cartilage by 35%, epiphyseal trabecular bone volume by 30%, and metaphyseal trabecular bone volume by 66% relative to those in age-matched control tissues. T4 treatment led to a full restoration of the epiphyseal trabecular bone and surpassed by 40% the control value. The magnitude of the articular cartilage and the epiphyseal trabecular bone volume returned to control values, while that of metaphyseal trabecular bone was 68% of control values. Treatment with hGH did not improve the epiphyseal growth plate cartilage or articular cartilage. It did restore epiphyseal trabecular bone to almost normal values, but metaphyseal trabecular bone improved to only a small though significant level (45% of control value). The combination of T4 and hGH resulted in an additional enlargement in the width of the epiphyseal growth plate cartilage and an increase in metaphyseal trabecular bone volume compared to those in the T4 group. Qualitative examinations indicated that it was only in the T4 and T4 plus hGH groups that the lowest chondrocytes in the epiphyseal growth plate cartilage resumed their normal hypertrophied size. These results suggest that the change in the hypothyroid state do not rely solely on the lack of pituitary GH synthesis and secretion, as replacement by exogenous GH did not restore normal epiphyseal growth plate cartilage morphology or its remodeling into metaphyseal trabecular bone. Treatment with T4 (which restored endogenous pituitary GH to 30% of control levels) results in full recovery of the epiphyseal growth plate cartilage morphology along with its associated metaphyseal trabecular bone. In addition, it can also be concluded that the decrease in epiphyseal trabecular bone volume observed in the hypothyroid animals was due solely to the GH-deficient state that accompanied hypothyroidism.
Animals↗
Effects of copper on bone component in the femoral tissues of rats: anabolic effect of zinc is weakened by copper.
The effects of copper on biochemical components in the femoral-diaphyseal (cortical bone) and -metaphyseal (trabecullar bone) tissues of rats in vivo and in vitro were investigated. Rats were orally administered copper sulfate (50, 100, or 200 microg Cu/100 g body weight) once daily for 7 d. Calcium content in the diaphyseal and metaphyseal tissues was significantly decreased with the administration of copper (200 microg/100 g), while alkaline phosphatase activity in these tissues was not significantly changed by copper administration. The diaphyseal DNA content was significantly decreased with the administration of copper (50, 100, or 200 microg/100 g). Moreover, the femoral-diaphyseal and -metaphyseal tissues were cultured for 48 h in serum-free medium containing either vehicle or copper (10(-7) - 10(-4) M). Culture with copper (10(-7) - 10(-4) M) caused a significant decrease in alkaline phosphatase activity in the diaphyseal and metaphyseal tissues, while calcium and DNA contents in these tissues were not significantly changed. Culture with parathyroid hormone [PTH (1-34); 10(-7) M], a bone-resorbing factor, caused a significant decrease in calcium content in the diaphyseal and metaphyseal tissues. This decrease was completely inhibited in the presence of copper (10(-6) or 10(-5) M). Culture with zinc sulfate (10(-4) M) caused a significant increase in calcium content and alkaline phosphatase activity in the diaphyseal and metaphyseal tissues. The effects of zinc (10(-4) M) in increasing femoral calcium content and alkaline phosphatase activity were not seen in the presence of cycloheximide (10(-6) M), an inhibitor of protein synthesis, suggesting that the effects of zinc are involved in newly synthesized protein components. The effects of zinc in increasing calcium content and alkaline phosphatase activity in the diaphyseal and metaphyseal tissues were significantly weakened in the presence of copper (10(-4) M). The inhibitory effects of copper were further enhanced in the presence of cycloheximide. This study demonstrates that supplementation with copper in adequate copper nutrition does not have anabolic effects on bone components in vivo and in vitro and that copper weakens the anabolic effects of zinc in vitro.
Administration, Oral↗
Osteoprogenitor cells as targets for ex vivo gene transfer.
We transduced osteoprogenitor cells with recombinant retrovirus and analyzed proviral integration patterns into chromosomal DNA to detect for the first time the clonal and cellular fate of osteoprogenitor-derived progeny cells. Metaphyseal bone cells and diaphyseal stromal cells were isolated from the distal femurs of young rats, transduced with the vM5neolacZ recombinant retrovirus, and selected in the neomycin analog, G418. Following surgical marrow ablation of a femur in one leg of mature rats, retroviral-transduced metaphyseal or diaphyseal cells were injected into the ablated site. These rats were killed 5-6 days later. Metaphyseal and diaphyseal cells were isolated from distal femurs, selected in G418, and stained for beta-galactosidase (beta-gal+). The number and clonal origin of transduced progenitor cells were determined. High numbers of beta-galactosidase colonies with an osteoblast phenotype were obtained following metaphyseal transplants and detected in 100% of metaphyseal and none of diaphyseal specimens. In contrast, beta-galactosidase colonies derived from diaphyseal transplants were detected in 50% of specimens in both the metaphysis and diaphysis, and the absolute number of progenitor cell colonies was 60-fold less than metaphyseal transplants. Provirus was only detected in the ablated bones and not in the contralateral bone or other tissues. Proviral integration fragment analysis showed a single integration site for recovered metaphyseal cell clones, consistent with their origination from a common single progenitor. This is one of the first demonstrations of successful transplantation of clonal osteoprogenitors to their site of origin in bone. It may be possible to use these cells to target genes to bone for therapeutic use in skeletal and hematopoietic diseases.
Animals↗
Stimulatory effect of zinc and growth factor on bone protein component in newborn rats: enhancement with zinc and insulin-like growth factor-I.
The effect of zinc and growth factor on bone protein component in newborn rats was investigated. The characterization of protein component in the femoral-diaphyseal and metaphyseal tissue of newborn rats (3-35 days old) was examined using sodium dodecyl sulfate-polyacrylamide gel electrophoresis analysis. The diaphyseal and metaphyseal tissues of 7 days-old rats were cultured for 24 or 48 h in a medium containing either vehicle, zinc sulfate (10-4 M) or dipicolinate (10-3 M), a chelator of zinc ion, in the presence or absence of insulin-like growth factor-I (IGF-I; 10-8 M) or transforming growth factor-beta (TFG-beta; 10-10 M) with an effective concentration. Many cellular protein molecules were present in the diaphyseal and metaphyseal tissues; potent bands were seen in protein molecules with about 66 and 46 kDa. Protein molecule of about 66 kDa was greatly released in the medium cultured with the diaphyseal and metaphyseal tissues. This protein in the medium was increased by culture with zinc, IGF-I or TGF-beta. Total protein content in the medium cultured with the diaphyseal and metaphyseal tissues was significantly increased in the presence of zinc, IGF-I or TGF-beta. The IGF-I-increased medium protein content was significantly enhanced by zinc. This enhancing effect was not seen in TGF-beta. Alkaline phosphatase activity and deoxyribonucleic acid (DNA) content in the diaphyseal and metaphyseal tissues was significantly increased by culture with zinc, IGF-I or TGF-beta. The effect of IGF-I was significantly enhanced by zinc, while it was not found in TGF-beta. The effect of IGF-I or TGF-beta in increasing the bone components was seen in the presence of dipicolinate. This study demonstrates that zinc, like IGF-I and TGF-beta, can increase protein components in the femoral-diaphyseal and metaphyseal tissues of new-born rats. Zinc may especially play a role in bone growth in collaboration with IGF-I.
Alkaline Phosphatase↗
The postnatal development of the insertions of the medial collateral ligament in the rat knee.
Bone soft tissue remodelling at the femoral and tibial insertions of the medial collateral ligament (MCL) of the rat knee was monitored at regular intervals from birth to 120 days of age in 40 Sprague Dawley rats. At birth the femoral insertion originated from the perichondrium of the epiphysis. By day 8 the perichondrium within the insertion had turned into fibrocartilage. Secondary ossification of the femoral epiphysis had progressed in the region near to the insertion site by day 15. The epiphyseal cartilage was entirely replaced by bone by day 40 except for the fibrocartilage within the insertion. After that stage, no qualitative change in zonal insertion characteristics was observed, but only increase in size and decrease in cellularity. At birth, the tibial ligament inserted onto the thin cortical bone of the metaphysis via periosteum. At day 8, osteoclasts started to resorb the thin cortical bone at the ligament insertion, thus forming a metaphyseal depression between days 10 and 20. From days 20 to 120, the insertion remained qualitatively unchanged, showing three zones, the ligament, periosteum, and metaphyseal trabecular bone. The deep periosteal layer showed osteoclastic activity in the proximal part and osteoblastic activity in the distal part. The migration-mechanism of the ligament insertion during growth seems to be caused by this growth-related osteoclastic resorption of the proximal metaphyseal bone and by simultaneous osteogenic activity, which successively cements the distal part of the ligament to bone. The persistence of the periosteal layer and the metaphyseal depression for up to 120 days may be regarded as a sign of continuing growth in this animal model. This is the first investigation showing that the formation of the metaphyseal depression is a purely postnatal event, and suggests that this process might be initiated by the change in mode of growth and joint biomechanics after birth, enabling ligament development and migration in a growing and increasingly loaded weight-bearing joint. The mainly resorptive process, which takes place during development of the tibial MCL insertion, may account for the tensile failure of this ligament that commonly occurs at this site during growth. The pronounced morphological differences between the chondral femoral and the periosteal tibial attachment of the adult MCL are apparently caused by the different postnatal development processes at epiphyses and metaphyses.
Animals↗
RMRP gene sequence analysis confirms a cartilage-hair hypoplasia variant with only skeletal manifestations and reveals a high density of single-nucleotide polymorphisms.
Mutations in the RMRP gene that codes for an RNA subunit of the MRP RNAse complex are the cause of cartilage-hair hypoplasia (CHH; MIM 250250). We tested the hypothesis that recessive metaphyseal dysplasia without hypotrichosis (M1M 250460), a disorder presenting with short stature and metaphyseal dysplasia similar to CHH, but lacking hair anomalies, immunodeficiency and other extra skeletal features, might be allelic to CHH. We identified four mutation-carrying alleles segregating with the skeletal phenotype in two unrelated boys and their parents. One allele carried the common Finnish mutation +70A--> G; the remaining three carried +195C--> T, +238C--> T, and dupAAGCTGAGGACG at -2. Sequencing 120 alleles from a control group revealed an unusually high density of single-nucleotide polymorphisms in and around the RMRP gene: the biological significance of this finding is unclear. We conclude that recessive metaphyseal dysplasia without hypotrichosis is a variant of CHH, manifesting only as short stature and metaphyseal dysplasia. Precise diagnosis of this form of metaphyseal dysplasia is not without importance because of recessive inheritance with corresponding recurrence risk, as well as because of potential complications such as anaemia, susceptibility to infections and the increased likelihood of developing cancer. The short stature and metaphyseal changes associated with cone-shaped epiphyses of the hands should raise the diagnostic possibility of a CHH-related disorder that can then be confirmed by mutation analysis.
Alleles↗
Radiographic measurement of infantile tibia vara.
The reproducibility of the radiographic measurement of 30 pairs of tibiae being treated for bowing was evaluated. Two methods of measurement were used. The first was the metaphyseal-diaphyseal angle of Levine and Drennan; the second, a metaphyseal-metaphyseal angle based on Cobb's angle for measuring scoliosis. Statistical analysis comparing the two methods showed no significant differences except for an increase in the angle in more severe cases measured by the metaphyseal-metaphyseal method, indicating distal tibial as well as proximal involvement. Computer digitization correlated extremely well with manual measurements. The metaphyseal-diaphyseal measurement is quite reproducible, and may allow early diagnosis and treatment of tibia vara.
Child, Preschool↗
Cementless stem fixation and primary stability under physiological-like loads in vitro.
Primary stability and in consequence osteointegration are commonly related to the stem anchorage but also to the complex musculoskeletal loading of the hip region. This study investigated the influence of metaphyseal and meta-diaphyseal anchorage on the primary stability of cementless stems under physiological-like loading in vitro. Metaphyseal and meta-diaphyseal anchoring stems (n=6 each) were implanted into composite femora. Musculoskeletal loads, validated by in vivo data (peak joint force 2348 N), were applied using a mechanical set-up. Interface movements were recorded by seven displacement transducers and primary stability was compared. Both stems exhibited similar movement patterns and principally moved distally with a retroversional twist. Although elastic movements were comparable, the metaphyseal stem exhibited higher plastic deformations than the meta-diaphyseal stem, particularly for the metaphyseal, medio-lateral and antero-posterior components. Under physiological-like loading, the metaphyseal stem allowed higher interface movements and tended to initially migrate faster than the meta-diaphyseal stem and then stabilized. Elastic movements were comparable and seemed to be less influenced by the anchoring concept than by the mechanical properties of the bone. The analyses emphasize the importance of metaphyseal bone in proximal anchorage and the necessity of an accurate canal preparation to prevent excessive initial migration.
Arthroplasty, Replacement, Hip↗
[Use of the elastic stable intramedullary nailing technique in non-typical pediatric fractures].
PURPOSE OF THE STUDY: The "Elastic Stable Intramedullary Nailing" (E.S.I.N.) of French authors as a method of treatment of children's diaphyseal long bone fractures is well known from nineties of the last century. After we got familiar with the technique we wanted to introduce it in other indications, especially some metaphyseal long bone fractures and injuries of metacarpals, meta-tarsals and phalangeal bones in the growing skeleton. MATERIAL: During two last years, i.e. 1999-2000, ESIN procedure in 97 children was performed in our Regional Pediatric Trauma Centre. That was 39% of all osteosyntheses and 3.05% of all children's fractures treated during this period. Children treated by ESIN procedure were divided into three groups (see in Methods), 52 being in group one, 28 in group two and 17 in group three. METHODS: In our series of patients managed with the use of ESIN procedure according to the type of fractured bone and physical behavior of the implants three groups were formed. In the first group called "Classic ESIN Procedure" were children with diaphyseal long bone fracture (femur, tibia, humerus and forearm bones), in the second group "Non-typical ESIN Procedure" metaphyseal long bone fractures (humerus, radius, tibia) and in the third group, "ESIN-like Procedure" patients with metacarpal and phalangeal digital fractures were included. In all children the technique was very similar to original French (Nancy) description. Supraphyseal trepanation of the bone and introduction of usually two prebent elastic nails intramedullary. RESULTS: All 97 children healed good. In four children we saw little problems. In one patient it was necessary to change the ESIN osteosynthesis of open tibia fracture to external fixation. In the other boy we saw delayed union of forearm bones and in the third a 2 centimeter overgrowth of the affected tibia was recorded. In the last boy where non-typical ESIN procedure was performed for proximal radial fracture separation the angulation of radial head, overgrowth and subluxation occurred. However, we were surprised by simplicity, short time of the procedure and good results in two new groups of patients with metaphyseal and metacarpal resp. phalangeal fractures. DISCUSSION: Though in metaphyseal long-bone fractures (Non-typical ESIN Procedure) and metacarpal and phalangeal digital fractures (ESIN-like Procedure) the technique does not respect original physical suggestions of three point fragment fixation it works very well. Already the French authors from Nancy mention the indication of the ESIN technique for some metaphyseal fractures (supracondylar humeral and proximal radial). We do not use the method in supracondylar fractures but found it excellent in earlier problematic proximal humeral and radial fractures and especially phalangeal digital fractures. Also we were able to establish three main indication groups for this procedure. CONCLUSIONS: The ESIN technique can be used in three groups of children's fractures. "Classic ESIN Procedure" in diaphyseal long bone fractures, "Non-typical ESIN Procedure" in metaphyseal long bone fractures and "ESIN-like Procedure" in metacarpal and phalangeal digital fractures.
Bones of Upper Extremity↗