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The occurrence of metaphyseal cortical irregularities in adolescents in many different bones indicates a commonality of these lesions as a variation of normal growth, rather than a stress or avulsive by-product. The histologically recognized incomplete nature of the metaphysial cortex in children offers an attractive explanation for this phenomenon.
1. Spondylo-epi-metaphyseal dysplasia with progressive, severe kyphoscoliosis and gross joint laxity is a distinctive entity. The clinical and radiographic manifestations of seven affected children are presented and it is concluded that this disorder is probably more common than previously suspected. Autosomal recessive transmission is likely. 2. Skeletal survey is indicated in all patients with infantile idiopathic scoliosis, kyphosis, or kyphoscoliosis. In this context, radiographic studies of the pelvis may be of great diagnostic value. 3. Referal for expert orthopaedic management is essential for all patients with this disorder, as profound disability with pulmonary and spinal complications may be expected.
Blount stapling of the growth plate induced changes in the metaphyseal architecture, which progressed in correlation to the postoperative follow-up. The stereological investigations revealed a subtile reaction of the medial stapled tibial plate in a total of 37 domestic pigs (10 weeks old) during the postoperative follow-up (up to 17 weeks). Zone 1, 380-1120 microns distal to the plate representing "bone modelling", and zone 2, 1180-2360 microns distally representing "bone remodelling", were investigated separately. The findings in zone 2 were very similar to zone 1 but were less extensive. The parameter most sensitive to stapling was the surface density; the trabecular distance showed parallel but less impressive findings. The specific surface and trabecular diameter changed with some delay. The last parameter to change was the volume density.
For some reasons skull findings in Jansen's Metaphyseal Dysplasia have been largely neglected. A survey of the seven known cases (three of them being primarily observed and described by two of the authors) disclose important and constant alterations, namely pronounced basilar thickening and sclerosis, prominent supraorbital and zygomatic arches, underdevelopment of the paranasal sinuses with sclerosis of the adjacent bone, and hypoplasia of the mandible. These alterations give rise to distinct and fairly specific features. The relationship to other craniotubular disorders, such as craniodiaphyseal dysplasia, craniometaphyseal dysplasia, and frontometaphyseal dysplasia is discussed.
The types and distribution of glycosaminoglycans (GAGs) were studied immunocytochemically in osteoid, mineralized bone matrix, and cartilage matrix of growing rat metaphyseal bone after aldehyde fixation and EDTA demineralization, using four monoclonal antibodies (mAbs 1-B-5, 2-B-6, 3-B-3 and 5-D-4). These mAbs specifically recognize epitopes in non-sulphated chondroitin (C0-S); chondroitin 4-sulphate (C4-S) and dermatan sulphate (DS); chondroitin 6-sulphate (C6-S) and C0-S; and keratan sulphate (KS) respectively. In osteoid, all mAbs except 1-B-5 weakly stained matrix material on and between collagen fibrils, and moderately stained organic material corresponding to bone nodules, which are known sites of mineralization. However, the staining of osteoid abruptly decreased at the mineralization front; weak staining was confined mostly to the organic material of bone nodules in mineralized bone matrix, with very weak or no staining of the rest of the bone matrix. This staining progressively decreased toward the mineralized cartilage matrix and became negative. The mineralized cartilage matrix and lamina limitans reacted strongly with all mAbs except 5-D-4. These results indicate that osteoid contains sulphated proteoglycans containing C4-S and/or DS, C6-S and KS, and subsequent bone matrix mineralization appears to require accumulation of these macromolecules within bone nodules and eventual loss of these substances for complete mineralization, whereas proteoglycans containing C0-S, C4-S and/or DS, and C6-S still exist in mineralized cartilage matrix and lamina limitans.
The authors describe two siblings, a male and a female, with disproportionate short stature, rhizomelic-mesomelic shortening of the limb bones, marked bowing of the femora, moderate bowing of the humeri, radii and ulnae, straight tibiae and fibulae, normal hands, flared cupped metaphyses of the tibiae, ulnae, radii and ribs, and narrow chest. There was some improvement of the bone changes with advancing age. These two patients are similar to five other cases from the literature and strongly support Hall and Spranger's view that this pseudocampomelic condition most likely represents a distinct familial bowing syndrome. The differential diagnosis and the hereditary aspects in the two patients, are also briefly discussed.
We report here a recurrent frameshift mutation within the carboxyl-terminal noncollagenous domain coding region of the type X collagen gene (COL10A1) in a Japanese patient with Schmid metaphyseal chondrodysplasia. The mutation involves deletion of a CT dinucleotide from position 1992 (1992delCT), and produces a frameshift which creates a premature termination codon close to the site of the deletion. The predicted length of the mutant polypeptide is 664 amino acids, which is shorter than the wild type polypeptide (680 amino acids). A 1992delCT mutation of COL10A1 has been previously reported in one family. The independent occurrence of de novo mutation of this specific dinucleotide repeat suggests that this region is a possible mutational hot spot on COL10A1.
A case of mitochondrial encephalomyopathy (Kearns-Sayre syndrome) demonstrating bilateral symmetric metaphyseal dysplasia is reported. This represents a new association with the syndrome.
A case of congenital syphilis is described in which the diagnosis was not made until the age of 9 and with persistance of metaphyseal changes in spite of prolonged penicillin treatment.
This is the report of a boy who presented at birth with severe generalized platyspondyly, a vertebral "coronal cleft", and an abnormal configuration of the pelvis with short and broad iliac and ischial bones and horizontal acetabular roofs. The rest of the skeleton was normal. In the ensuing years the vertebral bodies and pelvis assumed a near normal configuration, but the patient developed changes of metaphyseal chondrodysplasia in the long bones of the lower limbs with progressive shortness of stature.
Analysis of 20 cases of metaphyseal chondrodysplasia, Schmid type as well as a review of the world literature reveals a specific autosomal dominant disorder that was often over-diagnosed in the past, sometimes resulting in incorrect genetic counselling. Significant radiologic features include an enlarged capital femoral epiphysis in early childhood, coxa vara, greater involvement of the distal femoral metaphysis than the proximal, anterior rib changes and a normal spine. Chondroosseous morphology is not specific. Presentation in nonfamilial cases is no earlier than the second year of life.
Young weanling guinea piglets were placed on a diet deficient only in vitamin C. When they reached a state of severe scorbutus, they were given vitamin C and the morphological differentiation of various mesenchymal cells in the proximal end of the tibia was followed over 7 days. The altering metaphyseal cellular pattern is recorded descriptively as well as quantitatively. Levels of mesenchymal cells, preosteoblasts, osteoblasts, and osteocytes remained relatively steady for 24 h. However, by 48 h there was a precipitous decline of mesenchymal cells with a concomitant rise in recognizable osteogenic cells; this change continued until the 7th day of repletion. At this time, with the exception of the preosteoblasts, the cellular population had returned to about the level in the control animals. Osteoclastic and endothelial cellular movements fluctuated widely during the period of repletion under examination. These results support the concept of separate lines of differentiation for osteoclasts and osteoblasts in the postnatal animal. Moreover, mesenchymal cells appear to be precursors of osteogenic cells.
The surface structure of the growing portion of bones, called the metaphysis, contains clues about the locomotor characteristics of various species. Present methods of capturing this anthropologically interesting surface are time-consuming and subject to human error. The research implements a digital stereo imaging technique for bone metaphyses and joints in skeletal collections. The corresponding points in two images collected from different angles are determined using an area-based correlation matching method. The depths of matched points are computed from the difference in location of the points in the two images. The paper presents a practical implementation of computer vision for anthropology using an 80286-based personal computer, a camera and a video digitiser. The stereo matching algorithm, a practical implementation of classical stereo imaging methods, takes less than 1 min and produces reasonable representations of mammal bones. The accuracy of the depth measurements ranged from 0.7 to 12 per cent for 45-150 cm object-camera distances. False matches occurred in approximately 6 per cent of the total matched points.
Transverse midshaft spurs allowed a diagnosis of hypophosphatasia in a newborn infant without metaphyseal irregularity.
To assess the efficacy of PGE2 in inducing in vivo bone formation, graded doses of prostaglandins E2 were administered to 255 g rats. Histomorphometric analyses of selected sequential fluorescent-labeled bones of rats treated with 0, 0.3, 1.0, 3, or 6 mg PGE2/kg/d for 21 days showed that the doses PGE2 depressed longitudinal bone growth, increased growth cartilage thickness slightly, decreased degenerative cartilage cell size and cartilage cell production slightly, and increased proximal tibial metaphyseal hard-tissue mass markedly. Periosteal bone formation was depressed at the higher doses, and an early, slight depression in endosteal bone formation was also observed, along with a striking late increase in endosteal bone formation and in the formation of trabecular bone in the marrow cavity of the tibial shaft. The characteristics and magnitude of these responses were quite similar to those observed in our previous study of the effects of PGE2 on weanling rats except for the delayed increase in cortico-endosteal bone formation.
Patients with acute lymphatic leukaemia frequently are osteoporotic. A small subset of these develop disabling metaphyseal transverse fractures, usually bilateral and in the lower limb. These impaction fractures have a characteristic appearance and develop in recently laid down bone. They may develop ab initio or during therapy. Magnesium deficiency is found in these patients.
From Jan. 1995 to June 1996, 6 cases of traumatic defect of the tibia were treated by remodeling and reduction of fractured ends and osteotomy and distraction at the proximal metaphysics of the tibia. In 6 patients, aged 19 to 54, with the limb-length discrepancy being at least 3 to 4.5 cm, the tibia was lengthened by 3 cm in 3 cases, 4 to 4.5 cm in 3 cases. The consolidation and maturation of the bone were accomplished within 3 to 6 months. The authors believe that this procedure is simple, less invasive and involves no bone-graft.
45 trauma patients with fracture-related metaphyseal bone defects filled with hydroxyapatite ceramic (Endobon) were followed for three to four years after surgery. Osseointegration of Endobon could be confirmed histologically in all biopsates, which could be obtained during scheduled reintervention for hardware removal. The objective of clinical and radiological follow-up was to evaluate the extent of ceramic remodeling as well as long-term implant outcome. Thirteen patients undergoing surgery in 1992 or 1993 were available for follow-up. None of these patients showed radiologic evidence of loss of bony reconstruction or HAC sintering, nor was there evidence of HAC resorption or fragmentation. Our results--with respect to the examined few patients--suggest that Endobon is useful for filling bony defects in carefully selected trauma patients if certain surgical technique prerequisites are met. Like cancellous autografts special alloplasts appear to go some way toward solving problems of defect filling encountered in clinical practice.