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PubMed1996

Constitutively activated receptors for parathyroid hormone and parathyroid hormone-related peptide in Jansen's metaphyseal chondrodysplasia.

BACKGROUND: An activating mutation of the receptor for parathyroid hormone (PTH) and parathyroid hormone-related peptide (PTHrP) was recently found in a patient with Jansens's metaphyseal chondrodysplasia, a rare form of short-limbed dwarfism associated with hypercalcemia and normal or low serum concentrations of the two hormones. To investigate this and other activating mutations and to refine the classification of this unusual disorder, we analyzed genomic DNA from six additional patients with Jansen's disease. METHODS: Exons encoding the PTH-PTHrP receptor were amplified by the polymerase chain reaction (PCR), and the products were analyzed by gel electrophoresis or direct nucleotide-sequence analysis. Nucleotide changes were confirmed by restriction-enzyme digestion of genomic DNA or the PCR products. RESULTS: The previously reported mutation, which changes a histidine at position 223 to arginine (H223R), was found in genomic DNA from three of the six patients but not in DNA from their healthy relatives or 45 unrelated normal subjects. A novel missense mutation that changes a threonine in the receptor's sixth membrane-spanning region to proline (T410P) was identified in another patient but not in 62 normal subjects. In two patients with radiologic evidence of Jansen's metaphyseal chondrodysplasia but less severe hypercalcemia, no receptor mutations were detected. In COS-7 cels expressing PTH-PTHrP receptors with the T410P or H223R mutation, basal cyclic AMP accumulation was four to six times higher than in cells expressing wild-type receptors. CONCLUSIONS: The expression of constitutively active PTH-PTHrp receptors in kidney, bone, and growth-plate chondrocytes provides a plausible genetic explanation for mineral-ion abnormalities and metaphyseal changes in patients with Jansen's disease.

Base Sequence↗

PubMed1990

Diagnosis of metaphysitis in foals.

Acute metaphysitis was induced in the third metacarpal bone of five foals by injecting lipopolysaccharide intraosseously adjacent to the growth plate, and synovial fluid was collected from the metacarpophalangeal joint 6 hours later. A control injection of an equal volume of physiological saline was given in a similar site in the opposite limb. Mild transient lameness, fever, depression and anorexia occurred after the lipopolysaccharide injection. On the lipopolysaccharide-injected side there was an increased leucocyte count in the synovial fluid, with neutrophil predominance; this did not occur on the control side. From this study in foals we conclude that in cases of suspected bacterial metaphysitis, arthrocentesis of the joint adjacent to the metaphysitis should be conducted immediately.

Journal Article↗

PubMed1996

Blade plate reconstruction of metaphyseal nonunion of the tibia.

Sixteen patients with nonunion of the metaphyseal region of the proximal or distal tibia after fracture or reconstructive procedures were surgically treated using a fixed angle blade plate and autogenous bone grafting. Eight of these nonunions were complicated by prior sepsis. The average time of followup assessment was 23 months. Fourteen of these 16 nonunions went on to uncomplicated union after the index procedure. Failure of the tibial fracture to unite in 2 patients required further surgical intervention with a ring external fixator. The authors recommend blade plate reconstruction for metaphyseal nonunion of the proximal and distal tibia, especially when complicated by angular deformity, small osteopenic metaphyseal fragments, and stiff adjacent joints.

Adult↗

PubMed2002

Aneurysmal bone cyst arising from a fibrous metaphyseal defect in a toddler.

This is a case of aneurysmal bone cyst arising from a fibrous metaphyseal defect of the proximal tibia in a toddler. A 32-month-old girl presented with physiologic bowing with a small metaphyseal defect at the medial aspect of the left proximal tibia. A large aneurysmal bone cyst, occupying almost the entire metaphysis of the proximal tibia, evolved from this lesion during the following 1 year 3 months. The lesion healed uneventfully after curettage and bone grafting. This case emphasizes the importance of regular followups to observe the clinical course of a fibrous metaphyseal defect during childhood.

Bone Cysts, Aneurysmal↗

PubMed2003

Salvage of distal tibia metaphyseal nonunions with the 90 degrees cannulated blade plate.

Nonunion of distal tibia metaphyseal fractures after trauma is a major problem. Treating these nonunions is made more challenging by the presence of symptomatic ipsilateral tibiotalar arthrosis. The current study examined the use of the 90 degrees cannulated blade plate as an alternative method of stable internal fixation for 13 distal tibia metaphyseal nonunions and simultaneous fusion of three arthritic tibiotalar joints in 13 patients (seven males and six females) with an average age of 42.4 years (range, 21-73 years). Each patient had an average of three prior procedures (range, 2-6). Patients were followed up for an average of 34.2 months (range, 24-55 months). All 13 patients achieved radiographic and clinical union an average of 15.6 weeks (range, 12-20 weeks) from the date of the definitive procedure. There were two broken screws, but no secondary procedures were required to obtain fusion. All patients were ambulatory without support at the last followup. The implant proved effective for stable internal fixation of distal tibia metaphyseal nonunions alone or with simultaneous fusion of the tibiotalar joint.

Adult↗

PubMed2006

Bone remodeling is different in metaphyseal and diaphyseal-fit uncemented hip stems.

Femoral component stability in uncemented total hip arthroplasties depends on periprosthetic bone remodeling. Stem design is an important factor influencing bone remodeling, however the design that promotes the most bone remodeling is unclear. We examined metaphyseal and diaphyseal-fit stems to determine the effect of stem design on bone remodeling and stability. Twenty-three patients who had total hip arthroplasties (28 hips) with metaphyseal-fit stems were matched with 27 patients (32 hips) who had uncemented total hip arthroplasties with diaphyseal-fit stems. We assessed preoperative radiographs for canal fill, canal shape, and bone quality. We then assessed postoperative radiographs for periprosthetic bone remodeling including spot welds, cortical hypertrophy, and pedestal formation. Patients were examined clinically using a modified Harris hip score. Patients with metaphyseal stems had increased cortical hypertrophy 1 year postoperatively. However, there was no functional difference 2 years postoperatively. Both stem designs resulted in bone remodeling by 2 years postoperatively with similar clinical results.

Adult↗

PubMed2006

A randomized, controlled trial of distal radius fractures with metaphyseal displacement but without joint incongruity: closed reduction and casting versus closed reduction, spanning external fixation, and optional percutaneous K-wires.

OBJECTIVES: To compare closed reduction and casting with closed reduction and external fixation with optional K-wire fixation for distal radius fractures with metaphyseal displacement but without joint incongruity. DESIGN: Prospective study. SETTING: Multicenter study at 3 University teaching hospitals. PATIENTS/PARTICIPANTS: A total of 113 skeletally mature patients with distal radius fractures with metaphyseal displacement, but without joint incongruity, were randomized to receive 1 of 2 standardized treatment protocols. Patients were evaluated at 6 weeks, 6 months, 1 year, and 2 years. INTERVENTION: Closed reduction and casting (n = 59) or closed reduction and external fixation (n = 54). MAIN OUTCOME MEASUREMENTS: Upper extremity function was measured using upper extremity MFA domain scores, overall Jebsen Taylor scores, and pinch and grip strength tests. Global function and pain were measured using the SF-36. Radiographic evaluation and range of motion were documented. RESULTS: Upper extremity MFA scores, Jebsen Taylor scores, SF-36 bodily pain scores, and grip strength improved significantly during the first year for all patients. By 2 years, mean Jebsen Taylor scores and SF 36 bodily pain scores for patients in both groups were similar to scores for normal age- and gender-matched population controls. At all points, there was a trend for better function in the external fixation; however, this did not reach statistical significance. There was a trend for better length and palmar tilt restoration with external fixation. CONCLUSIONS: For distal radius fractures with metaphyseal displacement but with a congruous joint, there exists a trend for better functional, clinical, and radiographic outcomes when treated by immediate external fixation and optional K-wire fixation.

Adult↗

PubMed1982

Fronto-metaphyseal dysplasia. Further delineation of the clinical syndrome.

Fronto-metaphyseal dysplasia is a rare but clinically striking condition characterized by skeletal and other abnormalities. Typical clinical and radiological features of the disease were present in four individuals in the family in this report. One of these, a male, had an obstructive uropathy at birth, making this the fourth male reported with fronto-metaphyseal dysplasia and an associated renal abnormality. In addition, he had severe congenital stridor associated with sub-glottic narrowing of the trachea. His affected sister also had severe congenital stridor associated with sub-glottic stenosis and a tracheal web. Both children had recurrent respiratory tract infection requiring frequent hospital admission. Respiratory problems have been reported in several other individuals with fronto-metaphyseal dysplasia and it would appear that renal and respiratory tract abnormalities are part of the syndrome. Although sex-linked inheritance has been considered the most likely mode of transmission of the disease, the segregation and clinical severity of the condition in this family could be equally well explained on the basis of dominant inheritance with varying expressivity.

Abnormalities, Multiple↗

PubMed1995

Dutch variant of Bellini metaphyseal dysplasia: report of two siblings.

Two sibling girls with cone-shaped knee epiphyses and metaphyses are described. Bone dysplasia with this rare, distinctive, radiographic finding, was first reported by Bellini and Bardare with only few cases reported thereafter. Velores et al. divided bone dysplasias with cone-shaped epiphyses and metaphyses of the knee in two entities which they named trichoscyphodysplasia and metaphyseal acroscyphodysplasia. Although the authors agree that there is more than one bone dysplasia that presents with these distinctive radiographic knee appearances, they consider that two few cases have been reported to satisfactorily classify this group of disorders.

Bone Diseases, Metabolic↗

PubMed1995

Transphyseal vessel involvement in repair of metaphyseal retained cartilage.

A foal with retained cartilage in the distal metaphysis of the right and left radii and third metacarpal bones had abnormal physeal vasculature. In areas where cartilage was retained, vessels crossed the physis, and branched at the physeal-metaphyseal junction or at the site of retained cartilage. Vessels appeared to be involved in re-initiation of endochondral ossification and in the repair reponse to the presence of retained cartilage. In areas where the physis was radiographically and histologically normal, no vessels crossed the physis. A function of transphyseal vessels may be as a reserve blood supply in areas of metaphyseal abnormality, at a stage of maturity when metaphyseal vessels are not well developed.

Animals↗

PubMed2003

Metaphyseal osteomyelitis in an immature Abyssinian cat.

Metaphyseal osteomyelitis is a rarely described condition in small animals, especially the cat. Infection, most commonly Staphylococcus sp, is considered to occur in the metaphyseal region of the immature animal due to vascular anomalies that predispose to the haematogenous seeding of bacteria in this area. There is also speculation that the characteristics of the bacteria that allow them to adhere to cartilage matrix, rather than vascular linings and erythrocytes, may provide an advantage for colonisation in the metaphysis, resulting in infection. This case describes the successful management of a case of distal radial metaphyseal osteomyelitis in an immature cat using surgical intervention and antibacterial therapy.

Animals↗

PubMed2003

Metaphyseal osteomyelitis in an immature Abyssinian cat.

Animals↗

PubMed1990

Microspherophakia-metaphyseal dysplasia: a 'new' dominantly inherited bone dysplasia with severe eye involvement.

We report a father and son affected by a hitherto unpublished bone dysplasia with moderately severe dwarfism. On initial radiographs, thickening of the diaphyses of the long bones was striking. The small bones of the extremities were almost unaffected. With age, the metaphyseal deformation became more prominent. The epiphyses became irregular and their growth was delayed (particularly the femoral heads). The femoral neck showed an unusual 'lip' on the inner edge. Later, the stubby appearance of the long bones faded and, in adulthood, only enlarged metaphyses and deformed femoral necks persisted. The vertebrae showed moderate deformation with irregular flattening, and narrowing of the spinal canal with a shortened interpedicular distance. The eye defects consisted of high grade myopia, microspherophakia, lens coloboma, lens luxation, and retinal detachment. The name 'microspherophakia-metaphyseal dysplasia' is suggested for this probably autosomal dominant bone dysplasia.

Adult↗

PubMed2004

Adrenal steroid hormones and metaphyseal bone in children.

BACKGROUND/OBJECTIVES: The responses of metaphyseal bone tissue to physiological variations of endogenous adrenal steroid hormones during childhood are unclear. Therefore, we studied potential hormonal influences in children before the appearance of pubic hair (onset of pubarche). METHODS: Excretions of major glucocorticoid metabolites (C21), cortisol, sum of adrenarchal dehydroepiandrosterone and its immediate 16-hydroxylated metabolites (DHEA&M), and 5-androstene-3beta,17beta-diol (hermaphrodiol) were analyzed in a cross-sectional study in 24-hour urine samples of 109 healthy boys and girls, aged 6-13 years, using steroid profiling by gas chromatography-mass spectrometry. Total and trabecular volumetric bone mineral densities, bone mineral content (BMC) and bone strength strain index were determined with peripheral quantitative computed tomography at the distal forearm. RESULTS: In multiple regression analyses significant associations with the metaphyseal radius were seen for grip force, age, or BMI depending on gender and bone variable analyzed. DHEA&M did not contribute to the explanation of the variance of any bone variable. However, hermaphrodiol positively explained a significant part of variation of bone mineral densities, and BMC (p < 0.01) in girls. Significantly negative associations with all bone variables were seen in boys for cortisol. CONCLUSIONS: The steroid hormones, cortisol and hermaphrodiol, in their physiological ranges, but not the adrenarche marker DHEA&M, appear to associate with metaphyseal bone in a sex-dependent manner during childhood.

Adolescent↗

PubMed1995

Histologic and immunocytochemical characterization of canine distemper-associated metaphyseal bone lesions in young dogs following experimental infection.

The proximal metaphyses of the humerus of weanling gnotobiotic dogs experimentally infected with canine distemper virus (CDV) were investigated histologically and immunocytochemically between 4 and 41 days after infection. Viral antigen was demonstrated in hematopoietic marrow and bone cells at postinfection day (PID) 5 and PID 7, respectively. Between PID 8 and 27, CDV antigen was abundantly present in marrow cells, osteoclasts, and osteoblasts and less frequently in osteocytes. Immunopositive cells in both osseous tissues and bone marrow declined between PID 29 and PID 36 and were absent by PID 41. Chondrocytes of the growth plate were negative for viral antigen throughout the observation period. In bone, viral antigen was more frequently observed in bone cells of the primary spongiosa than in the secondary spongiosa. There was a strong correlation between occurrence of CDV antigen and osseous changes. Associated metaphyseal bone lesions were mild and most prominent between PID 8 and PID 32. Lesions consisted of necrosis of osteoclasts, which was associated with subsequent persistence of the primary spongiosa (growth retardation lattice). Atrophy and necrosis of osteoblasts and marrow cells were also noted. Infection of metaphyseal bone cells appears to be common in young dogs with experimental systemic distemper. Bone cell infection is preceded by infection of marrow cells, and infected bone cells may experience degeneration and necrosis. This subtle viral effect may result in defects in bone modeling in CDV-infected dogs.

Animals↗

PubMed1990

The use of the powered metaphyseal stapler for reconstructive procedures in the adult foot.

We have evaluated the Powered Metaphyseal Stapler in various procedures in the adult foot to determine the effectiveness of this form of internal fixation. Thirty-five patients had 40 operations (five bilateral). In all but one case there was both clinical and radiographic evidence of a solid bony union. Backing out of the staples has been a minor problem that has required staple removal in three patients. The Powered Metaphyseal Stapler appears to offer a most satisfactory option for internal fixation in the foot, when used in metaphyseal or cancellous bone, as either primary or adjuvant fixation.

Adult↗

PubMed1978

Metaphyseal multifocal osteosarcoma.

Two patients, aged six and eight years, having metaphyseal osteolytic and osteoblastic multifocal osteosarcoma are described. The condition is named "metaphyseal multifocal osteosarcoma". The metaphyseal areas are the sites of the pathological process. It can be postulated that a certain intensity of growth activity is the predominant factor in this condition and that this accounts for its multicentered presentation.

Arm↗

PubMed1993

Use of the metaphyseal-diaphyseal angle in the evaluation of bowed legs.

We evaluated the accuracy of the angle described by Levine and Drennan, the metaphyseal-diaphyseal angle of the proximal aspect of the tibia, for the differentiation of physiological bowing from Blount disease. We compared this angle, as measured at presentation, in 106 children (179 extremities) who had physiological bowing with the angle in nineteen children (thirty-two extremities) who had documented Blount disease. The angle averaged 9 +/- 3.9 degrees for the patients who had physiological bowing and 19 +/- 5.7 degrees for the patients who had Blount disease (p < 0.0000001). Linear regression analysis, performed to evaluate any changes in the metaphyseal-diaphyseal angle in relation to age, showed that the older the child was at the time of presentation the more likely it was that the angle would be smaller in a child who had physiological bowing and larger in a child who had Blount disease. The chance for false-positive and false-negative errors was greater than 5 per cent if the angle was more than 9 degrees but less than 16 degrees. Sixty-six (37 percent) of the 179 extremities in the group that had physiological bowing had an angle of at least 11 degrees; one extremity affected by Blount disease had an angle of less than 11 degrees. In this study, we found that the metaphyseal-diaphyseal angle may be helpful in the identification of Blount disease but should not be the sole criterion used to determine the diagnosis.

Age Factors↗