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At least 217 records · Page 12Linked to original sources

Coralline hydroxyapatite bone-graft substitutes in a canine metaphyseal defect model. Radiographic-histometric correlation.

Radiographic and histometric evaluation of a new form of bone-graft substitute derived from reef-building sea coral was performed in a canine metaphyseal defect model. Blocks of this material were implanted into the proximal tibial metaphyses of eight dogs, with radiographic densitometry and harvesting performed at two, four, six, and 12 months. Histometric analysis demonstrated progressive apposition of host compact bone at the margins and trabecular bone at the interior of the implants with time following surgery. Corrected transmission density determinations correlated significantly with degree of osseous ingrowth (R = -0.78), void volume fraction (R = 0.88), and postoperative interval (R = 0.88). These results support the successful early application of coralline hydroxyapatite bone-graft substitutes as an alternative to autogenous grafting in the clinical setting, and indicate that the course of incorporation into host bone can be noninvasively monitored using densitometric techniques.

Absorptiometry, Photon↗

Functional outcome after blade plate reconstruction of distal tibia metaphyseal nonunions: a study of 11 cases.

OBJECTIVES: To present the functional outcome of a cohort of 11 patients prospectively followed before and after blade plate reconstruction of a distal tibia metaphyseal nonunion. DESIGN: Prospective case series. SETTING: University hospital tertiary referral center. PATIENTS: Eleven patients with an average age of 48 years. Average duration of nonunion was 11 months. Patients had undergone an average of 3.1 procedures before the index surgery. Three patients had prior deep infections, and one patient had an active infection. INTERVENTION: A precontoured 4.5-mm cannulated blade plate was applied to the medial tibial surface through a posteromedial approach. Autograft was added in eight patients to fill bone voids. MAIN OUTCOME MEASURES: AOFAS scores were assigned to each patient preoperatively and at most recent follow-up. RESULTS: All patients healed their nonunions after the index surgery. Average time to radiographic union was 16 weeks. Average time to full weight bearing was 12 weeks. AOFAS scores improved in all patients from an average preoperative score of 29 to an average postoperative score of 89. The only complication was a deep infection, which was treated successfully with one irrigation and débridement and 6 weeks of intravenous antibiotics. CONCLUSION: Blade plate reconstruction of distal tibia metaphyseal nonunion is a safe and reliable method that results in a high union and low complication rate.

Adult↗

Metaphyseal nonunion: a diagnostic dilemma.

Twenty-eight patients with metaphyseal nonunion were evaluated retrospectively. Initially, all patients were thought to have healing of their fractures. Mild pain and/or instability with weightbearing or discomfort localized around the hardware were common symptoms of this nonunion; however, substantial pain was not a predominant feature. In the majority of cases, routine roentgenograms were not helpful in establishing the diagnosis. Overlapping hardware made visualization difficult; furthermore, the presence of a callus gave a false impression of fracture healing. Tomograms were done in 13 patients, but were not helpful in four patients. Stress views and examination under fluoroscopy were reliable means of making this diagnosis, particularly around the knee region. It was done, however, only in a few patients. Subtle changes in the position of hardware or the position of the fragments are also an indication of failure of union. The average time from the initial injury to the diagnosis of nonunion was 36 weeks. Five patients whose pain was attributed to the hardware were found to have a nonunion intraoperatively at time of attempted hardware removal. In one patient, the nonunion was diagnosed during a quadricepsplasty. In two patients, the diagnosis was missed a second time following revision of fixation and bone grafting. Diagnosis of metaphyseal nonunion requires a high index of suspicion, because it occurs among all age groups. It is not as rare as previously described and poses many difficulties. The authors recommend the use of additional studies, including tomograms or stress views, oblique to establish difficult diagrams.

Adolescent↗

Growth arrest of the distal radius following a metaphyseal fracture: case report and review of the literature.

We report a 12-year-old girl who developed growth arrest of the distal radius physis 9 months after sustaining a complete fracture of the distal radial and ulnar metaphysis with no involvement of the physis evident at time of injury. The girl sustained a fracture of the metaphysis of her right distal radius and ulna after a fall. Anterior-posterior, lateral and oblique radiographs at injury, and during subsequent healing show no evidence of the fracture involving the physis. She was treated with closed reduction and casting for 6 weeks and healed uneventfully. She returned 4 month later concerned about distal ulnar prominence. Radiographs revealed a loss of radial tilt and with suspicion of a physeal bar. Magnetic resonance imaging confirmed a physeal bar located in the dorsal radial region. A literature search of the Medline database was used to obtain prior case reports for review purpose. The patient underwent an epiphysiodesis of the distal radius and ulna along with an opening wedge osteotomy and bone grafting of the distal radius to restore radial height and inclination. She healed without complication and with restoration of the normal relationship of the distal radius and ulna. A review of the literature reveals five reported case of distal radial metaphyseal fractures not invloving the physis leading to growth arrest. By comparison, there are 31 reported cases of distal radius physeal arrest following fractures involving the physis. The physician should be aware that common distal radius metaphyseal fractures may rarely lead to growth arrest.

Child↗

Does fibular plating improve alignment after intramedullary nailing of distal metaphyseal tibia fractures?

OBJECTIVE: Evaluate whether supplementary fibular fixation helped maintain axial alignment in distal metaphyseal tibia-fibula fractures treated by locked intramedullary nailing. DESIGN: Retrospective chart and radiographic review. SETTING: Three, level 1, trauma centers. PATIENTS: Distal metaphyseal tibia-fibula fractures were separated into 2 groups based on the presence of adjunctive fibular plating. Group 1 consisted of fractures treated with small fragment plate fixation of the fibula and intramedullary (IM) nailing of the tibia, whereas group 2 consisted of fractures treated with IM nailing of the tibia without fibular fixation. OUTCOME MEASURES: Malalignment of the tibial shaft was defined as 1) >5 degrees of varus/valgus angulation, or 2) >10 degrees anterior/posterior angulation. Measures of angulation were obtained from radiographs taken immediately after the surgery, a second time 3 months later, and at 6-month follow-up. Leg length and rotational deformity were not examined. RESULTS: Seventy-two fractures were studied. In 25 cases, the associated fibula fracture was stabilized, and in 47 cases the associated fibula fracture was not stabilized. Cases were more likely to have the associated fibula fracture stabilized where the tibia fracture was very distal. In multivariate adjusted analysis, plating of the fibula fracture was significantly associated with maintenance of reduction 12 weeks or later after surgery (odds ratio = 0.03; P = 0.036). The use of 2 medial-lateral distal locking bolts also was protective against loss of reduction; however, this association was not statistically significant (odds ratio = 0.29; P = 0.275). CONCLUSIONS: In this study, the proportion of fractures that lost alignment was smaller among those receiving stabilization of the fibula in conjunction with IM nailing compared with those receiving IM nailing alone. Adjunctive fibular stabilization was associated significantly with the ability to maintain fracture reduction beyond 12 weeks. At the present time, the authors recommend fibular plating whenever IM nailing is contemplated in the unstable distal tibia-fibular fracture.

Adolescent↗

Metaphyseal growth arrest lines in psychosocial short stature.

Metaphyseal growth arrest lines are seen in children who experience significant physical stress such as infection or malnutrition over a sufficient period of time. These lines have not been reported previously in children with psychosocial short stature (PSS). Two boys and a girl with PSS with metaphyseal growth arrest lines on skeletal radiographs at the time of maximal stress in their homes are described. All three had reversible growth hormone insufficiency during admission, which is pathognomic for PSS. Multiple growth arrest lines in the distal end of the radius or vertebrae should alert clinicians to an alternative diagnosis in a child with growth hormone insufficiency. This may provide a clue to the diagnosis of occult PSS.

Body Height↗

Recessive metaphyseal dysplasia without hypotrichosis. A syndrome clinically distinct from McKusick cartilage-hair hypoplasia.

Among children with recessive metaphyseal dysplasia involving the knees and extremities, two types can be distinguished. In true cartilage-hair hypoplasia, as described by McKusick, many patients show clinical hair involvement and variable immunodeficiency. We present a series of six patients with the same radiological changes, but without apparent hypotrichosis. We suggest that they should be considered as having a variant form of cartilage-hair hypoplasia, with a clinically distinct phenotype, which could be as common as 'true' cartilage-hair hypoplasia among non-Amish populations. Microscopic examination of the hair may show reduction in the diameter of the hair shaft. This form of metaphyseal dysplasia may result from allelic heterogeneity.

Bone Diseases, Developmental↗

Genetic changes in the RNA components of RNase MRP and RNase P in Schmid metaphyseal chondrodysplasia.

BACKGROUND: The Schmid type of metaphyseal chondrodysplasia (MCDS) is generally due to mutations in COL10A1 encoding for type X collagen of cartilage. METHODS: We performed a study on the genes coding for the RNA components of RNase MRP (MRPR) and RNase P (H1RNA) among 20 patients with diagnosis of MCDS and no mutations in COL10A1. RESULTS: Two patients were found to be homozygous for a base substitution G for A at nucleotide 70 of RMRP, which is the major mutation causing cartilage-hair hypoplasia. No pathogenic mutations were detected in H1RNA. CONCLUSION: Cartilage-hair hypoplasia diagnosis should be considered in patients with metaphyseal chondrodysplasia even in the absence of any extra-skeletal manifestations if no mutation in COL10A1 can be found and the family history is compatible with autosomal recessive inheritance. Correct diagnosis is important for genetic counselling and for proper follow up of the patients.

Child, Preschool↗

Metaphyseal chondrodysplasia.

Any review of the metaphyseal chondrodysplasias is complicated by their variety and mainly unknown pathogenesis. The more familiar types display considerable clinical and radiological diversity: even more so the rarer disorders which still require complete definition, but differences in their mode of inheritance make diagnostic precision mandatory. These dysplasias present in infancy or in childhood, when the patient, usually dwarfed, may be proportionate, so that some forms may be confused with rickets or other lesions. Mental retardation is unusual, but the skin, hair, nails and facies provide valuable diagnostic features. Radiological abnormalities mainly affect the metaphyses of the shortened limb bones, less often the skull, vertebrae, pelvis, ribs and extremities, and sometimes their distribution may indicate the specific type of dysplasia. In a further complex group multiple systems are involved, notably the pancreas, intestine and lympho-reticular, causing malabsorption and haematological or immunological disorders.

Adenosine Deaminase↗

Radiation absorbed-dose estimates for the liver, spleen, and metaphyseal growth complexes in children undergoing gallium-67 citrate scanning.

Quantitative conjugate-view external counting techniques were applied to estimate the radiation dose to the liver, spleen, and metaphyseal growth complexes (distal femur and proximal tibia) for ten pediatric patients undergoing gallium-67 scanning procedures. The effective half-life of Ga 67 in these organs was approximately 78 hours. The dose per unit of administered activity for the liver and spleen was between 0.3 and 4.0 rad/mCi (0.08 to 1.08 Gy/GBq) and 0.5 and 7.0 rad/mCi (0.13 to 1.89 Gy/GBq), respectively. For the metaphyseal growth plates, the range was 2.3 to 14.3 rad/mCi (0.62 to 3.86 Gy/GBq).

Adolescent↗

Epi- and metaphyseal changes in children caused by administration of bisphosphonates.

Nitrogen-containing bisphosphonates (NCBs) are potent inhibitors of bone resorption and are used in the treatment of adults with various skeletal disorders. Little is known about their effects on the growing skeleton. The authors retrospectively studied the skeletal radiographs obtained in nine children before, during, and after NCB administration. Bandlike metaphyseal sclerosis and concentric epi- and apophyseal sclerosis developed in all patients. The extent of sclerosis depended on the duration of treatment and was related to local, and probably general, skeletal growth activity. In the maturing spine, NCBs caused a "picture-frame" or "bone-within-bone" appearance, depending on continuation or cessation of administration. In addition, metaphyseal undertubulation of long bones was noted in five patients. After discontinuation of bisphosphonate treatment and/or closure of the growth plates, the degree of sclerosis decreased, and sclerosis tended to disappear, indicating that this is a reversible phenomenon.

Adolescent↗

Reversible metaphyseal dysplasia, a novel bone phenotype, in two unrelated children with autoimmunepolyendocrinopathy-candidiasis-ectodermal dystrophy: clinical and molecular studies.

We report the association of an undescribed, reversible metaphyseal dysplasia (RMD) with autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) in two patients, one homozygous and one heterozygous for a 13-bp deletion in exon 8 of the autoimmune regulator (AIRE) gene. One patient also had a novel deletion in exon 6, resulting in a frameshift mutation and introduction of a STOP codon in exon 10. Their APECED phenotypes differed, but both patients developed progressive skeletal deformities and growth failure from early childhood. Radiological examination suggested a generalized abnormality of endochondral ossification, with irregular, flared, radioopaque regions in the metaphyses, subjacent to the growth plates. Histopathology in patient 1 showed islands of calcified cartilage within bone, consistent with impaired coupling of cartilage resorption with vascular invasion and ossification. Despite discordance for puberty, both patients experienced radiological resolution of their bone disease in their mid-teens, with improvement in histopathology in patient 1. RMD may constitute a rare phenotypic variation of APECED, possibly resulting from autoimmunity directed against skeletal proteins. We also demonstrated AIRE expression in chondrocytes derived from human fetal growth plates, primary culture of human chondrocytes, and two chondrosarcoma cell lines, suggesting a potential role for abnormal AIRE expression in the development of RMD.

Adult↗

IMAGe, a new clinical association of intrauterine growth retardation, metaphyseal dysplasia, adrenal hypoplasia congenita, and genital anomalies.

We report three boys with adrenal hypoplasia congenita (AHC) and additional findings that represent a new syndrome, IMAGe: Intrauterine growth retardation, Metaphyseal dysplasia, AHC, and Genital anomalies. Each presented shortly after birth with growth retardation and severe adrenal insufficiency. Each of the three patients had mild dysmorphic features, bilateral cryptorchidism, a small penis, and hypogonadotropic hypogonadism. Skeletal surveys revealed metaphyseal dysplasia in all three and epiphyseal dysplasia in two. The patients had documented or suspected hypercalciuria and/or hypercalcemia, resulting in nephrocalcinosis in one and in prenatal liver and spleen calcifications in another. AHC presents most often either as an isolated abnormality, caused by mutations in the DAX1 gene, or as part of an Xp21 contiguous gene syndrome, caused by a deletion of the Duchenne muscular dystrophy, glycerol kinase, and DAX1 genes. All three patients with the IMAGe association had normal creatine kinase levels and no evidence of glycerol kinase deficiency. Sequence analysis of DNA from these patients revealed no mutation in the DAX1- or steroidogenic factor-1-coding sequences, nor was a deletion of DAX1 detected. Identification of the molecular basis of the IMAGe association will give new insight into the pathogenesis of this syndromic relationship involving bone, adrenal cortical, and pituitary development.

Adrenal Insufficiency↗

A new type of leukoencephalopathy with metaphyseal chondrodysplasia maps to Xq25-q27.

BACKGROUND: The authors report a three-generation family with four male patients presenting with a novel type of X-chromosomal leukoencephalopathy associated with skeletal abnormalities. METHODS: The index patient and his brother reached their early motor milestones in due time and had normal language development. Between the ages of 2 and 3 years, first signs of spastic paraplegia were noticed. Furthermore, the patients developed tremor, ataxia, optic atrophy, and spastic tetraparesis. Both boys had broad wrists and knees without significant contractures. A maternal uncle and a granduncle had the same disease. RESULTS: Leukoencephalopathy (MRI, MRS) and metaphyseal chondrodysplasia (X-ray, MRI) were diagnosed. MRS showed a reduction of choline-containing compounds in the white matter. An autopsy on one of the patients, who died at age 37 years, revealed an orthochromatic type of leukoencephalopathy. In bone and cartilage tissue, unspecific signs of a mild chondrodysplasia were found. At the PLP gene locus an obligate recombination was observed, which excludes the Pelizaeus-Merzbacher locus on Xq21-22. However, affected males share a fragment of the long arm of chromosome X. CONCLUSION: The authors report a new type of leukoencephalopathy associated with metaphyseal chondrodysplasia located on Xq25-q27.

Adult↗

Metaphyseal abnormalities in fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva (FOP) is a rare, congenital disorder characterized by diffuse ossification of extraskeletal connective tissue. The classical features and progression of the disease are described and three cases are presented which fall into the general pattern of FOP clinically and radiologically. A constant feature seen was a slight metaphyseal flaring with spiking at the edges of the metaphyses, compatible with minor alteration in bone morphology during growth. These changes cannot be seen after epiphyseal fusion. The major abnormalities persist into adult life.

Bone and Bones↗

Management of completely displaced metaphyseal fractures of the distal radius in children. A prospective, randomised controlled trial.

In a prospective, randomised controlled trial, 68 children who had a completely displaced metaphyseal fracture of the distal radius were treated either by manipulation (MUA) and application of an above-elbow cast alone or by the additional insertion of a percutaneous Kirschner (K-) wire. Full radiological follow-up to union was obtained in 65 children and 56 returned for clinical evaluation three months after injury. Maintenance of reduction was significantly better in the K-wire group and fewer follow-up radiographs were required. There was no significant difference in the clinical outcome measured three months after injury. Seven of 33 patients in the MUA group had to undergo a second procedure because of an unacceptable position compared with none of the 35 in the K-wire group (chi-squared test, p < 0.01). One patient in the K-wire group required exploration for recovery of a migrated wire. We conclude that the use of a percutaneous K-wire to augment the reduction of the fracture in children who have a completely displaced metaphyseal fracture of the distal radius is a safe and reliable way of maintaining alignment of the fracture.

Adolescent↗

Targeted disruption of cadherin-11 leads to a reduction in bone density in calvaria and long bone metaphyses.

The migration and adhesion of osteoblasts requires several classical cadherins. Cadherin-11, one of the classical cadherins, was expressed in mouse osteoblasts in skull bone and femur, revealed by immunohistochemistry. To elucidate the function of cadherin-11 in osteoblastogenesis, cadherin-11 null mutant mice were investigated. Although apparently normal at birth, Alizarin red staining of null mutant mice showed a reduced calcified area at the frontal suture that caused a round-shaped calvaria with increasing animal age to 3 months. Consequently, there was a reduction in bone density at the femoral metaphyses and the diploë of calvaria in null mutant mice. In the in vitro culture of newborn calvarial cells, the calcified area of mutant cells was smaller than those derived from wild-type littermates. These results show that absence of cadherin-11 leads to reduced bone density in some parts of skeletons including calvaria and long bone metaphyses, and thus suggest that cadherin-11 plays roles in the regulation of osteoblast differentiation and in the mineralization of the osteoid matrix.

Animals↗

The development of metaphyseal cortex--implications for distal radius fractures during growth.

Fractures of the distal radial metaphysis are very common in otherwise healthy children. The reasons for this high fracture incidence are not entirely clear. To address this problem, we undertook a detailed analysis of distal radius development using peripheral quantitative computed tomography (pQCT) at a site 4% proximal to the radial articular surface. The study population comprised 337 healthy children and adolescents (aged 6-18 years; 171 girls) and 107 adults (aged 29-40 years; 88 women). Total volumetric bone mineral density (vBMD) remained stable at about 70% of the adult value between the ages of 6-7 years and 14-15 years in both genders. Cortical thickness increased little between 6-7 years and 12-13 years in girls and 14-15 years in boys. Strength-Strain Index (SSI; a parameter combining geometry and density) was still at only 20% of the adult value in girls aged 10-11 years and at 21% of the adult level in boys aged 12-13 years. At these ages, factors that contribute to the mechanical challenge to the distal radius in case of a fall (forearm length and body weight) had already reached 49% and 36% of the adult value in girls and boys, respectively. The shaping of the distal radius cortex (metaphyseal inwaisting) was assessed by analyzing the decrease in cross-sectional bone size between adjacent bone slices in a separate population of 44 children (aged 8-19 years; 26 girls). The rates of periosteal resorption and endocortical apposition were estimated to average 8 microm/day and 10 microm/day, respectively, during the growth period. In conclusion, during growth the increase in distal radius strength lags behind the increase in mechanical challenges caused by a fall, because metaphyseal cortical thickness does not increase sufficiently. The endocortical apposition rate is already very high at that site and apparently cannot be further increased to levels that would be necessary to keep bone strength adapted to the mechanical requirements.

Adolescent↗