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Valgus deformity following proximal metaphyseal fractures in children: experiences in the African tropics.

Three cases of valgus deformity following proximal metaphyseal fractures of the tibia in childhood are reported. All the cases seen required corrective osteotomies for management. In one case, a 4-year-old girl, recurrence was noted to have occurred despite the corrective osteotomy. Subsequent revision and suturing of the pes anserinus tendon plate was considered to be pertinent in the eventual correction and prevention of recurrence obtained. Although conservative management technics and the possibility of spontaneous correction are also considered in the management of the ailment, the role of the pes anserinus tendon plate is highlighted in terms of loss of its tethering effect and medial overgrowth due to hemichondrodiastasis. Primary repair by removal of the infolding of the periosteum into the fracture space and resuturing of same is the proposed method of management.

Adolescent↗

Distal metaphyseal fractures of tibia: a prospective randomized trial of closed reduction and intramedullary nail versus open reduction and plate and screws fixation.

BACKGROUND: To compare closed intramedullary nailing with open plate and screw fixation and set the indications for each treatment modality in distal metaphyseal fractures of tibia, 64 consecutive cases of fractures that had been randomly treated with either method were prospectively followed up. METHOD: Thirty-four patients were in the group treated with closed intramedullary nailing (Group I) and 30 patients were treated with open reduction and internal fixation with anatomic plates and screws (Group II). They were observed for 2 years, and the end results were compared between the two groups. RESULTS: The duration of operation was 72 minutes in Group I and 89 minutes in Group II (p = 0.02). The period of time before radiologic union was 18 weeks in Group I and 20 weeks in Group II (p = 0.89). There was one superficial infection in Group I and six superficial infections and one deep infection in Group II (p = 0.03). The average angulation was 2.8 degrees in Group I and 0.9 degrees in Group II (p = 0.01). The ankle dorsiflexion at the final follow-up was 14 degrees in Group I and 7 degrees in Group II (p = 0.001). The Olerud and Molander functional ankle score was 88.5% of normal side in Group I and 88.2% in Group II (p = 0.71). CONCLUSION: Our results have shown that locked intramedullary nails have an advantage in the duration of operation, restoration of motion, and reduced wound problems, and anatomic plate and screws can restore alignment better than intramedullary nails. It can be concluded from this study that intramedullary nails are recommended for fractures associated with soft-tissue damage of Tscherne C2 or higher. In other cases, the authors think that either treatment modality can yield expected results.

Adolescent↗

Physeal, metaphyseal, and diaphyseal injuries of the lower extremities in children with myelomeningocele.

A study of 16 patients with myelomeningocele who sustained 37 fractures revealed that children with diaphyseal and metaphyseal fractures presented with local warmth, redness, swelling, and increased general body temperature, leukocytosis, and sedimentation rate. These fractures were the result of a single stress or trauma and healed uneventfully by splinting for approximately 4 weeks. In physeal injuries, which probably resulted from repetitive stresses, the systemic response was less pronounced. These injuries had to be immobilized more rigidly in plaster casts for a minimum of 8 weeks.

Adolescent↗

The etiology of valgus angulation after metaphyseal fractures of the tibia in children.

We conducted a retrospective study of proximal metaphyseal fractures of the tibia in children who developed valgus deformities. We reviewed the cases in an effort to determine the most likely etiology of post-fracture tibia valga, which occurs despite careful treatment and follow-up. We reviewed seven major theories presented in the literature regarding the etiology of this problem. Our conclusion was that all the theories were not prerequisites for the development of valgus angulation, but rather may be secondary mechanisms. We suggest that the most likely primary mechanism is an increased vascular response causing an asymmetric growth stimulation of the medial metaphysis of the proximal tibia.

Child↗

Autosomal recessive inheritance of metaphyseal dysplasia (Pyle disease).

Two young adults with Pyle disease have been investigated in a large Afrikaner kindred in South Africa. Consanguinity was present in the family, and it is likely that the condition was inherited as an autosomal recessive. This contention is supported by the radiographic demonstration of minor degrees of widening of the distal femora in obligatory and potentially heterozygous relatives. Apart from genu valgus of moderate degree, the patients enjoyed good health and their gross radiographic skeletal abnormalities contrasted with the innocuous clinical presentation. Differentiation of Pyle disease from the autosomal dominant and autosomal recessive forms of cranio-metaphyseal dysplasia is of prognostic importance in view of the potentially serious complications in these latter disorders.

Adult↗

The manifestations and natural history of spondylo-epi-metaphyseal dysplasia with joint laxity.

Spondylo-epi-metaphyseal dysplasia with joint laxity (SEMDJL) is characterized by severe dwarfism, articular hypermobility and progressive spinal malalignment. The clinical manifestations of 18 affected persons in 13 families of the Afrikans-speaking community of South Africa have been analysed and it has become evident that survival into adulthood is unusual. SEMDJL is inherited as an autosomal recessive trait.

Adolescent↗

In vitro evaluation of screws and suture anchors in metaphyseal bone of the canine tibia.

OBJECTIVE: To compare ease of insertion, load to failure, and mode of failure of cortical and cancellous screws, BoneBiter, IMEX, and TwinFix suture anchors in canine metaphyseal tibial bone. STUDY DESIGN: Experimental biomechanical study. ANIMALS: Canine cadaveric tibias. METHODS: One investigator inserted all anchors and subjectively evaluated ease of placement. Anchor systems were loaded to failure along axis of insertion with audio-video recording to determine failure mode. RESULTS: BoneBiter was the most difficult anchor to insert successfully. Mean+/-SD loads to failure were cancellous screw (711+/-193 N), IMEX 4.7 mm 18 g wire (661+/-163 N), IMEX 4.0 mm 18 g wire (661+/-165 N), cortical screw (635+/-184 N), BoneBiter #5 Kevlar suture (393+/- 109 N), and TwinFix 5.0 mm #2 polyester (267+/-73 N). No significant differences were noted among the cortical screw, cancellous screw, IMEX 4.7 and 4.0 mm, all of which were significantly (P<.001) greater than BoneBiter and TwinFix . Failure modes were pullout of bone, suture-wire breakage, eyelet breakage, or no failure to 1000 N: screws (18,0,0,2), IMEX (18,1,1,0), BoneBiter (2,8,0,0), and TwinFix (0,10,0,0). CONCLUSIONS: Fixation devices were user friendly, with the exception of BoneBiter. Mode of failure is dependent on suture material and anchor design. Cortical and cancellous screws, and IMEX anchors with 18 g wire have significantly greater load to failure compared with BoneBiter and TwinFix suture anchors. CLINICAL RELEVANCE: Based on load to failure, ease of use, design characteristics, and cost, IMEX anchors may have advantages over other comparable soft tissue fixation devices.

Animals↗

Unusual metaphyseal disturbance in two kittens.

This report describes the presenting features, radiographic changes, biochemical alterations and clinical progress of two kittens, from separate litters, which were found to have a growth plate disturbance initially diagnosed and treated as vitamin D3-dependent rickets, but subsequently suspected to be a metaphyseal chondrodysplasia.

Animals↗

Chemical self-organization, complexification, and process metaphysics.

Recognizing the self-activity of matter through complexification can lead to seeing evolution as a process of union. This process approach emphasizes a metaphysics of becoming rather than of being. Advances in the science of thermodynamics lead to understanding chemical self-organization as a stage in this process of complexification.

Journal Article↗

A constitutively active mutant PTH-PTHrP receptor in Jansen-type metaphyseal chondrodysplasia.

A single heterozygous nucleotide exchange in exon M2 of the gene encoding the parathyroid hormone-parathyroid hormone-related peptide (PTH-PTHrP) receptor was identified in a patient with Jansen-type metaphyseal chondrodysplasia, which changes a strictly conserved histidine residue at position 223 in the receptor's first intracellular loop to arginine. Constitutive, ligand-independent adenosine 3',5'-monophosphate accumulation was observed in COS-7 cells expressing the mutant PTH-PTHrP receptor but not in cells expressing the wild-type receptor. This finding explains the severe ligand-independent hypercalcemia and hypophosphatemia, and most likely the abnormal formation of endochondral bone, in this rare form of short-limbed dwarfism.

Amino Acid Sequence↗

Metaphyseal chondrodysplasia, neutropenia, and pancreatic insufficiency presenting with respiratory distress in the neonatal period.

Two pairs of brothers suffered respiratory distress in the newborn period because their ribs were abnormally short. The diagnostic radiological features of metaphyseal chondrodysplasia appeared only in the second year. Pancreatic insufficiency and neutropenia were present. One died of overwhelming infection and his brother survived a life-threatening episode of gangrenous proctitis.

Agranulocytosis↗

Retinitis pigmentosa, metaphyseal chondrodysplasia, and brachydactyly: an affected brother and sister.

A brother and sister, children of normal parents are described. They had retinitis pigmentosa, causing near-blindness as a result of very narrow fields of vision, associated with metaphyseal chondrodysplasia and marked shortening of the metacarpals and terminal phalanges. Autosomal recessive inheritance is suggested with a common biochemical cause for all these defects. This apparently new association of retinitis pigmentosa with a systemic bone dysplasia emphasises that this not uncommon clinical diagnosis has a variety of different possible causes.

Adolescent↗

Femoral neck metaphyseal osteopathy in the cat.

This paper describes 17 cats that developed an idiopathic necrosis of the femoral neck. In four cats the lesions were bilateral when they were first examined and five cats developed lesions in the other limb within five months. They were all male cats, two years old or younger, and 15 had been neutered. The initial sign was a vague lameness which typically progressed, often acutely, to a more severe lameness. Radiography demonstrated radiolucency and loss of definition within the proximal femoral metaphysis, the femoral neck. In 12 cases there was a complete radiolucent line across the femoral neck. An excision arthroplasty was carried out on all the affected hips and the lameness resolved in all cases. The clinical and radiological signs suggest a primary bone resorption with secondary fracture of the femoral neck. The lesions have some similarities with Legg-Calve-Perthes' disease, traumatic fracture of the femoral neck, canine metaphyseal osteopathy, bacterial osteomyelitis and experimental feline herpes virus osteomyelitis.

Animals↗

Dose to the metaphyseal growth complexes in children undergoing 99mTc-EHDP bone scans.

The spatial temporal distribution of radionuclides in children may differ greatly from that accepted for adults. Following injection of a bone-seeking agent (99mTc-EHDP), radioactivity in the metaphyseal growth complexes of the distal femur and proximal tibia was quantitated in a series of children 4 to 16 years of age, using a gamma camera/computer system. The dose to the growth plate was fount to range from 0.8 to 4.7 rads when adjusted to an administered activity of 200 muCi/kg, compared to approximately 0.6 rad to the adult skeleton for a corresponding study.

Adolescent↗

Spondylo-megaepiphyseal-metaphyseal dysplasia: a new bone dysplasia resembling cleidocranial dysplasia.

Eight patients are presented who have a generalized bone dysplasia that resembles severe cleidocranial dysplasia but lacks the cranial and clavicular features of that well-defined condition. Vertebral-body ossification is markedly defective, and ossification centers of the tubular bones are grossly enlarged. These patients also differ from those with variant forms of cleidocranial dysplasia described in the literature. It is suggested that they constitute a separate entity sharing some features of cleidocranial dysplasia, and the descriptive term spondylo-megaepiphyseal-metaphyseal dysplasia is offered as a provisional term until it can be more specifically categorized. The condition appears to be transmitted as an autosomal recessive trait.

Adolescent↗

Prenatal diagnosis for Schmid metaphyseal chondrodysplasia in twins.

We report prenatal diagnostic studies for metaphyseal chondrodysplasia of the Schmid type. Identification of a specific COL10A1 gene mutation in an affected father allowed prenatal diagnosis by chorionic villus sampling in a twin pregnancy. Neither of the nonidentical twins received the abnormal COL10A1 gene from their affected father. This result was confirmed by postnatal DNA analysis. Prenatal diagnosis can be offered to all families with characterized COL10A1 gene mutations.

Chorionic Villi Sampling↗

A nonsense mutation in the carboxyl-terminal domain of type X collagen causes haploinsufficiency in schmid metaphyseal chondrodysplasia.

Type X collagen is a short-chain homotrimeric collagen expressed in the hypertrophic zone of calcifying cartilage. The clustering of mutations in the carboxyl-terminal NC1 domain in Schmid metaphyseal chondrodysplasia (SMCD) suggested a critical role for this type X collagen domain, but since no direct analysis of cartilage has been conducted in SMCD patients, the mechanisms of type X collagen dysfunction remain controversial. To resolve this problem, we obtained SMCD growth plate cartilage, determined the type X collagen mutation, and analyzed the expression of mutant and normal type X collagen mRNA and protein. The mutation was a single nucleotide substitution that changed the Tyr632 codon (TAC) to a stop codon (TAA). However, analysis of the expression of the normal and mutant allele transcripts in growth plate cartilage by reverse transcription PCR, restriction enzyme mapping, and a single nucleotide primer extension assay, demonstrated that only normal mRNA was present. The lack of mutant mRNA is most likely the result of nonsense-mediated mRNA decay, a common fate for transcripts carrying premature termination mutations. Furthermore, no mutant protein was detected by immunoblotting cartilage extracts. Our data indicates that a functionally null allele leading to type X collagen haploinsufficiency is the molecular basis of SMCD in this patient.

Animals↗