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PubMed2005

Fetal mesenchymal stem-cell engraftment in bone after in utero transplantation in a patient with severe osteogenesis imperfecta.

BACKGROUND: Mesenchymal stem cells (MSC) are progenitors of mesenchymal tissues such as bone, cartilage, and adipose. Adult human leukocyte antigen (HLA)-matched MSC have been used in cellular therapies of bone disorders such as osteogenesis imperfecta, with promising results. METHODS: A female fetus with multiple intrauterine fractures, diagnosed as severe osteogenesis imperfecta, underwent transplantation with allogeneic HLA-mismatched male fetal MSC in the 32nd week of gestation. Engraftment analyses of donor cells, immunologic reaction against donor cells, and the well-being of the patient were assessed. RESULTS: At 9 months of age, on slides stained for osteocalcin or osteopontin, a centromeric XY-specific probe revealed 0.3% of XY-positive cells in a bone biopsy specimen. Whole Y genome fluorescent in situ hybridization staining showed a median of 7.4% Y-positive cells (range, 6.8%-16.6%). Bone histology showed regularly arranged and configurated bone trabeculae. Patient lymphocyte proliferation against donor MSC was not observed in co-culture experiments performed in vitro after MSC injection. Complementary bisphosphonate treatment was begun at 4 months. During the first 2 years of life, three fractures were noted. At 2 years of corrected age, psychomotor development was normal and growth followed the same channel, -5 SD. CONCLUSIONS: The authors' findings show that allogeneic fetal MSC can engraft and differentiate into bone in a human fetus even when the recipient is immunocompetent and HLA-incompatible.

Adult↗

PubMedDate not supplied

Mechanical induction of osteogenesis: the importance of pin rigidity.

Eight dogs, divided into two groups of four by varying pin rigidity, underwent 15% left tibial lengthening by the Ilizarov method. In group I, "tensioned" 1.6-mm wires maintained a rigidity approaching that of 4.0-mm pins. In group II, the wires, maintained at half the tension, averaged 45% of the rigidity measured in group I. All dogs in group I filled the experimental gap with de novo osteogenesis, whereas all of the dogs in group II prematurely bridged the gap, arresting the process of osteogenesis. From these experimental results, clinical trials have been started using commercially available external fixation devices utilizing pins with equivalent rigidity.

Adolescent↗

PubMedDate not supplied

The prevention of recurrent fractures of the lower extremities in severe osteogenesis imperfecta using vacuum pants: a preliminary report in four patients.

Severe osteogenesis imperfecta results in frequent fracturing of lower extremity bones when the child assumes upright weightbearing. To prevent such breakage and to provide simple, comfortable standing, a special "vacuum pant" orthosis was designed and assessed in four patients. Not only has this enabled the family to help their children stand up safely and comfortably, but the frequency of fracturing has diminished. Bone densitometry studies of the tibia revealed an increase in both the mineral content (0.39 to 0.70 g/cm) and the bone density (0.44 g/cm2) above that expected for growth alone in one patient. The "vacuum pant" orthosis is a useful addition to the treatment program of the severe osteogenesis imperfecta child.

Child↗

PubMed1994

Production of overmodified type I procollagen in a case of osteogenesis imperfecta.

Collagen synthesis in cultured skin fibroblasts from a patient with osteogenesis imperfecta was studied. Approximately 2 fold accumulation of collagen in the cell layer was found. The slower mobility of pro alpha 1 (I) and pro alpha 2 (I) as well as alpha 1 and alpha 2 (I) polypeptide on sodium dodecylsulfate-polyacrylamide gel electrophoresis was detected, indicating that abnormal posttranslational modification could be present in type I procollagen in patient fibroblasts. The degrees of hydroxylation and subsequent glycosylation of lysine residues in the affected collagen were elevated 1.5 and 1.4 fold, respectively. There were no significant changes in the relative content of type III to type I collagen nor the incorporation of mannose into the carboxyterminal propeptide of pro alpha 1 (I) and pro alpha 2 (I). These results indicate that the patient produces an over-modified type I procollagen which is responsible for the clinical features and has a collagen abnormality already reported in type II osteogenesis imperfecta.

Cells, Cultured↗

PubMed1996

Osteogenesis imperfecta and malignant hyperthermia. Is there a relationship?

We describe a patient with osteogenesis imperfecta who developed tachycardia, metabolic and respiratory acidosis (pH 7.14, PCO2 8.4 kPa, BE -8.5 mmol.l-1) and hyperthermia up to 40 degrees C during anaesthesia with barbiturates, fentanyl, pancuronium, and nitrous oxide. Malignant hyperthermia was suspected and the patient treated accordingly. Two years later the in-vitro contracture test for malignant hyperthermia was completely normal. We conclude that hypermetabolism in patients with osteogenesis imperfecta is due to unknown mechanisms other than malignant hyperthermia.

Anesthesia, Intravenous↗

PubMed1991

Prenatal diagnosis of lethal osteogenesis imperfecta in twin pregnancy.

Lethal osteogenesis imperfecta was diagnosed at 27 weeks amenorrea in one fetus of a bichorial twin pregnancy. Sonographic findings included: short-limb dwarfism, hypotrophy and hypoechoic bones. The affected fetus was so translucent that only the normal fetus could be seen on plain in utero radiography. The affected fetus died immediately after birth. Postmortem radiography and histology were typical of lethal osteogenesis imperfecta of type IIA. Aids to the etiological diagnosis of in utero dwarfism are presented. Sonographic features correlated with neonatal death are described.

Diagnosis, Differential↗

PubMed2004

Alveolar distraction osteogenesis vs. vertical guided bone regeneration for the correction of vertically deficient edentulous ridges: a 1-3-year prospective study on humans.

The purpose of this prospective study was to compare vertical guided bone regeneration (GBR) and vertical distraction osteogenesis (DO) for their ability in correcting vertically deficient alveolar ridges and their ability in maintaining over time the vertical bone gain obtained before and after implant placement. Eleven patients (group 1) were treated by means of vertical GBR with autogenous bone and e-PTFE membranes, while 10 patients (group 2) were treated by means of DO. In group 1, six patients received implants at the time of GBR (subgroup 1A), while five patients had implants placed at the time of membrane removal (subgroup 1B). In group 2, implants were placed at the time of distraction device removal. A total of 25 implants were placed in group 1 and 34 implants were placed in group 2 patients. Three to 5 months after implant placement, patients were rehabilitated with implant-borne dental prostheses. The following parameters were evaluated: (a) bone resorption of the regenerated ridges before and after implant placement; (b) peri-implant clinical parameters 1, 2, and 3 years after prosthetic loading of implants; (c) survival and success rates of implants. Bone resorption values before and after implant placement were significantly higher in group 1. The results suggested that both techniques may improve the deficit of vertically resorbed edentulous ridges, although distraction osteogenesis seems to be more predictable as far as the long-term prognosis of vertical bone gain is concerned. Implant survival rates as well as peri-implant clinical parameters do not differ significantly between the two groups, whereas the success rate of implants placed in group 2 patients was higher than that obtained in group 1 patients.

Adolescent↗

PubMed2004

Novel development for intraoral distraction osteogenesis by individually fabricated traction prostheses.

Modified fixed or removable partial dentures are used for intraoral distraction osteogenesis. These traction prostheses rest on teeth or endosseous implants and direct distraction forces to an endosseous implant in the osteotomy segment. This article explains the fabrication and function of custom-made traction prostheses for 1-, 2- and 3-dimensional distraction osteogenesis.

Dental Abutments↗

PubMed2005

Reconstruction of mandibular defects following tumor ablation by vertical distraction osteogenesis using intraosseous distraction devices.

OBJECTIVES: Distraction osteogenesis has recently evolved a challenging technique to overcome major drawbacks of conventional augmentation procedures. We, therefore, report the application of miniaturized intraosseous distraction devices for the rehabilitation of mandibular defects due to ablative tumor surgery. METHODS: In 10 patients who had undergone box-shaped or segmental resections, augmentation of the residual mandibular bone or of full thickness iliac crest grafts was performed by intraosseous implant-shaped distractors. Distraction and reconsolidation was monitored by ultrasonography. Implants were inserted within 1 week after active distraction. Median follow-up after implant insertion was 38 months. RESULTS: On average, a vertical gain of 7.3 mm was obtained by distraction. Except for one case (local infection), all distraction zones showed complete ossification by radiologic and ultrasonographic evaluation. Overall 28 implants were placed in the distracted bone. Two implants were lost at 2.4 and 22 months after placement. The estimated 4-year implant survival rate in this population was 90%. For the endpoint 'bone loss' (>1.5 mm in the first and >1 mm in following years), the estimated 4-year success rate was 59% with four out of seven events occurring in a single patient (patient No. 1 of this series). CONCLUSION: Vertical distraction by means of implant distractors could be performed with reasonable success in tumor patients with box-shaped resection defects or undercontoured bone grafts. Overall morbidity was very low. Even though blood supply is continuously maintained in distraction osteogenesis, bone resorption remains a critical issue for this reconstruction technique too.

Adult↗

PubMed1985

Incisor and jaw relationship in 27 persons with osteogenesis imperfecta.

The incisor relation and craniofacial morphology, as evaluated from lateral cephalograms, were studied in 14 women and 13 men with osteogenesis imperfecta. A negative overjet was found in one woman and five men, and for the total material a mesial basal jaw relationship was observed. Data for both women and men indicated that skeletal discrepancies had been partially compensated for by the incisor inclination. Probably, persons with osteogenesis imperfecta may be regarded as a potential high-risk group for development of malocclusion.

Adolescent↗

PubMed1980

An arthropathic form of osteogenesis imperfecta.

A 14-year-old girl gradually developed severe osteoporosis and destructive generalized joint disease, resulting in joint stiffness and anchyloses. She also had moderate hydroxyprolinemia and hydroxyprolinuria. Rheumatoid arthritis was highly unlikely. Anamnestic data revealed two long bone fractures. Collagen biosynthesis was studied in fibroblasts cultured from the patient's skin. Chromatograms of 3H-labelled culture media proteins on ion exchange celluloses revealed an increased ratio of type III collagen to type I collagen when compared with the chromatograms of age-matched control fibroblasts. This finding is typical of certain cell strains in osteogenesis imperfecta. The patient might thus express a new variety of osteogenesis imperfecta with chronic arthropathy.

Adolescent↗

PubMed1983

Effect of porcine calcitonin therapy on vitamin D metabolism and clinical response in a patient with osteogenesis imperfecta.

A 1 11/12-year-old girl with osteogenesis imperfecta was treated with porcine calcitonin. Eight bone fractures occurred in the previous 20 months before therapy, but none occurred during eight months of therapy. There was also a significant improvement in linear growth and radiographic bone density. This is the first study of the effect of calcitonin on vitamin D metabolism in a human. The high plasma levels of 1,25 dihydroxy-vitamin D (1,25-(OH)2-D) and 24,25 dihydroxy-vitamin D (24,25-(OH)2-D) before calcitonin therapy decreased after therapy. Plasma 25 hydroxy-vitamin D (25-OH-D) concentration, which normal in level before calcitonin therapy, was normal or slightly decreased during administration. It is concluded that calcitonin probably influences vitamin D metabolism in a patient with osteogenesis imperfecta.

Animals↗

PubMed1991

Clinical and genetic aspects in autosomal dominant inherited osteogenesis imperfecta type I.

In 30 fully investigated family pedigrees in which there were at least two generations of people suffering from osteogenesis imperfecta type I (McKusick no. 16620), the data on 144 random offspring could be used for segregation analysis. The major characteristics, blue sclerae, fractures, and hearing loss, were present in every pedigree. Their penetrance was also calculated. Precise definitions were used in the study. The segregation ratio or observed: expected ratio was 70:72. The incidence of blue sclerae was 70:70 (100%), for fractures 61:70 (87%), and for hearing loss 30:70 (43%). There was a very clear relationship between age and the progression of the hearing loss. Dividing the offspring into two groups depending on whether or not male-to-male inheritance was present and performing segregation and penetrance calculation on these data did not produce any indications that there are two genetically distinguishable subtypes of osteogenesis imperfecta type I. In a smaller group of 107 offspring, calculations could be made on several separate generations.

Age Factors↗

PubMed1983

Heritable bone fragility, joint laxity and dysplastic dentin in Friesian calves: a bovine syndrome of osteogenesis imperfecta.

A lethal syndrome of bone fragility, joint laxity, translucent teeth and other connective tissue defects occurred in Victoria in 37 of 84 progeny of a clinically normal, registered Friesian bull between 1978 and 1981. Test matings to unrelated cows indicated that the syndrome was transmitted as an autosomal dominant trait. Affected calves were readily recognized at birth by pink teeth and abnormal posture. At birth 14 of 19 affected calves examined had rib fractures and 4 of these calves also had fractures of long bones. Spontaneous fractures of long bones also occurred in 5 of the 11 calves which survived for more than 10 days. Hypoplasia of dentin, tendons, and ligaments were observed in all cases at necropsy. Osteopenia was only observed at necropsy in older calves. Histological lesions of bone and teeth were similar to those reported in descriptions of osteogenesis imperfecta with dentinogenesis imperfecta in man. The syndrome is provisionally designated as "bovine osteogenesis imperfecta". Semen has been collected from the progenitor bull to enable further investigations of this heritable collagenous tissue dysplasia.

Animals↗

PubMed1994

Bone mineral content and density in healthy subjects and in osteogenesis imperfecta.

Lumbar spine bone mineral density in a cross sectional study of healthy subjects increased by 0.012 and 0.016 g/cm2/year in boys and girls respectively between 5 and 11 years of age. These rates increased five-fold in girls and threefold in boys between the ages of 11 and 13 years as a result of the bone mineral content increasing more rapidly than the coronal area at this age. By the age of 11 years the girls had 66% of the coronal area, 61% of the bone mineral density, and 41% of the bone mineral content of subjects aged 18-23 years. The ratio (lumbar spine bone mineral content/body weight) was constant in boys aged 6-13 years, but there were significant variations in girls. Femoral neck bone mineral density in both sexes changed little between 6 and 11 years and at 11 years was 69% of the adult values. Subjects with osteogenesis imperfecta had a low bone mineral density and bone mineral content for their age and weight. The z score of bone mineral density at the femoral neck was significantly lower than at the lumbar spine. In patients with recurrent fractures a low bone mineral density may help in identifying those with osteogenesis imperfecta and assist in their subsequent management.

Absorptiometry, Photon↗

PubMed1997

Hypercalcaemia in osteogenesis imperfecta treated with pamidronate.

The response to the bisphosphonate, pamidronate, is reported in a child with osteogenesis imperfecta who had recurrent symptomatic hypercalcaemia after immobilisation following fractures. Oral clodronate was effective in the prevention of immobilisation hypercalcaemia in the same child. The bisphosphonates may have other roles in osteogenesis imperfecta by decreasing bone turnover.

Adolescent↗

PubMed1974

Instability of polymeric skin collagen in osteogenesis imperfecta.

The structural polymeric collagen of the skin of 19 patients with osteogenesis imperfecta has been examined. In those with severe bone disease, who often have white sclerae, this collagen fraction is less resistant to depolymerization than that of age-matched controls, though the total amount is normal. In patients with less severe bone disease, whose sclerae are usually blue, the polymeric collagen may have normal stability but the total amount is reduced. These results suggest defective cross-linking of collagen in severe osteogenesis imperfecta.

Adolescent↗

PubMed1978

Metacarpal morphometry in adults with osteogenesis imperfecta.

Osteogenesis imperfecta is often regarded as a form of osteoporosis. In many cases, particularly those in whom the first fracture occurs outside the neonatal period, bones that have not been fractured may appear radiologically normal. In a group of 24 adults with osteogenesis imperfecta the thickness of the metacarpal cortex was normal but their bones were often slender. Osteoporosis is probably not an inevitable feature of such cases, and some of the radiological abnormalities reported may be the results of previous fractures and their treatment.

Adult↗