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At least 973 records · Page 54Linked to original sources
PubMed1986

[Genetic heterogeneity of osteogenesis imperfecta. Study of 6 cases].

Osteogenesis imperfecta one of the most common disorders of connective tissue, has been known for centuries. The most characteristic alterations which define it are: osteoporosis, osseous fragility with multiple fractures, blue sclerae, deafness and imperfect dentinogenesis. Important advances in the biochemical, anatomopathological, genetic, therapeutic and prophylactic fields have resulted in a great present-day interest in this disease. In this work we report six cases of osteogenesis imperfecta according to the current classification and we review the most outstanding aspects.

Adult↗
PubMed1979

[A contribution to the recording and study of the disease pattern of osteogenesis imperfecta tarda (author's transl)].

This study aims at investigating in a group of thirty-five patients whether osteogenesis imperfecta tarda presents a uniform disease pattern in respect of clinical and roentgenological criteria, or whether it can be subdivided into clinical groups. Depending on the severity of the disease, it was possible to subdivide this into two groups: a severe form of osteogenesis imperfecta tarda, and a bland form. This subdivision was effected according to the most conspicuous symptom of frequency of the fracture. The other symptoms do not allow any subdivision into groups since their occurrence is independent of the severity of the disease pattern.

Activities of Daily Living↗
PubMed1973

The co-existence of primary hyperparathyroidism and osteogenesis imperfecta.

A 47-year-old patient suffering from osteogenesis imperfecta was found to have mild hypercalcemia. The latter proved to be due to a parathyroid adenoma. The clinical and laboratory features of this association are summarized, and the implications of serum calcium abnormalities in osteogenesis imperfecta are discussed.

Adenoma↗
PubMedDate not supplied

An in vitro study of electrical osteogenesis using direct and pulsating currents.

Although many investigators have electrically stimulated osteogenesis in vivo, this phenomenon has not been reported in vitro. In this work direct and pulsating currents were applied to in vitro fetal rat tibiae. The results indicated that electric current did cause osteogenesis in vitro, precipitates of calcium compounds formed in the cathode region, and direct current was more effective in stimulating bone growth than pulsed current delivering the same total charge (coulombs).

Animals↗
PubMed1977

Scanning electron microscopy findings in osteogenesis imperfecta fetalis.

A case of osteogenesis imperfecta fetalis in a stillborn black male infant is described. Scanning electron microscopic studies of the skeleton showed normal intercartilaginous septa in the metaphyses of long bones, covered by uniform, calcifying globules 1 mum in diameter. In the diaphyses, however, the orientation of bone trabeculae was abnormal. The trabeculae were thin, with wide and densely packed osteocytic lacunae, and with more resorbing surfaces than in the control specimens. It is concluded that in osteogenesis imperfecta there is abnormal periosteal ossification, together with increased osteocytic resorption.

Bone and Bones↗
PubMed1978

Osteogenesis imperfecta in one of twins. Case report.

The is a report of a 5-year-old girl with osteogenesis imperfecta, an uncommon abnormality considered to encompass several distinct disorders and biochemically classified as a connective tissue disease. Affected individuals, in the more severe form of the disease, often die in utero or shortly thereafter secondary to birth trauma. The patient was one of dizygotic twins discordant for osteogenesis imperfecta tarda inherited as an autosomal dominant trait.

Adolescent↗
PubMed1984

[Radionuclide studies of reparative osteogenesis during modeling the shape of the leg].

The time course of metabolic processes in the osseous tissue and the capillary blood circulation in the forming osseous regenerate and the bone as a whole were studied in 16 patients using pyrophosphate and DTPA. The results of radiometry made it possible to give a quantitative evaluation of regeneration intensity: during intensive osteogenesis the accumulation of labeled pyrophosphate increased up to 720%, and the capillary blood circulation speeded up to 380%. Before the completion of osteogenesis the drug accumulation decreased considerably up to 250%.

Adult↗
PubMed1978

Osteogenesis imperfecta as a complication of pregnancy.

Osteogenesis imperfecta is a complex disorder that rarely complicates pregancy. The successful obstetric management of a patient with severe osteogensis imperfecta is presented along with a detailed review of maternal osteogenesis imperfecta in the recent English literature. A review of the disease process, its complications, and associated disorders is presented.

Adult↗
PubMed1984

Brittle hair in osteogenesis imperfecta: transmission and scanning electron microscopy and mycologic assay.

The brittle hair fibres from a case of osteogenesis imperfecta were studied by scanning and transmission electron microscopy. Normal hair fibres are extremely difficult to infect with T. mentagrophytes. As a tentative assay for biochemical defects of hair fibres, experimental dermatophytic infection of the hair with T. mentagrophytes was performed. The massive growth induced suggested that the experimental fungal infection may indeed indicate a biochemical defect in the hairs from our case of osteogenesis imperfecta.

Adult↗
PubMed1984

Strangulated diaphragmatic hernia in a patient with osteogenesis imperfecta.

Hernias are common in patients with osteogenesis imperfecta, but to our knowledge there have been no reports of diaphragmatic hernias in such patients. We describe a patient with osteogenesis imperfecta in whom a diaphragmatic hernia contained a strangulated segment of the splenic flexure of the colon. Resection of the necrotic tissue and transverse colostomy resulted in an uneventful recovery.

Aged↗
PubMed1978

The onset of osteogenesis in the developing chick limb.

The pattern of the onset of osteogenesis in each of the skeletal elements of the developing limbs of the chick embryo is described using Alizarin-red-stained whole mounts. Grafting experiments where 6-day unossified cartilage rudiments are placed into 4-day host wings show the initiation of ossification to be a programmed event. Truncation experiments show this to be the case not only for the initial onset of osteogenesis, but also for each region of each element. These results are discussed in terms of morphogenetic events occurring in the early wing bud.

Animals↗
PubMed1983

Subhyaloid hemorrhage in osteogenesis imperfecta tarda.

The clinical characteristics of osteogenesis imperfecta are the result of a defect in the collagen fibrils. This paper presents a case of osteogenesis imperfecta tarda in which a large subhyaloid hemorrhage occurred. This complication, which has never before been reported in the literature, could be the result of a deficiency of collagen in and around the blood vessels of the eye.

Adult↗
PubMedDate not supplied

[Dynamics of the count of bone marrow cells forming fibroblast colonies in culture and its relation to osteogenesis activity in the reparative regeneration of the lengthened limb].

A comparative analysis of the number of bone marrow cells forming fibroblastic colonies in culture and of the roentgenological characteristics of bone regenerate during different periods of limb elongation was carried out in dogs. The increase in numbers of colony forming cells in the operated bone and decrease of their number in the intact ones were shown to correspond to the period of active osteogenesis. A distinct negative correlation between the number of colony forming cells in humerus and operated bone is considered as an indirect evidence of the migration of these cells from the intact bones. The number of colony forming cells can be used to estimate quantitatively the intensity of osteogenesis upon reparative regeneration under the conditions of limb elongation.

Animals↗
PubMed1983

[Osteogenesis of the rat mandible during development].

The author studied the mandibular osteogenesis process in Wistar rats during extrauterine development using macroscopic and microscopic investigations. Macroscopic investigations were carried out on rats aged 1, 5, 10, 20, 30 and 70 days, for establishing changes in the structure of the mandible and its elements, and autoradiographic determination of the sites of increased osteogenetic activity using Cr-51 chromium was obtained. Microscopic investigations were carried out in rats aged 1 and 5 days. The purpose of these investigations was a study of the morphological structure of the mandible, autoradiographic demonstration of the sites of increased cellular proliferation using 3H thymidine in rats aged 1 day, and osseous matrix formation in rats aged 1 and 5 days using 3H proline. The reported investigations showed that the periosteum and perichondrium were the source of mandibular bone formation. Proliferation of the cartilage of the condylar process and coronoid process occurs from the side of the perichondrium covering both these processes. This type of cartilage growth differs from the growth of the cartilage on the anterior part of mandibular corpus which showed additionally evidence of enchondral proliferation of cells. The process of osteogenesis was most active in the mandibular processes, anterior part of the mandibular corpus and the sites of attachments of muscles. This finding is explained as a result of function influence on the growing mandible. The results of macroscopic autoradiography performed with Cr-51 were confirmed by microscopic examinations.

Aging↗
PubMedDate not supplied

[Bone marrow hematopoietic function and its relation to osteogenesis activity during reparative regeneration in leg lengthening in the dog].

The cellular composition of bone marrow and peripheral blood was studied in different periods of the formation of bone regenerate between the ends of tibia fragments upon their dosed stretching. The intensive regeneration of a hemopoietic rudiment proceeded during the period of active osteogenesis in the elongated bone. A conclusion is drawn that the cellular composition of bone marrow and peripheral blood can be used as indices of the osteogenesis intensity.

Animals↗
PubMed1984

The prenatal ultrasonographic diagnosis of osteogenesis imperfecta lethalis.

The ultrasonographic features of osteogenesis imperfecta lethalis (OI Type II), including those not reported previously (*), are: In the skull: *Abnormal compressibility of the vault by the transducer, which may alter measurements of biparietal diameter. *Unusually good visualization of the orbits. *Increased visualization of arterial pulsations. *Increased through-transmission of the ultra-sound beam due to extremely poor mineralization. In the remainder of the skeleton. *Diminished visualization and increased through-transmission of the remainder of the skeleton, particularly of the spine, ribs and long bones due to defective mineralization. *Abnormally short limbs and poor visualization of the long bones due to defective mineralization. *Abnormally small thorax due to collapse of the rib cage. *Reduced fetal movement. Recognition of fetal fractures and deformities. Prenatal radiographs can be used to confirm the diagnosis and differentiate between osteogenesis imperfecta lethalis and other forms of neonatal dwarfism.

Adult↗
PubMed1982

Osteogenesis imperfecta 1982.

Osteogenesis imperfecta is a highly heterogeneous disorder comprising at least four major clinically discernible conditions. Clinical and genetic observations suggest further heterogeneity. Recent advances in collagen biochemistry suggest heterogeneity of clinically identical conditions and permit a tentative clinical-biochemical classification of osteogenesis imperfecta.

Bone and Bones↗
PubMed1982

Electrical callus formation and its osteogenesis.

Three different electric stimulation methods--direct current, electric Teflon film and PMLG piezoelectric film--could induce electrical osteogenesis around Donryu rat femur. The configuration of each type of electrical osteogenesis was different but the histological findings were essentially the same. 3H-thymidine autoradiographic studies were carried out on rat femurs stimulated by direct current to elucidate some mechanisms of electrical osteogensis. The results disclosed that the electric stimulation affected not only proliferation of osteoprogenitor cells from the periosteal cells but also differentiation of osteoprogenitor cells to osteoblasts and finally to osteocytes.

Animals↗