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Biomedical subjects

V Stanescu

Publications and source records attributed to V Stanescu.

At least 55 records · Page 3Linked to original sources
PubMed1984

Opsismodysplasia: a new type of chondrodysplasia with predominant involvement of the bones of the hand and the vertebrae.

The name opsismodysplasia is proposed for a new chondrodysplasia, which was studied in three patients. Clinically, the condition is recognized at birth on the basis of shortness, short hands, and facial abnormalities with a short nose and a depressed bridge of nose. The most characteristic radiographic signs are: very retarded bone maturation; marked shortness of the bones of the hands and of the feet with concave metaphyses; and thin, lamellar vertebral bodies. The growth cartilage studied in one case showed a wide hypertrophic area containing thick connective tissue septa, irregular provisional calcification, and vascular invasion. Type I collagen was detected in the hypertrophic area by immunohistochemical and microchemical tests. The transmission of opsismodysplasia is probably autosomal recessive.

Abnormalities, Multiple↗
PubMed1984

Labeling of articular cartilage surface with cationized ferritin: aged human normal and osteoarthritic cartilage.

The labeling of the articular surface with cationized ferritin (CF), an electron-dense marker, visualizes the anionic sites and may disclose abnormal penetration of the large CF molecule into the subsurface layers. Various areas of cartilage selected by unaided eye examination were taken from femoral heads excised in three cases of osteoarthritis and two cases of hip fracture. The fragments were examined by optical microscopy and by electron microscopy after labeling with CF. The labeling with and the penetration of CF were correlated with the morphological features of the surface. The surfaces belonging to the erosion border were disrupted and the CF penetrated approximately 2 microns into the matrix along the collagen fibers and in areas containing a patchy dense material. Prefixation with Karnovsky's fixative prevents CF penetration. The fragments taken at a distance from the erosion border showed at electron microscopical examination either an intact appearance of the surface that was labeled without penetration or a disrupted surface with penetration of the label. The osteophytes and the regeneration buds surface were labeled showing little or no penetration. The fragments from cartilage of hip fractures had either an intact surface regularly labeled or a slightly or moderately disrupted surface with moderate penetration of CF. The penetration of large molecules of CF in damaged cartilage demonstrates important permeability changes that may be significant for the pathogenetic mechanism of osteoarthritis. Similar permeability changes were previously shown in mice femoral heads treated in vitro with collagenase or trypsin and labeled with CF.

Age Factors↗
PubMed1984

Pathogenic mechanisms in osteochondrodysplasias.

UNLABELLED: We performed histochemical, immunohistochemical, electron-microscopic, and microchemical studies on cartilage growth plates from sixty-eight patients with nineteen different forms of human osteochondrodysplasia. Cartilage biopsies were obtained during orthopaedic procedures. Postmortem specimens were obtained within a short time after death. The combined morphological and biochemical studies revealed specific abnormalities suggestive of a particular biochemical defect in several chondrodysplasias. In pseudoachondroplasia, non-collagenous protein accumulated in the rough endoplasmic reticulum of chondrocytes and a proteoglycan species that normally is present in the extracellular matrix was not detected by gel electrophoresis. The accumulated material was stained with antibodies against the core protein of proteoglycan. This strongly suggested that in this syndrome an abnormal core protein of a proteoglycan species is not properly transferred to the Golgi system. In Kniest syndrome, intracytoplasmic accumulation of metachromatic material, dilatation of rough endoplasmic reticulum, and an abnormal gel-electrophoretic pattern of cartilage proteoglycans suggested an abnormality of cartilage proteoglycan metabolism. Abnormalities that probably are related to degradative lysosomal processes of proteoglycans in chondrocytes were found in spondylometaphyseal dysplasia of the Kozlowski type. An abnormal organization of type-II collagen was found in fibrochondrogenesis. In diastrophic dysplasia, an abnormal organization of collagen was found in areas of interterritorial matrix and around many degenerated cells, but also in the lacunae of cells without ultrastructural signs of degeneration. The segment-long-spacing form of collagen prepared from cartilage of three patients with diastrophic dysplasia showed an abnormal cross-striation pattern in a portion between bands 42 and 45, corresponding to the position of the alpha 1(II) cyanogen-bromide-derived 10,5 peptide. This suggested that in this syndrome there is a structural alteration of the type-II collagen molecule. There was an accumulation of intracellular lipid in pyknodysostosis and in hypochondrogenesis, and of glycoproteins in several atypical cases of spondyloepiphyseal dysplasia. In a pair of twins with an atypical form of spondyloepiphyseal dysplasia, the presence of many multinucleated chondrocytes suggested a primary impairment of cell division. CLINICAL RELEVANCE: A knowledge of the pathogenic mechanisms in osteochondrodysplasias might improve the classification; aid in diagnosis, prognosis, and genetic counseling; and contribute to the understanding of normal endochondral growth.(ABSTRACT TRUNCATED AT 400 WORDS)

Achondroplasia↗
PubMed1984

[Spondyloepiphyseal dysplasia with an accumulation of glycoproteins in chondrocytes].

A case presenting a peculiar type of spondylo-epiphyseal dysplasia was studied. Clinically, the normal height was striking. The X-rays showed large epiphysis and wide metaphysis. Bilateral coxa valga with very large femoral necks was present. The height of the vertebral bodies was slightly reduced. The study of the upper tibial growth cartilage showed glycoprotein inclusions in the chondrocytes and large dilatations of the rough endoplasmic reticulum. The gel electrophoresis of the non collagenous proteins extracted from the growth cartilage had an abnormal densitometric tracing. The type of inheritance of this syndrome is unknown, the patient being an isolated case in a normal family.

Adolescent↗
PubMed1983

The distribution of proteoglycans of high electrophoretic mobility in cartilages from different species and of different ages.

The distribution of small proteoglycans of high relative electrophoretic mobility in cartilage of various species and of different ages was studied. Proteoglycans extracted by 4 M guanidinium chloride were purified by ion-exchange chromatography and assessed by gel electrophoresis. Proteoglycans fractionated by equilibrium density gradient centrifugation under 'dissociative' conditions were similarly purified and assessed. A rapid migrating population was found in articular cartilages of young humans, baboons, calves, pigs, rabbits, rats, chickens and in mandibular and vertebral cartilages of dog-fish. It was not detected in unfractionated proteoglycans extracted from fetal rat, pig, calf, baboon and human cartilages. In baboon and human fetal cartilages of advanced gestational age, however, small amounts of the rapid population were present being detected in the low density fractions of dissociative gradients. The rapid migrating population was not found either in unfractionated or in fractionated proteoglycans obtained from articular cartilages of humans aged over 40. It was also absent from human osteoarthritic cartilages but was detected even at advanced age in cartilages covering osteophytes.

Adult↗
PubMed1983

Hypochondrogenesis.

Three clinicopathological observations of a mild form of type II achondrogenesis are presented. The cases were selected from a group of 21 similar cases to illustrate the various degrees of clinical and roentgenological signs that can be found. The cases had various survival periods after birth but not exceeding several months. The roentgenological signs were less severe than those of type II achondrogenesis. Some cases similar to case no. 3 have roentgenological signs very close to spondylo-epiphyseal dysplasia congenita and probably were confused previously with the latter. The name of hypochondrogenesis was proposed for these cases because the lesions of the growth plate are similar although less marked to those found in type II achondrogenesis: high cellularity with poor matrix development; irregular columnization and vascular penetration; large chondrocytes and even more enlarged lacunae; large sclerotic cartilage canals. The clinical and roentgenological diagnosis of hypochondrogenesis could be difficult especially in the less severe forms. The delay in vertebral ossification, the absence of all the epiphyseal nuclei and of the tarsal bones might suggest the diagnosis of hypochondrogenesis, rather than that of spondyloepiphyseal dysplasia. The evolution which seems to be always lethal in a period of several weeks or months would make the diagnosis still more likely and it could be confirmed by histopathological examination. Cases of spondylo-epiphyseal dysplasia congenita might have at birth, roentgenological signs indistinguishable from those of hypochondrogenesis, as was illustrated by case no. 4.(ABSTRACT TRUNCATED AT 250 WORDS)

Achondroplasia↗
PubMed1982

Atelosteogenesis.

The name atelosteogenesis is proposed for a lethal chondrodysplasia characterized by deficient ossification of various bones, notably the humerus, femur, thoracic spine, and hand bones. Clinically, the patients have micromelic dwarfism with incurvated legs, club feet, often dislocation of the elbows, and, rarely, a cleft palate. The most characteristic radiographic signs are incomplete ossification of the vertebral bodies with coronal clefts of the lumbar and hypoplasia of the upper thoracic vertebral bodies, a distal hypoplasia and club shape of the humerus and the femur, and the lack of ossification of single phalanges and metacarpals in most patients. Histologically, there are clusters of chondrocytes surrounded by fibrous capsules and, more frequently, degeneration zones containing degenerated chondrocytes and copious amounts of metachromatic material in the epiphyses and the basal zone of the growth plate.

Abnormalities, Multiple↗
PubMed1982

Noncollagenous proteins in cartilage of normal subjects and patients with degenerative joint disease. A gel electrophoretic study.

Normal articular cartilage from subjects of various ages and cartilage from patients with degenerative joint disease were extracted with 4M guanidinium chloride. After dialysis against 8M urea pH 6.8, a 0.2M NaCl fraction was obtained by ion exchange chromatography on DE-52 in 8M urea. This fraction was concentrated, reduced, and analyzed by sodium dodecyl sulfate--polyacrylamide gel electrophoresis (7% gels). Six major noncollagenous protein bands (P1-P6) were found; 2 were identified as the link proteins. The approximate molecular weights of P1-P6 were: 87,000, 64,000, 56,000, 46,000, 41,000, and 27,000. A similar sodium dodecyl sulfate-polyacrylamide gel electrophoresis pattern of P1-P6 was found in young baboons, in normal young and aged humans, and in patients with degenerative joint disease. Peaks corresponding to extracted collagen were decreased in older patients and increased in patients with degenerative joint disease, even those of advanced age.

Adolescent↗
PubMed1982

[Heterogeneity of formes frustes of Morquio's disease].

Two children presenting with a mild form of Morquio's syndrome are reported. Clinically, there was a characteristic brevity of the trunk and slit lamp examination showed discrete corneal opacities. On X-ray films, generalized plastyspondylia was moderate but it was associated with hypoplasia of the odontoid process. Acetabula were enlarged with coxa valga; obliquity of inferior radio-cubital extremity was associated with a sharp pattern of the proximal end of metacarpi. Epiphyseal cartilage chondrocytes also looked like those of Morquio's syndrome: large cells containing numerous vacuoles, limited by a single smooth membrane. On the other hand, no keratosulfate was found in urines and N-acetylgalactosamine-6-sulfate-sulfatase and beta-galactosidase assays in fibroblasts were normal. Thus, this mild form is different from the so-called Morquio's syndromes types A and B.

Child↗
PubMed1981

Characterization of a proteoglycan of high electrophoretic mobility.

Proteoglycans from articular cartilage of young baboons (Papio papio) were fractionated on an associative density gradient. Material from the top of the gradient was shown to contain a proteoglycan of high electrophoretic mobility on large porosity gels. Associated and/or contaminating proteins were removed by ion-exchange chromatography on DEAE-cellulose and subsequent gel filtration on Sepharose 4B in the presence of 0.1% SDS. The electrophoretically homogeneous proteoglycan (Kd 0.43 on Sepharose 4B SDS) contained 39.7% protein, was rich in aspartate, glutamate, leucine and glycine and had a GalN : GluN molar ratio of 3.87.

Amino Acids↗
PubMed1981

[A special form of dominant spondylo-epiphyseal dysplasia (author's transl)]].

A special form of dominant spondylo-epiphyseal dysplasia has been observed in a 3 year-old boy and in his father. The roentgenological examination showed alterations involving chiefly the upper femoral extremities and the spine. This might suggest the diagnosis of spondylo-epiphyseal dysplasia congenita. However the clinical features were different. The alterations of the growth plate were also different from those found in typical forms of spondylo-epiphyseal dysplasia congenita. Large dilatations of the rough endoplasmic reticulum of chondrocytes are present in both syndromes but their content had different histochemical features. The cartilage alterations were also different from those found in the dominant form of poly-epiphyseal dysplasia as well as in pseudo-achondroplasia.

Adult↗
PubMed1980

Proteoglycan populations of baboon (Papio papio) cartilages from different anatomical sites: gel electrophoretic analysis of dissociated proteoglycans and of fractions obtained by density gradient centrifugation.

Proteoglycans extracted by 4 M guanidinium chloride from different cartilages and vertebral discs of young baboons (Papio papio) were purified by ion-exchange chromatography and assessed by gel electrophoresis. Proteoglycans fractionated by equilibrium density gradient centrifugation under 'dissociative' conditions were similarly purified and assessed. (1) Non-fractionated proteoglycans from articular, nasal, laryngeal, tracheal, costal, vertebral plate and ear cartilage and from anulus fibrosus gave three metachromatic bands on gel electrophoresis: two broad, closely running bands (I and II) and a faster, more discrete band (III). The migrations rates of corresponding bands of various cartilages were similar, with minor differences in staining intensity. Gel electrophoresis of material fractionated on the gradient indicated the presence of bands with the same migration and appearance as those found for the unfractionated proteoglycans and also partial separation of bands (buoyant density I greater than II greater than III). (2) Non-fractionated proteoglycans from growth cartillage yielded bands I and II only. Small amounts of material corresponding to band III were detected in the upper fractions of the gradients. (3) Proteoglycans from nucleus pulposus migrated as two diffuse bands corresponding to bands I and II. The two bands were partially separated in the gradients and an additional very slow band was formed in the middle fractions of the gradients. (4) Proteoglycans from knee menisci contained a densely stained band of corresponding mobility to band III. Faint bands corresponding to band I and II were also detected. The bands were partially separated in the gradients.

Animals↗