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[The heart in various connective tissue diseases].

In this study is presented the experience of 294 patients with different connective tissue diseases such as: a) rheumatoid arthritis, b) systemic lupus erythematosus, c) ankylosing spondylitis, d) progressive systemic sclerosis, e) dermatomyositis-polymyositis and f) mixed connective tissue disease. All these patients were studied prospectively during the last eight years through non-invasive methods to detect cardiovascular complications. The connective tissue diseases can provoke inflammation and fibrosis in any of the diverse cardiac structures, with the consequent complications. These alterations are frequent, with a high degree of morbidity, and have a direct relationship with the severity and chronicity of the primary connective tissue disease. Some of these patients respond favorably to cardiological and anti-inflammatory management, while others are refractory to such treatments. The prognosis of the observed complications in these patients depends in great part on the development of the basic rheumatic disease. This kind of patients should be studied systematically from the cardiological view point, so as to detect these complications as early as possible.

Adult↗

[The pathological action of surrounding connective tissues of alar cartilage in the cleft lip nose].

OBJECTIVE: This study was to explore the pathological action of the adjacent connective tissues of alar cartilage in unilateral cleft lip nose. METHODS: Anatomic-histological and pathoanatomical study was carried on 40 normal cadavers and 30 patients with secondary deformities of unilateral cleft lip during their operations. RESULTS: Six kinds of connective tissues with different histological features were found between the alar cartilage and adjacent skin, cartilage or bone. There was no histological difference in each connective tissue between the normal and the cleft lip nose. In the cleft lip nose, due to histological differences among the six connective tissues, there existed various changes of pathological dislocations between the alar cartilage and adjacent skin, cartilage or bone. CONCLUSION: The authors suggest that the surrounding connective tissues of the alar cartilage play an important role and be the intrinsic basis to produce the characteristic deformities of the cleft lip nose. The result is helpful in understanding the anatomical and histological features of the connective tissues around the alar cartilage and the deformities of the unilateral cleft lip nose so as to select a reasonable operation method.

Abnormalities, Multiple↗

[Sharp's syndrome (mixed connective tissue disease). Clinical aspects diagnosis and prognosis].

Sharp syndrome (mixed connective tissue disease) is a distinct rheumatic syndrome with symptoms of various connective tissue diseases (rheumatoid arthritis, systemic lupus erythematodes, progressive systemic sclerosis, polymyositis and others). 15 patients with mixed connective tissue disease are described. The clinical picture and diagnostic criteria are evaluated and the course of the disease, treatment and prognosis are discussed.

Adolescent↗

Connective tissue metabolism in normal and atrophic skeletal muscle.

The first two enzymes of the pentose-phosphate pathway (G6PD and 6PGD) were studied in rabbit skeletal muscle. Their activities closely paralleled the connective tissue levels in each individual muscle, and they increased in atrophying muscles at the same rate as connective tissue. Skeletal muscle tendons and subcutaneously implanted polyvinyl sponges, both of which consist of relatively pure connective tissue, displayed much higher activity of these enzymes than the muscle homogenates. These results suggest that pentose synthesis in muscle is mostly confined to its connective tissue elements, raising the possibility of a role for connective tissue in the biosynthetic processes of skeletal muscle. The contribution of connective tissue to the results of muscle quantitative studies may be significant, and even overshadow other values in atrophic muscles, where the muscle cell population may be greatly reduced. The interpretation of quantitative biochemical studies on pathological muscle samples should take this factor into consideration.

Animals↗

Effect of fixation on the light microscopical visualization of mast cells in the mucosa and connective tissue of the human duodenum.

Mast cells have been studied in the mucosa and connective tissue of 28 biopsies of human duodenum. They were collected from individuals who for various reasons had to undergo upper intestinal endoscopy. Tissues were fixed in both standard formalin and a formalin-acetic acid (FA) fixative developed for visualization of mucosal mast cells. With both fixatives connective tissue mast cells (CTMC) were equally well recognized. Only with the FA fixative, an additional population of mast cells was observed both in the mucosa and the connective tissue. The population consisted of round cells, always smaller than CTMC, with a number of granules staining violet with toluidine blue. These mast cells comprised two cell types, differing in size, the larger being present in both the mucosa and the connective tissue, the smaller ones in a subepithelial position in the crypts of Lieberkühn and in the adjacent submucosa. It was suggested that this first mentioned additional population was similar to the mucosal mast cells (MMC) observed in rats and mice. The subepithelially located metachromatic cells differed in morphology from the globule leukocytes found in rats and mice. It was concluded that in the human duodenum a population of cells with MMC staining characteristics is present, which can only be visualized under special fixation conditions. Their further characterization and role in various disease conditions remains to be elucidated.

Acetates↗

Effects of surface topography on the connective tissue attachment to subcutaneous implants.

PURPOSE: A major concern for implants that penetrate stratified epithelia is aggressive epithelial proliferation and migration. This epithelial downgrowth on the implant can be inhibited by a firm attachment between the underlying connective tissue and the implant. This study evaluates the connective tissue attachment to titanium implants with various well-defined surface topographies. MATERIALS AND METHODS: Titanium-coated epoxy replicas of polished (PO; R(a) = 0.06 microm), finely blasted (FB; R(a) = 1.36 microm), coarsely blasted (CB; R(a) = 5.09 microm), acid-etched (AE; R(a) = 0.59 microm), coarsely blasted and acid-etched (SLA; R(a) = 4.39 microm), titanium plasma-sprayed (TPS; R(a) = 5.85 microm), machined-like (ML; R(a) = 2.15 microm), and micromachined grooved (GR; V-shaped grooves 30 microm deep) surfaces were implanted subcutaneously in 74 rats for 1 to 11 weeks. Animals were sacrificed weekly. Surfaces were processed for histomorphometric evaluation of connective tissue attachment, capsule thickness, and where applicable, the degree of separation between the tissue and implant. RESULTS: A total of 153 test surfaces were analyzed. Statistical analysis revealed that textured and rough substrata, namely the GR, TPS, AE, CB, and SLA surfaces, exhibited significantly greater (P < .05) connective tissue attachment and thinner fibrous encapsulation when compared to the PO surface. Tissue separation from the implant interface was of significantly lower magnitude and frequency with the rough surfaces than with the PO surface. CONCLUSIONS: The results indicate that rough implant surfaces are associated with stable connective tissue attachment, which has implications for their use in percutaneous and permucosal applications. In addition, data from the AE surface may indicate that the geometry of the surface irregularities can also be a significant determinant of the connective tissue response.

Animals↗

Mechanical properties and hydroxyproline content of connective tissue in porous ceramic implants.

The present study describes a model applying ceramic implants (A1203) for in vivo studies of connective tissue regeneration. Two types of implants have been developed: a one-piece model for histological examination and chemical analyses, and a two-piece implant which can also be used for mechanical testing of connective tissue. When these were implanted subcutaneoulsly on the back of rats, a correlation was found between the mechanical strength and the hydroxyproline content of connective tissue in the implants. The peak synthesis occurred between the 7th and the 14th day after implantation, and a plateau was reached for both strength-increases and hydroxyproline-formation between the 14th and the 21st days. For histological examination, the implants were embedded in plastic materials and prepared as hard tissue specimens. The model presented can be applied to study connective tissue regeneration in normal and pathological conditions, including studies of the effects of various drugs on the connective tissue.

Aluminum Oxide↗

The interaction of Kaposi's sarcoma with monoclonal antibodies to human sarcoma and connective tissue differentiation antigens.

Four monoclonal antibodies (McAbs) previously generated against human soft tissue sarcomas and reacting with connective tissue differentiation antigens were evaluated for their interaction with tissues obtained from patients with classic Kaposi's sarcoma. Biopsy was performed on active neoplastic lesions from the skin of 26 patients, frozen sections were prepared, and the binding of the McAbs was tested using the indirect immunofluorescence assay. Clinically uninvolved skin from the same patients as well as skin and muscle from eight non-cancer patients were treated similarly and served as controls. McAbs IXG11, 23H7, IIIE5, and 15G5 interacted strongly with the Kaposi's sarcoma lesions and weakly with the uninvolved skin in 22 of 26 (84%), 23 of 26 (88%), 12 of 14 (85%), and 1 of 6 (16%) of the patients, respectively. IXG11, 23H7, and IIIE5 interacted weakly with the skin of seven of eight non-cancer patients. McAb 15G5 was found to bind strongly to tumor lesions, to the respective uninvolved skin in four of five Kaposi's sarcoma patients, and also to skin and connective tissues of muscle from non-cancer patients. The mode of interaction was morphologically different for each McAb. It is suggested that McAbs IXG11, 23H7 and IIIE5 identify markers whose expression is markedly increased in Kaposi's sarcoma lesions as compared with uninvolved skin of the same patients. These markers may serve as immunologic probes for the investigation of this neoplastic process.

Adult↗

Nipple connective tissue and its development: insights from the K14-PTHrP mouse.

Parathyroid hormone-related protein (PTHrP) regulates a wide variety of developmental processes. Keratin 14 (K14) promoter-mediated overexpression of PTHrP in the epidermis during development converts the entire murine ventral skin to hairless nipple-like skin. In this report, we characterize the morphology and processes that influence the development of nipple connective tissue. The connective tissue of the nipple displayed increased levels of proteoglycans, and collagen bundles with atypical morphology, as well as increased numbers of mast cells, capillaries, nerve fibers and dermal melanocytes. The unique characteristics of nipple connective tissue were not present until mice reach 3-4 weeks of age. The adult male K14-PTHrP mouse has a less dramatic ventral skin phenotype, and does not manifest a nipple-like dermis. Ovariectomy or orchiedectomy prior to sexual maturity had no impact on the ventral skin of the male or female K14-PTHrP mice, but exposure to androgens in utero repressed many of the nipple-like characteristics in the ventral skin of the female K14-PTHrP mice.

Animals↗

Pressure-induced connective tissue synthesis in pulmonary artery segments is dependent on intact endothelium.

Physiologic stimuli of connective tissue accumulation in pulmonary vascular remodeling are poorly defined. We postulated that increased pressure within central pulmonary arteries is a stimulus for connective tissue synthesis and the response is dependent on an intact endothelium. Mechanical tension equivalent to 50 mmHg pressure was applied for 4 h to isolated rat main pulmonary arteries (endothelium intact or removed), and incorporation of [14C]proline into collagen, [14C]valine into elastin, [3H]thymidine into DNA and pro alpha 1 (I) collagen mRNA levels were measured. In intact vessels, tension induced synthesis of collagen (3.1 +/- 0.4 vs. 2.3 +/- 0.5 [SEM] dpm X 10(2) [14C]-hydroxyproline/[mg protein.h]) (n = 10) and elastin (6.1 +/- 2.4 vs. 2.9 +/- 0.4 dpm X 10(3) [14C]valine/[mg protein.h]) (n = 5) (both P less than 0.05). Steady state mRNA levels of pro alpha 1 (I) collagen were also increased by tension (46 vs. 30 X 10(2) dpm hybridized/100 ng total RNA). However, the stimulus did not increase [3H]thymidine incorporation into DNA. In denuded vessels, tension had no effect on connective tissue synthesis or mRNA level of pro alpha 1 (I) collagen. Messenger RNA levels for v-sis were induced by tension in intact but not denuded vessels. Our findings establish that induction of vascular connective tissue synthesis by mechanical tension is dependent on an intact endothelium.

Actins↗

Connective tissue disease: an approach.

The connective tissue diseases are a poorly defined group of conditions characterised by abnormal activity of the immune system. This article will not detail the features of any one condition, but rather attempt to provide a system for the evaluation and classification overall and thus hopefully enable the reader to approach these complex conditions with greater confidence.

Connective Tissue Diseases↗

[Myofibroblasts in connective tissue of the human amnion].

Connective tissue of the human amnion at 14, 19, 21 and 33 weeks of gestation and at full term was examined by electron microscopy. Myofibroblasts which have not been described in the amnion so far were identified in addition to fibroblasts and macrophages (Hofbauer cells). Myofibroblasts display the following morphological characteristics: 1. The nuclei have multiple indentations and deep folds. 2. Numerous bundls of filaments (5 nm and 10 nm in diameter) are present within the cytoplasm. The bundles of 5 nm-filaments contain dense bodies. 3. Frequently they are connected to each other by desmosomes or by gap junctions. 4. The plasma membrane shows many hemidesmosome-like zones of thickening. 5. The cell surface is partly covered by basement membrane. Since these cells possess the ability of contraction, we suggest that they may contribute to the protection of the amniontic membrane from overdistension.

Amnion↗

[Vitamins K and E and the functional state of connective tissue].

The function of the connective tissue in the skin of albino rats with the vitamin K deficiency was studied. Changes both in the cellular elements and in the fibrous structures (deranged differentiation of cellular elements, changes in the collagenformation, accumulation of acid mucopolysaccarides, etc.) were revealed. All of the mentioned changes are eliminated not only by introduction of vicasol, but also by that of alpha-tocopherol.

Animals↗

[Hypophosphoremic osteomalacia of connective tissue tumors].

The authors undertake a general review of the association between hypophosphoraemia and connective tissue tumour, based upon three personal cases and 27 cases of benign connective tissue tumours, as well as cases of hypophosphoraemia related to malignant tumours or to diffuse dysplasia of connective tissue origin, collected from the literature. This syndrome is distinguished from hypophosphoraemia induced by other tumours (myeloma, carcinoma of the prostate) which are based upon different mechanisms. Hypophosphoraemia, associated with a fall in plasma levels of 1-25 (OH)2 D3 by inhibition of renal 1 alpha hydroxylase, suggests the existence of a complex tubular deficit. Removal of the tumour, most often vascular and intra- or para-osseous, results in rapid normalisation of laboratory then radiological and clinical abnormalities. The physiopathology of the syndrome remains very mysterious. It may be likened to certain tubulotoxic syndromes due to cadmium and in particular to maleic acid. However no precise data yet exists regarding any possible abnormal tumour secretion. In practice, any case of hypophosphoraemic osteomalacia requires investigation to locate a possible tumour of connective tissue, and this all the more so when it is accompanied by very low plasma levels of 1-25(OH)2 D2.

Adolescent↗

Toward a kinetic theory of connective tissue micromechanics.

The aim of this study is to develop unifying concepts at the microstructural level to account for macroscopic connective tissue dynamics. We establish the hypothesis that rate-dependent and rate-independent dissipative stresses arise in the interaction among fibers in the connective tissue matrix. A quantitative theoretical analysis is specified in terms of geometry and material properties of connective tissue fibers and surrounding constituents. The analysis leads to the notion of slip and diffusion boundary layers, which become unifying concepts in understanding mechanisms that underlie connective tissue elasticity and energy dissipation during various types of loading. The complex three-dimensional fiber network is simplified to the interaction of two ideally elastic fibers that dissipate energy on slipping interface surfaces. The effects of such interactions are assumed to be expressed in the aggregate matrix. Special solutions of the field equations are obtained analytically, whereas the general solution of the model field equations is obtained numerically. The solutions lead to predictions of tissue behavior that are qualitatively, if not quantitatively, consistent with reports of a variety of dynamic moduli, their dependencies on the rate and amplitude of load application, and some features associated with preconditioning.

Connective Tissue↗

[Sharp's syndrome: a new entity amongst the connective tissue diseases?].

Sharp and coworkers have coined the name "Mixed connective tissue disease" to an association of symptoms seen in different connective tissue diseases. Those symptoms are associated with a special antibody directed against ribonucleoproteins (RNA-P). The presence of this antibody accounts for a high titer of antinuclear fluorescent antibody, of the speckled type. The most frequents symptoms in the association described by Sharp are Raynaud's phenomenon, swollen fingers, non deforming arthritis. We have observed those symptoms without any further symptoms of connective tissue disease, but with the presence of anti-RNA-P antibody. We suggest to coin the name of Sharp's syndrome to that clinical association. The significance of anti-RNA-P, and their correlation with a remarkable benignity of the disease, is discussed.

Antibodies, Antinuclear↗

Respiratory manifestations of connective tissue disease.

Pleuropulmonary disease is a common feature of the following connective tissue diseases: systemic lupus erythematosis (SLE), rheumatoid arthritis (RA), progressive systemic sclerosis (PSS), mixed connective tissue disease (MCTD), polymyositis/dermatomyositis (PM/DM), and Sjogren's syndrome (SS). Features common to most of these disorders include pleurisy with effusion and interstitial lung disease. Pleural effusions caused by SLE and RA have certain characteristics on pleural fluid analysis that aid in diagnosis, but infection and other causes of effusion must be excluded. Interstitial lung involvement is usually indolent in onset, but a more rapidly progressive course over weeks to a few months may mimic infection. Several drugs used to treat connective tissue diseases may cause interstitial disease, increase susceptibility to infection, or both. This complicates differential diagnosis. Acute lupus pneumonitis and SLE-related alveolar hemorrhage are usually fulminant processes, often associated with fever. Diagnosis of these conditions always requires exclusion of infection. Rheumatoid nodules may mimic infectious and neoplastic lung diseases. Needle biopsy helps reduce the likelihood of infection or malignancy, but open lung biopsy is needed if a firm diagnosis of rheumatoid nodules is required.

Arthritis, Rheumatoid↗

Esophageal motor abnormalities in children and adolescents with scleroderma and mixed connective tissue disease.

To determine the frequency and nature of esophageal motor abnormalities in children and adolescents with scleroderma syndromes and mixed connective tissue disease, esophageal manometry was performed on seven patients with progressive systemic sclerosis, four patients with mixed connective tissue disease, and two patients with linear scleroderma. A total of 73% of patients with progressive systemic sclerosis and mixed connective tissue disease had symptoms of esophageal dysfunction. A significant association between the presence of Raynaud phenomenon and esophageal symptoms was noted. Esophageal motor abnormalities were detected in 73% of patients with progressive systemic sclerosis and mixed connective tissue disease; these abnormalities were characterized by decreased lower esophageal sphincter pressure and abnormal peristalsis in the distal two thirds of the esophageal body. They resemble those described among adults with progressive systemic sclerosis and mixed connective tissue disease but were not related to disease duration or to the presence of Raynaud phenomenon. Patients with linear scleroderma did not have esophageal symptoms and demonstrated only nonspecific motor abnormalities that did not worsen during several years of follow-up.

Adolescent↗