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125421. Development of the coronary vessel system. (Abstract)

Development of the coronary vessel system. Formation of the coronary vessels is a fundamental event in heart development. Congenital abnormalities in the coronary system can have major deleterious effects on heart function. It is also possible that subtle variation in the patterning of coronary vessels has significant but uncharacterized effects on myocardial structure and function. In addition, generation of the coronary vascular system represents a complex system for analysis of regulation (...) of cell fate determination, cell and epithelial migration, epithelial/mesenchymal transition, and patterning of a complex three-dimensional structure. In this review, we present the descriptive embryology of this process as well as the recent data that shed light on the unique developmental mechanisms underlying generation of coronary vessels. This review also attempts to identify areas where additional research is needed and highlights the questions that must be answered for a meaningful

2002 Circulation Research

125422. Circadian rhythm in the cardiovascular system: chronocardiology. (Abstract)

Circadian rhythm in the cardiovascular system: chronocardiology. We reviewed recent progress in the study of the chronobiological aspects of the cardiovascular system.Medline was used as the main search tool, and the full texts of selected papers were obtained.More than 300 references were found, and 52 of them, representing the major findings in this field, were included in the reference list. Results of these studies confirm that most cardiovascular physiological parameters (such as heart (...) rate, blood pressure, electrocardiogram indices) and pathophysiological events (myocardial ischemia/infarction, sudden cardiac death) show circadian rhythms. Results also suggest that consideration of these rhythms is important for the diagnosis and treatment of cardiovascular disorders and that restoration of normal circadian rhythms may be associated with clinical improvement.The study of circadian rhythms in the cardiovascular system is emerging as an important area of investigation because

2003 American Heart Journal

125423. Novel therapeutic agents for systemic lupus erythematosus. (Abstract)

of these approaches will be discussed in this review. Some therapies are currently in use in clinical rheumatology practice (mycophenolate mofetil) and others are entering phase I trials (anti-BLyS monoclonal antibody). While some of these new therapies target specific inflammatory mechanisms in SLE (anti-CD40L monoclonal antibody), others work by nonspecific inhibition of the immune system (immunoablation). (...) Novel therapeutic agents for systemic lupus erythematosus. The last significant breakthrough in the treatment of systemic lupus erythematosus (SLE) was the use of cyclophosphamide and methylprednisolone in the treatment of lupus nephritis. Recent advances in immunology, oncology, and endocrinology have resulted in many potential therapies for SLE. These therapies include new immunosuppressants, biologic medications, tolerizing agents, immunoablation techniques, and hormonal medications. Each

2002 Current Opinion in Rheumatology

125424. The role of infectious agents in the pathogenesis of systemic sclerosis. (Abstract)

The role of infectious agents in the pathogenesis of systemic sclerosis. Over the past few years, increasing evidence has accumulated to implicate infectious agents in the etiology of systemic sclerosis (SSc) and Raynaud phenomenon. Infection rates in patients with SSc compared with those in control populations do not provide clear support for any specific pathogen. However, increased antibody titers, a preponderance of specific strains in patients with SSc, and evidence of molecular mimicry (...) inducing autoimmune responses suggest mechanisms by which infectious agents may contribute to the development and progression of SSc. Here we review studies examining the potential involvement of, cytomegalovirus, and parvovirus B19 in SSc pathogenesis.

2002 Current Opinion in Rheumatology

125425. The expanding spectrum of systemic autoinflammatory disorders and their rheumatic manifestations. (Abstract)

The expanding spectrum of systemic autoinflammatory disorders and their rheumatic manifestations. The authors review the genes, and their respective proteins, responsible for eight autoinflammatory conditions. Familial Mediterranean fever is caused by mutations in pyrin, which is the prototype of a new family of proteins belonging to the death-domain superfamily. This new group of proteins, which regulate apoptosis, inflammation, and cytokine processing, share an approximately 90-amino-acid N

2003 Current Opinion in Rheumatology

125426. Immune dysregulation, polyendocrinopathy, enteropathy, and X-linked inheritance (IPEX), a syndrome of systemic autoimmunity caused by mutations of FOXP3, a critical regulator of T-cell homeostasis. (Abstract)

Immune dysregulation, polyendocrinopathy, enteropathy, and X-linked inheritance (IPEX), a syndrome of systemic autoimmunity caused by mutations of FOXP3, a critical regulator of T-cell homeostasis. Immune dysregulation, polyendocrinopathy, enteropathy, and X-linked inheritance (IPEX) is one of a group of clinical syndromes that present with multisystem autoimmune disease suggesting a phenotype of immune dysregulation. Clinically, IPEX manifests most commonly with diarrhea, insulin-dependent (...) diabetes mellitus, thyroid disorders, and eczema. FOXP3, the gene responsible for IPEX, maps to chromosome Xp11.23-Xq13.3 and encodes a putative DNA-binding protein of the forkhead family. Recent data indicate that FOXP3 is expressed primarily in the CD4+CD25+ regulatory T-cell subset, where it may function as a transcriptional repressor and key modulator of regulatory T-cell fate and function. This review describes the clinical features of IPEX and the structure, function, and known mutations of FOXP3

2003 Current Opinion in Rheumatology

125427. A comparison between anti-Th/To- and anticentromere antibody-positive systemic sclerosis patients with limited cutaneous involvement. (Abstract)

A comparison between anti-Th/To- and anticentromere antibody-positive systemic sclerosis patients with limited cutaneous involvement. To compare anti-Th/To-positive and anticentromere antibody (ACA)-positive patients with limited cutaneous systemic sclerosis (lcSSc).We reviewed the medical records of 107 anti-Th/To-positive patients and 365 ACA-positive patients who were first evaluated during 1985-2000. ACA was detected by indirect immunofluorescence on HEp-2 cell substrate, and anti-Th

2003 Arthritis and Rheumatism

125428. Interim mortality in infants with systemic-to-pulmonary artery shunts. (Abstract)

Interim mortality in infants with systemic-to-pulmonary artery shunts. This retrospective review examines the risks and causes of death in infants with aortopulmonary shunts between the time of hospital discharge and planned reintervention.From January 1991 through December 2000, a total of 146 infants aged 60 days or less underwent placement of systemic-to-pulmonary artery shunts and were discharged from the hospital alive. Inpatient, outpatient, and autopsy records were reviewed.Indications

2003 Annals of Thoracic Surgery

125429. Epidermal growth factor receptors and cyclooxygenase-2 in the pathogenesis of non-small cell lung cancer: potential targets for chemoprevention and systemic therapy. (Abstract)

Epidermal growth factor receptors and cyclooxygenase-2 in the pathogenesis of non-small cell lung cancer: potential targets for chemoprevention and systemic therapy. The epidermal growth factor receptor (EGFR) is part of a family of plasma membrane receptor tyrosine kinases that control many important cellular functions, from growth and proliferation to cell death. Cyclooxygenase (COX)-2 is an enzyme which catalyses the conversion of arachidonic acid to prostagladins and thromboxane (...) . It is induced by various inflammatory stimuli, including the pro-inflammatory cytokines, Interleukin (IL)-1beta, Tumour Necrosis Factor (TNF)-alpha and IL-2. Both EGFR and COX-2 are over-expressed in non-small cell lung cancer (NSCLC) and have been implicated in the early stages of tumourigenesis. This paper considers their roles in the development and progression of lung cancer, their potential interactions, and reviews the recent progress in cancer therapies that are directed toward these targets

2003 Lung Cancer

125430. Outcome of children with prenatally diagnosed central nervous system malformations. (Abstract)

Outcome of children with prenatally diagnosed central nervous system malformations. To study the outcome of pregnancies with a prenatally diagnosed central nervous system (CNS) malformation.Leiden University Medical Centre is a tertiary referral center for fetal ultrasound and invasive prenatal diagnosis. Maternal and neonatal records of prenatally diagnosed CNS malformations were retrospectively reviewed over a 6-year period (1993-1998). Information on current development of surviving children

2003 Ultrasound in Obstetrics and Gynecology

125431. Comparative biology of the IGF system in endometrium, decidua, and placenta, and clinical implications for foetal growth and implantation disorders. (Abstract)

in placental and foetal development. This paper reviews the diversity in the expression and regulation of IGF system in the decidua and placenta at the foetal-maternal interface in the human and different animal species, which may benefit in directing future studies in understanding of various complications of human pregnancy. (...) Comparative biology of the IGF system in endometrium, decidua, and placenta, and clinical implications for foetal growth and implantation disorders. The insulin like growth factors and their binding proteins appear to play a central role during implantation and establishment of pregnancy in all species studied. Although there are similarities among species in the cell types that express IGFs and IGFBPs and their regulation during implantation and pregnancy, there are also significant

2003 Placenta

125432. Chronic obstructive pulmonary disease. 5: systemic effects of COPD. Full Text available with Trip Pro

Chronic obstructive pulmonary disease. 5: systemic effects of COPD. The role of body cell mass wasting, muscle wasting, and changes in muscle metabolism in the pathogenesis of chronic obstructive pulmonary disease is reviewed.

2002 Thorax

125433. Fibroblast and endothelial apoptosis in systemic sclerosis. (Abstract)

to the vascular endothelium and defective apoptosis of skin fibroblasts may also contribute to disease. The purpose of this review is to discuss these findings in the context of the pathophysiology of systemic sclerosis.This review highlights concepts and recent findings relating to apoptosis of vascular endothelium and skin fibroblasts. Important paradigms of fibroblast cell death in wound healing and keloid formation are discussed. Recent observations describing resistance of systemic sclerosis fibroblasts (...) Fibroblast and endothelial apoptosis in systemic sclerosis. Systemic sclerosis is a disease characterized by vascular and skin changes associated with activation of fibroblasts and increased synthesis of matrix components. These abnormalities lead to fibrosis and impaired function of internal organs such as the lung, kidney, and gastrointestinal tract. Recent evidence suggests that although activation of cells in and around the blood vessels and in the skin occurs in systemic sclerosis, injury

2003 Current Opinion in Rheumatology

125434. Evaluation and management of pulmonary hypertension in systemic sclerosis. (Abstract)

Evaluation and management of pulmonary hypertension in systemic sclerosis. This review summarizes the most recent studies on pulmonary hypertension related to systemic sclerosis, emphasizing new insights into pathophysiology, identification of new markers, and advances in the diagnosis and treatment of this complication that greatly worsens the prognosis of patients with systemic sclerosis.

2003 Current Opinion in Rheumatology

125435. Update on vascular disease in systemic lupus erythematosus. (Abstract)

atherogenesis in the general population will provide clues to unraveling the complexity of why systemic lupus erythematosus and atherosclerosis are linked so frequently.Inflammation is involved in all aspects of atherogenesis from the initial endothelial "response to injury," to foam cell formation leading to the atherosclerotic lesion, to the rupture of the "vulnerable" fibrous cap, resulting in the acute coronary syndrome and potentially in death. The authors review how factors commonly seen in systemic (...) Update on vascular disease in systemic lupus erythematosus. Young women with systemic lupus erythematosus have strikingly high rates of coronary heart disease. Current knowledge indicates that atherosclerosis is an active inflammatory and immune-mediated process. Therefore, the chronic inflammation and immune dysregulation characteristic of systemic lupus erythematosus undoubtedly contribute to the accelerated vascular disease seen in these patients. Carefully considering what is known about

2003 Current Opinion in Rheumatology

125436. Apoptosis in systemic lupus erythematosus. (Abstract)

plays a central role in tolerizing B cells and T cells to both tissue-specific and ubiquitously expressed self-antigens, and may drive the autoimmune response in systemic autoimmune disease. The authors review the significant recent advances in this area. Recent studies suggest that predisposing factors to subsequent development of systemic autoimmunity may be the incomplete induction of tolerance to apoptotic antigens, potentially through abnormal apoptotic signaling and effector pathways (...) Apoptosis in systemic lupus erythematosus. Systemic lupus erythematosus is a complex, multisystem autoimmune disease characterized by production of high-titer autoantibodies directed against ubiquitously expressed self-antigens. Autoantigens in systemic lupus erythematosus are highly diverse in terms of structure and location in control cells, but become clustered in and on the surface blebs of apoptotic cells. The past several years have provided significant evidence that the apoptotic cell

2003 Current Opinion in Rheumatology

125437. Interleukin 6 as a therapeutic target in systemic-onset juvenile idiopathic arthritis. (Abstract)

levels of interleukin 6 brings children severe growth impairment, which was strongly suggested by the recent establishment of interleukin 6 transgenic mice.This review will provide the evidences of the relation between the imbalance of interleukin 6 homeostasis and systemic-onset juvenile idiopathic arthritis. Also reviewed will be the author's recent trials of anti interleukin 6 receptor antibody, named temporally as MRA, for children with acute systemic disease intractable to long-term, high-dose (...) Interleukin 6 as a therapeutic target in systemic-onset juvenile idiopathic arthritis. Systemic-onset juvenile idiopathic arthritis is a severe and steroid-dependent disease that sometimes progresses to a fatal disease, macrophage activation syndrome. The investigation of proinflammatory cytokine levels revealed the increases of interleukin 6 in serum of systemic-onset disease. To avoid the disease progression and the adverse events of high-dose corticosteroids, it might be a reasonable

2003 Current Opinion in Rheumatology

125438. European Consensus Lupus Activity Measurement is sensitive to change in disease activity in childhood-onset systemic lupus erythematosus. (Abstract)

courses of 66 newly diagnosed cSLE patients were reviewed. The ECLAM and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) were scored for all clinic visits and hospitalizations. Damage was assessed at the end of the followup period using the Systemic Lupus International Collaboration Clinics/American College of Rheumatology Damage Index. Disease activity at the time of diagnosis, 6 months after diagnosis, at the time of first flare, and 6 months after first flare was used to estimate (...) European Consensus Lupus Activity Measurement is sensitive to change in disease activity in childhood-onset systemic lupus erythematosus. To evaluate the European Consensus Lupus Activity Measurement (ECLAM) for responsiveness to change in disease activity when used in childhood-onset systemic lupus erythematosus (cSLE). To confirm the construct validity and to characterize the measurement properties of the ECLAM by assessing its ability to predict damage and steroid requirements.The disease

2003 Arthritis and Rheumatism

125439. The epidemiology of the primary systemic vasculitides in northwest Spain: implications of the Chapel Hill Consensus Conference definitions. (Abstract)

The epidemiology of the primary systemic vasculitides in northwest Spain: implications of the Chapel Hill Consensus Conference definitions. To assess the epidemiology of the primary systemic vasculitides (PSV) in a well-defined population of southern Europe over a 14-year period using the Chapel Hill Consensus Conference (CHCC) definitions.The case records of all patients 15 years or older with vasculitis diagnosed between January 1988 and December 2001 at a single reference hospital (...) in the Lugo region of northwest Spain were reviewed. Incidence rates were age- and sex-adjusted to the European standard population. Patients were classified as having PSV according to the CHCC definitions.Fifty-four Lugo residents (29 men) fulfilled the CHCC definitions for PSV. The mean age was 60.7 +/- 13.5 years (men: 61.0 +/- 13.4; women: 60.4 +/- 13.8 years). The overall annual incidence of PSV was 13.07/million (95% confidence interval [95% CI] 8.89-19.22). PSV were slightly more common in men

2003 Arthritis and Rheumatism

125440. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. (Abstract)

Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. To estimate the prevalence, incidence, survival, and disease characteristics of systemic sclerosis (SSc) in the Detroit tricounty area.A census of SSc cases for the period 1989-1991 was conducted in the Detroit area, using multiple sources for case identification. Diagnoses were verified by medical record review. Capture-recapture analysis was used to estimate the total SSc population

2003 Arthritis and Rheumatism

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