Pediatric rheumatology

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Format: Buch
Sprache:English
Veröffentlicht: Philadelphia, Pa. Saunders 2012
Schriftenreihe:Pediatric clinics of North America 59,2
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adam_text Pediatrie Rheumatology Contents Preface: Pediatric Rheumatology xiii Ronald M. Laxer and David D. Sherry Principles of Inflammation for the Pediatrician 225 Alessia Omenetti, Sabrina Chiesa, and Marco Gattorno The immune system consists of 2 branches: innate and adaptive. The for¬ mer represents the first line of host defense during infection and plays a key role in the early recognition and protection against invading patho¬ gens. The latter orchestrates elimination of pathogens in the late phase of infection and leads to the generation of immunologie memory. Innate and adaptive immunity should not be considered separate compartments. Innate and adaptive immune responses represent an integrated system of host defense. The authors review the mechanisms driving the induction and perpetuation of the inflammatory responses observed during patho¬ gen-associated, autoimmune, and autoinflammatory diseases. Approach to the Child with Joint Inflammation 245 Roberta Berard Arthritis is manifested as a swollen joint having at least 2 of the following conditions: limited range of motion, pain on movement, or warmth overly¬ ing the joint. This article discusses an approach to the evaluation of a child with arthritis of one (mono) or several (poly) joints. Laboratory Testing in Pediatrie Rheumatology 263 Jay Mehta In children, laboratory evaluations can assist in the screening of patients for inflammatory disorders, confirm diagnoses, allow for monitoring of dis¬ ease activity and response to therapy, and suggest prognoses and risk of morbidities associated with rheumatic diseases. This review provides an overview of the usefulness and interpretation of both the commonly or¬ dered tests ordered by the general pediatrician as well as those frequently used in the pediatric rheumatology clinic for diagnosis and disease moni¬ toring. Studies discussed include the complete blood count, acute phase reactants, autoantibodies, serum complement, urinalysis, streptococcal antibody tests, and commonly used genetic studies. Rheumatologic Emergencies in Newborns, Children, and Adolescents 285 Jonathan D. Akikusa This article presents five clinical scenarios in which the initial manifesta¬ tions of pediatric rheumatic diseases constitute life-threatening medical emergencies. It is intended as a problem-oriented guide for pediatricians to assist in the recognition of rheumatologic differentials in children pre¬ senting with critical illness and provides an approach to their initial inves¬ tigation and management. Contents Juvenile Idiopathic Arthritis 301 Peter J. Gowdie and Shirley M.L. Tse Juvenile idiopathic arthritis (ЛА) encompasses a complex group of disor¬ ders with arthritis as a common feature. This article provides the pediatrician with a review of the epidemiology, classification, clinical manifestations, and complications of JIA. It also provides an update on the current understand¬ ing of the cause of JIA and recent developments in management and a recent review of the long-term outcome in JIA. Making Sense of the Cytokine Storm: A Conceptual Framework for Understanding, Diagnosing, and Treating Hemophagocytic Syndromes 329 Scott W. Canna and Edward M. Behrens Cytokine storm syndromes (CSS) are a group of disorders representing a variety of inflammatory causes. The clinical presentations of all CSS can be strikingly similar, creating diagnostic uncertainty. However, clini¬ cians should avoid the temptation to treat all CSS equally, because their inciting inflammatory insults vary widely. Failure to identify and address this underlying trigger results in delayed, inoptimal, or potentially harmful consequences. This review places the hemophagocytic syndromes hemo¬ phagocytic lymphohistiocytosis and macrophage activation syndrome within a conceptual model of CSS and provides a logical framework for diagnosis and treatment of CSS of suspected rheumatic origin. Systemic Lupus Erythematosus in Children and Adolescents 345 Deborah M. Levy and Sylvia Kamphuis Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a wide range of disease manifestations that can involve any organ system, and can lead to significant morbidity and even mortality. This article reviews the epidemiology, common clinical features, and complications of the disease, and briefly discusses the available treatment options. In addition, important medical and psychosocial issues relevant to the pediatrician caring for children and adolescents with SLE are discussed. Idiopathic Inflammatory Myopathies in Childhood: Current Concepts 365 Adam M. Huber The juvenile idiopathic inflammatory myopathies are a group of rare, chronic, multisystem, autoimmune diseases associated with muscle weakness. This article reviews practical issues of relevance to pediatricians, including clinical presentation, differential diagnosis, investigation, therapy, and prog¬ nosis. The importance of early recognition and specialist involvement is emphasized. Pediatrie Scleroderma: Systemic and Localized Forms 381 Kathryn S. Torok Pediatrie scleroderma includes 2 major groups of clinical entities, systemic sclerosis (SSc) and localized scleroderma (LS). Although both share a com¬ mon pathophysiology, their clinical manifestations differ. LS is typically confined to the skin and underlying subcutis, with up to a quarter of Contents ix patients showing extracutaneous disease manifestations such as arthritis and uveitis. Vascular, cutaneous, gastrointestinal, pulmonary, and muscu- loskeletal involvement are most commonly seen in children with SSc. Treatment of both forms targets the active inflammatory stage and halts disease progression; however, progress needs to be made toward the development of more effective antifibrotic therapy to help reverse disease damage. Pediatric Vasculitis 407 Pamela F. Weiss Childhood vasculitis is a challenging and complex group of conditions that are multisystem in nature and often require integrated care from multiple subspecialties, including rheumatology, dermatology, cardiology, nephrol- ogy, neurology, and gastroenterology. Vasculitis is defined as the presence of inflammation in the blood vessel wall. The site of vessel involvement, size of the affected vessels, extent of vascular injury, and underlying pathology determine the disease phenotype and severity. This article explores the classification and general features of pediatric vasculitis, as well as the clin¬ ical presentation, diagnostic evaluation, and therapeutic options for the most common vasculitides. Kawasaki Disease 425 Rosie Scuccimarri Kawasaki disease is a systemic vasculitis and the leading cause of ac¬ quired heart disease in North American and Japanese children. The epide¬ miology, cause, and clinical characteristics of this disease are reviewed. The diagnostic challenge of Kawasaki disease and its implications for cor¬ onary artery outcomes are discussed, as are the recommended treatment, ongoing treatment controversies, concerns associated with treatment resistance, and the importance of ongoing follow up. Autoinflammatory Syndromes 447 Philip J. Hashkes and Ori Toker There has been an expansion of the autoinflammatory syndromes due to the discovery of new diseases related to mutations in genes regulating the innate immune system and the knowledge gained from these diseases as applied to more common nongenetic inflammatory conditions. Autoinflammatory syndromes are characterized by unprovoked (or triggered by minor events) recurrent episodes of systemic inflammation involving various body sys¬ tems, which are often accompanied by fever. Inflammation is mediated by polymorphonuciear and macrophage cells through cytokines, particularly interleukin-1 . This article reviews the clinical approach to patients with sus¬ pected autoinflammatory syndromes, several of the main and new (mostly genetics) syndromes, advances in treatment, and prognosis. Approach to the Patient with Noninflammatory Musculoskeletal Pain 471 Peter Weiser Musculoskeletal pain is one of the most common presenting symptoms at the pediatrician s office. Etiology ranges from benign conditions to serious Contents ones requiring prompt attention. This article addresses entities that pres¬ ent as musculoskeletal pain but are not associated with arthritis. The most common nonarthritic conditions are benign limb pain of childhood (grow¬ ing pains), hypermobility, overuse syndromes with or without skeletal abnormalities, malignancies, and pain amplification syndromes. The| initial decision process, diagnosis, and treatment options for each of these con¬ ditions are discussed. Immunodeficiency Diseases with Rheumatic Manifestations 493 Troy R. Torgerson Most clinicians associate primary immunodeficiency disorders (PIDDs) with susceptibility to frequent or severe infections. It is less commonly rec¬ ognized, however, that PIDDs are frequently associated with autoimmune or rheumatologic manifestations. This review provides a synopsis of the rheumatic manifestations associated with immunodeficiencies in each of the major compartments of the immune system. Index 509
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publishDate 2012
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series Pediatric clinics of North America
series2 Pediatric clinics of North America
spellingShingle Pediatric rheumatology
Pediatric clinics of North America
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title Pediatric rheumatology
title_auth Pediatric rheumatology
title_exact_search Pediatric rheumatology
title_full Pediatric rheumatology guest ed. Ronald M. Laxer ...
title_fullStr Pediatric rheumatology guest ed. Ronald M. Laxer ...
title_full_unstemmed Pediatric rheumatology guest ed. Ronald M. Laxer ...
title_short Pediatric rheumatology
title_sort pediatric rheumatology
topic Kind (DE-588)4030550-8 gnd
Rheumatismus (DE-588)4049836-0 gnd
topic_facet Kind
Rheumatismus
Aufsatzsammlung
url http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=025112601&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA
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