Juvenile Spondyloarthropathies

Oct 25, 2017 by in RHEUMATOLOGY Comments Off on Juvenile Spondyloarthropathies

Arthritis and enthesitis Or Arthritis and enthesitis, or arthritis or enthesitis with at least two of the following  Presence of or a history of sacroiliac joint tenderness and/or inflammatory lumbosacral…

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Clinical Examination of the Child with a Rheumatic Disease

Oct 25, 2017 by in RHEUMATOLOGY Comments Off on Clinical Examination of the Child with a Rheumatic Disease

Life-threatening conditions Malignancy (leukaemia, lymphoma, bone tumour) Sepsis (septic arthritis, osteomyelitis) Non-accidental injury Joint pain with minimal or no swelling Hypermobility syndromes Orthopaedic syndromes (e.g. Osgood–Schlatter disease, Perthes’ disease) Idiopathic…

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Behçet’s Disease

Oct 25, 2017 by in RHEUMATOLOGY Comments Off on Behçet’s Disease

Fig. 38.1 Deep vein thrombosis of the right leg in a young boy Introduction Behçet’s disease is a systemic vasculitis of unknown origin, which is seen in the pediatric age…

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Overview of Juvenile Idiopathic Arthritis

Oct 25, 2017 by in RHEUMATOLOGY Comments Off on Overview of Juvenile Idiopathic Arthritis

Inflammatory Infection: septic arthritis, osteomyelitis, tuberculosis, Lyme arthritis Reactive arthritis secondary to extra-articular bacterial (e.g., streptococcal, enteric bacteria) or viral infections (e.g., hepatitis B, parvovirus, Epstein-Barr virus, varicella, rubella) Malignancies:…

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Basic Immunology for the Pediatrician

Oct 25, 2017 by in RHEUMATOLOGY Comments Off on Basic Immunology for the Pediatrician

  Innate immunity Adaptive immunity Physical barriers Skin, mucous membrane Mucosal immune system Cellular component Neutrophils Macrophages and dendritic cells Natural killer cells T and B lymphocytes Soluble molecules (circulating…

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Autoinflammatory Syndromes in Children

Oct 25, 2017 by in RHEUMATOLOGY Comments Off on Autoinflammatory Syndromes in Children

Disease Gene defect Attack duration Clinical features Risk of amyloidosis Treatment Familial Mediterranean fever (FMF) MEFV 1–3 days Episodic fever, peritonitis, pleuritis, erysipeloid erythema, monoarthritis M694V and M694I mutations Colchicine…

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Juvenile Localized Scleroderma

Oct 25, 2017 by in RHEUMATOLOGY Comments Off on Juvenile Localized Scleroderma

Type Subtype Features 1. Circumscribed morphea (a) Superficial Oval or round circumscribed areas of induration limited to the epidermis and dermis, often with altered pigmentation and violaceous, erythematous halo (lilac…

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Pediatric Lupus Nephritis

Oct 25, 2017 by in RHEUMATOLOGY Comments Off on Pediatric Lupus Nephritis

Class – name Light microscopy Immunofluorescence/electron microscopy (location of immune deposits) Proteinuria Hematuria Hypertension I – minimal mesangial Normal Mesangial Absent Absent Absent II – mesangial proliferative Mesangial expansion and/or…

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Juvenile Systemic Sclerosis

Oct 25, 2017 by in RHEUMATOLOGY Comments Off on Juvenile Systemic Sclerosis

Fig. 30.1 The scleroderma patterns in systemic sclerosis are characterised by progressive loss of capillaries. In an early pattern, there is a prevalence of giant capillaries without loss of capillaries….

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Macrophage Activation Syndrome

Oct 25, 2017 by in RHEUMATOLOGY Comments Off on Macrophage Activation Syndrome

Fig. 22.1 Cytolytic pathway proteins mutated in MAS. A diagram of the immunologic synapse between a cytolytic (to the left) lymphocyte and an APC (to the right) is portrayed. Proteins…

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