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1、Rheumatoid Disease in Children 小儿风湿性疾病,Childrens Hospital of Fudan University Xu Hong ,Rheumatoid Disease Connective tissue diseases （包含有120种与关节、骨骼、肌肉等相关的疾病）,过敏性紫癜（anaphylactoid purpura）类风湿性关节炎（JRA）SLE（systemic lupus erythematosis）皮肌炎（dermatomyositis）硬皮病（scleroderma）风湿热（rheumatic fever）川崎病 (Kawasaki
2、 disease),Rheumatoid Disease in Children,The most common diseases：Juvenile Rheumatiod Arthritis Henoch-Schonlein purpura Kawasaki Disease (Mucocutaneous Lymph Node Syndrome, MCLS) Rheumatic Fever,冠状动脉造影 Coronary angiogram,Henoch-Schonlein purpura,Alternative names:Anaphylactoid purpuraVascular purpu
3、ra,Definition,Henoch-Schonlein purpura is a disease that has the symptoms purple spots on the skin joint pain gastrointestinal symptoms glomerulonephritis (a type of kidney disorder),History story,Schonleins Purpura ( In 1837, Johan Schonlein ) the skin and joints are affected but the gastrointestin
4、al tract is not,Henochs Purpura (Later, his student, Eduard Henoch ) purpura spots on the skin and acute abdominal problemsnot affected by joint disease,General conditions,a type of hypersensitivity vasculitis and inflammatory response within the blood vesselIt is caused by an abnormal response of t
5、he immune system The exact cause for this disorder is unknown. The syndrome is usually seen in children, but people of any age may be affected It is more common in boys than in girls Many people with Henoch-Schonlein purpura had an upper respiratory illness in the previous weeks,Causes and pathogene
6、sis,An inflammatory disease of the small blood vessels (capillaries) usually a self-limited diseaseIt is the most common form of childhood vascular inflammation (vasculitis) and results in inflammatory changes in small blood vessels,Common allergens include,Medications (such as certain antibiotics,
7、antihistamines and thiazide diuretics ) Pathogens( bacterium/virus/parasite, such as group A strep, varicella, parvovirus and hepatitis B ) An acute respiratory infection,Common allergens include,foods ( such as berries, shellfish, fish, nuts, eggs, milk, and others ) pollen animal dander ( scales o
8、f shed skin ) insect bites Certain vaccinations, such as for measles, yellow fever and cholera,Pathophysiology,a small-vessel vasculitis characterized by immunoglobulin A (IgA), C3, and immune complex deposition in arterioles, capillaries, and venulesHSP and IgA nephropathy are related disorders. Bo
9、th illnesses have elevated serum IgA levels and identical findings on renal biopsy,Pathophysiology,Lower Leg Direct immunofluoresence (DIF) showed deposits of IgA about dermal vessels.,Renal biopsy showed deposits of IgA in the mensangium,Frequency: In the US: Approximately 14 cases occur per 100,00
10、0 school-aged children. HSP also occurs in adults, although less commonly than in children. Mortality/Morbidity: In general, HSP is a benign self-limited disorder. Fewer than 5% of cases cause chronic symptoms. Fewer than 1% of cases progress to end-stage renal failure. Sex: Male-to-female ratio is
11、about 2:1. Age: 75% of patients affected are aged 2-11 years. In some series, as many as 27% of the patients are adults.,Manifestations,History: The most common symptoms of HSP include the following: Rash (95-100%), especially involving the legs, may not be present on initial presentation Subcutaneo
12、us edema (20-50%) Abdominal pain and vomiting (85%) Joint pain (60-80%), especially involving knees and ankles Scrotal edema (2-35%) Bloody stools (“hematochezia“, “melena“ ),Manifestations,Physical: Palpable purpura, particularly on the buttocks and legs Edema of the hands, feet, scalp, and ears Ar
13、thritis, most commonly involving the knees and ankles Abdominal tenderness Gastrointestinal bleeding Acute scrotal edema that may mimic testicular torsion,WithAngioneurotic edemaSwelling eyes, lip, hand,On the buttocks 臀部,in rare cases the brain and spinal cord (central nervous system) involved,Arth
14、ritis, the knee,Lab Studies,Routine lab test results are usually within reference ranges. Some lab studies help in excluding other diagnoses and in evaluating renal function:urinalysis / CBC with platelet count and differential / BUN, creatinine / prothrombin time (PT), activated partial thromboplas
15、tin time (aPTT) / lipase,Lab Studies,Hematuria and/or proteinuria are present in 10-20% of patients Platelet count is usually in the reference range but may be elevated A normal lipase makes acute pancreatitis very unlikely WBC count may be in the reference range or elevated. Eosinophilia is sometim
16、es present ESR in the reference range or elevated BUN and creatinine levels may be elevated from renal involvement of HSP or from dehydration,Imaging Studies:,Abdominal ultrasound may be better than barium enema to diagnose intussusception, since HSP-related intussusception is more often ileoileal instead of ileocolic as is typical in idiopathic intussusception. Doppler or radionuclide testicular scan results show normal or increased blood flow in HSP, in contrast to the decreased blood flow seen in testicular torsion.,
