临床医学小儿风湿性疾病徐虹课件

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 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 purpura Vascular purpura,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 gastrointestinal tract is not,Henochs Purpura (Later, his student, Eduard Henoch ) purpura spots on the skin and acute abdominal problems not affected by joint disease,General conditions,a type of hypersensitivity vasculitis and inflammatory response within the blood vessel It is caused by an abnormal response of the 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 pathogenesis,An inflammatory disease of the small blood vessels (capillaries) usually a self-limited disease It 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, 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 of 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 venules HSP and IgA nephropathy are related disorders. Both 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 100,000 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 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 Subcutaneous 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 Arthritis, most commonly involving the knees and ankles Abdominal tenderness Gastrointestinal bleeding Acute scrotal edema that may mimic testicular torsion,With Angioneurotic edema Swelling eyes, lip, hand,On the buttocks 臀部,in rare cases the brain and spinal cord (central nervous system) involved,Arthritis, 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 thromboplastin 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 sometimes 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.,Diagnosis and differentiation,Diagnosis is clinical and not based on laboratory evaluation A physical examination reveals skin lesions and joint tenderness A urinalysis shows microscopic blood in the urine A skin biopsy shows vasculitis,Diagnosis and differentiation,A normal platelet count rules out idiopathic thrombocytopenic purpura (ITP) A normal platelet count and normal coagulation studies (ie, PT, aPTT, fibrin split products) rule out thrombotic thrombocytopenic purpura (TTP),Other Problems to be Considered,Essential mixed cryoglobulinemia Waldenstrm macroglobulinemia Systemic lupus erythematosus Rickettsial diseases Bowel infarction/perforation,Treatment,Anti-inflammatory medications, such as aspirin or ibuprofen, to relieve joint pain and swelling Stopping medication that may be the underlying cause Corticosteroids If symptoms persist, therapy with corticosteroids such as prednisone, tried to treat abdominal pain, subcutaneous edema, and nephritis,Treatment,Patients with severe abdominal pain, significant gastrointestinal bleeding, or marked renal insufficiency may require hospitalization.,Treatment in HSPN (HSP nephritis),many different modalities in severe cases IV or oral steroids with or without: Azathioprine （硫唑嘌呤） Cyclophosphamide （CTX） Cyclosporine （环孢素A） Dipyridamole (潘生丁) Plasmapheresis （血浆置换） high-dose IV immunoglobulin G (IVIg),Prognosis,HSP is generally a benign disease with an excellent prognosis More than 80% of patients have a single isolated episode lasting a few weeks Approximately 10-20% of patients have recurrences Fewer than 5% of patients develop chronic HSP Abdominal pain resolves spontaneously within 72 hours in most patients,General conditions,A chronic systemic disease Primarily of the joints Marked by inflammatory changes in the synovial membranes（滑膜） and articular structures Widespread fibrinoid degeneration of the collagen fibers in mesenchymal tissues, and by atrophy and rarefaction of bony structures （纤维化和骨质疏松） Etiology is unknown Autoimmune mechanisms have been implicated,General conditions,Arthritis can be short-term - lasting for just a few weeks or months, then going away forever or it can be chronic and last for months or years. In rare cases, it can last a lifetime,Causes and pathogenesis,Causes unknown exactly Research indicates that it is an autoimmune disease White blood cells lose the ability to tell the difference between the bodys own healthy cells and harmful invaders like bacteria and viruses The immune system, which is supposed to protect the body from these harmful invaders, instead releases chemicals that can damage healthy tissues and cause inflammation and pain,Clinical manifestations,Signs and Symptoms The first signs of arthritis can be subtle or obvious. Signs may include limping (跛行) or a sore wrist, finger, or knee Joints may suddenly swell and remain enlarged. Stiffness in the neck, hips, or other joints Rashes may suddenly appear and disappear developing in 1 area and then another. High fevers that tend to spike in the evenings and suddenly disappear are characteristic of systemic juvenile rheumatoid arthritis,High fevers in Systemic JRA,Clinical manifestations,Typically appears between the ages of 6 months and 16 years Not a single disease, but a group of diseases What they all have in common is chronic joint inflammation. the first signs are often joint pain or swelling and reddened or warm joints The diseases are very different in their symptoms, their treatments, and their outcomes There are 3 major types of juvenile rheumatoid arthritis (according to onset within the first 6 months),Clinical manifestations,Polyarticular arthritis (多关节型) affects more girls than boys Symptoms include swelling or pain in 5 or more joints The small joints of the hands are affected as well as the weight-bearing joints such as the knees, hips, ankles, feet, and neck In addition, a low-grade fever may appear, as well as bumps or nodules on the body on areas subjected to pressure from sitting or leaning,swelling of small joints of the hands,a lot of stiffness when they first wake up in the morning. Once their joints warm up, they can usually move more easily.,Clinical manifestations,Pauciarticular JRA (少关节型) affects 4 or fewer joints Symptoms include pain, stiffness, or swelling in the joints The knee and wrist joints are the most commonly affected iridocyclitis or iritis or uveitis may occur with or without active joint symptoms ( can be detected early by an ophthalmologist ),Clinical manifestations,Systemic JRA （全身型） affects the whole body Symptoms include high fevers that often increase in the evenings and then may suddenly drop to normal During the onset of fever, the child may feel very ill, appear pale, or develop a rash The rash may suddenly disappear and then quickly appear again The spleen and lymph nodes may also become enlarged Eventually many of the bodys joints are affected by swelling, pain, and stiffness,Lab Studies,Abnormalities in the numbers and appearances of CBC can be useful in the diagnosis of many medical conditions A blood culture may be taken to rule out infections Bone marrow examination is a test to rule out conditions such as leukemia,Lab Studies,Erythrocyte sedimentation rate often increases in people when inflammation is occurring in the body Rheumatoid factor（RA 类风湿因子） is an antibody, common in rheumatoid arthritis Rare in persons with systemic JRA a marker for persistence of polyarticular JRA into adulthood,Lab Studies,ANA (antinuclear antibody) is a test performed on the blood to detect autoimmunity. It is also useful in predicting which children are likely to have eye disease with JRA Test for certain viral infections such as Lyme disease that may cause similar symptoms or occur along with the arthritis.,Imaging Studies,Radiography of affected joints When only a single joint is affected, radiography is important to exclude other diseases, such as osteomyelitis 骨髓炎or septic arthritis Bone scanning: When physical findings do not document definite arthritis, consider bone scanning as a means of identifying a potential focus of osteomyelitis or other abnormality,Imaging Studies,MRI is helpful when considering trauma in the differential diagnosis CT scanning of long bones: Perform when considering osteoid osteoma in a child with lower extremity pain (often at night) and unremarkable findings on physical examination.,Imaging Studies,Echocardiography This is performed in a child with possible systemic JRA and with fevers. In a person who has nonspecific rash, adenopathy, and possible mucocutaneous changes, to exclude coronary arterial dilation resulting from (possibly atypical) Kawasaki disease. In an individual who has findings suggestive of SLE (eg, nephritis, pleuritic chest pain, thrombocytopenia),Others,Synovial biopsy This procedure may be helpful to exclude other diagnoses particularly when the knee is affected (eg, villonodular synovitis 绒毛膜结节样滑膜炎 granulomatous arthritis),Diagnosis and differentiation,Diagnosis To effectively manage and minimize the effects of arthritis, an early and accurate diagnosis is essential A detailed physical examination is a critical tool in diagnosing JRA Physical findings are important to detect abnormalities suggestive of other possible diagnoses,Diagnosis and differentiation,The diagnosis of JRA is based on the physical finding of arthritis (or synovitis) in at least one joint that persists for at least 6 weeks With other causes being excluded and with onset when the individual is younger than 16 years Arthritis on examination is defined as either joint swelling or the combination of limited motion with pain (on motion or to palpation),Diagnosis and differentiation,DIFFERENTIALS Acute Lymphoblastic Leukemia Autoimmune Chronic Active Hepatitis Crohn Disease Endocarditis, Bacterial Fever in the Toddler Kawasaki Disease Systemic Lupus Erythematosus,Treatment,The goals of treatment: To relieve pain and inflammation To slow down or prevent the destruction of joints To restore use and function of the joints to promote optimal growth, physical activity, and social and emotional development Combination of medication, physical therapy, and exercise (physician, rheumatologist, and physical therapist),Treatment,Initial treatment limited to the use of salicylates then other nonsteroidal anti-inflammatory drugs (NSAIDs) resulted in many patients becoming wheelchair bound Other patients underwent synovectomies to remove excess tissue resulting from uncontrolled arthritis The addition of second-line drugs, starting with gold salt injections and then replaced by the much more effective methotrexate (MTX),improved the outlook for these patients,Treatment,In specific situations, injection of corticosteroids into the joint The approval of the biologic antagonist to tumor necrosis factor (TNF) - a new era of treatment more closely tailored to the pathophysiology of the disease,Treatment,Physical Therapy An appropriate physical therapy program is essential in the management of any type of arthritis To recommend range-of-motion exercises to restore flexibility in stiff, sore joints and other exercises to help build strength and endurance,Treatment,Regular Exercise When pain strikes, its natural to want to sit still Its important to maintain a regular exercise program, especially for those with arthritis Muscles must be kept strong and healthy so they can help support and protect joints Regular exercise also helps to maintain range of motion of joints,Patients with JRA may have a gradually responsive disease (particularly those with pauciarticular JRA) A small number of patients with pauciarticular JRA develop aggressive arthritis confined to a single joint, requiring more intensive medical treatment and physical therapy. Some patients with polyarticular JRA demonstrate rapid response to treatment; however, most have prolonged courses, requiring frequent adjustments in medical and nonmedical therapy,Treatment,Thank you for your attention!,我劝一个草率结婚的朋友离婚。她平静的告诉我，如果说当初鲁莽结婚是个错误。那么，现在草率离婚是一错再错。这位朋友后来还是离婚了，大家一致认为她的行为很理性。 同样的故事正在互联网搜索巨头谷歌身上发生，但是谷歌选择了草率“离婚”。 饮鸩止渴 由于急于抑制苹果iphone手机翻天覆地的产业冲击，谷歌采取急功近利的粗糙型开放策略。饮鸩止渴的策略一时取得了成果。市场研究公司尼尔森最近公布的数据显示，在通过Verizon Wireless、AT&T和Sprint Nextel三大运营商经销后，谷歌Android手机在美国市场上的销售量已经超过iPhone。另一家市场研究公司iSuppli甚至认为，全球范围内使用Android操作系统的手机数量将在 2012年超过苹果iPhone。 表面繁华的背后，是Android生态系统的一团糟，谷歌正在为自己的粗放型开放策略买单。 用户对谷歌手机的态度从开始的好奇、后来的犹豫，变成强烈的批评。“大多Android手机程序都是垃圾，乱七八糟的”，一位手机发烧友迅速投奔了iphone的阵营：“同样的植物人大战僵尸游戏，在谷歌手机和iphone手机上的体验简直没法比” 混乱，还是混乱。一切一切的乱象，折射出谷歌已经失去对Android生态系统的控制。这一切的根源，我的判断是开放策略初期过于宽松，导致失去控制权。混乱的生态系统表现在用户手机上，就是应用程序的混乱和粗燥。 一错再错 为此，谷歌开始采取对策。最近，有国内厂商称新的Android3.0开始关闭应用程序的API(应用程序编程接口)，统一Android界面。这意味着，谷歌将放弃其初始开放策略，开始封闭管理。 粗看之下，谷歌认识到自己的错误。既然是过度开放导致的错误，那么收紧开放尺度是很自然的逻辑，无懈可击。但我认为，谷歌仓促收紧开放策略仍然是个错误。打个比喻。如果过度开放的政策是草率结婚，那么草率的封闭就是草率离婚。这么判断的原因很简单，谷歌把Android开放出去的那一天，Android已经不属于谷歌。谷歌没有认识到这一点，还以为Android只是自己的。 合作伙伴对谷歌封闭政策的反应加强了我的判断结论。经济观察报报道，国内第一家生产基于Android平台手机的设计公司创杨通信，近日已经被迫出售。创杨通信负责人给出的出售理由是，“因为不愿意甘当炮灰而选择放弃。” 按照目前Android3.0将统一界面的想法，未来的手机市场将出现毫无差异化的产品。这对于企业来说，几乎意味着不可避免的价格战。利润空间的微薄，导致合作伙伴生存环境恶劣。于是大量退出几乎是一种必然。 除了为合作伙伴找到新的利益空间，谷歌还将面临开放阵营精神层面的声讨，这对谷歌的挑战会更大。如果说谷歌为了自己竞争的私利利用了开放，赢得了名声。那么，谷歌不能一脚把开放踢开，他现在还需要为这种名声买单。 如果只顾自己收网，谷歌会被面临铺天盖地的道德谴责。谷歌，希望你准备好了，三思而行。 木桶效应就是指一个水桶无论有多高，它盛水的高度取决于其中最低的那块木板。这在选购手机的时候也同样适用。尤其是很多用户在购买手机的时候， 都会专注于某一个参数，比如要求处理器主频要高达1GHz，但一部手机的整机表现是由多个因素组成，所以在购买手机的时候一定要从整体的角度上来看一部手 机的性除了处理器主频以外，其实还有很多影响整机表现的元素，比如运行内存(RAM)、机身内存(ROM)、操作系统、厂商对系统的优化都会有所影 响。不过在很多用户眼中，这几项却远没有处理器主频重要。而如果忽略这几项的话，可能买到一个主频很高，但整机性能却仍然不令人满意的机型。 用户在-财务部员工 老子不求人，人人求老子！于是乎，这帮狗屎们天天拽得好像自己是救世主是其他员工的再生父母一样，牛！超级牛！100牛！我活这么大了还真没看到过不牛的财务，这帮人其实在公司是同事们最不敢得罪、但更是最让人看不起，人际关系最差的一群人，当然，据我观察，也是离婚率最高的一类人。 最有城府最有心计的人-人力资源部员工 每天的工作就是算计如何搞出用最小代价换取最大回报的提议来讨好老板，看谁不顺眼就想方设法算计如何在考核、薪酬奖金分配方面给他穿小鞋的鸟人。：智商最高情商最差的人-研发部员工 技术过硬，为人木衲。上台发言三分钟 搞不出一句话来，向领导汇报工作结结巴巴没 更多精品文档请访问我的个人主页/611696569 谢，再见！个半小时理不出个头绪来。企业中最好管理的一群伪知识分子，可以被任意剥削，基本上不会反抗，或者从来就没有过反抗的意识 最吊儿郎当和无耻的人-销售部员工 老板们财富的来源，老板们最想讨好的一群人，这群人其实也是最无耻公司内口碑最差却又人际关系最和谐的一群人。天天吊儿郎当的来公司报个到，调戏一下前台，和狐朋狗友打打电话，10点不到就开始琢磨找借口出门拜见客户，其实下午就是上 所述，大家在选购手机的时候一定要综合考虑一款手机的硬件规格。除此之外，也不要把硬件看的太过重要，就比如苹果iPhone 3GS在硬件配置上并不出众，但却在操控手感以及软件资源上目前难有机型企及。更高分辨率能获得更为逼真细腻的显示效果，所以对于屏幕的分辨率绝大多数人都会偏向于分辨率更高的机型。但对于笔者 所说的高分辨率未必是好事会有所怀疑。其实这里说的高分辨率“不好”更多是指采用非主流的高分辨率机型。在此前，就有几款“悲情”机型在分辨率上吃了不小 的亏。 大名鼎鼎的HTC Diamond就是一款颇具代表意义的机型，Diamond上市的手机市场还处于QVGA时代、只有少数旗舰机皇采用WVGA这样级别的屏幕，由于HTC Diamond却采用了VGA这一过渡型的分辨率，而也正是因为这一点，Diamond很多软件都未有支持或无法完美运行，可谓是一个不小的遗憾。除此之 外，曾经非常经典的,附赠人生心语 人生太短，聪明太晚 人生太短，聪明太晚(1) 我们都老得太快 却聪明得太迟 把钱省下来，等待退休后再去享受 结果退休后，因为年纪大，身体差，行动不方便，哪里也去不成。钱存下来等养老，结果孩子长大了，要出国留学，要创业做生意，要花钱娶老婆，自己的退休金都被拗走了。 人生太短，聪明太晚(2) 当自己有足够的能力善待自己时，就立刻去做，老年人有时候是无法做中年人或是青少年人可以做的事，年纪和健康就是一大因素。小孩子从小就告诉他，养你到高中，大学以后就要自立更生，要留学，创业，娶老婆，自己想办法，自己要留多一点钱，不要为了小孩子而活我们都老得太快却聪明得太迟，我的学长去年丧妻。这突如其来的事故，实在叫人难以接受，但是死亡的到来不总是如此。学长说他太太最希望他能送鲜花给他，但是他觉得太浪费，总推说等到下次再买，结果却是在她死后，用鲜花布置她的灵堂。这不是太蠢愚了吗？！ 等到、