CL0093：Z[转帖]血友病性关节炎（顺便看看别人的发帖）

rjbjl

hemophilic arthropathy
abstract: a 36-year-old man with known hemophilia presented with pain and swelling of his left knee. three days earlier he had fallen onto the same knee. the physical examination revealed a grossly swollen and tender knee.

case summary

che denied illicit drug use or any recent fevers or chills. radiographs of the left knee at that time were significant only for a joint effusion (not shown). he was given nonsteroidal anti-inflammatory medication and was referred to physical therapy for range-of-motion (rom) exercises and modalities for pain and swelling control. although the pain and swelling gradually decreased over a 4-week period, the patient experienced progressive loss of motion and represented 10 months later with significant joint contractures. repeat radiographs (figure 1) were obtained, followed by magnetic resonance (mr) imaging of the left knee (figure 2).

diagnosis

hemophilic arthropathy

imaging findings

radiographs of the right knee were normal. the radiographic images of the left knee reveal extensive erosive and degenerative change about the femoral-tibial and femoral-patellar joints and diffuse, severe osteopenia (figure 1). mr imaging shows extensive subchondral cystic change and edema. the t2-weighted mr image shows, particularly well, multiple, intra-articular dark irregular foci corresponding to hemosiderin deposits (figure 2).

discussion

the coagulation dysfunction in hemophilia a is a sex-linked deficiency or abnormality of a plasma protein called factor viii (fviii) found in 1 of 5000 male births. physiologically, fviii is found circulating in the plasma bound to von willebrand&#39；s factor, which acts as a carrier protein. the degree of fviii deficiency correlates with the extent of bleeding, and hemophilia a can be classified as severe (<1% activity), moderate (1% to 5%), or mild (5% to 25%). typically, symptoms begin in childhood associated with hemorrhage after minor trauma. as the individual ages and becomes more prudent, symptoms become less frequent. although a presumptive diagnosis can be made based on age, sex, family history, and clinical presentation, the presentation is not always classic. up to 30% of patients may have a normal family history due to mild disease or lack of males in the family tree. mild disease, however, may not present until well into adulthood； there has even been a case in which the patient was diagnosed during his 60s.1 hemophilia has been well documented in females in whom x chromosome inactivation occurs at an early stage of embryogenesis, resulting in unusually low levels of fviii.2 the differential diagnosis for congenital bleeding disorders includes hemophilia b (deficiency of factor ix), factor xi deficiency, and von willebrand&#39；s disease (deficiency of vwf). acquired coagulation disorders should also be considered, such as vitamin k deficiency, liver disease, and factor deficiencies that may be seen in conditions like systemic lupus erythematosus or lymphoma.

radiographically, the diagnosis is often suggested by recurring changes of hemarthrosis in the knees, elbows, and ankles.3 although there is no universally accepted classification system, several common patterns of joint disease in hemophilic arthropathy have been described by arnold and hilgartner.4 the initial episode of intra-articular bleeding, often following minor trauma, is usually associated with joint effusion without osseous or articular involvement. with recurrent small bleeds or after a large bleed, peri-articular osteoporosis and regional soft-tissue swelling are common sequelae. in adolescents, the hyperemic joint may lead to localized, accelerated growth and limb-length discrepancies. eventually osseous irregularity and erosion develop, accompanied by subchondral cysts. synovial effusions are common and may appear radiodense due to hemosiderin deposition. an important diagnostic clue to hemophilic arthropathy during this phase is th

rjbjl

abstract: a 36-year-old man with known hemophilia
presented with pain and swelling of his left knee.
three days earlier he had fallen onto the same knee.
the physical examination revealed a grossly swollen
and tender knee.

case summary

che denied illicit drug use or any recent fevers or chills.
radiographs of the left knee at that time were significant
only for a joint effusion (not shown). he was given
nonsteroidal anti-inflammatory medication and was
referred to physical therapy for range-of-motion (rom)
exercises and modalities for pain and swelling control.
although the pain and swelling gradually decreased over
a 4-week period, the patient experienced progressive
loss of motion and represented 10 months later with
significant joint contractures. repeat radiographs
(figure 1) were obtained, followed by magnetic resonance
(mr) imaging of the left knee (figure 2).

diagnosis

hemophilic arthropathy

imaging findings

radiographs of the right knee were normal.
the radiographic images of the left knee reveal extensive
erosive and degenerative change about the femoral-tibial
and femoral-patellar joints and diffuse, severe osteopenia
(figure 1). mr imaging shows extensive subchondral cystic
change and edema. the t2-weighted mr image shows,
particularly well, multiple, intra-articular dark irregular foci
corresponding to hemosiderin deposits (figure 2).

discussion

the coagulation dysfunction in hemophilia a is a sex-linked
deficiency or abnormality of a plasma protein called factor viii
(fviii) found in 1 of 5000 male births. physiologically,
fviii is found circulating in the plasma bound to von willebrand&#39；s
factor, which acts as a carrier protein. the degree of fviii
deficiency correlates with the extent of bleeding, and hemophilia
a can be classified as severe (<1% activity), moderate
(1% to 5%), or mild (5% to 25%). typically, symptoms begin in
childhood associated with hemorrhage after minor trauma.
as the individual ages and becomes more prudent,
symptoms become less frequent. although a presumptive
diagnosis can be made based on age, sex, family history,
and clinical presentation, the presentation is not always classic.
up to 30% of patients may have a normal family history due to
mild disease or lack of males in the family tree. mild disease,
however, may not present until well into adulthood；
there has even been a case in which the patient was diagnosed
during his 60s.1 hemophilia has been well documented in females
in whom x chromosome inactivation occurs at an early stage of
embryogenesis, resulting in unusually low levels of fviii.2
the differential diagnosis for congenital bleeding disorders includes
hemophilia b (deficiency of factor ix), factor xi deficiency,
and von willebrand&#39；s disease (deficiency of vwf).
acquired coagulation disorders should also be considered,
such as vitamin k deficiency, liver disease, and factor deficiencies
that may be seen in conditions like systemic lupus erythematosus
or lymphoma.

radiographically, the diagnosis is often suggested by recurring
changes of hemarthrosis in the knees, elbows, and ankles.3
although there is no universally accepted classification system,
several common patterns of joint disease in hemophilic arthropathy
have been described by arnold and hilgartner.4 the initial episode
of intra-articular bleeding, often following minor trauma, is usually
associated with joint effusion without osseous or articular involvement.
with recurrent small bleeds or after a large bleed, peri-articular
osteoporosis and regional soft-tissue swelling are common sequelae.
in adolescents, the hyperemic joint may lead to localized, accelerated
growth and limb-length discrepancies. eventually osseous irregularity
and erosion develop, accompanied by subchondral cysts.
synovial effusions are common and may appear radiodense due to
hemosiderin de

men

讲一讲，确定是血友病引起的吗

guicai

[emb12]英文太差，不过片子不错

天上飘云

[emb20][emb19][emb10][emb11][emb12][emb13][emb27][emb26]

drzhang8888

英文太差

jinguoji

英文太差  
看不懂

tkp815

软件翻译的(呵呵,有点不对头,不过总比看天书强)
摘要: 由于已知的血友病的一个 36 岁的男人
以他的左边膝的痛苦和膨胀呈现了。
三年之前，他已经在相同的膝之上跌落。
身体检查显示一非常肿大的
而且投标膝。

情形摘要

che 否认不法的药物使用或任何的最近发烧或寒冷。
左边膝的 x 光照片那时是重要的
只有对于一个联合的流出物.(不显示) 他有
nonsteroidal 反激动的药物治疗和是
关于范围参照物理疗法了--运动 (rom)
对于痛苦和膨胀的练习和样式控制。
虽然痛苦和膨胀逐渐地结束减少了
4个星期期间, 病人经历了改革论者
运动的损失和表现 10 月之后由于
重要的联合挛缩。 重复 x 光照片
(图 1) 被获得, 被有磁性共呜跟随
(先生) 左边的描绘膝行 (图 2).

诊断

hemophilic arthropathy

描绘调查结果

正确膝的 x 光照片是常态。
左边膝的 x 光线照相术图像显示广泛的
关于大腿骨者的腐蚀和退步的变化-胫骨的
而且大腿骨-膝盖骨的关节和漫射, 严格的 osteopenia
(图 1). 先生描绘表演广泛的 subchondral 胞囊的
变化和水肿。 t2 重量的先生图像表演,
特别地好的，多样的，和 articular 内黑暗的不规则的焦距
对 hemosiderin 存款 (图 2) 符合。

讨论

在血友病一的凝结机能障碍是性别-联编
血浆蛋白质的缺乏或反常打电话给因素 8
(fviii) 在 5000 中的 1 男性的出生方面发现。 生理学地,
fviii 被发现在血浆中流通约束到出自 willebrand
因素, 担任运送者蛋白质。 fviii 的程度
和出血的范围的缺乏有相互关系的东西 , 和血友病
一能被归类为严格的 (<1% 活动), 温和主义者
(1-5%), 或温和的 (5-25%). 典型地, 症状开始在
与较小的外伤后的出血有关的孩童时期。
如个体变老而且变得更审慎,
症状变成较少。 虽然一根据推定的
诊断能被做基于年龄，性别，家庭历史,
而且临床的发表, 发表不总是第一流。
达 30% 的病人可能让正常的家庭历史适当到
在家谱中的温和疾病或男性的缺乏。 温和的疾病,
然而, 不可能呈现直到好的进入成人期之内；
已经甚至有一个情形在哪一个病人被诊断
在他的 60 年代期间。1 血友病已经很好地在女性被证明
在谁 x 染色体 inactivation 在早阶段发生
胚胎发生, 造成异常低度 fviii。2
对于天生的出血混乱的差别诊断包括
血友病 b(因素 9 的缺乏), 因素 11 缺乏,
而且出自 willebrand 的疾病 (vwf 的缺乏).
已取得的凝结混乱也应该被考虑,
如此的如维他命 k 缺乏，肝脏疾病和因素缺乏
那可能在像全身性红斑性狼疮一样的情况被见到
或 lymphoma。

x 光线照相术地, 诊断时常被藉由回到建议
在膝，手肘和脚踝中的 hemarthrosis 的变化。3
虽然没有普遍一般承认的分类制度,
在 hemophilic arthropathy 中的联合疾病的一些通常式样
已经被阿诺和 hilgartner 描述。4 开始的插曲
articular 内出血, 时常跟随较小的外伤, 通常是
以联合的流出物联合没有骨的或 articular 牵涉。
藉由再发生的小出血或在大的出血之后, 妖精-articular
骨质疏松症和地方的软-薄的纱织品膨胀是通常的后遗症。
在青少年中， hyperemic 关节可能带领本土化, 加速的
生长和四肢长相差。 最后骨的违法
而且腐蚀发展, 被 subchondral 包囊陪伴。
synovial 流出物是通常的而且可能出现 radiodense 适当的到
hemosiderin 沈淀。 对 hemophilic 的一个重要的诊断线索
arthropathy 在这状态期间是联合空间的保存。
然而,如骨的腐蚀继续,变窄的关节空间被见到,
和改革论者,对称的软骨破坏联合。
最后，联合空间的完全涂去将会发生和
中级的退步告示, 包括 osteophytes 和 eburnation,
发展。 藉由慢性病，肌肉不平衡和关节
挛缩可能发展。

描绘的先生能发现没被看得见的早的腐蚀在传统的之上
x 光线照相术和最好的样式或许为估定
与 hemophilic arthropathy 有关的 articular 内反常。
通常, hemarthrosis 能被视为对于中间物的低点的区域
在 t1 重量的先生上的信号在 t2 上的图像和增加的信号-重量
先生描绘。5 慢性的妖精-articular 的变化时常被看得见当做
在两者的 t1 上减少了信号强度- 而且 t2-重量了图像先生。
synovial 肥大起因于纤维症而且出现如小节的
对在 t1 上的中间的信号强度的低点的区域- 而且 t2- 重量
先生描绘。 subchondral 包囊包含激动的流动表演
在 t2 重量的先生上的增加的信号描绘, 当 fibrotic 包囊的时候是
hypointense。6 因为 articular 软骨很好地在先生上被看得见
描绘, 稀薄或缺席软骨的焦点区域能是
容易地发现了。

为血友病的治疗典型地包括一些形式的因素
替换仰赖疾病的严重。
与痛苦和膨胀有关的联合的受伤能被对待
藉由保守的措施 , 像是固定，冷却,
而且 nonsteroidal 反激动的药物治疗,
哪一个不干扰凝结路或
血小板动作。 热望治疗是 contraindicated
除非坏疽, 适当的连接紧张或区划压力,
即将来临。 给脉管的硬化症的放射线<

hexue

我怎么看不到图片啊

yuhongjun

装斯文。