标题: CL0093：Z[转帖]血友病性关节炎（顺便看看别人的发帖） [打印本页] 作者: rjbjl 时间: 2004-10-11 23:35 标题: CL0093：Z[转帖]血友病性关节炎（顺便看看别人的发帖） 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'；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'；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作者: rjbjl 时间: 2004-10-11 23:45
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'；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'；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作者: men 时间: 2004-10-15 22:13
讲一讲，确定是血友病引起的吗作者: guicai 时间: 2004-11-21 03:45
[emb12]英文太差，不过片子不错作者: 天上飘云 时间: 2005-1-14 07:41
[emb20][emb19][emb10][emb11][emb12][emb13][emb27][emb26]作者: drzhang8888 时间: 2006-8-14 06:20
英文太差作者: jinguoji 时间: 2006-10-22 06:40
英文太差
看不懂作者: tkp815 时间: 2007-2-25 18:42
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).