血友病性骨关节病X线诊断(附18例报告)

目的:探讨血友病性骨关节病X线特点,以提高对本病的认识。方法:回顾性分析18例血友病性骨关节病的X线表现,结合文献进行分析。结果:18例共累及36个关节,其中膝关节25个,踝关节10个,肩关节1个。关节囊软组织肿胀、密度增高15例,软组织内斑片状钙化3例,关节囊内血肿3例,关节周围软组织及远端肢体肌肉萎缩3例。关节间隙明显狭窄11例,轻度狭窄5例,关节间隙轻度增宽1例,无明显狭窄1例。关节面硬化、凹凸不平16例,无明显改变2例。股骨髁间凹增宽加深14例,骨骺骨端增粗变方、骨干变细11例,髌骨呈方形5例,骨质囊变9例,骨质疏松15例。并发假性骨肿瘤2例。结论:血友病性骨关节病好发于膝部,骨端骨骺增大变方、骨干变细,股骨髁间凹增宽加深,髌骨呈方形和血友病性假肿瘤表现具有特征性。     

血友病性骨关节病：附30例临床X线分析

本文报告30例血友病性骨关节病,其中1例为女性(血友病丙),X线片上可有以下10种改变:(1)关节囊、软组织肿胀;(2)关节间隙增宽或变窄;(3)骨性关节凹凸不整;(4)股骨髁间凹或鹰咀窝加深;(5)骨骺或骨端过度发育增大变方;(6)方形膑骨;(7)骨骺线或骨骺不规则;(8)关节骨性僵直;(9)骨内囊肿样变;(10)软组织内钙化。文中对上述表现进行了评价并对病理改变与X 线表现的关系进行了讨论。     

抗维生素D佝偻病的X线表现（附一家族八例报告）

目的：探讨抗维生素Ｄ佝偻病（ＶＤＲＲ）的Ｘ线表现。材料和方法：报告一家族８例ＶＤＲＲ，对该家族７例病人进行全身骨骼Ｘ线摄片观察。结果：经观察其Ｘ线表现计有：（１）松质骨骨小梁粗疏、细疏或呈网状，肋骨呈“丝瓜筋”状；（２）骨皮质松化；（３）骨转换表现；（４）骨端膨大，关节面模糊伴囊变；（５）肢体弯曲畸形，胫骨弓形弯曲，严重者呈“Ｌ”形，膝内翻或外翻，呈“Ｏ”形或“Ｘ”形腿；（６）椎体“双框征”，附件呈棉絮状改变；（７）髂骨骺下疏松带；（８）耻骨联合、骶髂关节毛刷征；（９）骨盆口三角状变形；（１０）承重肢体出现Ｌｏｏｓｅｒ带及骨间隔；（１１）儿童干骺端喇叭口样膨大，干骺端毛刷征；（１２）囟门闭合延迟，颅缝增宽、模糊。结论：通过对本病Ｘ线表现的认识，有助于提高对ＶＤＲＲ的诊断和鉴别诊断的水平。     

血友病性骨关节病影像学表现(附20例分析)

目的 探讨血友病性骨关节病临床及影像学表现.方法 回顾性总结 20例经过临床及实验室检查证实的血友病患者的影像学资料,结合文献资料对血友病患者病变关节的影像学表现进行分析.结果 20例共累及45个关节,其中膝关节25个,肘关节9个,踝关节8个,髋关节3个.影像学表现为关节囊软组织肿胀13例,关节间隙狭窄7例,关节面硬化和囊变6例,关节强直7例, 骨端或骨骺增大变方6例,髁间窝变宽、加深7例,板障内出血1例.结论 血友病性关节炎有其比较特征性的影像学特点,结合临床及相关血液学检查可以明确诊断.     

蛇毒所致肢端骨溶解X线分析（附10例报告）

蛇毒所致肢端骨溶解Ｘ线分析（附１０例报告）张吉才肢端指（趾）骨溶骨症不是独立疾病而是一种Ｘ线征象，主要表现为肢端指（趾）骨骨质吸收、破坏及残缺，可由多种病因引起。蛇毒所致肢端骨溶解报告甚少，本文报告４例并结合文献资料６例共１０例一并复习，并对其成因、...     

长骨溶骨型骨巨细胞瘤的X线诊断

目的：探讨长骨溶骨型骨巨细胞瘤的Ｘ线特征。方法：回顾分析了２２例经手术及病理证实的长骨溶骨型骨巨细胞瘤的Ｘ线征像。结果：（１）２２例均有不同程度的骨端松质骨破坏,破坏形态均趋于类球状,１０例侵及关节面。（２）骨皮质破坏１９例。（３）局限性骨膜反应４例。结论：长骨溶骨型骨巨细胞瘤的特征Ｘ线表现为骨端松质骨溶骨性骨质破坏,破坏形态趋于类球状,骨膜反应少见     

抗维生素D佝偻病的X线表现

探讨抗维生素Ｄ佝偻病的Ｘ线表现。材料和方法；报告一家族８例ＶＤＲＲ，对该家族７例病人进行全身骨骼Ｘ摄片观察。结果：经观察其Ｘ线表现计有：（１）松质骨骨小梁粗疏，细疏或呈网状，肋骨呈“丝瓜筋”状；（２）骨皮质松化；（３）骨转换表现；（４）骨端膨大，关节面模糊伴囊变；（５）肢体弯曲畸形，胫骨弓形弯曲，严重者呈“Ｌ”形，膝内翻或外翻，虽“Ｏ”形或“Ｘ”形腱。     

血友病性假肿瘤（附4例报告）

血友病性假肿瘤又称之为血友病性假囊肿、血友病性骨血囊肿，或称为骨吸收性肿瘤，为血友病的一种罕见而严重的并发症，其发生率仅占血友病的1％～2％。本病由斯他克（Starker）1918年首先提出。为加深及提高对本病的认识，本文报告我院近几年来发现的经实验室检查证实的4例血友病性似肿瘤，结合文献着重分析其X线表现。     

颅面骨骨化性纤维瘤的影像诊断(附10例报告)

研究颅骨骨化性纤维瘤的ＣＴ、Ｘ线表现，着重探讨颅面骨骨化性纤维瘤的ＣＴ诊断和鉴别诊断要点。 材料与方法。回顾分析１０例颅骨、颌面骨骨化性纤维瘤的临床及影像学资料。结果１０例中，７例经ＣＴ检查，３例经Ｘ线检查。其主要影像表为（１）骨性肿块；（２）膨胀性破坏；（３）病灶边界清楚。     

血友病性关节病的MRI分析

目的:探讨血友病性关节病的MRI表现及临床价值.方法:回顾性分析11例22个膝关节血友病性关节病患者的MRI表现.结果:22个膝关节均有积血,表现为关节腔、骨骼和/或软组织血肿,早期血肿在T1WI为等信号、T2WI呈高信号;中期血肿在T1WI呈略高信号或混杂信号,T2WI呈高信号或混杂信号;晚期血肿在T1WI和T2WI均表现为混杂信号,血肿逐渐被吸收.病变早期关节结构基本保持正常,中晚期关节结构均有损害,表现为关节软骨、骨皮质破坏吸收,关节间隙狭窄,骨端或骨骺增大变形.早中期滑膜、关节囊、肌肉及韧带肿胀,而晚期则萎缩、退变.结论:MRI能准确显示血友病性关节病的病理改变,对疾病分期、指导临床治疗及同位素放射治疗效果评价具有重要意义.     

先天发育性（骨）关节病——关节病的新概念

目的：关节病的传统概念其病因是获得性的或由后天继发因素所致，本文提出先天发育性（骨）关节病的概念。材料与方法：本组通过对成骨不全（６例），Ｍａｒｆａｎ综合征（４例），粘多糖病（８例），多发骨骺发育异常（２２例），致密性骨发育不全（２例），干骺软骨发育异常（４例），石骨症（３例），遗传性多发外生骨疣（６例），内生软骨瘤病（４例），Ｍａｆｆｕｃｃｉ病（２例），Ｍａｆｆｕｃｃｉ病（２例），系统性骨纤维发     

Haemophilia imaging: a review

Haemophilia disorders are characterised by a blood coagulation anomaly leading to prolonged and excessive bleeding. Imaging provides an essential role in the investigation of both the musculoskeletal and the non-musculoskeletal complications of haemophilia. Our institution is home to a large tertiary referral centre for haemophilia treatment. Using our broad experience, we present a multi-modality pictorial review of the musculoskeletal manifestations of haemophilia, including haemophilic arthropathy, intra-muscular haemorrhage and haemophilic pseudotumour. The main imaging features of haemophilic arthropathy are described, including synovial hypertrophy, haemosiderin deposition, sub-chondral cyst formation and loss of joint space.     

邻关节骨囊肿的影像诊断

目的 探讨邻关节骨囊肿(骨内腱鞘囊肿)的影像表现特征.方法 对经病理证实的54例邻关节囊肿影像表现进行回顾性分析.46例行X线平片检查,30例行CT检查,14例行MR检查.结果 54例邻关节骨囊肿位于踝关节27例(含多发),膝关节16例,髋关节7例,位于肱骨近端、尺骨近端、大多角骨及第1趾骨近节近端各1例;其中多发1例(距骨和胫骨远端).病变位于踝关节及膝关节共43例44个病变,其中位于内侧关节而下29个病变(65.9%).44例为类圆形单房状溶骨病变,10例呈多房状,内有分隔,均有轻度的硬化边.15例关节面有中断,与关节腔相通,1例关节面塌陷,3例病变内见气体密度,1例见液.液平面.具体为:(1)46例47个病灶x线表现为骨内邻近关节面的圆形、半圆形或不规则囊样透亮区,边缘清晰、硬化,6个病灶相邻关节面可见裂隙,关节间隙无改变;(2)30例30个病灶CT表现为19个病灶呈圆形、3个呈半圆1形、8个呈不规则形,边缘骨质硬化,14个病灶相邻关节面可见裂隙,27个病灶密度均匀,3个病灶内可见气体;(3)14例共15个病灶MR T1WI表现为低到中等信号,T2WI为高信号,1例见液-液平面,8个病变近关节而骨质断裂,7例见软组织肿胀.结论 邻近关节的特殊发病部位结合典型影像表现,对邻关节囊肿能够作出正确的影像诊断.     

骶髂关节病变的CT诊断

目的 阐明骶髂关节病变的ＣＴ表现。材料与方法 搜集经临床确诊或病理证实的35例骶髂关节病变者,男22例,女13例。其中强直性脊柱炎（AS）13例,类风湿性关节炎（RA）7例,化脓性骶髂关节炎2例,骶髂关节结核5例,髂骨致密性骨炎6例,创伤性关节炎2例,结果 CT表现：AS常双侧对称发病,自关节下部开始,关节面硬化与破坏,间隙狭窄或消失,骨桥形成,RA常一侧发病,易侵犯关节上壮举剖,关节面密度减低,骨质疏松,关节面下出现周围硬化的小囊状骨缺损;化脓性骶髂关节炎常单发,骨一侧发病,易侵犯关节上半部,关节面密度减低,骨质疏松,关节面下出现周围硬化的小囊状骨缺损;化脓性骶髂关节炎常单发,骨质疏松,破坏,半生,关节间隙增宽或变窄,关节囊肿胀,关节强直,周围软组织肿胀或钙化;骶髂关节结核常单侧发病,多位于关节中下部,关节面模糊,骨质破坏及死骨形成,关节间隙增宽,常伴冷脓肿和窦道形成;髂骨致密性骨炎示髂骨面硬化区,不累及关节;创伤性关节炎骨关节面增生,浓密,关节间隙狭窄,可伴骨性强直。结论 骶髂关节病变的CT表现各不相同,CT能清楚显示骶髂关节及其周围结构,是目前诊断骶髂关节病变最理想的检查手段。     

Hemophilic arthropathy: assessment with MR imaging

Fifteen patients with hemophilia, 14 of whom had hemophilic arthropathy, were examined with magnetic resonance (MR) imaging to determine if it could be used to assess hemophilic arthropathy, especially synovial hypertrophy and the status of the articular cartilage. Thirty-five joints of the appendicular skeleton were imaged. Four joints in two patients were clinically normal. Synovial hypertrophy was detected in 28 joints and appeared as areas of low to intermediate signal intensity on T1- and T2-weighted images, with foci of increased signal intensity on T2-weighted images (presumed to be due to areas of fluid or inflammation) in 16 joints. Abnormal articular cartilage was demonstrated in 26 joints; bone lesions, fluid collections, and joint space narrowing could also be seen. MR imaging appears to be useful in depicting the components of hemophilic arthropathy.     

成人股骨头缺血坏死关节囊(腔)异常CT研究

目的探讨成人股骨头缺血坏死关节囊(腔)异常的出现率、发病机制和CT表现。材料与方法回顾性分析具有典型X线和CT表现或/和经病理或随访证实的138例成人ANFH病人的CT片,从中挑选出伴关节囊(腔)异常者进行分析。结果45个出现关节积液和其他关节囊(腔)异常,包括钙质样高密度游离体(6个),关节囊肥厚(15个),关节囊钙化(5个)和髂腰肌囊扩张(7个)。所有45个关节的股骨头均已发生塌陷。结论股骨头塌陷后,股骨头外移、应力改变和对髋关节囊的异常牵拉,是成人股骨头缺血坏死关节囊(腔)异常的主要原因。不同关节囊(腔)异常具有不同的发生率和CT表现。     

Use of echography for the diagnosis of joint amyloidosis in dialyzed patients

Amyloid arthropathy is an important complication of long-term hemodialysis. This condition may lead to destructive bone lesions and to severe functional impairment. We studied with sonography of both knees 32 long-term dialysis patients (mean dialytic age: 69.7 months), whose 7 were carrying palpable joint swellings. In 29/32 patients, ultrasound scans were associated with knee X-rays films, patellar views included. In one case, opaque arthrography was performed too. Sonography demonstrated the presence of fluid collections within the articular space and the synovial cavities in 19/32 patients (59%). Their content was transonic or mildly hypoechoic. X-ray films were positive for amyloid bone involvement in 19/29 cases (66%), with intra-osseous cysts and cortical erosions. There was a good general agreement between the results obtained with the two techniques: therefore, in some cases only one examination was positive. Sonography is proposed, in association with standard X-ray films, as a simple and reliable method for the diagnosis of articular amyloidosis in dialysis patients and for the follow-up of this disease.     

Radiographic and MRI features of deferiprone-related arthropathy of the knees in patients with beta-thalassemia

OBJECTIVE: This study was undertaken to describe the radiographic and MRI appearances of arthropathy of the knees in 14 patients with beta-thalassemia major undergoing chelation therapy with deferiprone (L1). MATERIALS AND METHODS: All available radiographs and MRI studies of the knees in 14 beta-thalassemia major patients (mean age, 16.3 years; age range, 7-33 years) undergoing chelation therapy with L1 were retrospectively assessed for changes in the synovium, cartilage, and bone. Imaging findings and signs of knee arthropathy were correlated with chelation therapy and average serum ferritin concentration. RESULTS: Nine (64%) of the 14 patients developed arthralgia of the knees during treatment with L1. Abnormal imaging findings were present in all symptomatic and two asymptomatic patients (12/14, 86%) and included joint effusion, subchondral bone irregularity, and patellar beaks. Additional MRI findings were thickening and enhancement of the synovium; hypointense bands in the synovium; irregularly thickened epiphyseal and articular cartilage overlying subchondral bone defects; and, on T2-weighted sequences, hyperintense articular cartilage lesions. The degree of knee symptoms at the time of imaging did not reflect the severity of cartilage and subchondral bone changes. CONCLUSION: Radiologic changes can be seen in L1-related arthropathy and should be recognized. MRI of the knees should be considered in symptomatic children and young adults with thalassemia undergoing L1 chelation therapy for iron overload.