HYPEROSTOSIS

骨肥厚症
  • 文章类型: Case Reports
    被认为是罕见的,滑膜炎-痤疮-脓疱病-骨肥大-骨炎(SAPHO)综合征是一个独特的临床实体,皮肤表现和骨关节症状。胸锁和胸肋关节中心的前胸壁疼痛是可以导致其诊断的重要且特征性的临床发现。放射科医生和临床医生必须意识到滑膜炎-痤疮-脓疱病-肥大-骨炎综合征,因为它可以模仿一些更常见的疾病实体,如佩吉特病。我们报道了一个63岁的男性患者,没有明显的病史,他介绍给皮肤科,伴有严重的手掌和足底脓疱病,并伴有多关节痛。计算机断层扫描显示胸锁骨增生,支持SAPHO综合征,非甾体抗炎药治疗后临床症状消退。
    Considered rare, the synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome is a distinct clinical entity, associating skin manifestations and osteoarticular symptoms. Anterior chest wall pain centered at sternoclavicular and sternocostal joints is an important and characteristic clinical finding that can lead to its diagnosis. Radiologists and clinicians must be aware of synovitis-acne-pustulosis-hyperostosis-osteitis syndrome as it can mimic some of the more common disease entities such as Paget\'s disease. We report the case of a 63-year-old male patient, with no significant medical history, who presented to the dermatology department, with severe palmar and plantar pustulosis associated with polyarthralgia. Computerized tomography scan showed sternoclavicular hyperostosis, in favor of SAPHO syndrome, with regression of clinical symptoms after non-steroidal anti-inflammatory drug treatment.
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  • 文章类型: Journal Article
    描述了来自Niobrara组的烟熏山粉笔成员的一系列12个连续的鱼形尾椎骨。椎骨中心表现出病理性骨的广泛过度生长,并且在中心和椎间隙内有其他病理性骨。这共同导致了大多数centra的骨化。病理范围在硬膜前椎骨1-3上最大,向前减小,这表明病理学开始向后发展并向前发展。除了骨骼上的病理性过度生长,标本保留了被解释为与神经和血管相关的钙化和/或骨化软组织的特征。病理与先前描述的鱼类骨骼病理的例子不同,有人认为它们是由细菌和真菌感染引起的。随着病理的发展,它们会对鱼的游泳和喂食能力产生不利影响,大概最终导致了鱼的死亡。
    A series of 12 contiguous caudal vertebrae of an ichthyodectiform fish from the Smoky Hill Chalk Member of the Niobrara Formation is described. The vertebral centra exhibit extensive overgrowth of pathological bone and there is additional pathological bone within the centra and intervertebral spaces, which together resulted in the coossification of most centra. The extent of the pathology is greatest on preural vertebrae 1-3 and decreases anteriorly, which suggests that the pathology began posteriorly and progressed anteriorly. In addition to the pathological overgrowth on bones, the specimen preserves features interpreted as calcified and/or ossified soft tissues associated with the neural and haemal canals. The pathologies are unlike previously described examples of bony pathologies in fish, and it is suggested that they resulted from combined bacterial and fungal infections. As the pathologies developed, they would have adversely impacted the fish\'s swimming and feeding abilities, and presumably eventually led to the fish\'s death.
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
    En斑块状脑膜瘤是一种罕见的脑膜瘤,具有浸润性,片状生长,有时侵入骨头。我们在这里报告一例斑块状脑膜瘤。患者是一名66岁的女性,表现为头痛和双侧眼球突出疼痛。脑磁共振成像显示双侧斑块状脑膜瘤,表现为双侧大翼蝶骨骨增生,伴有双侧颞前区和斜坡后区域的硬脑膜增厚和增强,两侧侵入海绵窦,视神经的交叉前部分,和眼眶外侧直肌通过眶上裂引起双侧眼球突出症。由于海绵窦和眶尖的侵入,小量但广泛的切除结合上眶裂和视神经管的骨性减压,然后进行辅助放疗通常会产生良好的功能和美容效果。在三年的时间里,随访磁共振成像扫描显示无明显复发征象。
    En plaque meningioma is a rare type of meningioma characterized by an infiltrative nature, sheet-like growth, and at times invading the bone. We report here a case of en plaque meningioma. The patient was a 66-year-old woman presenting with headache and painful bilateral proptosis. Cerebral magnetic resonance imaging revealed a bilateral en plaque meningioma showed as a bilateral hyperostotic of greater wing sphenoid bone associated with bilateral thickening and enhancement of the dura in the anterior temporal area and the retroclival region invading bilaterally the cavernous sinus, the prechiasmatic portion of the optic nerve, and the lateral rectus muscle of the orbit through the superior orbital fissure causing bilateral exophthalmia. Due to invasion of the cavernous sinus and the orbital apex, a subtotal but extensive removal combined with bony decompression of the cranial nerves at the superior orbital fissure and optic canal followed by adjuvant radiotherapy frequently produces good functional and cosmetic results, and over a 3-year period, follow-up magnetic resonance imaging scans showed no obvious signs of recurrence.
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  • 文章类型: Journal Article
    教学要点:肋骨良性肥厚是由应力现象组成的良性实体,不应与Paget混淆,纤维发育不良,或成骨细胞转移。
    Teaching point: Benign hyperostosis of the rib is a benign entity consisting of a stress phenomenon that should not be confused with Paget, fibrous dysplasia, or osteoblastic metastasis.
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  • 文章类型: Case Reports
    背景:石骨症是一种罕见的遗传性疾病,可以通过常染色体隐性遗传或常染色体显性遗传传播。
    方法:这里,我们报告了一个18岁男孩的股骨转子骨折病例,该病例带有解剖钢板。在最后一次随访中,手术后24个月,骨折愈合良好,患者活动不受限制。
    结论:石骨病是一种罕见的骨疾病,主要由破骨细胞功能障碍引起。它是由导致骨骼过度矿化的重塑缺陷引起的,导致骨骼脆弱。手术和非手术治疗各有优缺点。因此,切开复位和解剖钢板内固定仍然是治疗骨结石患者股骨转子骨折的有效方法。
    结论:对于我们的患者,如文献中所述,随着骨质疏松性骨折的巩固,一些原则得到尊重,并发症发生率降低。
    BACKGROUND: Osteopetrosis is a rare hereditary disease that can be transmitted in an autosomal recessive or autosomal dominant.
    METHODS: Here, we report a case of trochanteric fracture in an 18-year-old boy with an anatomical plate. At the last follow-up, 24 months after surgery, the fracture had healed well, and the patient was not restricted in his activities.
    CONCLUSIONS: Osteopetrosis is a rare bone disease that is mainly caused by osteoclast dysfunction. It results from a remodelling defect that leads to hypermineralization of the skeleton, resulting in bone fragility. Both surgical and nonsurgical management have advantages and disadvantages. Thus, open reduction and anatomic plate fixation remain effective management modalities for trochanteric fractures in osteopetrosis patients.
    CONCLUSIONS: For our patient and as described in the literature, the complication rate decreases as some principles are respected with better consolidation of the osteoporotic fracture.
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  • 文章类型: Journal Article
    报告我们对患者特异性植入物进行单步眼眶重建的经验,并通过三例手术病例描述该技术的发展。方法:描述了3例一步SWM去除和眼眶重建的病例。连续给出所有情况以描述技术的发展。电磁导航和切割引导器(模板)促进了骨肥大性骨切除。基于3D模型,使用CAD/CAM制造硅胶模具。然后由这些模具制造PMMA植入物。切除肿瘤后,用钛螺钉将植入物调整并固定在颅骨上。结果:以下步骤的程序改变了这些系列:骨增生切除,植入物厚度控制,植入物重叠特征,解剖调整,植入物固定。在所有情况下都解决了突起。在随访期间,一名患者的视力逐渐下降。随访中未发现动眼神经紊乱和肿瘤再生长。
    CAD/CAM技术可以创建任何尺寸和配置的植入物,因此,增加骨切除的程度,降低肿瘤进展的风险。该程序在一个步骤中进行,从而降低了术后发病的风险。
    UNASSIGNED: To report our experience with patient specific implants for one-step orbit reconstruction following hyperostotic SWM removal and to describe the evolution of the technique through three surgical cases. Methods: Three cases of one-step SWM removal and orbit reconstruction are described. All cases are given consecutively to describe the evolution of the technique. Hyperostotic bone resection was facilitated by electromagnetic navigation and cutting guides (templates). Based on a 3D model, silicone molds were made using CAD/CAM. Then PMMA implant was fabricated from these molds. The implant was adjusted and fixed to the cranium with titanium screws after tumor removal. Results: Following steps of the procedure changed over these series: hyperostotic bone resection, implant thickness control, implant overlay features, anatomic adjustments, implant fixation. The proptosis resolved in all cases. In one patient the progressive visual acuity deterioration was recognized during the follow-up. No oculomotor disturbances and no tumor regrowth were found at the follow-up.
    UNASSIGNED: CAD/CAM technologies enable creation of implants of any size and configuration, and thereby, to increase the extent of bony resection and lower the risk of tumor progression. The procedure is performed in one step which decreases the risk of postoperative morbidity.
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  • 文章类型: Journal Article
    两名女性(FL1,FL2)和一名男性(ML)11周龄,完好无损,圈养的非洲狮子幼崽(Pantheraleoleo)具有轻度前庭体征的历史。ML的初始血清维生素A浓度较低(140nmol/L)。使用每个幼崽的头部和颈椎的计算机断层扫描(CT)证实了颅骨增生。CT测量根据颅骨宽度进行调整。ML显示小脑和枕骨的最明显增厚,小脑小脑与颅骨宽度比(TCR)为0.08(FL1:0.06,FL2:0.05),基底蝶骨与颅骨宽度比(BBR)为0.07(FL1:0.06,FL2:0.04)。磁共振成像(MRI)显示小脑疝和C1的颈髓内T2加权高强度,在所有幼崽中至少有两个颈椎向尾延伸。通过皮下补充维生素A并喂养整个尸体开始治疗。3周后发现共济失调的改善。在3和8个月后的ML中进行CT和MRI检查。3个月后,受影响的骨骼厚度略有减少,TCR和BBR下降至0.05。小脑仍然轻度突出,伴有宫颈T2w高信号的改善。早上8点之后,评估和诊断成像显示,尽管存在轻微的小脑疝,但在神经系统状态和测量方面(TCR0.05,BBR0.04)进一步改善。总之,在出现颅骨肥大的幼狮幼崽中,骨重建和临床体征的改善是可以实现的,这可能归因于高剂量维生素A的补充。
    Two female (FL 1, FL 2) and one male (ML) 11-wk-old, intact, captive African lion cubs (Panthera leo leo) were presented with a history of mild vestibular signs. Initial serum vitamin A concentrations were low (140 nmol/L) for ML. Calvarial hyperostosis was confirmed using computed tomography (CT) of the head and cervical vertebrae in each cub. CT measurements were adapted in relation to the skull width. ML showed the most pronounced thickening of the tentorium cerebelli and occipital bone, represented by a tentorium cerebelli to skull width ratio (TCR) of 0.08 (FL 1: 0.06, FL 2: 0.05) and a basisphenoid to skull width ratio (BBR) of 0.07 (FL 1: 0.06, FL 2: 0.04). Magnetic resonance imaging (MRI) revealed cerebellar herniation and cervical intramedullary T2-weighted hyperintensity from C1, extending caudally for at least two cervical vertebrae in all cubs. Treatment was initiated with subcutaneous vitamin A supplementation and feeding of whole carcasses. Improvement in ataxia was noticed 3 wk later. Follow-up CT and MRI examinations were performed in ML after 3 and 8 mon. The affected bones appeared slightly less thickened and TCR and BBR had decreased to 0.05 after 3 mon. The cerebellum remained mildly herniated, accompanied by amelioration of cervical T2w hyperintensities. After 8 mon, evaluation and diagnostic imaging revealed further improvement regarding the neurologic status and measurements (TCR 0.05, BBR 0.04) despite persistence of a subtle cerebellar herniation. In conclusion, bone remodeling and improvement in clinical signs may be achievable in young lion cubs presented with calvarial hyperostosis and may be attributable to high-dose vitamin A supplementation.
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  • 文章类型: Editorial
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  • 文章类型: Journal Article
    化脓性汗腺炎(HS)是一种终末毛囊的自身炎症性皮肤病,可以零星存在,家族性,或综合征形式。已经对后者提出了分类,区分与已知遗传状况相关的病例,滤泡角质化疾病或自身炎性疾病。这篇综述侧重于这些实体的临床和遗传特征(即,坏疽性脓皮病[PG],痤疮和HS;PG,痤疮,化脓性关节炎和HS;银屑病关节炎,PG,痤疮和HS;滑膜炎,痤疮,脓疱病,骨增生,骨炎;等等),提出了与HS相关的自身炎症综合征的统称。
    Hidradenitis suppurativa (HS) is an autoinflammatory skin disorder of the terminal hair follicle, which can present in sporadic, familial, or syndromic form. A classification has been proposed for the latter, distinguishing cases associated with a known genetic condition, with follicular keratinization disorders or with autoinflammatory diseases. This review focuses on the clinical and genetic features of those entities (ie, pyoderma gangrenosum [PG], acne and HS; PG, acne, pyogenic arthritis and HS; psoriatic arthritis, PG, acne and HS; synovitis, acne, pustulosis, hyperostosis, osteitis; and so forth) for which the collective term HS-related autoinflammatory syndromes is proposed.
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