Eosinophilic granuloma

嗜酸性肉芽肿
  • 文章类型: Case Reports
    背景:嗜酸性肉芽肿(EG)是一种影响骨组织细胞的增殖性疾病。在疾病的早期阶段没有特定的临床体征或影像学表现,容易忽视和误诊。因为这种疾病的稀有性,目前没有标准化的治疗原则。很少有关于此类事件影响儿童轴心的报道。我们发现一例儿童的EG导致寰枢关节脱位和轴向骨破坏。
    方法:在没有明确解释的情况下出现咽部不适超过六个月后,一个6岁的男孩被送到我们医院。经过仔细评估,病理提示轴性EG的可能性很大。最终,我们决定用后路椎弓根螺钉固定和局部类固醇注射治疗这个男孩。
    结论:上颈椎的EGs在儿童中并不常见,它们非常容易被忽视或误诊。后路椎弓根螺钉固定和局部类固醇注射是影响寰枢关节的轴向EGs患者的有效治疗方法。
    BACKGROUND: Eosinophilic granuloma (EG) is a proliferative condition that affects the cells of bone tissue. There are no specific clinical signs or imaging manifestations in the early stages of the disease, making it simple to overlook and misdiagnose. Because of the disease\'s rarity, there is presently no standardized treatment principle. There are few accounts of such occurrences affecting the axis among children. We discovered a case of a child whose EG resulted in atlantoaxial joint dislocation and destruction of the axial bone.
    METHODS: After having pharyngeal discomfort for more than six months without a clear explanation, a 6-year-old boy was brought to our hospital. Following a careful evaluation, the pathology indicated a strong likelihood of an axial EG. Ultimately, we decided to treat the boy with posterior pedicle screw fixation and local steroid injections.
    CONCLUSIONS: EGs of the upper cervical spine are quite uncommon in children, and they are exceedingly easy to overlook or misdiagnose. Posterior pedicle screw fixation and local steroid injections are effective treatments for patients with axial EGs affecting the atlantoaxial junction.
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  • 文章类型: Case Reports
    背景:嗜酸性肉芽肿(EG)-朗格汉斯细胞组织细胞增生症的最常见形式-很少发生,仅肋骨和锁骨受累的表现极为罕见。EG症状通常包括疼痛,肿胀,和软组织肿块。骨EG的临床诊断复杂,鉴别诊断包括尤因肉瘤,结核病,多发性骨髓瘤,淋巴瘤原发性骨恶性肿瘤,和其他溶骨性病变。
    方法:患者是一名11岁女性,在就诊前2天发现右锁骨和胸骨交界处皮下肿块,无明显诱因。最初,我们考虑皮下囊肿或炎性肿块。彩色超声和计算机断层扫描检查显示骨髓炎。最后,患者在病理组织活检后被诊断为EG,经手术和抗感染治疗后,患儿康复。
    方法:患者在专科医院行手术切除肿瘤,经病理检查确诊为EG。
    方法:患者到专科医院手术切除肿块,并接受抗感染治疗。
    结果:患者在手术切除和抗生素治疗后康复。
    结论:在本报告中,我们强调EG在儿童中的临床表现并不具有特异性.此外,检查年龄,历史,存在的症状,这些部位的数量对于做出正确的诊断至关重要,组织学检查是必要的,以确认诊断。
    BACKGROUND: Eosinophilic granuloma (EG) - the most common form of Langerhans cell histiocytosis - occurs rarely, and manifestations with only rib and clavicle involvement are extremely rare. EG symptoms often include pain, swelling, and soft tissue mass. The clinical diagnosis of bone EG is complex, and the differential diagnosis includes Ewing sarcoma, tuberculosis, multiple myeloma, lymphoma, primary bone malignancy, and other osteolytic lesions.
    METHODS: The patient was an 11-year-old female who found a subcutaneous mass at the junction of the right clavicle and sternum 2 days before presenting at the clinic without apparent triggers. Initially, we considered a subcutaneous cyst or inflammatory mass. Color ultrasound and computed tomography examination revealed osteomyelitis. Finally, the patient was diagnosed with EG after a pathological tissue biopsy, and the child recovered after surgery and anti-infective treatment.
    METHODS: The patient underwent surgery to remove the tumor at a specialist hospital and was diagnosed with EG by pathological examination.
    METHODS: The patient went to a specialist hospital for surgery to remove the mass and underwent anti-infective treatment.
    RESULTS: The patient recovered after surgical resection and antibiotic treatment.
    CONCLUSIONS: In this report, we emphasize that the clinical presentation of EG in children is not specific. Furthermore, examining age, history, presence of symptoms, and the number of sites is essential to make a correct diagnosis, and a histological examination is necessary to confirm the diagnosis.
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  • 文章类型: Journal Article
    UNASSIGNED:评估椎体后凸成形术结合短期后路器械治疗因嗜酸性肉芽肿(EG)引起的椎骨平面患儿的有效性和安全性。
    未经证实:EG患者的临床资料,对2019年3月至2020年3月接受椎体后凸成形术和短期器械治疗的患者进行回顾性分析.评估了病变椎骨的恢复情况,并与历史病例数据进行了比较。
    未经授权:9例EG患者接受了椎体后凸成形术和短期后路器械治疗。初始治疗的平均年龄为66.7个月(范围,28-132个月)。平均随访月数为26.7个月。(范围,24-30个月)。4例和5例表现为胸腰椎破坏,分别。根据Garg\的分类,7和2例分为IIA和IIB级,分别。术后1年和2年的平均病变椎体高度明显高于术前高度。术后1年和2年病变椎体高度占参考的平均百分比分别为72.0%和86.0%,分别。手术后2年,病变椎体高度占参考的平均百分比显着高于同期的历史病例。没有观察到轻微或主要的不良事件。
    UNASSIGNED:经椎弓根球囊椎体后凸成形术与短期后路器械结合用于直接修复椎骨平面似乎是可行且安全的。手术后2年观察到更好的短时间椎骨重塑。建议对因EG而患有椎骨平面的儿童进行积极的手术治疗,以保持恢复椎骨高度的能力。
    UNASSIGNED: To assess the validity and safety of kyphoplasty combined with short-term posterior instrumentation to treat children with vertebrae plana due to eosinophilic granuloma (EG).
    UNASSIGNED: Clinical data of EG patients, who received kyphoplasty and short-term instrumentation from March 2019 to March 2020, were retrospectively reviewed. The recovery of diseased vertebrae was assessed and compared with historical case data.
    UNASSIGNED: Nine patients with EG had received kyphoplasty and short-term posterior instrumentation. The mean age at initial treatment was 66.7 months old (range, 28-132 months). The average number of follow-up months was 26.7. (range, 24-30 months).Four and 5 cases presented with thoracic and lumbar vertebral destruction, respectively. Under Garg\'s classification, 7 and 2 cases were classified as Grade IIA and IIB, respectively. The average diseased vertebral heights at 1-year and 2-year after surgery were significantly higher than the preoperative heights. The average percentages of diseased vertebral heights to references at 1-year and 2-year after surgery were 72.0% and 86.0%, respectively. The average percentage of diseased vertebral heights to the references at 2-year after surgery was significantly higher than that of the historical cases at the same time. No minor or major adverse events were observed.
    UNASSIGNED: Transpedicular balloon kyphoplasty for the direct restoration of vertebrae plana seems feasible and safe in combination with short-term posterior instrumentation. Better short-time vertebrae remodeling was observed 2 years after surgery. Active surgical treatment is suggested for children who have vertebrae plana as a result of EG in order to maintain the ability to recover vertebral height.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    朗格汉斯细胞组织细胞增生症(LCH)是一种疾病谱,其特征是朗格汉斯细胞在受影响的组织中大量浸润/积累。嗜酸性肉芽肿是LCH的最温和形式,主要影响颅骨,下颌骨,儿童和年轻人的肋骨和股骨,但很少发生在上颌骨。这里,我们描述了一个35岁男性的嗜酸性肉芽肿异常病例,该病例影响了25号牙齿和46号牙齿(FDI编号系统)附近的组织。虽然病灶在局部放疗后得到临床缓解,第18号牙齿在初始治疗后11个月出现新的病变,表明嗜酸性肉芽肿复发。随后,患者接受了皮质类固醇治疗,在3年的随访后实现了缓解且无病。
    Langerhans\' cell histiocytosis (LCH) is a disease spectrum characterized by the intense infiltration/accumulation of Langerhans\' cells in the affected tissues. Eosinophilic granuloma is the mildest form of LCH which mainly affects the skull, mandible, ribs and femur in children and young adults but rarely occurs in the maxilla. Here, we describe an unusual case of eosinophilic granuloma affecting the tissues adjacent to tooth #25 and tooth #46 (FDI numbering system) in a 35-year-old male. Although lesions underwent clinical remission after local radiotherapy, a new lesion appeared 11 months after initial therapy in tooth #18, indicating recurrence of eosinophilic granuloma. Subsequently, the patient was treated with corticosteroid therapy, achieved remission and is disease free after 3 years of follow-up.
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  • 文章类型: Case Reports
    Eosinophilic granuloma, a rare disease, has various clinical manifestations and no specific X-rays features and is thus easily misdiagnosed. This paper reports a case of multifocal eosinophilic granuloma of jaw with long-term follow-up. The patient initially presented with periodontal tissue destruction.The diagnosis, treatment and prognosis of multifocal eosinophilic granuloma of jaw were discussed in combination with the literature to alert this disease in clinical practice.
    嗜酸性肉芽肿是一种较罕见的疾病,其临床表现多样,影像学无特异性,临床中易误诊。现对1例起初以牙周组织破坏为表现的颌骨多发性嗜酸性肉芽肿的长期追踪情况进行报道,并结合文献对其临床表现、诊断、治疗及预后等进行讨论,以提高对该疾病的认识与诊治水平。.
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  • 文章类型: Journal Article
    UNASSIGNED: Retrospective case series.
    UNASSIGNED: To evaluate the efficacy of growth-preserving posterior spinal column reconstruction surgery in children with collapsed vertebral eosinophilic granuloma (EG).
    UNASSIGNED: We retrospectively reviewed 15 confirmed cases of vertebral EG who met the inclusion and exclusion criteria from March 2015 to March 2018 in our hospital. The recovery of diseased vertebrae was assessed.
    UNASSIGNED: The cases were composed of 11 males and 4 females with a mean age of 74.3 ± 38.8 months. Nine cases presented with thoracic vertebral destruction, 6 cases with lumbar vertebral destruction. Under Garg\'s classification, 10, 3 and 2 cases were classified as Grade IIA, IB and IIB lesions, respectively. All patients had mild to moderate night-aggravated back pain and recovered at 1-week after surgery. Three patients had Frankel D neurologic symptoms and recovered at 1-week, 1-week and 4-weeks after surgery, respectively. Follow-up time after surgery ranged from 1.9 years to 4.5 years, with a mean of 2.9 years. The median vertebral height at 12 months after surgery was significantly higher than the preoperative height. Furthermore, the vertebral heights of all diseased vertebrae displayed significant recovery trends at 3, 6 and 12 months after surgical treatment. The percentages of vertebral height recovery to references at 12 months after surgery ranged from 34.7% to 92.5%, with an average of 71.2%.
    UNASSIGNED: In children with collapsed vertebral EGs, active surgical treatment is necessary to retain the ability to restore height and to reconstruct the spine stability.
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  • 文章类型: Journal Article
    BACKGROUND: Acetabular roof lesions (ARLs) in children are uncommon and may involve a variety of diseases. The acetabular roof is the main weight-bearing area of the hip joint, and lesions affecting the acetabular roof lead to fluid accumulation in the hip joint, causing hip pain and claudication. Methods for diagnosing and treating ARLs and the prognosis after treatment are rarely reported. We present our experience in a group of children and teenagers with ARLs to retrospectively explore the clinical and imaging features and histopathological diagnosis and report the treatment methods and follow-up observations.
    METHODS: Patients with ARLs admitted to the Children\'s Hospital of Chongqing Medical University from April 2011 to September 2018 were selected retrospectively. We collected the basic information of patients (name, sex, age), main symptoms and signs, results of various laboratory tests, treatment methods, and intraoperative observations through the hospital medical record system. We collected X-ray, computed tomography (CT), magnetic resonance imaging (MRI) and pathological examination data through the Picture Archiving and Communications System. Follow-up data were collected through an outpatient medical record system, telephone, and chat software (such as WeChat). We used descriptive methods to analyze the lesion structure and destruction mode based on the imaging findings and histopathological diagnosis.
    RESULTS: There were 14 ARL patients, including 6 with eosinophilic granuloma (EG), 5 with chronic osteomyelitis, 2 with bone cyst, and 1 with tuberculosis. One patient underwent percutaneous needle biopsy, 2 underwent open biopsy, and 11 underwent curettage; among them, 5 patients also underwent bone grafting. These lesions had no characteristic imaging findings, and the diagnosis was mainly based on histopathological examination. Most patients showed complete symptom resolution and good hip function at the 1-year follow-up.
    CONCLUSIONS: ARLs are not common in children. The types of lesions are diverse and mostly benign, with EG being most common. Malignant tumors may also occur, such as Ewing\'s sarcoma, non-Hodgkin\'s lymphoma, metastases and neuroblastoma. CT and MRI can be helpful in diagnosing certain cases, but incisional biopsy is required in most cases.
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  • 文章类型: Journal Article
    Objective: To analyze the clinical and prognostic characteristics in patients with eosinophilic granulomatosis with polyangitis (EGPA). Methods: The clinical data of 146 EGPA patients hospitalized in Peking Union Medical College Hospital from 2000 to 2019 were analyzed retrospectively, including clinical manifestations, laboratory results, treatment, complications and outcome at discharge. Birmingham Vasculitis activity score-V3 (BVAS-V3) was used to evaluate disease activity. Results: The ratio of male to female was 1.8∶1 with average age (41.7±16.1) year-old. The median time from disease onset to diagnosis was 18(6, 60) months (0.5~450). The most common clinical manifestations were lung [121(82.9%)] and nose/paranasal sinuses [119(81.5%)] involvement. The positive rate of anti-neutrophil cytoplasmic antibody (ANCA) was 24.7%, mainly peripheral (P)-ANCA/myeloperoxidase (MPO)-ANCA. Compared with ANCA-negative patients, the ANCA-positive patients had a higher incidence of renal involvement and nervous system involvement (66.7% vs. 20.9%, 80.6% vs. 51.8%, P<0.001), fever and optic neuropathy (66.7% vs. 40.9%,8.3% vs. 0, P<0.05), more active disease [median BVAS-V3 25(18,30)vs. 19(14,24),P=0.001] and more elevated erythrocyte sedimentation rate [40.5(20.5,82.8)mm/1h vs. 25.0(13.3,50.8)mm/1h,P=0.006] and C-reactive protein [37.1(11.8,72.9)mg/L vs.13.5(3.4,66.1)mg/L,P=0.036]. More ANCA-negative patients had pleural effusion (20.9% vs. 5.6%, P<0.04) compared with ANCA-negative patients. Pulmonary infection was the most common complication. A total of 12 EGPA patients (8.2%)achieved remission and 6 patients (4.1%)died or discharged themselves from the hospital. Conclusion: EGPA is a rare small vessel vasculitis. The clinical manifestations and outcomes are heterogenous. The mortality rate of EGPA is high.
    目的: 分析和总结嗜酸性肉芽肿性多血管炎(EGPA)的临床特征。 方法: 收集2000年至2019年北京协和医院确诊的146例EGPA住院患者资料,回顾性分析其临床表现、实验室检查、辅助检查、治疗、并发症及转归。 结果: 146例患者中男93例,女53例,年龄(41.7±16.1)岁,发病至确诊时间为0.5~450个月,中位时间18(6,60)个月。以肺部[121例(82.9%)]、鼻与鼻窦[119例(81.5%)]受累最多见;血清抗中性粒细胞胞质抗体(ANCA)阳性者36例(24.7%),以核周型或髓过氧化物酶ANCA为主。ANCA阳性者肾脏(66.7%比20.9%,P<0.001)、神经系统受累比例(80.6%比51.8%,P<0.001)明显高于ANCA阴性者,更易出现发热(66.7%比40.9%,P<0.05)及视神经病变(8.3%比0,P<0.05),疾病活动度更高[中位伯明翰血管炎活动度评分25(18,30)比19(14,24),P=0.001],红细胞沉降率[40.5(20.5,82.8)mm/1 h比25.0(13.3,50.8)mm/1 h,P=0.006]和超敏C反应蛋白[37.1(11.8,72.9)mg/L比13.5(3.4,66.1)mg/L,P=0.036]显著升高;而ANCA阴性者胸腔积液比例高于ANCA阳性者(20.9%比5.6%,P=0.04)。治疗以糖皮质激素和环磷酰胺为主。12例(8.2%)患者病情缓解,6例(4.1%)患者死亡或放弃治疗。并发症以肺部感染最多见。 结论: EGPA是一种少见的血管炎,临床表现与转归异质性强,病死率高。.
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  • 文章类型: Case Reports
    Eosinophilic granuloma (EG) is a benign bone tumor of unknown origin usually seen in children. It is typically found in flat and long bones of the skull and of the appendicular skeleton, respectively. Small bones are rarely affected. The diagnosis and treatment of EG can be challenging as differential diagnosis includes several disorders that can cause osteolytic lesions. Moreover, surgical treatment can be difficult due to the small size of the bone and surrounding structures. Here we describe a case of EG of the proximal phalanx of the thumb (P1 D1) presenting in a 4-month-old boy with persistent swelling and limited range-of-motion of the interphalangeal joint in the left thumb. Over a 6-year follow-up, no sign of relapse was observed. Moreover, the curetted bone did regenerate, and it appeared homogenously dense as normal trabecular bone on the last plain radiographs; the patient was symptom-free at the last follow-up visit. Clinical presentation, treatment, pathoanatomy, localization, and complication of this form of tumor are discussed, and all previously reported cases are reviewed to give a more comprehensive picture of EG of the hand and fingers.
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