Ameloblastoma

成釉细胞瘤
  • 文章类型: Journal Article
    目的:这项贝叶斯网络荟萃分析旨在分析成釉细胞瘤(AM)患者的临床病理特征与BRAF突变之间的关联,并评估诊断准确性。
    方法:从2010年至2024年检索了四个电子数据库。使用的搜索词特定于BRAF和AM。观察性研究或随机对照试验被认为是合格的。对AM患者BRAF基因突变的发生率及相应的临床病理特征进行贝叶斯网络分析和诊断准确性评价。
    结果:共纳入20项研究的937名AM患者。AM患者BRAF突变的合并患病率为72%。根据贝叶斯网络分析,BRAF突变更有可能发生在年轻人中(比值比[OR],2.3;可信区间[CrI]:1.2-4.5),下颌骨部位(或,3.6;95%CrI:2.7-5.2),和独症(或者,1.6;95%CrI:1.1-2.4)AM患者。同样,在年轻人中发现了更高的诊断准确性,下颌骨,和单纯性AM组。
    结论:发病率,风险,在年轻患者中,AM中BRAF突变的诊断准确性更高,那些有下颌骨参与的人,与单囊AM患者相比,其他临床病理特征患者。此外,分子检测和免疫组织化学分析在诊断准确性上有很强的一致性.
    OBJECTIVE: This Bayesian network meta-analysis was performed to analyze the associations between clinicopathological characteristics and BRAF mutations in ameloblastoma (AM) patients and to evaluate the diagnostic accuracy.
    METHODS: Four electronic databases were searched from 2010 to 2024. The search terms used were specific to BRAF and AM. Observational studies or randomized controlled trials were considered eligible. The incidence of BRAF mutation and corresponding clinicopathological features in AM patients were subjected to Bayesian network analyses and diagnostic accuracy evaluation.
    RESULTS: A total of 937 AM patients from 20 studies were included. The pooled prevalence of BRAF mutations in AM patients was 72%. According to the Bayesian network analysis, BRAF mutations are more likely to occur in younger (odds ratio [OR], 2.3; credible interval [CrI]: 1.2-4.5), mandible site (OR, 3.6; 95% CrI: 2.7-5.2), and unicystic (OR, 1.6; 95% CrI: 1.1-2.4) AM patients. Similarly, higher diagnostic accuracy was found in the younger, mandible, and unicystic AM groups.
    CONCLUSIONS: The incidence, risk, and diagnostic accuracy of BRAF mutation in AM were greater in younger patients, those with mandible involvement, and those with unicystic AM than in patients with other clinicopathological features. In addition, there was a strong concordance in the diagnostic accuracy between molecular tests and immunohistochemical analysis.
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  • 文章类型: English Abstract
    UNASSIGNED: To explore the feasibility and effectiveness of mixed reality technology for localizing perforator vessels in the repair of mandibular defects using free fibular flap.
    UNASSIGNED: Between June 2020 and June 2023, 12 patients with mandibular defects were repaired with free fibular flap. There were 8 males and 4 females, with an average age of 61 years (range, 35-78 years). There were 9 cases of ameloblastomas and 3 cases of squamous cell carcinomas involving the mandible. The disease duration ranged from 15 days to 2 years (median, 14.2 months). The length of mandibular defects ranged from 5 to 14 cm (mean, 8.5 cm). The area of soft tissue defects ranged from 5 cm×4 cm to 8 cm×6 cm. Preoperative enhanced CT scans of the maxillofacial region and CT angiography of the lower limbs were performed, and the data was used to create three-dimensional models of the mandible and lower limb perforator vessels. During operation, the mixed reality technology was used to overlay the three-dimensional model of perforator vessels onto the body surface for harvesting the free fibular flap. The length of the fibula harvested ranged from 6 to 15 cm, with a mean of 9.5 cm; the size of the flap ranged from 6 cm×5 cm to 10 cm×8 cm. The donor sites were sutured directly in 7 cases and repaired with free skin grafting in 5 cases.
    UNASSIGNED: Thirty perforator vessels were located by mixed reality technology before operation, with an average of 2.5 vessels per case; the distance between the exit point of the perforator vessels located before operation and the actual exit point ranged from 1 to 4 mm, with a mean of 2.8 mm. All fibular flaps survived; 1 case had necrosis at the distal end of flap, which healed after dressing changes. One donor site had infection, which healed after anti-inflammatory dressing changes; the remaining incisions healed by first intention, and the grafts survived smoothly. All patients were followed up 8-36 months (median, 21 months). The repaired facial appearance was satisfactory, with no flap swelling. Among the patients underwent postoperative radiotherapy, 2 patients had normal bone healing and 1 had delayed healing at 6 months.
    UNASSIGNED: In free fibular flap reconstruction of mandibular defects, the use of mixed reality technology for perforator vessel localization can achieve three-dimensional visualization, simplify surgical procedures, and reduce errors.
    UNASSIGNED: 探讨在游离腓骨皮瓣修复下颌骨缺损中,使用混合现实技术定位穿支血管的可行性和效果。.
    UNASSIGNED: 2020年6月—2023年6月,采用游离腓骨皮瓣修复12例下颌骨缺损患者。男8例,女4例;年龄35~78岁,平均61岁。下颌骨成釉细胞瘤9例,侵犯下颌骨口腔鳞癌3例。病程15 d~2年,中位病程14.2个月。下颌骨缺损长度5~14 cm,平均8.5 cm;软组织缺损范围为5 cm×4 cm~8 cm×6 cm。术前均行颌面部增强CT和下肢CT血管造影检查,将所得数据制成下颌骨、下肢穿支血管三维模型。术中使用混合现实技术将穿支血管三维模型重叠于患者体表,制取游离腓骨皮瓣修复缺损。腓骨切取长度为6~15 cm,平均9.5 cm;皮瓣切取范围6 cm×5 cm~10 cm×8 cm,供区拉拢缝合(7例)或游离植皮修复(5例)。.
    UNASSIGNED: 12例患者术前使用混合现实技术定位穿支血管30支,每例平均2.5支;术中测量术前定位穿支血管穿出点与实际穿出点距离为1~4 mm,平均2.8 mm。术后12例腓骨瓣顺利成活;1例皮瓣远端边缘坏死,换药后延期愈合。供区发生感染1例,经抗炎换药治疗后愈合;其余患者切口Ⅰ期愈合,植皮顺利成活。患者均获随访,随访时间8~36个月,中位时间21个月。修复面部外形好,皮瓣无臃肿。3例术后接受放化疗患者中,随访6个月时2例正常骨愈合、1例延迟愈合。.
    UNASSIGNED: 在游离腓骨皮瓣修复下颌骨缺损手术中使用混合现实技术,可以实现穿支血管三维可视化,手术操作简便,误差较小。.
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  • 文章类型: Journal Article
    靶向治疗有可能用于牙源性肿瘤的新辅助治疗,减少与大手术相关的发病率。在这方面,这项研究的目的是总结目前不同形式的靶向治疗的证据,有效性,和这个疗程的弊端。以电子方式搜索了四个数据库,而不考虑出版日期或语言。还进行了灰色文献检索和手动检索。需要有足够的关于牙源性肿瘤靶向治疗的临床数据的出版物来满足资格标准。对数据的分析是描述性的。共包括15篇论文,包括17例(15例成釉细胞瘤和2例成釉细胞癌)。发现了许多突变,与BRAFV600E是最常见的。Dabrafenib是靶向治疗中最常用的药物。除了一个案子,治疗减少了病变的大小(16/17例),显示承诺。记录的大多数不良事件都是轻度的,比如皮肤问题,声音变化,头发纹理异常,干眼,和全身症状(例如,疲劳,关节痛,和恶心)。结论:成釉细胞瘤和成釉细胞癌的靶向治疗可能是一种有用的治疗策略,根据纳入研究的结果。
    Targeted therapy has the potential to be used in the neoadjuvant setting for odontogenic tumors, reducing the morbidities associated with major surgery. In this regard, the aim of this study was to summarize the current evidence on the different forms of targeted therapy, effectiveness, and drawbacks of this course of treatment. Four databases were searched electronically without regard to publication date or language. Grey literature searches and manual searches were also undertaken. Publications with sufficient clinical data on targeted therapy for odontogenic tumors were required to meet the criteria for eligibility. The analysis of the data was descriptive. A total of 15 papers comprising 17 cases (15 ameloblastomas and 2 ameloblastic carcinomas) were included. Numerous mutations were found, with BRAF V600E being most common. Dabrafenib was the most utilized drug in targeted therapy. Except for one case, the treatment reduced the size of the lesion (16/17 cases), showing promise. Most of the adverse events recorded were mild, such as skin issues, voice changes, abnormal hair texture, dry eyes, and systemic symptoms (e.g., fatigue, joint pain, and nausea). It is possible to reach the conclusion that targeted therapy for ameloblastoma and ameloblastic carcinoma may be a useful treatment strategy, based on the findings of the included studies.
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  • 文章类型: Journal Article
    这项多中心研究的目的是评估“疏通-袋化-刮治”(D-M-C)策略在治疗下颌骨常规骨内成釉细胞瘤中的疗效。共有来自三个机构的31名患者,病理诊断为下颌骨常规成釉细胞瘤,用D-M-C策略治疗。手术方案包括疏浚和袋化(D-M)步骤,根据需要附加D-M步骤。一旦在随访期间实现了D-M步骤的明显效果,则患者进行刮宫(C)。8例患者随访时间≥36个月但<60个月,23例随访时间≥60个月。随访≥60个月的23例患者中,有19例末次随访时无病。没有复发的证据.D-M步骤对于减小肿瘤大小和保留重要结构是有效的。D-M-C手术策略可能是下颌骨常规成釉细胞瘤的可行治疗选择。
    The purpose of this multicentre study was to evaluate the efficacy of the \'dredging-marsupialization-curettage\' (D-M-C) strategy in the treatment of conventional intraosseous ameloblastoma of the mandible. A total of 31 patients from three institutions, who had a pathological diagnosis of conventional ameloblastoma of the mandible, were treated with the D-M-C strategy. The surgical protocol comprised a dredging and marsupialization (D-M) step, with additional D-M steps as required. The patients then underwent curettage (C) once an obvious effect of the D-M step had been achieved during follow-up. Eight patients were followed up for ≥36 months but <60 months, while 23 were followed up for ≥60 months. Nineteen of the 23 patients followed up for ≥60 months were disease-free at the last follow-up, with no evidence of recurrence. The D-M step is effective for reducing the tumour size and preserving vital structures. The D-M-C surgical strategy may be a feasible treatment option for conventional ameloblastoma of the mandible.
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  • 文章类型: Journal Article
    目的:口腔成釉细胞瘤(AME)和牙源性角化囊肿(OKC)的术前诊断一直是牙科的挑战。本研究使用影像组学方法和机器学习(ML)算法来表征锥形束计算机断层扫描(CBCT)图像特征,用于AME和OKC的术前鉴别诊断,并将ML算法与放射科医生进行比较以验证性能。
    方法:我们回顾性收集了326例AME和OKC患者的数据,所有诊断均通过组织病理学检查证实。总共选择了348个特征来训练六个ML模型,以通过五次交叉验证进行鉴别诊断。然后,我们将基于ML的诊断的性能与放射科医生的性能进行了比较。
    结果:在六个ML模型中,XGBoost在CBCT图像中有效区分AME和OKC,其分类性能优于其他模型。平均精度,召回,准确度,F1分数,曲线下面积(AUC)分别为0.900、0.807、0.843、0.841和0.872。与放射科医生的诊断相比,基于ML的放射学诊断表现更好。
    结论:基于Radiomic的ML算法可以准确区分AME和OKC的CBCT图像,促进AME和OKC的术前鉴别诊断。
    结论:ML和具有高分辨率CBCT图像的放射学方法为AME和OKC的鉴别诊断提供了新的见解。
    OBJECTIVE: Preoperative diagnosis of oral ameloblastoma (AME) and odontogenic keratocyst (OKC) has been a challenge in dentistry. This study uses radiomics approaches and machine learning (ML) algorithms to characterize cone-beam CT (CBCT) image features for the preoperative differential diagnosis of AME and OKC and compares ML algorithms to expert radiologists to validate performance.
    METHODS: We retrospectively collected the data of 326 patients with AME and OKC, where all diagnoses were confirmed by histopathologic tests. A total of 348 features were selected to train six ML models for differential diagnosis by a 5-fold cross-validation. We then compared the performance of ML-based diagnoses to those of radiologists.
    RESULTS: Among the six ML models, XGBoost was effective in distinguishing AME and OKC in CBCT images, with its classification performance outperforming the other models. The mean precision, recall, accuracy, F1-score, and area under the curve (AUC) were 0.900, 0.807, 0.843, 0.841, and 0.872, respectively. Compared to the diagnostics by radiologists, ML-based radiomic diagnostics performed better.
    CONCLUSIONS: Radiomic-based ML algorithms allow CBCT images of AME and OKC to be distinguished accurately, facilitating the preoperative differential diagnosis of AME and OKC.
    CONCLUSIONS: ML and radiomic approaches with high-resolution CBCT images provide new insights into the differential diagnosis of AME and OKC.
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  • 文章类型: Case Reports
    自1964年以来,颅骨原发性成釉细胞瘤(AM)或成釉细胞癌(AMCa)的报道病例很少。这种罕见情况在特定解剖部位的临床表现和独特特征仍不清楚。我们报告了一例位于额颞叶顶叶区域的颅骨原发性AM恶性转化的病例,并强调了其与文献中报道的其他病例的相似性。
    一名53岁女性患者,有20天的头痛和10天的双侧下肢无力病史。体格检查显示步态缓慢且不稳定。在颅骨成像上,在颅骨的右额叶-颞叶-顶叶区域观察到占位性病变。行右颅骨肿瘤边缘扩张切除术。手术后患者的运动功能恢复正常。术后影像学检查显示10例肿瘤切除。随访影像学检查显示肿瘤复发。患者接受了复发性肿瘤的切除术。术后病理分析显示AM恶变。随访影像学检查显示肿瘤复发。患者接受立体定向放疗。随访影像学检查显示没有肿瘤复发的证据,随后的胸部CT显示没有转移的迹象。
    颅骨的初级AM或AMCa在文献中越来越多地被描述,但是缺乏有关颅骨原发性AM恶性转化的详细报道。这种情况的发病机制尚不清楚。积极的治疗和密切的随访可能是预防疾病复发和恶变的关键。
    UNASSIGNED: Since 1964, there has been a scarcity of reported cases of primary ameloblastoma (AM) or ameloblastic carcinoma (AMCa) of the skull. The clinical presentation and distinctive features of this uncommon condition at specific anatomical sites remain unclear. We report a case of malignant transformation of a primary AM of the skull situated in the frontal-temporal-parietal region and highlight its similarities to other cases reported in the literature.
    UNASSIGNED: A 53-year-old female patient presented with a 20-day history of headaches and bilateral lower limb weakness for 10 days. Physical examination revealed slow and unsteady gait. An occupying lesion was observed in the right frontal-temporal-parietal region of the skull on the Cranial imaging. A right cranial bone tumor margin expansion resection was performed. The patient\'s motor functions recovered normally after surgery. Postoperative imaging examinations showed10 tumor resection. Follow-up imaging examinations showed tumor recurrence. The patient underwent resection of the recurrent tumor. Postoperative pathological analysis revealed malignant transformation of the AM.Follow-up imaging examinations showed tumor recurrence again. The patient was admitted for stereotactic radiotherapy. Follow-up imaging examinations demonstrated no evidence of tumor recurrence and subsequent chest CT revealed no signs of metastasis.
    UNASSIGNED: Primary AM or AMCa of the skull is increasingly being described in the literature, but detailed reports on the malignant transformation of primary AM of the skull are lacking. The pathogenesis of this condition remains unclear. Aggressive treatment and close follow-up may be crucial for preventing disease recurrence and malignant transformation.
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  • 文章类型: Journal Article
    口腔颌面部肿瘤由于其复发的趋势而构成了重大的临床挑战,尽管在手术切除技术的进步。颌骨的复杂结构进一步使治疗复杂化,并影响患者的生活质量。因此,重点已经转向药理干预,以潜在地减少侵入性外科手术。一种有希望的方法针对BRAF突变,特别是常见的V600E突变。BRAF,一种关键的蛋白激酶,通过RAS-RAF-MEK-ERK-MAP激酶途径调节细胞生长和分化。1799位的特定核苷酸变化,将胸腺嘧啶(T)换成腺嘌呤(A),导致V600E突变,导致不受控制的细胞生长。这种突变常见于某些口腔颌面部肿瘤,如成釉细胞瘤。最近一项针对BRAF的新辅助疗法,涉及dabrafenib和trametinib的使用,展示了一个有希望的,安全,下颌骨成釉细胞瘤治疗中器官保存的有效策略。这种分子见解和靶向治疗的融合是有效管理BRAF突变的口腔和颌面部肿瘤的关键,有望改善患者预后。
    Oral and maxillofacial tumors pose a significant clinical challenge due to their tendency to recur, despite advancements in surgical removal techniques. The jaw\'s intricate structure further complicates treatments and affects patient quality of life. Consequently, emphasis has shifted towards pharmacological interventions, to potentially reduce invasive surgical procedures. One promising approach targets BRAF mutations, specifically the common V600E mutation. BRAF, a critical protein kinase, regulates cell growth and differentiation via the RAS-RAF-MEK-ERK-MAP kinase pathway. A specific nucleotide change at position 1799, swapping Thymine (T) for Adenine (A), results in the V600E mutation, causing unchecked cell growth. This mutation is common in certain oral and maxillofacial tumors like ameloblastoma. A recent neoadjuvant therapy targeting BRAF, involving the use of dabrafenib and trametinib, has showcased a promising, safe, and effective strategy for organ preservation in the treatment of mandibular ameloblastoma. This convergence of molecular insights and targeted therapies holds the key to managing BRAF-mutated oral and maxillofacial tumors effectively, promising improved patient outcomes.
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  • 文章类型: Case Reports
    背景:成釉细胞瘤(AM)是最常见的良性牙源性肿瘤,在下颌骨比上颌骨更常见,尤其是下颌体和下颌角。小儿AM是一种罕见的疾病,尤其是10岁及以下的患者。与成人成釉细胞瘤的主流截骨和重建手术相比,小儿成釉细胞瘤的治疗还有更多的讨论空间。术后功能和心理影响不容忽视。特别是对于处于生长发育时期的儿童,截骨术通常很难被父母接受。我们报告了两名10岁以下的成釉细胞瘤患者,他们接受了刮治和开窗术治疗,这对成釉细胞瘤儿童是一种有益的方法。
    方法:我们介绍了2例儿童经典成釉细胞瘤。我们详细描述了患者的特征,处理过程,和后续结果。每次临床检查均记录开窗减压和刮除后病变区域的骨形成和重建。还描述和讨论了刮宫和减压的手术细节和原则。两组患者骨形状恢复较好,无复发。
    结论:儿童正处于生长发育时期,具有极强的骨形成和重建能力。基于微创和功能保存的原则,我们认为,刮宫联合减压可以作为治疗儿童AM的首选,尤其是下颌骨病变。
    BACKGROUND: Ameloblastoma (AM) is the most common benign odontogenic tumor, which is more often detected in the mandible than maxilla, especially the mandibular body and mandibular angle. Pediatric AM is a rare disease, especially in patients aged 10 and younger. Compared with the mainstream osteotomy and reconstructive surgery for adult ameloblastoma, there is more room for discussion in the treatment of pediatric ameloblastoma. The postoperative functional and psychological influence can not be ignored. Especially for children in the period of growth and development, an osteotomy is often challenging to be accepted by their parents. We report two patients with ameloblastoma under 10 years old who are treated with curettage and fenestration, which is a beneficial method for children with ameloblastoma.
    METHODS: We present two cases of classic ameloblastoma in children. We describe in detail the patients\' characteristics, treatment processes, and follow-up result. The bone formation and reconstruction in the lesion area after fenestration decompression and curettage are recorded at every clinic review. The surgical details and principles of curettage and decompression are also described and discussed. The two patients have good bone shape recovery and no recurrence.
    CONCLUSIONS: Children are in the growth and development period and possess an extremely strong ability of bone formation and reconstruction. Based on the principles of minimally invasive and functional preservation, we believe that curettage combined with decompression can be the first choice for treating AM in children, especially for mandibular lesions.
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    文章类型: English Abstract
    目的:研究成釉细胞瘤中BRAFV600E的蛋白和基因状态,成釉细胞癌和囊肿,并探讨成釉细胞瘤的复发和恶性转化是否取决于BRAF状态。
    方法:20个囊肿,25例原发性成釉细胞瘤,通过免疫组织化学分析了25例复发性成釉细胞瘤和8例成釉细胞癌,和2个囊肿,5例原发性釉叶瘤,5例复发性成釉细胞瘤,通过定量实时PCR(qPCR)分析了3个非典型成釉细胞瘤和6个成釉细胞癌。采用SPSS26.0软件包进行数据分析。
    结果:BRAFV600E表达与复发无相关性。囊肿和癌的BRAFV600E突变频率低于成釉细胞瘤。BRAFV600E蛋白阳性染色与BRAFV600E突变一致。
    结论:BRAFV600E突变可能有助于成釉细胞瘤伴囊肿和成釉细胞癌的鉴别诊断。免疫组织化学可能是筛选成釉细胞瘤中BRAFV600E突变的有益方法。
    OBJECTIVE: To investigate protein and genetic status of BRAF V600E in ameloblastomas, ameloblastic carcinomas and cysts, and to explore if recurrence and malignant transformation of ameloblastomas depends on BRAF status.
    METHODS: Twenty cysts, 25 primary ameloblastomas, 25 recurrent ameloblastomas and 8 ameloblastic carcinomas were analysed by immunohistochemistry, and 2 cysts, 5 primary ameloblatomas, 5 recurrent ameloblastomas, 3 atypical ameloblastomas and 6 ameloblastic carcinomas were analysed by quantitative real-time PCR(qPCR). SPSS 26.0 software package was used for data analysis.
    RESULTS: There was no correlation between BRAF V600E expression and recurrence. Cysts and carcinomas had lower frequencies of BRAF V600E mutations than ameloblastomas. There was a concordance between positive staining of BRAF V600E protein and BRAF V600E mutation.
    CONCLUSIONS: BRAF V600E mutation may be useful in the differential diagnosis of ameloblastomas with cysts and ameloblastic carcinomas. Immunohistochemistry may be a beneficial method to screen for BRAF V600E mutation in ameloblastoma.
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  • 文章类型: Journal Article
    成釉细胞瘤(AM)的特征在于局部侵袭性和骨吸收。据我们所知,先前尚未探索与AM相邻的骨的蛋白质组学特征。因此,我们从下颌骨观察了与AM和正常CB相邻的松质骨(CB)中的差异蛋白。提取CB蛋白,纯化,量化,并使用来自五名AM患者的样品通过液相色谱-质谱(LC-MS)进行分析。使用基因本体论进一步研究这些蛋白质以进行额外的功能注释和富集。满足表达差异倍性>1.5倍(上调和下调)和p<0.05的筛选要求的蛋白质随后被认为是差异蛋白质。进行免疫组织化学染色以证实上述发现。与正常下颌CB相比,在下颌AM附近的CB中鉴定出151种差异蛋白。这些主要与细胞分解代谢过程有关,脂质代谢,和脂肪酸(FA)代谢。LC-MS和免疫组织化学显示,与正常下颌CB相比,CD36是与AM接壤的CB中明显减少的蛋白质之一(分别为p=0.0066和p=0.0095)。CB中CD36的表达与AM中的骨重塑相关,使CD36成为治疗方法的可行靶标。
    Ameloblastoma (AM) is characterised by local aggressiveness and bone resorption. To our knowledge, the proteomic profile of bone adjacent to AM has not previously been explored. We therefore looked at the differential proteins in cancellous bone (CB) adjacent to AM and normal CB from the mandible. CB proteins were extracted, purified, quantified, and analysed by liquid chromatography-mass spectrometry (LC-MS) using samples from five patients with AM. These proteins were further investigated using gene ontology for additional functional annotation and enrichment. Proteins that met the screening requirements of expression difference ploidy > 1.5-fold (upregulation and downregulation) and p < 0.05 were subsequently deemed differential proteins. Immunohistochemical staining was performed to confirm the above findings. Compared with normal mandibular CB, 151 differential proteins were identified in CB adjacent to the mandibular AM. These were mainly linked to cellular catabolic processes, lipid metabolism, and fatty acids (FA) metabolism. LC-MS and immunohistochemistry showed that CD36 was one of the notably decreased proteins in CB bordering the AM compared with normal mandibular CB (p = 0.0066 and p = 0.0095, respectively). CD36 expression in CB correlates with bone remodelling in AM, making CD36 a viable target for therapeutic approaches.
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