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Epithelioid Inflammatory Myofibroblastic Sarcoma With Poor Response to Crizotinib: A Case Report. 对克唑替尼反应不良的上皮样炎症性肌纤维母细胞肉瘤:一例报告。
IF 1 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-10-12 eCollection Date: 2023-01-01 DOI: 10.1177/11795476231163954
Soheila Aminimoghaddam, Roghayeh Pourali

Introduction: Epithelioid type inflammatory myofibroblastic sarcoma (EIMS) is a subtype of inflammatory myofibroblastic tumor (IMT). It consists of round or epithelioid cells, and almost all types of EIMS contain rearrangements of the anaplastic lymphoma kinase (ALK) gene.

Case presentation: We describe a 20-year-old female presenting with abdominal pain and a rapidly growing intraabdominal mass who underwent surgical tumor resection. She was diagnosed with EIMS. ALK and ki-67 expressions were detected in immunohistochemistry assessment. She was started with Crizotinib 200 mg twice a day, and chemotherapy was also initiated due to the recurrence of the disease 4 months after the initial treatment. She was unresponsive to all the medical regimens and died in 8 months.

Conclusion: Approach to patients with EIMS is really challenging in terms of both diagnosis and treatment. Patients with combined surgical and non-surgical treatment regimen were seen to have a more favorable outcome in some EIMS cases. Therefore, it is essential to implement a multidisciplinary approach to diagnose and treat patients suspicious of EIMS.

简介:上皮样型炎性肌纤维母细胞肉瘤(EIMS)是炎性肌成纤维细胞瘤(IMT)的一种亚型。它由圆形或上皮样细胞组成,几乎所有类型的EIMS都含有间变性淋巴瘤激酶(ALK)基因的重排。病例介绍:我们描述了一名20岁的女性,她表现为腹痛和快速增长的腹部肿块,并接受了肿瘤切除手术。她被诊断为EIMS。免疫组化检测ALK和ki-67的表达。她开始服用克唑替尼200 mg,每天两次,由于疾病复发,也开始了化疗4 初次治疗后数月。她对所有的治疗方案都没有反应,于8月去世 月。结论:EIMS患者的治疗方法在诊断和治疗方面都具有挑战性。在一些EIMS病例中,手术和非手术联合治疗方案的患者有更有利的结果。因此,实施多学科方法来诊断和治疗怀疑EIMS的患者是至关重要的。
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引用次数: 1
Leucocytoclastic Vasculitis Presenting as Bilateral Ulcerative Keratitis: A Case Report. 以双侧溃疡性角膜炎为表现的白细胞增多性血管炎:一例报告。
IF 1 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-10-06 eCollection Date: 2023-01-01 DOI: 10.1177/11795476231204358
Hui Feng, Shang Li, Ying Jie

Introduction: Small artery disease caused by neutrophils and immune-mediated is known as leucocytoclastic vasculitis (LCV). Clinically, it manifests as palpable, asymptomatic purpuric papules on the limbs. Ocular manifestation is rare. Here, we describe a case of peripheral ulcerative keratitis (PUK) associated with LCV.

Case presentation: A 59-year-old man was referred to the hospital with blurred vision due to corneal perforation in his left eye. He complained of itchy nodules on his hands and lower legs for 15 years and the skin biopsy of the back of his hand revealed LCV 6 years ago, which suggested erythema elevatum diutinum. The patient was under treatment with anti-inflammatory and immunosuppressive drugs and physical features of LCV seen in him included erythema on his hands and legs. After receiving conjunctival flap covering surgery, the corneal perforation was resolved. Conjunctival flaps covered cornea that limited his vision to hand motion. Six months later, he was referred to our clinic again because of pain, redness, photophobia, and tearing in the right eye, presenting with PUK. Necrotic tissue was removed during surgery, which also included a conjunctival flap covering procedure. Following surgery, the symptoms were reduced, and the postoperative eye condition remained stable.

Conclusion: To our knowledge, it is the first case of PUK secondary to LCV which was diagnosed 6 years ago. This case demonstrates that PUK associated with LCV can be successfully treated by surgical interventions.

简介:由中性粒细胞和免疫介导的小动脉疾病被称为白细胞碎屑性血管炎(LCV)。临床上,表现为肢体上可触摸到的、无症状的purpuric丘疹。眼部表现罕见。在此,我们描述一例与LCV相关的外周溃疡性角膜炎(PUK)。病例介绍:一名59岁男子因左眼角膜穿孔导致视力模糊,被转诊至医院。15年来,他一直抱怨手和小腿上有发痒的结节 年,对他的手背进行的皮肤活检显示LCV 6 几年前,这表明红斑升高。患者正在接受抗炎和免疫抑制药物的治疗,其LCV的身体特征包括手和腿上的红斑。接受结膜瓣覆盖术后,角膜穿孔得到解决。结膜瓣覆盖了角膜,使他的视力仅限于手部活动。六个月后,由于疼痛、发红、畏光和右眼撕裂,他再次被转诊到我们的诊所,并出现PUK。坏死组织在手术中被切除,其中还包括结膜瓣覆盖手术。手术后,症状减轻,术后眼部状况保持稳定。结论:据我们所知,这是第一例继发于LCV的PUK,诊断为6 几年前。该病例表明,与LCV相关的PUK可以通过手术干预成功治疗。
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引用次数: 0
Nasal Leishmaniasis Misdiagnosed With Intranasal Polyp in a Patient Candidate for Rhinoplasty. 鼻利什曼病误诊为鼻内息肉一例鼻整形术患者。
IF 1 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-10-03 eCollection Date: 2023-01-01 DOI: 10.1177/11795476231186913
Zakaria Zakariaei, Mahdi Fakhar, Simin Bari, Majid Derakhshani, Elham Sadat Banimostafavi, Mostafa Soleymani

Mucosal leishmaniasis (ML) is a chronic and rare form of leishmaniasis that causes malignant lesions in the mucosa of the nasal, pharyngeal, and laryngeal regions. We describe a 29-year-old woman who had been suffering from an intranasal polyp for 3 years. The polyp recurred annually after surgical removal, and was diagnosed as nasal leishmaniasis.

粘膜利什曼病(ML)是一种慢性和罕见的利什曼原虫病,可导致鼻、咽和喉粘膜的恶性病变。我们描述了一位29岁的女性,她患有鼻内息肉3年 年。息肉每年手术切除后复发,诊断为鼻利什曼病。
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引用次数: 0
Progressive Memory Decline in a Patient With Atrial Septal Defect: Case Report and Literature Review. 心房间隔缺损患者的进行性记忆力衰退:病例报告与文献综述。
IF 0.8 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-05-25 eCollection Date: 2023-01-01 DOI: 10.1177/11795476231176713
Yaw Amo Wiafe, Gordon Manu Amponsah, George Asafu Adjaye Frimpong, Isaac Kofi Owusu

Atrial septal defect (ASD) is a common congenital anomaly that increases the risk of heart failure as well as strokes which can lead to cognitive impairment. The risk of stroke is higher when pulmonary hypertension develops and there is reversal of shunt. Stroke in ASD may be due to paradoxical emboli from the right heart or a left ventricular thrombus which develops as a result of atrial fibrillation, a common arrhythmia in ASD. We present a case of a 32-year-old Ghanaian man with history of ASD who presented with progressive memory loss with magnetic resonance imaging scan of the brain showing multiple infarcts, microvascular disease, and cerebral atrophy.

房间隔缺损(ASD)是一种常见的先天性畸形,会增加心力衰竭和中风的风险,并可能导致认知障碍。当出现肺动脉高压和分流逆转时,中风的风险更高。ASD 患者中风的原因可能是来自右心的矛盾性栓子或因心房颤动而形成的左心室血栓,心房颤动是 ASD 常见的心律失常。我们报告了一例 32 岁的加纳男子,他有 ASD 病史,出现进行性失忆,脑部磁共振成像扫描显示多发性梗死、微血管病变和脑萎缩。
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引用次数: 0
Blue Rubber Bleb Nevus Syndrome Presenting as Anemia, Hemorrhage, and Hemangiomas: A Rare Case Report. 表现为贫血、出血和血管瘤的蓝色橡胶痣综合征:罕见病例报告
IF 1 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-05-15 eCollection Date: 2023-01-01 DOI: 10.1177/11795476231173503
Qaisar Ali Khan, Christopher Farkouh, Arooba Khan, Zahir Uddin, Parsa Abdi, Michelle R Anthony, Faiza Amatul Hadi, Eyan Khan, Sara Parvez

Background: Blue Rubber Bleb Nevus syndrome (BRBNS) is a rare disorder, that results in congenital cutaneous hemangiomas of the skin and gastrointestinal tract. Although asymptomatic, the nevi present as soft, non-mobile, dark blue, compressible papules. Clinically it presents as iron deficiency anemia due to occult gastrointestinal bleeding.

Case presentation: A 22-year-old female patient presented with complaints of shortness of breath, fatigue, and palpitation for 2 months. On examination, she had a pale effect and widespread hemangiomas on her lips, hands, and feet. Laboratory results revealed iron deficiency anemia with hemoglobin (Hb) of 2.1 gm/dl and histopathology results of the hemangioma specimen showed angiokeratomas. Based on clinical manifestations and laboratory results, the patient was diagnosed with a case of BRBNS. The patient was transfused with red cell concentrate her symptoms improved but on the first follow-up visit her Hb again dropped to 8.6 mg/dl.

Conclusion: A high suspicion of BRBNS diagnosis should be considered if a patient presents with iron deficiency anemia and multiple cutaneous hemangiomas. Further screening should be done to explore internal bleeding and hemangiomas.

背景:蓝橡皮痣综合征(BRBNS)是一种罕见疾病,会导致皮肤和胃肠道出现先天性皮肤血管瘤。虽然没有症状,但痣表现为柔软、不活动、深蓝色、可压缩的丘疹。临床表现为因隐匿性消化道出血引起的缺铁性贫血:一名 22 岁的女性患者主诉气短、乏力和心悸 2 个月。经检查,她面色苍白,嘴唇、手和脚上有广泛的血管瘤。实验室结果显示她患有缺铁性贫血,血红蛋白(Hb)为 2.1 gm/dl,血管瘤标本的组织病理学结果显示为血管角化瘤。根据临床表现和实验室结果,患者被诊断为 BRBNS 病例。患者输注了浓缩红细胞,症状有所改善,但在第一次随访时,她的血红蛋白再次降至 8.6 mg/dl:结论:如果患者出现缺铁性贫血和多发性皮肤血管瘤,应高度怀疑 BRBNS 诊断。应做进一步检查,以发现内出血和血管瘤。
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引用次数: 0
A Case of Acute Respiratory Distress Syndrome Following Pleurodesis With Talc. 一例使用滑石粉进行胸膜穿刺术后的急性呼吸窘迫综合征病例
IF 0.8 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-04-24 eCollection Date: 2023-01-01 DOI: 10.1177/11795476231170196
Ali Hossein Samadi Takaldani, Nima Javanshir, Mohammad Negaresh, Helia Honardoost

Management of malignant pleural effusion is a medical challenge, and several methods have been proposed to deal with it including thoracentesis, indwelling pleural catheter placement, and chemical or mechanical pleurodesis. Each method, however, has its advantages and disadvantages. Talc pleurodesis is generally recognized as the most effective and safest method for the induction of chemical pleurodesis. However, in rare cases, it can lead to acute respiratory distress syndrome (ARDS). In this article, we report the case of a patient with metastatic adenocarcinoma to the pleura who presents with shortness of breath and malignant pleural effusion, develops ARDS after pleurodesis with talc, and expires despite the partial improvement of lung involvement. The symptoms and causes of this rare side effect as well as the methods that can be used to deal with it are reviewed in this article.

恶性胸腔积液的治疗是一项医学挑战,目前已提出了几种治疗方法,包括胸腔穿刺术、留置胸膜导管置入术、化学或机械胸膜穿刺术。然而,每种方法都有其优缺点。一般认为,滑石粉胸膜穿刺术是诱导化学性胸膜穿刺术的最有效、最安全的方法。但在极少数情况下,它可能会导致急性呼吸窘迫综合征(ARDS)。在本文中,我们报告了一例胸膜转移性腺癌患者的病例,该患者出现气短和恶性胸腔积液,在使用滑石粉进行胸膜腔穿刺后出现 ARDS,尽管肺部受累情况得到部分改善,但最终还是去世了。本文综述了这种罕见副作用的症状和原因,以及应对这种副作用的方法。
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引用次数: 0
Evaluation of Ayres Sensory Integration® Intervention on Sensory Processing and Motor Function in a Child with Rubinstein-Taybi Syndrome: A Case Report. 艾尔斯感觉统合®干预对鲁宾斯坦-泰比综合征患儿感觉处理和运动功能的评估:病例报告。
IF 0.8 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-02-01 eCollection Date: 2023-01-01 DOI: 10.1177/11795476221148866
Aymen Balikci, Teresa A May-Benson, Ayse Firdevs Aracikul Balikci, Ela Tarakci, Zeynep Ikbal Dogan, Gul Ilbay

The Rubinstein-Taybi Syndrome (RSTS) literature is limited about sensory integration, which is a foundational neurological function of the central nervous system that may affect the development of cognitive, social, and motor skills. The aim of this case report was to investigate the effects of Ayres Sensory Integration® (ASI) intervention on processing and integrating sensations, motor functions and parental goals of 3-year-old child with RSTS. Analysis of assessment data reviewed before and after treatment. Assessment collected by interview, Sensory Profile (SP), Sensory Processing Measure-Preschool (SPM-P) Home, Peabody Developmental Motor Scales-2 (PDMS-2), Gross Motor Function Measurement-88 (GMFM-88), and Gross Motor Function Classification System (GMFCS). Progress toward goals and objectives was measured with Goal Attainment Scale (GAS). ASI intervention was implemented 3 times per week for 8 weeks. At pre-intervention, SP and SPM-P Home revealed prominent sensory processing and integration difficulties in this case. PDMS-2 scores indicated the child was far behind his peers in fine and gross motor areas. In addition, systematic observations determined that the child's GMFCS level was III. After 8 weeks of ASI intervention significant improvements were found in parent reports of sensory processing in the areas of vestibular, tactile, and oral functioning on the Sensory Profile. Gains in functional motor skills were found on the GMFM-88 and the GMFCS. Consistent with these results, significant gains at or above expected levels of performance were found on GAS goals which reflected the family's main concerns for social participation, feeding, play, and movement. There are limited studies on sensory processing and integration in children with RSTS. This case report identified sensory processing and integration difficulties for the first time in a child with RSTS. Results also provide preliminary support for the positive effects of ASI intervention on sensory processing, functional motor skills, and parental goals of a child with RSTS.

鲁宾斯坦-泰比综合症(Rubinstein-Taybi Syndrome,RSTS)有关感觉统合的文献十分有限,而感觉统合是中枢神经系统的基础神经功能,可能会影响认知、社交和运动技能的发展。本病例报告旨在研究 Ayres Sensory Integration®(ASI)干预对 3 岁 RSTS 患儿的感觉处理和整合、运动功能以及父母目标的影响。分析治疗前后的评估数据。通过访谈、感觉档案(SP)、学前感觉处理测量(SPM-P)家庭、皮博迪运动发育量表-2(PDMS-2)、粗大运动功能测量-88(GMFM-88)和粗大运动功能分类系统(GMFCS)收集评估数据。目标达成量表(GAS)用于衡量目标和目的的进展情况。ASI 干预每周 3 次,为期 8 周。在干预前,SP 和 SPM-P 家庭显示该病例存在明显的感觉处理和统合困难。PDMS-2 分数表明,该儿童在精细和粗大运动方面远远落后于同龄人。此外,系统观察还确定孩子的 GMFCS 水平为 III 级。经过 8 周的 ASI 干预后,家长报告的感官档案中前庭、触觉和口腔功能方面的感官处理情况有了明显改善。功能性运动技能在 GMFM-88 和 GMFCS 中均有提高。与这些结果相一致的是,在 GAS 目标上也发现了明显的进步,达到或超过了预期水平,这些目标反映了家庭在社会参与、喂养、游戏和运动方面的主要关注点。有关 RSTS 儿童感官处理和整合的研究十分有限。本病例报告首次在一名 RSTS 患儿身上发现了感觉处理和统合方面的困难。研究结果还初步证实了 ASI 干预对 RSTS 患儿的感觉处理、功能性运动技能和父母目标的积极影响。
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引用次数: 0
Strangled by His Nerves-Cervical Plexiform Neurofibroma With Infantile Spinal Neurofibromatosis: Case Report in a 14 Years Old Child. 小儿脊髓神经纤维瘤病伴颈丛状神经纤维瘤:14岁儿童1例报告。
IF 1 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-01-01 DOI: 10.1177/11795476231164380
Ilias Tahiri, Abderrahim Bourial, Wahib Lahlou, Mounir Rghioui, Amal Hajjij, Abderrahmane Al Bouzidi, Mohamed Zalagh, Abdessamad El Azhari, Fouad Benariba

Background: Neurofibromatoses are a rare group of autosomal dominant tumor suppressor phacomatoses syndromes. Neurofibromatosis type 1 (NF1 or Von Recklinghausen's disease) is the most commonly found type of neurofibromatosis, and constitutes the most commonly found autosomal dominant disease of the nervous system.

Case presentation: We report a case of a 14-year-old boy who reported a 3-year-history of a slowly enlarging right lateral cervical mass. He has a medical history of a progressive limping gait disorder with scoliotic attitude. MRI identified a dumb-bell shaped intradural right cervical process through right paravertebral gutter on C2 to C4, a second intradural dorsal mass with the same characteristics through left paravertebral gutter on D4 and D5 and a large tissue-like mass infiltrating the lumbosacral subcutaneous soft tissues. A Surgical excision of the cervical and lumbar masses was performed with a good outcome after surgical excision.

Conclusions: This case illustrates the need for a collaboration of both neurological and head and neck surgeons in terms of managing difficulties related to a cervical neurofibroma. Benign plexiform neurofibromas are rapidly growing tumors, particularly in children and adolescents, which makes all the importance of early detection and appropriate treatment. Repeated interventions are usually needed in order to adapt and stabilize the tumors extension.

背景:神经纤维瘤病是一种罕见的常染色体显性肿瘤抑制性肉瘤综合征。1型神经纤维瘤病(NF1或Von Recklinghausen病)是最常见的神经纤维瘤病类型,构成了最常见的常染色体显性神经系统疾病。病例介绍:我们报告了一个14岁男孩的病例,他报告了3年的历史,右颈侧肿块缓慢扩大。他有进行性跛行步态障碍伴脊柱侧凸的病史。MRI发现右侧颈突经C2至C4右侧椎旁沟呈哑铃状,第二个硬膜内背侧肿块经D4和D5左侧椎旁沟呈相同特征,并发现一大块组织样肿块浸润腰骶皮下软组织。手术切除颈椎和腰椎肿块,手术切除后效果良好。结论:本病例说明了神经外科和头颈部外科医生在处理与宫颈神经纤维瘤相关的困难方面的合作的必要性。良性丛状神经纤维瘤是一种生长迅速的肿瘤,尤其在儿童和青少年中,因此早期发现和适当治疗非常重要。为了适应和稳定肿瘤的扩展,通常需要反复干预。
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引用次数: 0
An Approach to Acute SARS-CoV-2 Management with Complementary Neuraltherapeutic Medicine: A Case Report. 补充神经治疗药物治疗急性SARS-CoV-2 1例
IF 1 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-01-01 DOI: 10.1177/11795476231159584
Carlos Bustamante, Laura Pinilla, Oscar Amaris

Background: It has been proposed that the immunomodulatory capacity of neuraltherapeutic medicine (NTM) functions by means of stimuli to the nervous system, which influences the self-regulatory and plastic capacity of the nervous system, especially through the autonomic balance between the sympathetic and parasympathetic nervous systems. Several studies report the usefulness of NTM in inflammatory pathologies.

Case presentation: A case report through a retrospective review of the medical history of an 82-year-old male patient with a diagnosis of acute SARS-CoV-2 who received a therapeutic intervention of NTM at the beginning of his hospitalization and presented satisfactory clinical evolution, with a follow-up for 18 months without post-COVID sequelae. A patient diagnosed with acute pneumonia for SARS-CoV-2, and mild ARDS, with markers of severity given by the history of COPD, advanced age, and elevation of LDH, ferritin, and CRP. On the third day of hospitalization, he presented an episode of pulmonary thromboembolism. He presented significant clinical improvement with in-hospital management for 9 days and underwent out-patient control with no post-COVID sequelae.

Conclusions: NTM could be useful for the management of acute inflammatory diseases, including viral diseases such as SARS-CoV-2, in a mild or severe state of inflammation, when added to allopathic medicine, and it can improve clinical evolution and long-term sequelae. More studies are needed to validate this information.

背景:神经治疗药物(NTM)的免疫调节能力是通过刺激神经系统,影响神经系统的自我调节和可塑性,特别是通过交感和副交感神经系统之间的自主平衡发挥作用。一些研究报道了NTM在炎症病理中的作用。病例介绍:回顾性分析1例确诊为急性SARS-CoV-2的82岁男性患者的病史,该患者入院时接受NTM治疗干预,临床进展满意,随访18个月,无术后后遗症。诊断为SARS-CoV-2急性肺炎和轻度ARDS的患者,COPD病史、高龄、LDH、铁蛋白和CRP升高是严重程度的标志。在住院的第三天,他出现了肺血栓栓塞发作。经住院治疗9天,临床有明显改善,并进行了门诊控制,无术后后遗症。结论:NTM可用于治疗急性炎症性疾病,包括SARS-CoV-2等病毒性疾病,在轻度或重度炎症状态下,加入对抗疗法药物,可改善临床进展和长期后遗症。需要更多的研究来验证这一信息。
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引用次数: 0
Treatment of Orofacial Granulomatosis-9-Month Follow-up: A Case Report. 口腔面部肉芽肿病的治疗-9个月随访1例。
IF 1 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-01-01 DOI: 10.1177/11795476231160046
Mirlinda Sopi Krasniqi, Zana Sllamniku Dalipi, Donika Kastrati Dragidella, Labinota Kondirolli

Orofacial granulomatosis is a condition that manifests clinically as painless labial enlargement, perioral and mucosal edema, oral ulcers, and gingivitis. It is characterized by non-necrotizing granulomatous inflammation of the oral and maxillofacial region. When the swelling only affects the lips, the pathology is called Miescher's granulomatous cheilitis; however, when it also causes facial paresis and lingua plicata, it is known as Melkersson-Rosenthal syndrome. We report a case that was successfully treated with a combination of a local (intralesional) steroid, a systemic antibiotic, and a systemic steroid. After 6 months of therapy, we observed improvement in gingival hyperplasia and buccal mucosa and lip edema.

口腔面部肉芽肿病是一种临床表现为无痛性唇肿大、口周和粘膜水肿、口腔溃疡和牙龈炎的疾病。它的特点是口腔和颌面区域的非坏死性肉芽肿性炎症。当肿胀只影响到嘴唇时,病理称为米歇尔肉芽肿性唇炎;然而,当它也引起面部麻痹和口舌重叠时,它被称为梅尔克森-罗森塔尔综合征。我们报告一例成功地治疗了结合局部(病灶内)类固醇,全身性抗生素和全身性类固醇。治疗6个月后,我们观察到牙龈增生、口腔黏膜和唇部水肿的改善。
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引用次数: 0
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Clinical Medicine Insights. Case Reports
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