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Reactions to Misoprostol: A Case Report. 对米索前列醇的反应:病例报告。
IF 1 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-12-06 eCollection Date: 2023-01-01 DOI: 10.1177/11795476231215903
Arsalan Salari, Aseme Pourrajabi, Samaneh Karami, Zahra Hamidi Madani

Background: Most of abortions occur before the 13th week of pregnancy. Nowadays, non-surgical approaches for evacuation of uterine have been replaced with surgical ones due to the reduction in bleeding, fewer complications, ease of management, and cost-effectiveness. Misoprostol is a prostaglandin E1 analog that is used for labor induction. It is known as a safe drug with very few side effects.

Case presentation: A 29-year-old woman with the gestational age of 11 weeks and 6 days with a diagnosis of cystic hygroma introduced herself to the labor ward. At the time of hospitalization, the patient was conscious. The heart sounds were normal. A vaginal exam indicated no pathological findings. Totally, she received 1000 mg of Misoprostol. Approximately, 2 hours after the last placement of Misoprostol, the patient developed bending and mild cyanosis of fingers and showed tachycardia with a pulse rate of 140 beats/min. Her O2 saturation decreased to 78%. At this time, the patient had a successful miscarriage. Echocardiography showed an ejection fraction of 55% and normal right ventricular size. The electrocardiogram showed sinus tachycardia. Therefore, she was sent to CCU with a possible diagnosis of embolism. The cardiologist administered a heparin drip of 5000-unit IV stat, and 1000 unit/h heparin and asked for a D-Dimer test. However, the laboratory reported that the patient's blood sample was hemolyzed and they could only check her hemoglobin which was 4 g/dl. Immediately, the heparin drip was held and the patient received 3 packed cells. Her Hb was 6.5 g/dl. 12 hours later she showed tachycardia, and her O2 saturation reduced to 70%. She lost her consciousness. Nearly 40 minutes later, she had cardiorespiratory arrest and CPR wasn't successful and she died.

Conclusions: In Conclusion, even a frequently used drug such as Misoprostol can cause life-threatening side effects, leading to emergent situations.

背景:大多数流产发生在怀孕第 13 周之前。如今,由于出血量减少、并发症较少、易于处理和成本效益高,非手术方法已被手术方法所取代。米索前列醇是一种前列腺素 E1 类似物,用于引产。众所周知,米索前列醇是一种安全的药物,副作用极少:病例介绍:一名 29 岁的产妇,孕龄 11 周零 6 天,诊断为囊性息肉瘤。入院时,患者神志清醒。心音正常。阴道检查未发现病理结果。她总共接受了 1000 毫克的米索前列醇。在最后一次使用米索前列醇约 2 小时后,患者出现弯腰和手指轻度发绀,并出现心动过速,脉搏为 140 次/分。她的血氧饱和度降至 78%。此时,患者成功流产。超声心动图显示射血分数为 55%,右心室大小正常。心电图显示为窦性心动过速。因此,她被送往重症监护室,诊断可能是栓塞。心脏科医生为她静脉滴注了 5000 单位的肝素静滴和 1000 单位/小时的肝素,并要求进行 D-Dimer 检测。然而,化验室报告说,患者的血样已经溶血,他们只能检测她的血红蛋白(4 克/分升)。他们立即暂停了肝素点滴,并为患者输入了 3 个包装细胞。她的血红蛋白为 6.5 克/分升。12 小时后,她出现心动过速,氧气饱和度降至 70%。她失去了知觉。近 40 分钟后,她心跳呼吸骤停,心肺复苏失败,最终死亡:总之,即使是米索前列醇这样的常用药物,也可能产生危及生命的副作用,导致紧急情况的发生。
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引用次数: 0
Successful Recovery of COVID-19 Associated With Cardiomyopathy in Advanced Breast Cancer Patient With Pulmonary Lymphangitis Carcinomatosis. 晚期乳腺癌肺淋巴结炎癌肿患者成功治愈 COVID-19 相关心肌病变
IF 1 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-12-06 eCollection Date: 2023-01-01 DOI: 10.1177/11795476231210142
Hisham Alquaydheb, Abrar Alharbi, Ahmed Badran, Bader Alshamsan, Ghada Algwaiz, Mahmoud A Elshenawy, Dahish Ajarim

SARS-CoV-2 infection induces myocardiopathy in 19% of severe cases, with a mortality rate of up to 51%. The mainstay of treatment is supportive care, steroids, and tocilizumab (anti-IL-6). This is a case of a 43-year-old woman diagnosed with hormone-positive breast cancer with lung metastasis and pulmonary lymphangitis carcinomatosis (PLC). Her baseline cardiac function was within normal limits. She presented to the emergency department with respiratory distress. Chest CT showed multiple bilateral ground-glass opacities consistent with COVID-19 pneumonia and confirmed by COVID-19-PCR nasal swab. Her condition deteriorated, and she was urgently admitted to the intensive care unit with evidence of a cytokine storm. She was started on tocilizumab, dexamethasone, and meropenem. Echocardiogram (echo) showed a severely reduced ejection fraction with severe global hypokinesis. A second dose of tocilizumab was given, and the dexamethasone dose was increased. Fortunately, the patient had significant clinical and biochemical improvement and regained her normal cardiac function. In conclusion, dexamethasone and tocilizumab could be promising aids in treating cardiomyopathy secondary to SARS-CoV-2 infection.

在 19% 的严重病例中,SARS-CoV-2 感染会诱发心肌病,死亡率高达 51%。治疗的主要方法是支持性护理、类固醇和托西珠单抗(抗 IL-6)。这是一个 43 岁女性的病例,她被诊断为激素阳性乳腺癌,并伴有肺转移和肺淋巴管炎癌(PLC)。她的基线心脏功能在正常范围内。她因呼吸困难来到急诊科就诊。胸部CT显示双侧多发磨玻璃不透光,与COVID-19肺炎一致,并经COVID-19-PCR鼻拭子证实。她的病情恶化,被紧急送入重症监护室,并出现细胞因子风暴。她开始使用妥昔单抗、地塞米松和美罗培南。超声心动图(echo)显示射血分数严重下降,并伴有严重的全身运动功能减退。患者接受了第二剂托珠单抗治疗,并增加了地塞米松的剂量。幸运的是,患者的临床和生化指标得到了明显改善,恢复了正常的心脏功能。总之,地塞米松和托珠单抗是治疗继发于SARS-CoV-2感染的心肌病的有效辅助药物。
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引用次数: 0
Primary Hepatic Schwannoma: Case Report and Literature Review. 原发性肝脏许旺瘤:病例报告和文献综述。
IF 1 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-12-06 eCollection Date: 2023-01-01 DOI: 10.1177/11795476231215907
Nguyen Thi Khuyen, Truong Quoc Thanh, Hoang Van Trung, Trinh Cong Thao, Tran Nhu Tung

Primary hepatic schwannoma is an extremely rare tumor with a good prognosis. Preoperative diagnosis is often challenging due to nonspecific clinical symptoms and its rarity. Here, we report a case of a 56-year-old male patient misdiagnosed with malignant liver tumor, later identified as primary hepatic schwannoma. Furthermore, clinical and histopathological features of 19 cases of primary hepatic schwannoma are also documented. The age of the patients ranged from 38 to 72 years, with a mean age of 56.4 years, and the disease was more common in females. Patients typically presented without clinical symptoms and were not associated with neurofibromatosis type 1. Histopathological features of the tumor were similar to soft tissue schwannoma, characterized by a thick capsule consisting of Antoni A and Antoni B areas. Immunohistochemically, the tumor showed strong positivity and diffusely stained with S-100, while being negative for CD34, CD117, and SMA. Complete resection of the tumor was achieved in all patients. The prognosis was favorable, with no signs of recurrence. Follow-up examinations revealed disease-free survival ranging from 6 to 27 months. Differential diagnosis of primary hepatic schwannoma from malignant liver tumors and metastatic liver tumors can be made based on histopathological features and immunohistochemical staining with S-100.

原发性肝分裂瘤是一种极为罕见的肿瘤,预后良好。由于非特异性临床症状及其罕见性,术前诊断往往具有挑战性。在此,我们报告了一例被误诊为恶性肝肿瘤的 56 岁男性患者,后被确定为原发性肝分裂瘤。此外,我们还记录了 19 例原发性肝分裂瘤的临床和组织病理学特征。患者的年龄从 38 岁到 72 岁不等,平均年龄为 56.4 岁,女性患者较多。患者通常没有临床症状,也与神经纤维瘤病 1 型无关。肿瘤的组织病理学特征与软组织裂孔瘤相似,其特点是由安东尼A区和安东尼B区组成的厚囊。免疫组化显示,肿瘤呈强阳性,S-100呈弥漫性染色,而CD34、CD117和SMA均为阴性。所有患者均完全切除了肿瘤。预后良好,无复发迹象。随访检查显示无病生存期为 6 至 27 个月。根据组织病理学特征和 S-100 免疫组化染色,可对原发性肝分裂瘤与恶性肝肿瘤和转移性肝肿瘤进行鉴别诊断。
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引用次数: 0
Uncovering the Spectrum of Hemophagocytic Lymphohistiocytosis: A Nephrology Department's Analysis of 14 Cases. 吞噬血淋巴细胞增多症谱的揭示——附肾内科14例分析。
IF 1 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-10-31 eCollection Date: 2023-01-01 DOI: 10.1177/11795476231210137
Meriam Hajji, Samia Barbouch, Hayet Kaaroud, Khaoula Ben Abdelghani, Fethi Ben Hamida, Amel Harzallah, Ezzeddine Abderrahim

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a disease of multi-organ dysfunction due to excessive immune activation causing widespread inflammation and tissue destruction. It is a severe condition associated with high morbidity and mortality. Early identification is crucial for prompt treatment. The objective of this case series is to underscore the intricacy of managing HLH in individuals with renal dysfunction.

Methods: This is a retrospective study of patients diagnosed with HLH in a nephrology department over a period of 30 years. We retrospectively reviewed the medical files by applying the Revised HLH-2004 criteria.

Results: Among the 14 female patients included, the mean age was 45.2 years (range 23-78). Nine patients presented with sudden onset of fever and chills. Physical examination revealed purpura in 3 cases, hepatomegaly and splenomegaly in 6 and 5 cases respectively, and peripheral lymphadenopathy in 1 case. Hemorrhagic complications were observed in 5 cases, hypertriglyceridemia in 9 cases, and hyperferritinemia in all cases. Hypothyroidism was observed in all cases, and impaired renal function was detected in 11 of them, with 5 experiencing it as a result of lupus nephritis, and 1 case attributed to pre-eclampsia. Hemophagocytosis was confirmed through sternal puncture in 11 cases. Treatment involved etiological therapy with corticosteroids and immunosuppressants and/or anti-infectives. Intravenous immunoglobulins were administered in 6 cases, while 2 cases required coagulation factor transfusions. Unfortunately, 9 patients did not survive.

Conclusion: The study highlights the need for increased awareness and prompt recognition of HLH, particularly in patients with associated renal complications.

简介:吞噬细胞性淋巴组织细胞增多症(HLH)是一种由于过度免疫激活而引起广泛炎症和组织破坏的多器官功能障碍的疾病。这是一种严重的疾病,发病率和死亡率都很高。早期识别对于及时治疗至关重要。本系列病例的目的是强调肾功能障碍患者管理HLH的复杂性。方法:这是一项对肾内科诊断为HLH的患者进行的为期30年的回顾性研究 年。我们采用修订的HLH-2004标准对医疗档案进行了回顾性审查。结果:在纳入的14名女性患者中,平均年龄为45.2岁 年龄(23-78岁)。9名患者突然出现发烧和发冷。体检发现紫癜3例,肝肿大6例,脾肿大5例,外周淋巴结肿大1例。出血性并发症5例,高甘油三酯血症9例,高铁蛋白血症所有病例。所有病例均观察到甲状腺功能减退,其中11例肾功能受损,其中5例为狼疮性肾炎,1例为先兆子痫。11例经胸骨穿刺证实有吞噬细胞作用。治疗包括使用皮质类固醇、免疫抑制剂和/或抗感染药物进行病因治疗。6例患者静脉注射免疫球蛋白,2例患者需要输注凝血因子。不幸的是,9名患者未能存活。结论:该研究强调需要提高对HLH的认识和及时识别,特别是在有相关肾脏并发症的患者中。
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引用次数: 0
Secretory Breast Carcinoma: Report of Two Cases and Literature Review. 分泌性乳腺癌:两例报告及文献复习。
IF 1 Q3 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-10-31 eCollection Date: 2023-01-01 DOI: 10.1177/11795476231209182
Amal Mouadin, Laila Tahiri El Ousrouti, Sara Boukansa, Nawal Hammas, Laila Chbani, Hinde El Fatemi

Secretory Breast Carcinoma (SBC) is a rare subtype of breast cancer, predominantly affecting young women, and characterized by hormone receptor-negative and HER2-negative tumors with distinctive histological features, including secretory droplets within tumor cells. This article presents 2 unique cases of SBC, Case 1 involving a 42-year-old woman with triple-negative mammary carcinoma later diagnosed with triple-negative secretory carcinoma, and Case 2 featuring a 48-year-old woman with poorly differentiated adenocarcinoma subsequently identified as invasive mammary carcinoma of secretory type. Both cases received diverse treatment regimens, incorporating surgery, chemotherapy, radiotherapy, and hormone therapy. The importance of accurate diagnosis and the need for further research to optimize the management of this rare breast cancer subtype are emphasized. Raising awareness of SBC and reporting additional cases can enhance understanding and improve patient outcomes. Additionally, the integration of clinical, radiological, and histopathological findings, alongside specific molecular markers like S-100 and mammaglobin, is crucial for accurate SBC diagnosis. Given the lack of established guidelines for SBC management, collecting additional cases can aid in defining a more effective strategy for diagnosis, monitoring, and treatment, ultimately contributing to advancements in the field. Herein, we report 2 cases of this rare disease that were diagnosed and treated in our institution.

分泌性乳腺癌(SBC)是癌症的一种罕见亚型,主要影响年轻女性,以激素受体阴性和HER2阴性肿瘤为特征,具有独特的组织学特征,包括肿瘤细胞内的分泌液滴。本文介绍了两个独特的SBC病例,病例1涉及一名42岁的女性,患有三阴性乳腺癌,后来被诊断为三阴性分泌型癌,病例2涉及一名48岁的女性患有低分化腺癌,随后被诊断为分泌型浸润性乳腺癌。两个病例都接受了不同的治疗方案,包括手术、化疗、放疗和激素治疗。强调了准确诊断的重要性和进一步研究以优化这种罕见的癌症亚型的管理的必要性。提高对SBC的认识并报告更多病例可以增强理解并改善患者的预后。此外,整合临床、放射学和组织病理学结果,以及S-100和乳腺珠蛋白等特定分子标记物,对于准确诊断SBC至关重要。鉴于缺乏SBC管理的既定指南,收集更多病例有助于制定更有效的诊断、监测和治疗策略,最终有助于该领域的进步。在此,我们报告了2例在我们机构诊断和治疗的这种罕见疾病。
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引用次数: 0
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
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