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Retroperitoneal Liposarcoma: The Giant Type. 腹膜后脂肪肉瘤:巨型型。
Pub Date : 2022-10-01 Epub Date: 2022-10-31 DOI: 10.14740/jmc4014
Subhi Mansour, Nassim Azzam, Yoram Kluger, Safi Khuri

Retroperitoneal tumors (RPTs) are very rare tumors that arise in the large space of the retroperitoneum. About two-third of these tumors are malignant, of which soft tissue sarcoma (STS) is the most common and comprises almost one-third of malignant RPTs. Twenty to thirty percent of RPTs are benign. The retroperitoneal cavity has a very large potential space for tumor enlargement to a very high diameters without causing specific symptoms, especially during the initial phase of tumor enlargement. On diagnosis, the average tumor weight is 15 - 20 kg and tumor diameter is 20 - 25 cm. The most common retroperitoneal sarcoma type is liposarcoma, which account for 20% of all sarcoma types and 40% of all retroperitoneal sarcomas (RPSs). Other less common STS arise in the retroperitoneum include leiomyosarcoma and undifferentiated pleomorphic type. Giant liposarcoma is usually defined either as tumor diameter of 30 cm or more or tumor weight of 20 kg or higher. This specific type of sarcoma is very uncommon, with few case reports published in the English literature. Herein, we present a case of a healthy 33-year-old male patient, who was admitted due to abdominal distension and increased body weight since few months. An abdominopelvic computed tomography (CT) scan demonstrated a giant retroperitoneal mass of almost 40 cm in diameter in its largest dimension, located in the right retroperitoneal space. Ultrasound (US)-guided fine needle biopsy (FNB) was consistent with well differentiated liposarcoma. Surgical resection of the tumor along with the right colon, right ureter and kidney, third and fourth duodenal parts and part of the right iliopsoas muscle was contemplated. Histopathological report revealed well-differentiated liposarcoma of 50 cm in diameter, with foci of dedifferentiation, presented by pleomorphic sarcoma. Surgical margins were microscopically negative.

腹膜后肿瘤(RPTs)是非常罕见的肿瘤,发生在腹膜后的大空间。这些肿瘤中约有三分之二是恶性的,其中软组织肉瘤(STS)最为常见,占恶性rpt的近三分之一。20%到30%的rpt是良性的。腹膜后腔有很大的潜在空间使肿瘤扩大到非常大的直径,而不会引起特定的症状,特别是在肿瘤扩大的初期。诊断时,肿瘤平均重量为15 - 20公斤,肿瘤直径为20 - 25厘米。最常见的腹膜后肉瘤类型是脂肪肉瘤,占所有肉瘤类型的20%,占所有腹膜后肉瘤(rps)的40%。其他不常见的发生在腹膜后的STS包括平滑肌肉瘤和未分化多形性。巨大脂肪肉瘤通常定义为肿瘤直径大于或等于30cm,或肿瘤重量大于或等于20kg。这种特殊类型的肉瘤非常罕见,在英语文献中很少有病例报告。在此,我们报告一位健康的33岁男性患者,因腹胀和体重增加几个月而入院。腹部骨盆计算机断层扫描(CT)显示一个巨大的腹膜后肿块,最大直径约40厘米,位于右侧腹膜后间隙。超声引导下细针活检(FNB)符合高分化脂肪肉瘤。考虑将肿瘤连同右结肠、右输尿管、右肾、十二指肠第三、第四部分及部分右髂腰肌一并切除。组织病理学报告显示直径50厘米的高分化脂肪肉瘤,伴有去分化灶,表现为多形性肉瘤。手术缘镜下呈阴性。
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引用次数: 1
Isolated Renal Relapse in a Post-Allogenic Transplant Adult Patient With Acute Lymphoblastic Leukemia. 急性淋巴细胞白血病成人患者同种异体移植后孤立性肾复发。
Pub Date : 2022-10-01 Epub Date: 2022-10-31 DOI: 10.14740/jmc4003
Marco Alejandro Jimenez-Ochoa, Maria Margarita Contreras-Serratos, Martha Leticia Gonzalez-Bautista, Constantino Lopez-Macias, Diego Alberto Lozano-Jaramillo

Acute lymphoblastic leukemia (ALL) is an aggressive hematological neoplasm typically more common in children than adults. More prolonged remissions and a potential cure can be achieved if allogeneic hematopoietic stem cell transplantation (allo-HSCT) is performed. Outcomes after allo-HSCT vary significantly among patients, and multiple factors contribute to these outcomes. Isolated extramedullary relapse (iEMR) after allo-HSCT is rare. We present the case of a 43-year-old man who was diagnosed with Philadelphia chromosome-negative (Ph-neg), B-cell ALL and underwent haploidentical allo-HSCT because of high-risk features at diagnosis. One year later, he was admitted to the hospital with facial and peripheral edema, proteinuria, elevated serum creatinine levels, and hypertension. Renal biopsy was performed immediately. Renal infiltration of TdT+ leukemic cells was detected by immunohistochemistry. Bone marrow aspiration, lumbar puncture, and computed tomography (CT) scans were performed to identify other sites of possible relapse. No other sites were identified, and an extramedullary isolated renal relapse was diagnosed. Intensive re-induction with chemotherapy was not possible because of the coronavirus disease 2019 (COVID-19) infection. Six weeks later, a medullary relapse was noted. Medullary infiltration of B-cell ALL after allo-HSCT has a historically poor prognosis; however, iEMR appears to have a better overall prognosis. The optimal treatment for renal iEMR is still a matter of debate.

急性淋巴细胞白血病(ALL)是一种侵袭性血液肿瘤,儿童比成人更常见。如果进行同种异体造血干细胞移植(allo-HSCT),则可以实现更长时间的缓解和潜在的治愈。不同患者的同种异体造血干细胞移植后的结果差异很大,多种因素导致了这些结果。同种异体造血干细胞移植后孤立性髓外复发(iEMR)是罕见的。我们报告了一例43岁的男性,他被诊断为费城染色体阴性(Ph-neg), b细胞ALL,由于诊断时的高风险特征,他接受了单倍相同的同种异体造血干细胞移植。一年后,他因面部和周围水肿、蛋白尿、血清肌酐水平升高和高血压入院。立即行肾活检。免疫组化检测TdT+白血病细胞的肾浸润。骨髓抽吸、腰椎穿刺和计算机断层扫描(CT)来确定其他可能复发的部位。未发现其他部位,诊断为髓外孤立性肾复发。由于2019冠状病毒病(COVID-19)感染,无法进行强化再诱导化疗。6周后,髓质复发。同种异体造血干细胞移植后b细胞骨髓浸润预后较差;然而,iEMR似乎有更好的整体预后。肾脏iEMR的最佳治疗方法仍存在争议。
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引用次数: 0
Kluri Anomaly: Newly Introduced Pancreatic Duct Anomaly. Kluri异常:新引入的胰管异常。
Pub Date : 2022-10-01 Epub Date: 2022-10-31 DOI: 10.14740/jmc4012
Safi Khuri, Yoram Kluger

Anomalies of the pancreas organ, especially congenital anomalies, are very uncommon, and can involve the pancreatic ductal as well as the pancreatic extra-ductal systems. While these anomalies usually present as an incidental radiological finding during adulthood, sometimes, they can present as recurrent upper abdominal pain as a presentation of recurrent episodes of acute idiopathic pancreatitis. Thus, these anomalies should be regarded in the differential diagnosis list, as a cause, for recurrent idiopathic pancreatitis, especially in the adult age group of patients. Multiple different variations, mainly in duct course and configuration of the pancreatic ductal system have been reported. In addition, duplication anomalies and cystic dilatation of the pancreatic duct are well known anomalies as well. A combined anomaly of the aforementioned anomalies is even rarer. Herein, we present the case of a male patient, 47 years old, with recurrent admissions to different hospitals due to upper abdominal pain and episodes of severe idiopathic acute pancreatitis. Imaging tests, mainly abdomino-pelvic computed tomography (CT) scan and magnetic resonance imaging (MRI)/magnetic resonance cholangiopancreatography (MRCP) showed a dominant duct of Santorini without divisum along with cystic dilation of the proximal portion of the Santorini duct. Being a very rare pancreatic duct anomaly, the patient was treated by a multidisciplinary team (MDT) of physicians, including pancreas surgeons, gastroenterologists and radiologists. A surgical resection in the form of total pancreatectomy with Roux-en-Y gastrointestinal reconstruction was contemplated. Perioperative and postoperative periods were uneventful. The previously mentioned anomaly is unknown in the English literature and is introduced as new anomaly known as "Kluri".

胰腺器官的异常,特别是先天性异常,是非常罕见的,并可涉及胰腺导管以及胰腺导管外系统。虽然这些异常通常表现为成年期偶然的影像学发现,但有时,它们可以表现为复发性上腹部疼痛,表现为急性特发性胰腺炎复发发作。因此,这些异常应在鉴别诊断清单中被视为复发性特发性胰腺炎的原因,特别是在成年年龄组的患者中。多种不同的变化,主要是在胰管系统的导管过程和配置已被报道。此外,复制异常和胰管囊性扩张也是众所周知的异常。上述异常的组合异常更为罕见。在此,我们提出的情况下,男性患者,47岁,反复入院不同的医院,由于上腹部疼痛和发作严重特发性急性胰腺炎。影像学检查,主要是腹部-骨盆计算机断层扫描(CT)和磁共振成像(MRI)/磁共振胆管造影(MRCP)显示圣托里尼管的优势,无分裂,并伴有圣托里尼管近端部分的囊性扩张。作为一种非常罕见的胰管异常,患者接受了多学科团队(MDT)的治疗,包括胰腺外科医生,胃肠病学家和放射科医生。考虑手术切除全胰切除术和Roux-en-Y胃肠重建。围手术期和术后均无异常。前面提到的异常在英国文献中是未知的,并作为新的异常被称为“Kluri”。
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引用次数: 1
Intra-Atrial Right Coronary Artery and Anomalous Origin of Left Circumflex Artery Found Concurrently. 心房内右冠状动脉与左旋动脉异常起源同时发现。
Pub Date : 2022-10-01 Epub Date: 2022-10-31 DOI: 10.14740/jmc3986
Peter C Olson, Michael Cinelli, Hamfreth S Rahming, Marc Assaad, Jonathan Spagnola, James C Lafferty

Coronary artery anomalies (CAAs) are known to be anatomical aberrations in the origin and structure. Due to the diverse anatomical variants, surgeons and angiographers have struggled when faced with patients who have CAA. To frame the complicated issues surrounding CAA, we present a case of a young patient found to have two CAAs, concurrently on coronary computed tomography angiography (CCTA), while in the emergency room being evaluated for chest pain. Patient was medically observed without any invasive procedure since he was deemed to have low cardiovascular risk. Subsequently, literature on prevalence, as well as high risk findings are reviewed. Further studies to evaluate pharmacological, angiographic, and surgical interventions may have additional benefit for both patients and practitioners. Our aim is to help shed the light on difficulties cardiologists are facing during angiography. Additionally, our paper offers some guidance for how to evaluate and follow patients with similar findings into the future.

冠状动脉异常(CAAs)被认为是起源和结构上的解剖畸变。由于不同的解剖变异,外科医生和血管造影师在面对CAA患者时一直很挣扎。为了说明围绕CAA的复杂问题,我们报告了一个年轻患者的病例,在冠状动脉计算机断层血管造影(CCTA)中发现有两个CAAs,同时在急诊室接受胸痛评估。由于认为患者心血管风险较低,因此对患者进行了医学观察,没有进行任何侵入性手术。随后,回顾了有关患病率的文献以及高风险的发现。进一步研究评估药理学、血管造影和手术干预可能对患者和医生都有额外的好处。我们的目的是帮助阐明心脏病专家在血管造影时面临的困难。此外,我们的论文为未来如何评估和跟踪有类似发现的患者提供了一些指导。
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引用次数: 0
Be Patient: Prolonged Extracorporeal Membrane Oxygenation Support Including Full System Switch With Favorable Outcome. 耐心:延长体外膜氧合支持,包括全系统切换,结果良好。
Pub Date : 2022-10-01 Epub Date: 2022-10-31 DOI: 10.14740/jmc3979
Mathias Schmandt, Christian Putensen, Tatjana Stiehl, Julia Wagenpfeil, Jens-Christian Schewe, Stefan Felix Ehrentraut

Despite tremendous advances in treatment, acute respiratory distress syndrome (ARDS) remains a disease with high mortality (42-48%). Veno-venous extracorporeal membrane oxygenation (VV-ECMO) is often used as a last treatment option, which poses complex problems for the treatment team, especially with prolonged ECMO support. We report an interesting case of a 40-year-old female patient who developed influenza pneumonia leading to ARDS and subsequently requiring ECMO. Due to severe clotting complications, a prolonged ECMO run time with numerous filter changes was required. After a total of 56 days of ECMO therapy, the patient was successfully weaned. Fortunately, further in the course of treatment, complete recovery with restitutio ad integrum was achieved. A distinguishing feature of this case report is the description of a complete ECMO system change and the concurrent use of two ECMO systems for the same patient. Additionally, we provide data on the patient's current health-related quality of life as measured using the World Health Organization Disability Assessment Schedule 2.0.

尽管治疗取得了巨大进步,急性呼吸窘迫综合征(ARDS)仍然是一种死亡率很高的疾病(42-48%)。静脉-静脉体外膜氧合(VV-ECMO)通常被用作最后的治疗选择,这给治疗团队带来了复杂的问题,特别是长时间的ECMO支持。我们报告了一个有趣的病例,一位40岁的女性患者发展为流行性感冒肺炎导致ARDS,随后需要体外膜肺切除术。由于严重的凝血并发症,需要延长ECMO运行时间并多次更换过滤器。经过56天的ECMO治疗,患者成功断奶。幸运的是,在进一步的治疗过程中,实现了完全恢复和整合。本病例报告的一个显著特征是描述了一个完整的ECMO系统的变化和同一患者同时使用两个ECMO系统。此外,我们提供了使用世界卫生组织残疾评估表2.0测量的患者当前与健康相关的生活质量的数据。
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引用次数: 0
The Management of Traumatic Abdominal Wall Flank Hernia Along the Spigelian Aponeurosis Using Component Separation, Synthetic, and Biological Mesh. 利用成分分离、合成和生物补片治疗沿Spigelian腱膜的外伤性腹壁侧翼疝。
Pub Date : 2022-10-01 Epub Date: 2022-10-31 DOI: 10.14740/jmc4006
Aldin Malkoc, Kerry E Fine, Ramisa Anjum, Joseph Vivian Davis

Blunt abdominal trauma is associated with a variety of medical complications. Traumatic abdominal wall hernias (TAWHs) are a rare sequela of blunt trauma. Of the various forms of TAWH, a rare subtype described as a "spontaneous lateral ventral hernia" or flank hernia occurs in less than 1% of all blunt abdominal traumas. We present a case of a 39-year-old male with a past medical history of epilepsy who was involved in a rollover motor vehicle collision. It was reported that the patient had a seizure while driving. On physical exam, the patient had a large left lower flank contusion. Computed tomography revealed a complex TAWH with complete avulsion of the abdominal wall musculature from the iliac crest and near to total disruption of the internal oblique. To address this, we used a biological mesh inlay, reinforced with a synthetic Ventralight™ mesh secured to the iliac crest. In this article, we describe the patient's experience and management of a complex TAWH.

钝性腹部创伤与多种医学并发症有关。外伤性腹壁疝(TAWHs)是一种罕见的钝性创伤后遗症。在各种形式的TAWH中,一种罕见的亚型被描述为“自发性外侧腹疝”或腹侧疝,在所有钝性腹部创伤中发生率不到1%。我们提出一个病例39岁的男性与过去的病史癫痫谁是参与翻车机动车碰撞。据报道,病人在开车时癫痫发作。体检时发现患者左下腹有大面积挫伤。计算机断层扫描显示一个复杂的TAWH与腹壁肌肉完全撕脱从髂骨和接近完全破坏的内斜肌。为了解决这个问题,我们使用了一种生物网状物嵌体,并用合成的Ventralight™网状物固定在髂骨上。在这篇文章中,我们描述了患者的经验和管理一个复杂的TAWH。
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引用次数: 1
Immunocompetent Patient With Primary Bone Marrow Hodgkin Lymphoma. 免疫功能正常的原发性骨髓霍奇金淋巴瘤患者。
Pub Date : 2022-09-01 Epub Date: 2022-09-28 DOI: 10.14740/jmc3973
Armaan Dhaliwal, Vanessa F Eller, Jeffrey J Pu

Hodgkin lymphoma (HL) is a hematologic malignancy that comprises about 10% of all lymphomas with the most common type being classical HL (cHL). The typical clinical presentation of cHL involves multiple region lymphadenopathy and a chest mass found on imaging. However, not all patients present with the typical symptomology of cHL which poses a diagnostic challenge. Extranodal HL, especially primary bone marrow HL (PBMHL), has been described in immunocompromised patients with human immunodeficiency virus (HIV). In this case report, we present a PBMHL case in an immunocompetent patient with no HIV exposure. We discuss a 51-year-old immunocompetent female who presented with 2 - 3 months of fever, confusion, generalized myalgias, and fatigue. She had no lymphadenopathy on physical exam. On further testing, the patient's blood work demonstrated cytopenia and imaging confirmed no lymphadenopathy. Eventually, a bone marrow evaluation established her diagnosis of PBMHL. The patient expired after receiving one cycle of a modified chemotherapy regimen. This case illustrates that HL can be associated with an atypical clinical presentation which may delay diagnosis and treatment. PBMHL can occur in the normal population who is not immunocompromised nor HIV positive. In this situation, the best diagnostic approach is a thorough medical history, physical exam, and bone marrow aspiration and biopsy. Presence of constitutional symptoms without any lymphadenopathy or chest mass should raise the concern for possible atypical HL such as PBMHL. Accurate and timely identification of PBMHL allows for timely initiation of appropriate therapy. While cHL is responsive to chemotherapy, further research is required to improve the therapy for PBMHL.

霍奇金淋巴瘤(HL)是一种血液系统恶性肿瘤,约占所有淋巴瘤的10%,最常见的类型是经典HL (cHL)。cHL的典型临床表现包括多区域淋巴结病变和影像学上发现的胸部肿块。然而,并不是所有的患者都有典型的cHL症状,这给诊断带来了挑战。结外HL,尤其是原发性骨髓HL (PBMHL),已被描述为人类免疫缺陷病毒(HIV)免疫功能低下患者。在这个病例报告中,我们提出了一个PBMHL病例在免疫功能正常的病人没有艾滋病毒暴露。我们讨论了一位51岁的免疫功能正常的女性,她表现为2 - 3个月的发烧,精神错乱,全身肌痛和疲劳。体格检查未见淋巴结病变。在进一步的检查中,患者的血液检查显示细胞减少,影像学证实无淋巴结病。最终,骨髓评估确定了她的PBMHL诊断。患者在接受一个周期的改良化疗方案后死亡。本病例表明,HL可能伴有不典型的临床表现,这可能会延误诊断和治疗。PBMHL可发生在没有免疫功能低下或HIV阳性的正常人群中。在这种情况下,最好的诊断方法是全面的病史,体检,骨髓穿刺和活检。没有任何淋巴结病变或胸部肿块的体质症状应引起对非典型HL(如PBMHL)的关注。准确和及时地识别PBMHL可以及时开始适当的治疗。虽然cHL对化疗有反应,但需要进一步研究以改进PBMHL的治疗方法。
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引用次数: 0
Polyethylene Glycol 3350 Crystal Nephropathy in Association With Glomerular Mesangial Immunoglobin A Deposition. 聚乙二醇3350晶体肾病与肾小球系膜免疫球蛋白A沉积有关。
Pub Date : 2022-09-01 Epub Date: 2022-09-28 DOI: 10.14740/jmc4000
Rasha Aly, Ratna Acharya, Xu Zeng, Kiran Upadhyay

Polyethylene glycol (PEG) 3350, an active ingredient of over-the-counter MiraLAX, is a commonly used laxative in children and is produced by polymerization of ethylene glycol (EG). Masked EG toxicity secondary to contamination of PEG 3350 could occur. We present a 7-year-old child with developmental delay who presented with altered mental status and acute kidney injury (AKI) following intake of generic PEG 3350 for few days prior to presentation. There was high anion gap metabolic acidosis, hypernatremia, elevated osmolar gap, lactic acidosis, and AKI. Urinalysis showed tubular proteinuria, microscopic hematuria, and calcium oxalate crystals. Prior urinalyses were normal without hematuria or proteinuria. Renal biopsy revealed evidence of mesangial dominant immunoglobulin A (IgA) and complement 3 (C3) deposits along with dense tubular deposition of calcium oxalate crystals. He subsequently developed worsening oliguric AKI and required hemodialysis (HD) for several sessions. The AKI resolved within 2 weeks and further HD was not required. Mental status improved in few days. Follow-up urinalyses showed resolution of microscopic hematuria and crystalluria. We hypothesized that the generic PEG 3350 most likely was contaminated with EG leading to the presentation. A high index of suspicion of contamination of PEG 3350 with EG is required in patients presenting with unexplained high anion gap metabolic acidosis, elevated osmolar gap, lactic acidosis, AKI, calcium oxalate crystalluria, and oxalate crystals on renal biopsy. Further studies are needed to determine whether there is an association between transient glomerular mesangial IgA deposition and crystal nephropathy.

聚乙二醇(PEG) 3350,是非处方MiraLAX的有效成分,是一种常用的儿童泻药,由乙二醇(EG)聚合产生。PEG 3350污染后可能会出现隐蔽性EG毒性。我们报告了一名7岁的发育迟缓儿童,在就诊前几天服用通用PEG 3350后,出现了精神状态改变和急性肾损伤(AKI)。存在高阴离子间隙代谢性酸中毒、高钠血症、渗透压间隙升高、乳酸性酸中毒和AKI。尿分析显示管状蛋白尿,镜下血尿,草酸钙结晶。既往尿分析正常,无血尿或蛋白尿。肾活检显示肾小球系膜显性免疫球蛋白A (IgA)和补体3 (C3)沉积,同时伴有草酸钙晶体密集的管状沉积。随后,他出现了越来越严重的低尿酸AKI,并需要血液透析(HD)几个疗程。AKI在2周内消退,不需要进一步的HD治疗。几天后精神状态有所改善。随访尿液分析显示显微镜下血尿和结晶尿消失。我们假设,通用PEG 3350极有可能被EG污染导致呈现。对于出现不明原因的高阴离子间隙代谢性酸中毒、渗透性间隙升高、乳酸性酸中毒、AKI、草酸钙结晶尿和肾活检显示草酸盐结晶的患者,需要高度怀疑PEG 3350被EG污染。短暂性肾小球系膜IgA沉积与结晶肾病之间是否存在关联尚需进一步研究。
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引用次数: 0
SARS-CoV-2 Unmasks Type 1 Diabetes Mellitus With an Episode of Diabetic Ketoacidosis. SARS-CoV-2揭示了伴有糖尿病酮症酸中毒发作的1型糖尿病
Pub Date : 2022-09-01 Epub Date: 2022-09-28 DOI: 10.14740/jmc3963
Asimenia Halioti, Maria Kitinou, Varvara-Maria Chalioti, Georgios Chaliotis

During the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, coronavirus disease 2019 (COVID-19) has been significantly studied for its relationship with diabetes mellitus in general. Still, the association of SARS-CoV-2 infection with diabetic ketoacidosis (DKA) is more specific and warrants a meticulous investigational approach. In this case report, we present a 23-year-old female who developed DKA as the first manifestation of SARS-CoV-2 infection. During hospitalization, the diagnosis of type 1 diabetes mellitus (T1DM) was made and the patient was treated successfully for the metabolic disorder and for SARS-CoV-2. The potential of SARS-CoV-2 to induce DKA in type 1 diabetics is highlighted. We point out that DKA and COVID-19 may have similarities in clinical presentation when gastrointestinal features predominate. In addition, we describe mechanisms that have been hypothesized to explain the negative impact of SARS-CoV-2 on the endocrine pancreatic function.

在严重急性呼吸综合征冠状病毒2 (SARS-CoV-2)大流行期间,冠状病毒病2019 (COVID-19)与糖尿病的关系得到了重要研究。尽管如此,SARS-CoV-2感染与糖尿病酮症酸中毒(DKA)的关联更为具体,需要细致的研究方法。在本病例报告中,我们报告了一名23岁的女性,她以SARS-CoV-2感染的第一表现为DKA。住院期间诊断为1型糖尿病(T1DM),并成功治疗代谢紊乱和SARS-CoV-2。强调了SARS-CoV-2在1型糖尿病患者中诱导DKA的潜力。我们指出,DKA和COVID-19在以胃肠道特征为主的临床表现上可能有相似之处。此外,我们描述了已经假设的机制来解释SARS-CoV-2对胰腺内分泌功能的负面影响。
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引用次数: 1
Steven-Johnson Syndrome: A Rare but Serious Adverse Event of Nivolumab Use in a Patient With Metastatic Gastric Adenocarcinoma. 史蒂文-约翰逊综合征:转移性胃腺癌患者使用尼武单抗的罕见但严重的不良事件。
Pub Date : 2022-09-01 Epub Date: 2022-09-28 DOI: 10.14740/jmc3992
Eltaib Saad, Pabitra Adhikari, Drashti Antala, Ahmed Abdulrahman, Valiko Begiashvili, Khalid Mohamed, Elrazi Ali, Qishou Zhang

Nivolumab is a humanized monoclonal anti-programmed cell death receptor-1 (PD-1) antibody that has been authorized for use in the treatment of advanced malignancies. Cutaneous reactions are the most common immune-related adverse events reported with anti-PD-1 agents, and they range broadly from mild localized reactions to rarely severe or life-threatening systemic dermatoses. The occurrence of Steven-Johnson syndrome (SJS) or toxic epidermal necrolysis (TEN) with nivolumab use is an exceedingly rare phenomenon that was only documented in a handful of cases in the current literature, but it deserves careful attention as SJS/TEN may be associated with fatal outcomes. We present a case of nivolumab-induced SJS/TEN in a middle-aged female patient with metastatic gastric adenocarcinoma that was successfully treated with immunosuppressive therapy and supportive care. Prompt recognition of SJS/TEN with discontinuation of nivolumab is warranted when SJS/TEN is suspected clinically. Multidisciplinary management in a specialized burn unit is the key to improving outcomes of SJS/TEN.

Nivolumab是一种人源化单克隆抗程序性细胞死亡受体-1 (PD-1)抗体,已被批准用于治疗晚期恶性肿瘤。皮肤反应是抗pd -1药物报道的最常见的免疫相关不良事件,其范围从轻微的局部反应到罕见的严重或危及生命的全身性皮肤病。使用纳武单抗后发生史蒂文-约翰逊综合征(SJS)或中毒性表皮坏死松解症(TEN)是一种极其罕见的现象,目前文献中仅记录了少数病例,但值得注意的是,SJS/TEN可能与致命的结果有关。我们报告了一例尼伏单抗诱导的转移性胃腺癌中年女性患者的SJS/TEN,该患者成功地接受了免疫抑制治疗和支持性护理。当临床怀疑SJS/TEN时,应及时识别SJS/TEN并停用纳武单抗。烧伤专科多学科管理是改善SJS/TEN预后的关键。
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引用次数: 1
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