edema

硬肿病
  • 文章类型: Journal Article
    背景:TAFRO综合征是一种全身性炎症性疾病,表现为血小板减少症(t),anasarca(a),发烧(f),网织蛋白骨髓纤维化/肾功能不全(r),和器官肿大(o),并被认为是特发性多中心Castleman病(iMCD)的独特临床亚型。这种综合征引起的临床表现类似于结缔组织疾病或自身免疫性疾病。
    方法:一名中国年轻女性,最初表现为关节痛,雷诺现象,全身性水肿,抗小核核糖核蛋白颗粒抗体阳性被诊断为混合性结缔组织病。肾活检显示血栓性微血管病变。骨髓涂片显示骨髓增生,活检提示怀疑轻链限制性表达,巨核细胞增殖,和中度至重度骨髓纤维化。进行了淋巴结活检,组织病理学发现与混合型Castleman病的亚型一致。常规化疗后临床症状缓解。
    方法:经过以上检查结果和临床表现,最终诊断为TAFRO综合征.
    方法:她开始用硼替佐米化疗,环磷酰胺,还有地塞米松.
    结果:化疗后,血小板减少症等症状,血尿和蛋白尿消失,淋巴结肿大和VEGF水平下降,骨髓纤维化缓解。
    结论:我们的病例说明了第一例混合性结缔组织病和iMCD-TAFRO综合征的共同特征。细胞因子可能在iMCD-TAFRO综合征和全身性自身免疫性疾病的共同致病性中起作用。直接针对炎症因子如糖皮质激素或化疗的治疗具有重要的治疗意义。
    BACKGROUND: TAFRO syndrome is a systemic inflammatory disorder, manifesting as thrombocytopenia (t), anasarca (a), fever (f), reticulin myelofibrosis/renal insufficiency (r), and organomegaly (o), and considered as a unique clinical subtype of idiopathic multicentric Castleman disease (iMCD). Such syndrome gave rise to a clinical picture similar to that of either a connective tissue disease or an autoimmune disease.
    METHODS: A Chinese young female initially presenting with arthralgia, Raynaud phenomenon, generalized edema, and a positive anti-small nuclear ribonucleoprotein particle antibody was diagnosed as mixed connective tissue disease. The kidney biopsy showed thrombotic microangiopathy. Bone marrow smear showed bone marrow hyperplasia and biopsy revealed suspected light chain restricted expression, megakaryocyte proliferation, and moderate to severe bone marrow fibrosis. A lymph node biopsy was conducted and the histopathological findings were consistent with the subtype of mixed Castleman disease. The clinical symptoms were relieved after regular chemotherapy.
    METHODS: After above examination results and clinical manifestations, the final diagnoses was TAFRO syndrome.
    METHODS: The she was started on chemotherapy with bortezomib, cyclophosphamide, and dexamethasone.
    RESULTS: After chemotherapy, symptoms such as thrombocytopenia, hematuria and proteinuria disappeared, lymphadenopathy and VEGF level decreased, and bone marrow fibrosis relieved.
    CONCLUSIONS: Our case illustrated the first cases of shared characteristics of mixed connective tissue disease and iMCD-TAFRO syndrome. Cytokines may play a role in the shared pathogenicity of the iMCD-TAFRO syndrome and systemic autoimmune diseases. Therapy directly against inflammatory factors such as corticosteroids or chemotherapy have an important therapeutic implication.
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  • 文章类型: Case Reports
    A 31-year-old man sought medical evaluation for a 2-year history of edema and proteinuria, with prior pathology suggesting atypical membranous nephropathy (MN). Despite treatment with a combination of steroids, calcineurin inhibitors, and four courses of rituximab (1 g, intravenous injection), the patient\'s nephrotic syndrome showed no relief (24 h urine protein peaked at 31.18 g/d), indicating refractory nephrotic syndrome. Later in the disease course, a sudden surge of creatinine level (322.5 μmol/L) prompted a renal biopsy, which revealed concurrent acute interstitial nephritis. Further treatment involving steroids, cyclophosphamide, and a fifth rituximab infusion (1 g, intravenous injection) resulted in improvement in renal function (serum creatinine: 322.5➝147 μmol/L), but the MN failed to achieve partial relief. Subsequent treatment with the novel humanized CD20 monoclonal antibody obinutuzumab (1 g, intravenous injection) was initiated. In the latest follow-up, anti-phospholipase-A2-receptor antibody (PLA2R) antibody were negative, B cells were eliminated, serum albumin was 36 g/L, urine protein-to-creatinine ratio was 4 810 mg/g, and serum creatinine was 162 μmol/L. This case underscores the potential efficacy of obinutuzumab in refractory MN. For advanced MN cases, prompt identification of the cause of acute kidney injury is crucial, emphasizing the need for targeted interventions to potentially stall renal function decline.
    患者男性,31岁。因水肿、蛋白尿2年余就诊,外院肾脏病理提示不典型膜性肾病。糖皮质激素联合钙调磷酸酶抑制剂、4程利妥昔单抗(1 g,静脉输注)治疗后肾病综合征无缓解,24 h尿蛋白仍高达31.18 g,考虑为难治性肾病综合征。后期病程中短期内出现肌酐升高(322.5 μmol/L),重复肾组织活检病理示膜性肾病合并急性间质性肾炎,予足量糖皮质激素联合环磷酰胺加第5程利妥昔单抗1 g静脉输注,间质性肾炎导致的肾功能损害呈现缓解趋势(血肌酐322.5 μmol/L➝147 μmol/L),但膜性肾病仍未能部分缓解,随后尝试使用奥妥珠单抗(1 g,静脉输注)。患者门诊规律随诊,末次随访抗磷脂酶A2受体抗体阴性,B细胞清零,血白蛋白36 g/L,尿总蛋白肌酐比4 810 mg/g,血肌酐162 μmol/L。提示奥妥珠单抗在难治性膜性肾病中的疗效。此外,在肾病综合征的任何时期,合并急性肾损伤时均需要鉴别原因,寻找可逆因素并行针对性治疗,尽可能延缓肾功能恶化。.
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  • 文章类型: Case Reports
    特发性毛细血管渗漏综合征,也被称为克拉克森病,是医生应该意识到的低血容量性休克的罕见原因。它的特征是低血容量状态,具有广泛的液体第三间距的特征,并提出了诊断和治疗挑战。这里,我们介绍了一例具有挑战性的病例,一例36岁女性经历了反复发作的广泛水肿和低血容量性休克症状,提示毛细血管渗漏综合征.在此病例报告中描述了用于治疗该疾病的复苏和治疗措施。
    Idiopathic capillary leak syndrome, also known as Clarkson\'s Disease, is a rare cause of hypovolemic shock that physicians should be aware of. It is characterized by a state of hypovolemia with features of widespread fluid third spacing and poses diagnostic and therapeutic challenges. Here, we present a challenging case of a 36-year-old woman who experienced recurrent episodes of widespread edema and hypovolemic shock symptoms suggestive of capillary leak syndrome. The resuscitative and therapeutic measures employed in managing this disease are described in this case report.
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  • 文章类型: Case Reports
    背景:色素沉着绒毛结节性滑膜炎(PVNS)是一种影响滑膜关节的良性增生性疾病,法氏囊,和肌腱鞘.迄今为止,很少有研究报道PVNS患者术后疼痛和水肿的治疗。在这里,我们介绍了一例女性,在滑膜切除术和关节镜下半月板部分切除术后1周出现左下肢疼痛和水肿,由于左膝屈伸受限而无法行走。
    方法:一名32岁妇女先后接受了滑膜切除术和关节镜下半月板部分切除术,并在我院接受了手动淋巴引流(MLD)和运动贴膜(KT)的联合治疗,以减轻术后疼痛和水肿。在治疗后2周和出院后1周随访时评估以下参数:髌上围,髌下圆周,视觉模拟量表评分,膝盖的运动范围,匹兹堡睡眠质量指数评分,汉密尔顿焦虑量表(HAMA)评分,和汉密尔顿抑郁量表(HAMD)评分。治疗后,术后患者左膝关节疼痛及水肿得到有效缓解,改善睡眠质量,显著降低HAMA和HAMD评分。
    结论:MLD和KT联合应用可能是缓解PVNS患者术后疼痛和水肿的有效方法。
    BACKGROUND: Pigmented villonodular synovitis (PVNS) is a benign proliferative disorder that affects the synovial joints, bursae, and tendon sheaths. To date, few studies have reported on the treatment of postoperative pain and edema in patients with PVNS. Herein, we present the case of a woman who developed pain and edema in the left lower limb 1 wk after synovectomy and arthroscopic partial meniscectomy and was unable to walk due to limited flexion and extension of the left knee.
    METHODS: A 32-year-old woman underwent synovectomy and arthroscopic partial meniscectomy successively and was treated with a combination of manual lymphatic drainage (MLD) and kinesio taping (KT) in our hospital to alleviate postoperative pain and edema. The following parameters were assessed at 2 wk post-treatment and 1 wk post-discharge follow up: suprapatellar circumference, infrapatellar circumference, visual analog scale score, knee range of motion, pittsburgh sleep quality index score, hamilton anxiety rating scale (HAMA) score, and hamilton depression rating scale (HAMD) score. After treatment, the postoperative pain and edema in the patient\'s left knee were effectively relieved, resulting in improved sleep quality and remarkably attenuated HAMA and HAMD scores.
    CONCLUSIONS: Combined MLD and KT may be an effective approach for relieving postoperative pain and edema in patients with PVNS.
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  • 文章类型: Case Reports
    这个病例报告描述了一个40多岁的男人,有慢性吸烟史,出现发音障碍的人.他接受了显微喉镜检查和活检,发现右声带前可疑病变。由于固体病变充当球形阀进入声门,因此面罩通气在诱导全身麻醉时很困难。此肿块被激光切除并送去组织病理学检查。这证明了血肿,可能是创伤性的,具有一些息肉状特征,与Reinke的晚期水肿一致。莱因克的水肿是一种良性疾病,慢性炎症会导致声带内的液体积聚。长期的炎症导致声带固有层紊乱,引起液体积聚,从而导致声带水肿。这个过程随后可能导致息肉形成,并可能导致沙哑的声音。此病例报告描述了这种良性疾病的潜在气道后遗症。
    This case report describes a man in his mid 40s, with a history of chronic smoking, who presented with dysphonia. He underwent microlaryngoscopy and biopsy for a suspicious lesion on the anterior right vocal cord. Mask ventilation proved difficult on induction of general anaesthesia due to a solid lesion acting as a ball valve into the glottis. This mass was LASER debulked and sent for histopathology. This demonstrated a haematoma, likely traumatic in origin, with some polypoidal features, consistent with advanced Reinke\'s oedema. Reinke\'s oedema is a benign condition where chronic inflammation causes fluid accumulation within the vocal cords. Long-standing inflammation leads to disarrangement of the vocal cord lamina propria, causing fluid accumulation and thereby resulting oedema of the vocal cords. This process can subsequently lead to polyp formation and can cause gravelly voice. This case report describes the potential airway sequelae of this benign condition.
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  • 文章类型: Case Reports
    背景摩比汉病,也被称为Morbihan综合征,是一种罕见的医学疾病,其特征是慢性面部水肿主要影响面部的上三分之二。尽管几十年来一直在医学文献中得到认可,其真正的患病率和潜在的病理生理学仍然知之甚少。各种假设,包括淋巴引流受损,血管通透性异常,免疫失调,和蠕形螨感染的炎症反应,已提出解释病因。案例报告我们介绍了一例61岁的男性,面部上三分之一有组织的眼周水肿,最终导致莫比汉病的诊断。病人做了面部中部拉皮手术,允许组织检索用于眼睑水肿皮肤的组织病理学检查,显示慢性炎症,小淋巴管扩张,和蠕形螨在滤泡内定位的特征。这些发现并不具体,但与诊断假设一致.患者被转诊至风湿病学家进行进一步评估和治疗。他对全身性皮质类固醇和免疫抑制治疗反应不佳。相反,这导致水肿延伸到上眼睑。患者选择不接受进一步治疗。结论Morbihan病由于其罕见且临床特征与其他面部疾病重叠,常被误诊。它的管理具有挑战性,可能需要医疗和外科干预相结合。全身性皮质类固醇,免疫抑制剂,局部治疗取得了不同的成功。外科手术,如眼睑成形术或激光治疗,可以在严重的情况下考虑。早期识别和适当的管理对于改善患者预后和生活质量至关重要。
    BACKGROUND Morbihan disease, also known as Morbihan syndrome, is a rare medical condition characterized by chronic facial edema predominantly affecting the upper two-thirds of the face. Despite being recognized in medical literature for decades, its true prevalence and underlying pathophysiology remain poorly understood. Various hypotheses, including impaired lymphatic drainage, abnormal vascular permeability, immune dysregulation, and inflammatory reactions to demodex infestation, have been proposed to explain the etiology. CASE REPORT We present a case of a 61-year-old man with organized periocular edema of the upper third of the face, ultimately leading to Morbihan disease diagnosis. The patient underwent a midface lift, allowing for tissue retrieval for histopathological examination of the eyelid edematous skin, which revealed chronic inflammation, ectasia of small lymphatic vessels, and features of demodex intrafollicular localization. These findings were not specific, but consistent with the diagnostic hypothesis. The patient was referred to a rheumatologist for further evaluation and treatment. He did not respond well to systemic corticosteroids and immunosuppressive therapy. Rather, this resulted in extension of the edema to the upper eyelid. The patient opted not to undergo further treatment. CONCLUSIONS Morbihan disease is often misdiagnosed due to its rarity and overlapping clinical features with other facial conditions. Its management is challenging and can require a combination of medical and surgical interventions. Systemic corticosteroids, immunosuppressive agents, and topical treatments have had varying success. Surgical procedures, such as blepharoplasty or laser therapy, can be considered in severe cases. Early recognition and appropriate management are crucial to improving patient outcomes and quality of life.
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  • 文章类型: Case Reports
    背景:TAFRO综合征是一种引起血小板减少症的罕见疾病,全身性水肿,发烧,器官增大,和肾功能损害。很少有报告表明与疫苗有关,少数病例接受了肾活检。TAFRO综合征通常是严重和致命的,原因不明.我们报告了一例在接种2019年冠状病毒病(COVID-19)疫苗后发生的TAFRO综合征。
    方法:一名82岁女性每隔3周接受两剂BNT162b2mRNA疫苗。两周后,她因水肿入院,伴有肾功能衰竭和血小板减少症。仔细检查后,她被诊断为TAFRO综合征。她接受了类固醇治疗,环孢菌素,和血小板生成素受体激动剂。患者在缓解数月后出院。
    结论:尽管先前有报道称接种COVID-19后发生TAFRO综合征,这是一个罕见的病例,患者病情缓解并出院。在这种情况下还进行了肾活检,这与以前的报告一致。TAFRO综合征的良好治疗过程提供了有价值的见解。
    BACKGROUND: TAFRO syndrome is a rare disorder that causes thrombocytopenia, generalized oedema, fever, organ enlargement, and renal impairment. Few reports have suggested an association with vaccines, and few cases have undergone renal biopsy. TAFRO syndrome is often severe and fatal, and its cause is unknown. We report a case of TAFRO syndrome that occurred after vaccination with the coronavirus disease 2019 (COVID-19) vaccine.
    METHODS: An 82-year-old woman received two doses of the BNT162b2 mRNA vaccine 3 weeks apart. Two weeks later, she was admitted to the hospital with oedema, accompanied with renal failure and thrombocytopenia. After close examination, she was diagnosed with TAFRO syndrome. She was treated with steroids, cyclosporine, and thrombopoietin receptor agonists. The patient was discharged after several months in remission.
    CONCLUSIONS: Although an incident of TAFRO syndrome after COVID-19 vaccination has been previously reported, this is a rare case in which the patient went into remission and was discharged. A renal biopsy was also performed in this case, which was consistent with previous reports. The favorable treatment course for TAFRO syndrome provides valuable insights.
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  • 文章类型: Case Reports
    皮肌炎是一种罕见的,病因不明的自身免疫性系统性疾病,表现为一系列主要影响皮肤和肌肉的临床症状和体征。皮肌炎患者可出现罕见的“非规范”表现。局灶性或全身性水肿是该疾病的罕见且经常被忽视的症状,虽然自发性肌内出血更为罕见且未得到充分认可,危及生命的并发症,构成临床医生的医疗紧急情况。没有已知的诱发因素能够预测哪些患者会发生这种并发症,目前缺乏考虑治疗方法的具体说明。在这里,我们介绍了一例皮肌炎患者,同时伴有脱囊和自发性肌内出血。为了提高对此类患者的认识和及时诊断,我们提供了相关文献和迄今为止报道的病例的综述。
    Dermatomyositis is a rare, autoimmune systemic disorder of unknown aetiology that presents as a constellation of clinical symptoms and signs primarily affecting skin and muscles. Patients with dermatomyositis can present with rare \"non-canonical\" manifestations. Focal or generalised oedema is an infrequent and often overlooked symptom of the disease, while spontaneous intramuscular haemorrhage is an even rarer and under-recognised, life-threatening complication that constitutes a medical emergency for clinical physicians. There are no known predisposing factors able to predict which patients will develop this complication and specific instructions considering treatment approach are currently lacking. Herein, we present a case of a patient with dermatomyositis complicated by both anasarca and spontaneous intramuscular haemorrhage. In order to raise awareness and timely diagnosis of such patients, we provide a review of the relevant literature and of the cases reported this far.
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  • 文章类型: Case Reports
    背景和目的:阴囊肿胀或鞘膜积液是文献中描述的急性胰腺炎的罕见并发症。我们介绍了一例年轻男性患者因急性间质性水肿性酒精诱发的胰腺炎首次发作而引起的阴囊肿胀。病例报告:一名22岁男子因早上腹泻和呕吐入院,随后出现严重腹痛。紧急腹部多层螺旋CT扫描显示脂肪变性,胰腺肿胀和急性胰周积液(间质性水肿性胰腺炎)。此外,扫描显示小肠环和肾前筋膜之间有液体,而道格拉斯空间中的液体很少。没有阴囊肿胀的迹象。入学的第二天,患者出现左阴囊肿胀和轻度疼痛,无红斑。第四天,对照CT扫描显示进展为中度重症胰腺炎(CT严重程度指数4).两侧均存在带睾丸静脉口径增加的大血管型静脉丛的阴囊静脉扩张,从阴囊到腹股沟管的水平。出院时阴囊肿胀明显减轻。结论:阴囊肿胀是胰腺急性炎症的罕见并发症或表现。确定由胰腺炎引起的阴囊肿胀很重要,因为在严重的情况下,它可能与将来可能的不孕症有关。
    Background and Objectives: Scrotal swelling or hydrocele is a rare complication of acute pancreatitis described in the literature. We present a case of penoscrotal swelling caused by the first attack of acute interstitial edematous alcohol-induced pancreatitis in a young male patient. Case report: A 22-year-old man was admitted to the emergency unit due to diarrhea and vomiting since morning which was followed by severe abdominal pain. Urgent abdominal multislice CT scan showed steatosis, pancreatic swelling and acute peripancreatic fluid collection (interstitial edematous pancreatitis). Also, scan showed fluid between small bowel loops and along the anterior renal fascia, while there was minimal amount of fluid in the Douglas space. There was no sign of penoscrotal swelling. On the second day of admission, the patient developed left scrotal swelling and mild pain without erythema. On the fourth day, a control CT scan showed progression to moderately severe pancreatitis (CT severity index 4). Dilated scrotal veins of the pampiniform venous plexus with an increased caliber of the testicular veins were present on both sides, from the scrotum to the level of the inguinal canal. Penoscrotal swelling was significantly reduced on discharge. Conclusions: Penoscrotal swelling is a rare complication or manifestation of acute inflammation of the pancreas. It is important to identify scrotal swelling caused by pancreatitis because in severe cases it can be related to possible infertility in the future.
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  • 文章类型: Case Reports
    背景:垂体微腺瘤是常见的,发病率为27%。甲状腺功能减退导致眶周水肿(PO)在文献中仍然罕见,但它仍在病因因素之内。
    方法:本研究显示了一名53岁的患者,该患者出现PO并到牙科诊所就诊以排除牙齿感染。
    方法:基于对适当的血液学和放射学检查的评估,垂体微腺瘤的甲状腺刺激激素(TSH)水平突然升高。
    对患者进行进一步研究以确定PO的主要原因。结论是,药物方案的中断导致TSH升高,并导致PO而不是微腺瘤。
    BACKGROUND: Pituitary microadenomas are said to be common, with an incidence of 27%. Hypothyroidism causing periorbital oedema (PO) remains rare in the literature, but it is still within the etiological factors.
    METHODS: This study presents a 53-year-old patient who presented with PO and visited the dental clinic to exclude dental infection.
    METHODS: Based on the evaluation of proper haematological and radiological investigations, pituitary microadenoma was identified with an abrupt increase in the thyroid stimulating hormone (TSH) level.
    UNASSIGNED: The patient was followed with further studies to identify the primary cause of PO. It was concluded that the disruption in the drug regimen caused an increase in the TSH and led to PO rather than microadenoma.
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