Acquired Hemophilia A in Aged People: A Systematic Review of Case Reports and Case Series

Acquired hemophilia A (AHA) is a rare disease that requires urgent management. Currently, there is no consensus regarding optimal management in aged people. This systematic review aimed to describe diagnosis, clinical features, management, and endpoints in population aged 65 years or over with AHA.A literature search up to and including 31 May 2017 was performed in Medline, Embase, and Scopus. The search strategy on article titles comprised the following terms: “acquired” AND (“hemophilia A” OR “haemophilia A”). Filters were applied for age (65 years or older), publication type (case reports and case series), and studies including human beings only. There was no language restriction in the search strategy. Studies with no data on immunosuppressive therapy, and studies in other languages than English or French were excluded. Patient-level and study-level information was extracted.In total, 270 studies were identified by the literature search. After exclusion of duplicates, and studies presenting exclusion criteria, 80 articles including 159 cases were included in the final review. These 159 cases were 76.1 ± 7.2 years old, and were mainly men (64%).There is wide variety in the therapies used to eradicate the Factor VIII autoantibody, and efficacy is difficult to assess. The majority of patients with AHA receive immunosuppressants. Mortality is high, and likely depends on the rapidity of diagnosis and implementation of adequate management and monitoring.     

Myocardial Stunning Following Combined Modality Combretastatin‐Based Chemotherapy: Two Case Reports and Review of the Literature

Myocardial stunning, known as stress cardiomyopathy, broken‐heart syndrome, transient left ventricular apical ballooning, and Takotsubo cardiomyopathy, has been reported after many extracardiac stressors, but not following chemotherapy. We report 2 cases with characteristic electrocardiographic and echocardiographic features following combined modality therapy with combretastatin, a vascular‐disrupting agent being studied for treatment of anaplastic thyroid cancer. In 1 patient, an ECG performed per protocol 18 hours after drug initiation showed deep, symmetric T‐wave inversions in limb leads I and aVL and precordial leads V₂ through V₆. Echocardiography showed mildly reduced overall left ventricular systolic function with akinesis of the entire apex. The patient had mild elevations of troponin I. Coronary angiography revealed no epicardial coronary artery disease. The electrocardiographic and echocardiographic abnormalities resolved after several weeks. The patient remains stable from a cardiovascular standpoint and has not had a recurrence during follow‐up. An electrocardiogram performed per protocol in a second patient showed deep, symmetric T‐wave inversions throughout the precordial leads and a prolonged QT interval. Echocardiography showed mildly reduced left ventricular function with hypokinesis of the apical‐septal wall. Acute coronary syndrome was ruled out, and both the electrocardiographic and echocardiographic changes resolved at follow‐up. Although the patient remained pain‐free without recurrence of anginal symptoms during long‐term follow‐up, the patient developed progressive malignancy and died. Copyright © 2009 Wiley Periodicals, Inc.     

Idiopathic Granulomatous Mastitis: Case Reports and Review of Literature

Idiopathic granulomatous mastitis (IGM) is an uncommon benign disorder of the breast that can mimic two frequent breast disorders, breast carcinoma and breast abscess. In this report, we present two patients seen in a community teaching hospital over a period of one year, diagnosed with IGM after histological evaluation. One patient responded well to immunosuppressive therapy, but the second patient required bilateral mastectomy due to the severe and recurrent nature of the disease. IGM is a disorder that should be considered in the evaluation of women who present with painful breast disease. We discuss the diagnosis, clinical presentation and management of IGM.     

Left Ventricular Assist Devices and Gastrointestinal Bleeding: A Narrative Review of Case Reports and Case Series

Background

The use of left ventricular assist devices (LVADs) has become a state‐of‐the‐art therapy for advanced cardiac heart failure; however, multiple reports in the literature describe an increased risk for gastrointestinal (GI) bleeding in these patients. We characterized this association by reviewing recent studies on this topic.

Hypothesis

GI bleeding occurs frequently in patients with LVADs, especially with devices with nonpulsatile flow patterns.

Methods

We performed a comprehensive literature review to identify articles that reported GI bleeding in patients with LVADs. Databases used included PubMed, EMBASE, Scopus, Web of Knowledge, and Ovid. Baseline and outcome data were then ed from these reports.

Results

We identified 10 case reports and 22 case series with 1543 patients. The mean age was 54.2 years. Most patients had nonpulsatile LVADs (1316, 85.3%). Three hundred and seventeen patients (20.5%) developed GI bleeding; this occurred more frequently in patients with nonpulsatile LVADs. Multiple procedures were performed without complications but often did not identify a definite bleeding site. Suspect lesions occurred throughout the GI tract but were more frequent in the upper GI tract. Many patients had arteriovenous malformations. All patients received medical therapy. None of the patients had their LVAD replaced. The use of anticoagulation did not appear to predispose these patients to more GI bleeding episodes. Conclusions: Patients with LVADs have frequent GI bleeds, especially from arteriovenous malformations, which can occur throughout the GI tract. Most diagnostic and therapeutic interventions can be used safely in these patients. The pathogenesis of the GI bleeding in these patients may involve the use of anticoagulant medications, the formation of arteriovenous malformations, loss of von Willebrand factor activity, and mucosal ischemia. The authors have no funding, financial relationships, or conflicts of interest to disclose.     

Customized Root-Analogue Implants: A Review on Outcomes from Clinical Trials and Case Reports

(1) It is estimated that 10% of the world’s population will need a dental implant in their lifetime. Despite all the advances in the comprehension of dental implant designs, materials and techniques, traditional implants still have many limitations. Customized root-analogue implants are, therefore, gaining increased interest in dental rehabilitation and are expected to not only preserve more hard and soft tissues but also avoid a second surgery and improve patient overall satisfaction. In this sense, the aim of this review was to collect and analyse the clinical trials and case reports on customized root-analogue implants available in the literature; (2) This review was carried out according to the PRISMA Statement. An electronic database search was performed using five databases: PubMed, Google Scholar, Medline, Science Direct, and Scopus. The following keywords were used for gathering data: custom-made, dental implants, root-analogue, anatomical, customized and tooth-like; (3) 15 articles meeting the inclusion criteria—articles reporting clinical trials, case reports or animal studies and articles with root-analogue implants and articles with totally customized implant geometries—were selected for the qualitative synthesis. The design and manufacturing techniques, implant material and surface treatments were assessed and discussed; (4) The performance of some root-analogue implants with specific features (i.e., macro-retentions) was successful, with no signs of infection, periodontitis nor bleeding during the follow-up periods.     

三甲氧苄嗪和缺血性心脏病

心肌缺血主要是一种代谢性事件。通过药物干预可以直接刺激葡萄糖代谢或抑制脂肪酸beta氧化间接促进葡萄糖代谢,减少组织损害。三甲氧苄嗪（TMZ）可以有效的改善心肌在缺氧状态下的葡萄糖代谢水平,可以增加心肌对缺血再灌注损伤的耐受性,维持细胞膜电活动的稳定性,缩小心肌梗死面积,改善心功能,而对血流动力学没有明显影响。近年来逐渐受到关注。本文回顾了近年来国外进行的临床观察和基础研究的结果。     

Neonatal Myocardial Infarction: Case Report and Review of the Literature

Myocardial infarction in a neonate is rare. We describe the case of a full‐term male who presented with respiratory distress. A chest radiograph demonstrated cardiomegaly. An electrocardiogram revealed ST segment changes suggestive of ischemia. Cardiac enzymes were elevated and an echocardiogram revealed a regional wall motion abnormality. Cardiac catheterization was performed demonstrating occlusion of the ramus intermedius branch of the left main coronary artery. The patient decompensated, requiring extracorporeal membrane oxygenation (ECMO). The infant was able to be decannulated from ECMO support in 5 days and was ultimately discharged on hospital day 25. We review this case as well as the literature on neonatal myocardial infarction.     

Intermittent Left Bundle Branch Block and Myocardial Ischemia in Patient Without Coronary Artery Stenosis:A Case Report

Left bundle branch block （ LBBB ）, traditionally viewed as an electrophysiologic abnormality, is increasingly recognized for its effects on hemodynamics and patient＇s prognosis^[1]. Exercise nuclear studies frequently show reversible perfusion defects in the absence of obstructive coronary artery disease^[2] and some patients with intermittent LBBB develop angina coincident with the onset of LBBB^[3]. We report a case of intermittent LBBB with abnormal stress technetium 99m TC single-photon emission computed tomography （SPECT） study and normal coronary artery angiography.     

Primary Intestinal Lymphangiectasia: Four Case Reports and a Review of the Literature

Background  

Primary intestinal lymphangiectasia (PIL) is a rare digestive disease and most articles on this condition are isolated case reports.     

Nontyphoidal Cardiac Salmonellosis: Two Case Reports and a Review of the Literature

Nontyphoidal Salmonella, especially Salmonella enterica, is a rare cause of endocarditis and pericarditis that carries a high mortality rate. Proposed predisposing conditions include immunodeficiency states, congenital heart defects, and cardiac valve diseases. We present 2 cases of cardiovascular salmonellosis. The first case is that of a 73-year-old woman with mechanical mitral and bioprosthetic aortic valves who died from sequelae of nontyphoidal Salmonella mitral valve vegetation, aortic valve abscess, and sepsis. The second case is that of a 62-year-old man with a recent systemic lupus erythematosus exacerbation treated with oral steroids, who presented with obstructive features of tamponade and sepsis secondary to a large S. enteritidis purulent pericardial cyst. He recovered after emergent pericardial drainage and antibiotic therapy. Identifying patients at risk of cardiovascular salmonellosis is important for early diagnosis and treatment to minimize sequelae and death. We reviewed the literature to identify the predisposing risk factors of nontyphoidal Salmonella cardiac infection.     

Coronary Artery Embolism: Two Case Reports and a Review of the Literature

Coronary embolism (CE) is an uncommon and unique cause of acute myocardial infarction. In this report, we review 216 cases of CE including 2 new cases from our institution. The mean patient age was 52.5 years and 62% of the patients were males. Chest pain was the most common presenting symptom followed by dyspnea, and the most commonly affected vessel was the left anterior descending artery. Leading etiologies of the embolus were atrial fibrillation, septic emboli, and iatrogenic causes. Treatment approaches varied with thrombus aspiration being used in 30% of cases. In-hospital mortality rate was 36% and 13% of the cases were complicated by cerebrovascular accident. CE is a unique pathology that leads to acute myocardial infarction. It portends a high mortality rate and requires a high level of suspicion as symptoms may be misleading. Further research is needed in order to improve recognition and management and to lower associated mortality.     

心肌缺血后心肌存活性的评价

心肌缺血后如何评价其存活性的问题近年来日益引人注目,存活心肌主要指急性心肌缺血时间＜20分钟后引起的“顿抑心肌”及慢性心肌缺血后导致的“冬眠心肌”两种状况。本文从心肌代谢、超声心动图及心电图三个方面总结了目前常用的评价心肌存活性的方法。     

心肌桥合并Tako-tsubo心肌病1例报告并文献复习

目的：探讨Tako-tsubo心肌病的临床表现。方法：对1例心肌桥合并Tako-tsubo心肌病患者的诊治过程进行追踪随访并复习相关文献。结果：二维超声心动图结合室壁运动分析是冠状动脉病变最有效的无创筛查手段;心室造影及冠脉造影有利于除外冠心病和先天性心脏畸形,是诊断Tako-tsubo心肌病的影像学＂金标准＂。结论：选择性地对急性胸痛患者进行包括心室造影在内的多种影像学检查可以有效提高Tako-tsubo心肌病的诊断率。     

Spiked Helmet Electrocardiographic Sign-A Systematic Review of Case Reports

First reported in 2011, the spiked helmet sign (SHS) is an electrocardiographic pattern of ST-segment elevation anecdotally associated with poor prognosis. This study aims to systematically evaluate the electrocardiographic characteristics, clinical presentations, and outcomes of all cases of SHS reported in the literature. PubMed, Scopus, Web of Science, and EMBASE were searched electronically from their inception until November 2022. The Joanna Briggs Institute Critical Appraisal Checklist for Case Reports was used to critically appraise included studies. Studies written in English describing at least one patient with SHS were included. Altogether, 26 case reports or series describing 39 patients with SHS were included. All included studies were rated of acceptable quality. Associated conditions were heterogeneous, with intracranial hemorrhagic complications being the most common (9 patients), followed by pneumothorax (6 patients) or severe pneumonia (4 patients), bowel ischemia or obstruction (6 patients), and autonomic dysfunction (3 patients with Takotsubo cardiomyopathy and 3 patients with spinal injury, cocaine overuse, and stellate gangliectomy). Two patients had multiple complications and 12 other patients suffered from sepsis, myocardial infarction, etc. Clinical outcomes were reported for 32 patients, of whom 19 (59%) died during hospitalization (6 patients with pneumothorax or pneumonia, 4 patients with intracranial hemorrhagic complications, 2 patients with bowel ischemia or obstruction, and 7 patients due to other reasons). SHS may be associated with poor prognosis, necessitating its prompt recognition by clinicians and swift evaluation for underlying causes. Larger studies are needed to elucidate its prevalence, clinical implications, and precipitating mechanisms.     

A Conservative Approach to Granular Cell Tumors of the Esophagus: Four Case Reports and Literature Review

We present here four patients with six granular ceil tumors of the esophagus: all are women, 22,38,47, and 49 yr old. In three of them, single polyps were encountered. In the fourth, age 38, two lesions were found in 1982, and a new one, a satellite polyp, has appeared after 5 yr of follow-up. Ali of the patients'conditions were followed up [mean time 65 months (16 months to 10 yr)]. In connection with these case reports, we include an up-to-date review of the world literature on this subject.