Irritable bowel syndrome: A concise review of current treatment concepts

Irritable bowel syndrome (IBS) is one of the most common gastrointestinal disorders causing patients to seek medical treatment. It is relatively resource intensive and the source of significant morbidity. Recent insights into the pathophysiology and treatment of IBS has given clinicians more options than ever to contend with this disorder. The purpose of our paper is to review older, “classic” treatments for IBS as well as newer agents and “alternative” therapies. We discuss the evidence base of these drugs and provide context to help develop appropriate treatment plans for IBS patients.     

Pancreatic trauma: A concise review

Traumatic injury to the pancreas is rare and difficult to diagnose.In contrast,traumatic injuries to the liver,spleen and kidney are common and are usually identified with ease by imaging modalities.Pancreatic injuries are usually subtle to identify by different diagnostic imaging modalities,and these injuries are often overlooked in cases with extensive multiorgan trauma.The most evident findings of pancreatic injury are posttraumatic pancreatitis with blood,edema,and soft tissue infiltration of the anterior pararenal space.The alterations of post-traumatic pancreatitis may not be visualized within several hours following trauma as they are time dependent.Delayed diagnoses of traumatic pancreatic injuries are associated with high morbidity and mortality.Imaging plays an important role in diagnosis of pancreatic injuries because early recognition of the disruption of the main pancreatic duct is important.We reviewed our experience with the use of various imaging modalities for diagnosis of blunt pancreatic trauma.     

A case of takotsubo cardiomyopathy associated with Kounis syndrome

In-stent restenosis (ISR) occurs in 20% to 40% of de novo coronary lesions treated with bare-metal stents (BMS), depending on lesion and patient-related factors. Drug-eluting stents coated with antiproliferative agents, represent a valid rationale for treatment and prevention of recurrent ISR, with low MACE rates. However, case reports and observational studies reported a definite increase in the incidence of late stent thrombosis after drug-eluting stents use, particularly in off-label cases and after clopidogrel withdrawal. The case we present shows target vessel occlusion occurring at the site of a previously implanted BMS, suggesting that in-stent restenosis was the main pathological mechanism leading to abrupt thrombotic vessel closure and acute myocardial infarction.     

Kounis综合征一例

<正>1 病例资料患者男性,61岁,因"皮疹、腹泻12 h"入院。患者12 h前进食蚕蛹及鸭肠,进食3 h后躯干出现大面积红色斑丘疹,伴瘙痒,腹泻,皮疹进行性加重,渐出现周身大汗、湿冷、胸闷气短,不伴胸痛、黑曚、晕厥,症状持续不缓解。120送至当地医院,入院后即刻给予吸氧、心电血氧监护。查体:血压76/42 mmHg,心率98次/min,呼吸24次/min,血氧饱和度98%(鼻导管吸氧),意识清楚,烦躁,全身大汗,面色苍白、眼睑水肿明显、     

Acquired hemophilia A: a concise review

Acquired hemophilia A is a rare but severe autoimmune bleeding disorder. It is more frequent in the elderly and results from the presence of autoantibodies directed against clotting factor VIII. In this review, we briefly report on the present state of knowledge regarding acquired hemophilia A, analyzing its epidemiology, pathogenesis, diagnostic, and clinical features. We also describe the main characteristics of this disorder according to its association with different conditions and the most important advances in the treatment of bleeding episodes and the eradication of the autoantibody.     

Pleural tuberculosis: A concise clinical review

Tuberculosis (TB) is the leading infectious cause of death worldwide, and the commonest cause of death in people living with HIV. Globally, pleural TB remains one of the most frequent causes of pleural exudates, particularly in TB‐endemic areas and in the HIV positive population. Most TB pleural effusions are exudates with high adenosine deaminase (ADA), lymphocyte‐rich, straw‐coloured and free flowing, with a low yield on mycobacterial culture. TB pleurisy can also present as loculated neutrophil‐predominant effusions which mimic parapneumonic effusions. Rarely, they can present as frank TB empyema, containing an abundance of mycobacteria. Up to 80% of patients have parenchymal involvement on chest imaging. The diagnosis is simple if M. tuberculosis is detected in sputum, pleural fluid or biopsy specimens, and the recent advent of liquid medium culture techniques has increased the microbiological yield dramatically. Where the prevalence of TB is high the presence of a lymphocyte‐predominant exudate with a high ADA has a positive predictive value of 98%. In low prevalence areas, the absence of an elevated ADA and lymphocyte predominance makes TB very unlikely, and pleural biopsy should be performed to confirm the diagnosis. Pleural biopsy for liquid culture and susceptibility testing must also be considered where the prevalence of drug resistant TB is high. Treatment regimens are identical to those administered for pulmonary TB. Initial pleural drainage may have a role in symptom relief and in hastening the resolution of the effusion. Surgical intervention may be required in loculated effusions and empyemas.     

Exosomes biological significance: A concise review

Exosomes were initially thought to be a mechanism for removing unneeded membrane proteins from reticulocytes. Current studies have shown that the process of exosome formation extends to many mammalian cells. This concise review highlights the findings reported at a Workshop on Exosomes. Full knowledge of the contribution of exosomes to intercellular information transmission and the potential medical application of this knowledge will depend on the ingenuity of future investigators and their insight into biological processes.     

Therapeutic management of patients with Marfan syndrome: focus on cardiovascular involvement

Marfan syndrome is an autosomal dominant disorder of the connective tissues, with mutation on the fibrillin-1 gene encoding for fibrillin, a major component of the extracellular microfibrils. The prevalence of the syndrome is 7 to 17 in 100,000. The mean life expectancy for untreated patients with Marfan syndrome is 32 years, with aortic dissection, aortic rupture, or cardiac failure from mitral or aortic valve regurgitation as the predominant cause of death in >90% of patients. However, with optimal clinical management of patients with Marfan syndrome, life expectancy may be improved substantially to a nearly normal life span. Cornerstones of clinical management include modern noninvasive imaging modalities, molecular analysis at the complementary deoxyribonucleic acid/deoxyribonucleic acid level of the fibrillin-1 gene, genetic counseling, lifestyle changes, and cardiovascular surveillance, the latter including regular imaging of the aorta and endocarditis prophylaxis. Prophylactic use of beta-blocking agents, the timing of elective surgery, and the optimal surgical technique are difficult issues that need to be evaluated individually. Juvenile, postoperative, and pregnant patients with Marfan syndrome pose particular problems. The goal of this review is to facilitate differential decision-making based on referenced evidence and to provide guidelines for medical and surgical therapeutic strategies.     

Cefuroxime-axetil induced allergic angina: An insight into classification management of Kounis syndrome

Echocardiographic finding of mitral annulus calcification and aortic sclerosis have been shown to be associated with higher incidence and prevalence of cardiovascular risk factors. Here, we report direct visualization of coronary artery disease of the LAD by non-invasive transthoracic echocardiography confirmed by coronary angiography. Further study is needed to evaluate septal calcification as a novel cardiovascular risk marker.     

Metabolic syndrome, peripheral vascular disease and coronary artery disease: A concise review

Metabolic syndrome has a very strong association with both forms of cardiovascular (CV) disease - coronary artery disease and peripheral vascular disease. There is also a marked interrelationship between the two forms of CV disease. When one form of CV disease is present, the other should be strongly suspected and the possible occurrence of associated vascular events should be clinically evaluated. When three of the five major components that define metabolic syndrome are present, the clinical evidence indicates that one or both forms of CV disease are present, at least in incipient form. To give maximum clinical benefit to the patient at risk, the clinician must be aware of these associations, look for them and institute the measures that can reduce the risk of progression.     

Cutaneous light chain amyloidosis with multiple myeloma: A concise review

Objective/BackgroundCutaneous immunoglobulin (Ig) amyloid light-chain (AL) amyloidosis associated with overt multiple myeloma (MM) is rare and optimal treatment is not well defined. The recently developed highly efficacious MM therapy has brought on a new set of challenges to this field for consideration. The goal of this paper is to describe the characteristics of cutaneous manifestations of systemic AL amyloidosis associated with MM according to age, sex, race, Ig type, plasma cell percentage, and cytogenetic and fluorescent in situ hybridization studies along with their outcomes.MethodsAn electronic search of the PubMed database was performed to obtain key literature in AL amyloidosis and MM, using the following search terms: multiple myeloma, immunoglobulin light chain amyloidosis, and cutaneous amyloidosis. The search results were narrowed by selecting studies in English. Results were confined to the following articles types: case reports, case series, and systematic reviews.ResultsWe identified 32 cases from the PubMed database search and examined their potential relevance. We found the following: (a) higher prevalence in women (two-thirds) and white population; (b) IgG and IgA were equally distributed with lambda (λ) light chain occurring in 53–66% of cases; (c) majority of cases (56%) presented as hemorrhagic bullous lesions, followed by purpura/ecchymosis in 25% of cases; and (d) majority (64%) died within 6 months since diagnosis.ConclusionsWe reviewed the constellation of the cutaneous manifestations of AL amyloidosis with concurrent MM. We found a female predominance, and more than half presented as hemorrhagic bullous lesions. There is a preponderance of λ light chains over kappa (κ) light chains, both as a free light chain (15% vs. 4%) and as an intact Ig (38% vs. 24%; absolute number of 14 vs. 7 patients, respectively). In the subgroup of patients with bullous skin lesions, λ light chain was present in eight cases and κ light chain in seven cases. All κ light chain subtypes presented with bullous lesions and no other cutaneous types of lesions. They carried very poor prognosis with majority of cases surviving only 6 months, much worse than overall patients with AL amyloidosis without myeloma or myeloma without amyloidosis.     

Kounis syndrome associated with hypersensitivity to hymenoptera stings

Hymenoptera stings can induce acute coronary syndromes by different pathogenetic mechanisms including direct action of the venom constituents on the coronary endothelium or allergic reaction with mediators acting on the coronary vasculature. Two patients were stung by wasps and honeybees and developed Kounis syndrome as a consequence of allergic reaction. Kounis syndrome is the concurrence of acute coronary syndromes with mast cell activation induced by allergic or hypersensitivity and anaphylactic of anaphylactoid reactions. It is caused via inflammatory mediators released through mast cell activation. The patients had pre-existing coronary artery disease (type II variant of Kounis syndrome) and the allergic reaction induced by hymenoptera stings seems to have triggered inflammatory mediator release. The pathophysiology and clinical implications of this association are discussed.     

Acute pulmonary embolism: a concise review of diagnosis and management

An acute pulmonary embolism (aPE) is characterised by occlusion of one or more pulmonary arteries. Physiological disturbance may be minimal, but often cardiac output decreases as the right ventricle attempts to overcome increased afterload. Additionally, ventilation‐perfusion mismatches can develop in affected vascular beds, reducing systemic oxygenation. Incidence is reported at 50–75 per 100 000 in Australia and New Zealand, with 30‐day mortality rates ranging from 0.5% to over 20%. Incidence is likely to increase with the ageing population, increased survival of patients with comorbidities that are considered risk factors and improving sensitivity of imaging techniques. Use of clinical prediction scores, such as the Wells score, has assisted in clinical decision‐making and decreased unnecessary radiological investigations. However, imaging (i.e. computed tomography pulmonary angiography or ventilation‐perfusion scans) is still necessary for objective diagnosis. Anti‐coagulation remains the foundation of PE management. Haemodynamically unstable patients require thrombolysis unless absolutely contraindicated, while stable patients with right ventricular dysfunction or ischaemia should be aggressively anti‐coagulated. Stable patients with no right ventricular dysfunction can be discharged home early with anti‐coagulation and review. However, treatment should be case dependent with full consideration of the patient’s clinical state. Direct oral anti‐coagulants have become an alternative to vitamin K antagonists and are facilitating shorter hospital admissions. Additionally, duration of anti‐coagulation must be decided by considering any provoking factors, bleeding risk and comorbid state. Patients with truly unprovoked or idiopathic PE often require indefinite treatment, while in provoked cases it is typically 3 months with some patients requiring longer periods of 6–12 months.     

Inherited factor XI deficiency: A concise review

Inherited factor XI (FXI) deficiency, also called Hemophilia C, is an uncommon autosomal recessive disorder, which is associated with a variable bleeding tendency that usually manifests after trauma or surgery. This concise report reviews current knowledge regarding the pathogenesis, genetics, diagnosis, clinical manifestations and management of this inherited bleeding disorder.     

Kounis syndrome: a new twist on an old disease

Kounis syndrome is the concurrence of acute coronary syndromes with conditions associated with mast cell activation, such as allergies or hypersensitivity and anaphylactic or anaphylactoid insults that can involve other interrelated and interacting inflammatory cells behaving as a 'ball of thread'. It is caused by inflammatory mediators such as neutral proteases including tryptase and chymase, arachidonic acid products, histamine, platelet activating factor and a variety of cytokines and chemokines released during the activation process. Platelets with FCεRI and FCεRII receptors also participate in the above cascade. Vasospastic allergic angina, allergic myocardial infarction and stent thrombosis with occluding thrombus infiltrated by eosinophils and/or mast cells constitute the three reported variants of this syndrome. Kounis syndrome is a ubiquitus disease that represents a magnificent natural paradigm and nature's own experiment, in a final trigger pathway implicated in cases of coronary artery spasm and plaque rupture. Kounis syndrome can complicate anesthesia, vaccination, medical therapy and stent implantation and it seems to be associated with coronary allograft vasculopathy and takotsubo syndrome, it can often be confused with hypersensitivity myocarditis and can be the cause of unexplained sudden death. Kounis syndrome has revealed that the same mediators released from the same inflammatory cells are present in acute coronary events of nonallergic etiology. These cells are not only present in the culprit region before plaque erosion or rupture but they release their contents just before an actual coronary event. Therefore, does Kounis syndrome represent a magnificent natural paradigm and nature's own experiment in a final trigger pathway implicated in cases of coronary artery spasm and plaque rupture showing a novel way towards our effort to prevent acute coronary syndromes? Drugs, substances targeting the stem cell factor that is essential for mast cell development, proliferation, survival, adhesion and homing as well as monoclonal antibodies and natural molecules that protect mast cell surface and stabilize mast cell membrane could emerge as novel therapeutic ways capable to prevent acute coronary and acute cerebrovascular events.     

A concise review of pulmonary sarcoidosis

This is an update on sarcoidosis, focusing on etiology, diagnosis, and treatment. In the area of etiopathogenesis, we now have a better understanding of the immune response that leads to the disease as well as genetic factors that modify both the risk for the disease and its clinical outcome. Several groups have also identified possible agents as a cause for sarcoidosis. Although none of these potential causes has been definitely confirmed, there is increasing evidence to support that one or more infectious agents may cause sarcoidosis, although this organism may no longer be viable in the patient. The diagnosis of sarcoidosis has been significantly aided by new technology. This includes the endobronchial ultrasound, which has been shown to increase the yield of needle aspiration of mediastinal and hilar lymph nodes. The positive emission tomography scan has proven useful for selecting possible biopsy sites by identifying organ involvement not appreciated by routine methodology. It has also helped in assessing cardiac involvement. The biologic agents, such as the anti-tumor necrosis factor antibodies, have changed the approach to refractory sarcoidosis. There is increasing evidence that the clinician can identify which patient is most likely to benefit from such therapy. As new and more potent antiinflammatory agents have been developed, it is clear that there are other factors that burden the patient with sarcoidosis, including fatigue and sarcoidosis-associated pulmonary hypertension. There have been several recent studies demonstrating treatment options for these problems.     

Drug-induced thrombotic thrombocytopenic purpura/hemolytic uremic syndrome: a concise review

An extensive variety of drugs have been associated with thrombotic thrombocytopenic purpura and hemolytic uremic syndrome (TTP/HUS). Although a direct causal effect has usually not been proven, the cumulative evidence linking several drugs with TTP/HUS is strong. This paper reviews several categories of drugs including antineoplastics, immunotherapeutics and anti-platelet agents that have been reported to induce TTP/HUS. The pathogenesis of drug-induced TTP/HUS and the effectiveness of treatment regimens are also reviewed. A consensus on diagnostic criteria to accurately and consistently diagnose drug-induced TTP is needed.     

Parathyroid carcinoma. A concise review

Parathyroid carcinoma is a very rare malignancy and the least common among endocrine malignat tumors. Its etiology is still unclear and some hypotheses have been suggested: previous external radiation to the neck, pregression from benign to malignant lesion, familial hyperparathyroidism. An early accurate diagnosis is very important since surgery is the most effective therapeutic approach to parathyroid carcinoma. Prognosis is quite variable and stress is laid on the early diagnosis and radical surgery which seem to be most favorable prognostic factors. An accurate follow-up should include frequent measurements of serum calcium and PTH levels to promptly detect recurrence or metastases which are usually associated with severe hyeprcalcemia.     

Hypertrophic cardiomyopathy: state-of-the-art review, with focus on the management of outflow obstruction

Abstract
Significant advances in our understanding and management of hypertrophic cardiomyopathy have been made in the last decade, as the complex genetics and ­phenotype−genotype correlations that characterize the disease are gradually unravelled. The well-described clinical heterogeneity of hypertrophic cardiomyopathy is now understood to be based on profound genetic vari­ability, with at least 10 genes and over 150 mutations implicated. Several new therapeutic tools have entered clinical practice. The implantable cardioverter-­defibrillator is now strongly indicated in those at high risk of sudden arrhythmic death. Our ability to abort sudden death in this subgroup has placed added emphasis on risk stratification in newly diagnosed patients. New procedures have also been developed for the relief of outflow obstruction in patients with refractory symptoms and a significant subaortic outflow gradient. Although not as efficacious as the 'gold-standard'­surgical myectomy−myotomy, dual-chamber pacemaker implantation can be of modest benefit in select patients. Percutaneous transluminal septal myocardial ablation is an emerging catheter-based procedure for the relief of left ventricular outflow obstruction. Long-term follow-up data are still awaited. However, intermediate-term results suggest equivalent efficacy to surgical ­myectomy−myotomy. (Intern Med J 2003; 33: 521−529)