Clues to detect tumor necrosis factor receptor-associated periodic syndrome (TRAPS) among patients with idiopathic recurrent acute pericarditis: results of a multicentre study

Background

The potential clinical expression of tumor necrosis factor receptor-associated periodic syndrome (TRAPS), in the form of idiopathic recurrent acute pericarditis (IRAP) has not been explored in the medical literature. The aim of this study was to evaluate the incidence of TRAPS mutations in patients with recurrent pericarditis and identify possible clues to TRAPS diagnosis.

Methods

Therefore, 131 consecutive Caucasian IRAP patients were investigated for mutations of the TRAPS gene and prospectively evaluated.

Results

Out of 131 patients, 8 (6.1%) carried a mutation in the TNFRSF1A gene. Compared with those without genetic mutations, patients with TRAPS mutations had more frequently a positive family history for pericarditis and periodic fever syndromes (p?p?p?p?Conclusion TRAPS is a cause of recurrent pericarditis in 6% of unselected cases with recurrent pericarditis. A positive family history for pericarditis or periodic fever syndromes, a poor response to colchicine, recurrences after the first year from the index attack or on colchicine treatment, as well as the need of immunosuppressive agents are clues of the possible presence of TNFRSF1A gene mutations in patients with recurrent pericarditis.     

Acute pericarditis

Although acute pericarditis is most often associated with viral infection, it may also be caused by many diseases, drugs, invasive cardiothoracic procedures, and chest trauma. Diagnosing acute pericarditis is often a process of exclusion. A history of abrupt-onset chest pain, the presence of a pericardial friction rub, and changes on electrocardiography suggest acute pericarditis, as do PR-segment depression and upwardly concave ST-segment elevation. Although highly specific for pericarditis, the pericardial friction rub is often absent or transient. Auscultation during end expiration with the patient sitting up and leaning forward increases the likelihood of observing this physical finding. Echocardiography is recommended for most patients to confirm the diagnosis and to exclude tamponade. Outpatient management of select patients with acute pericarditis is an option. Complications may include pericardial effusion with tamponade, recurrence, and chronic constrictive pericarditis. Use of colchicine as an adjunct to conventional nonsteroidal anti-inflammatory drug therapy for acute viral pericarditis may hasten symptom resolution and reduce recurrences.     

Peritoneal hydatid cyst perforation: a rare cause of emergency abdominal surgeries

Introduction  In hydatid cyst disease, perforation and spontaneous fistula formation are rare, yet very serious, complications. In this study we describe five cases of hydatid cyst disease in which the cysts perforated. Methods  In this retrospective study we evaluated five patients who were surgically treated for perforated hydatid cysts between 2002 and 2006. All patients were admitted to a local hospital with acute abdominal symptoms. Results  The mean age of the patients (four males and one female) was 39 years. The perforations occurred spontaneously in four patients, and were the result of trauma in one. Three of the hydatid cysts were located in the liver and two in the spleen. Only one patient had a recurrent hydatid cyst. Diagnostic tools included ultrasonography (n=5), computerized tomography (n=1), and diagnostic peritoneal lavage (n=1). The cysts were treated by radical (n=2) or conservative (n=3) methods. There was no postoperative morbidity or mortality, and no recurrences of hydatid disease after follow-up (mean: 14 months). Conclusion  Cyst perforation in the peritoneal cavity is a serious complication of hydatid disease and should be treated immediately. The literature suggests that recurrences may be related to the operative technique, the location of the cyst, and inadequate irrigation of the peritoneal cavity with scolicidal agents. The findings of this study suggest that if treated promptly, cyst perforation can be successfully treated.     

Management of recurrent otitis media.

Recurrent otitis media is defined as three or more episodes of acute otitis media in six months or four or more episodes in one year, with the bouts of acute infection separated by intervals of full resolution. The disorder is common in children and is different from otitis media with effusion, the other frequently encountered chronic ear disease. Daily low-dose antibiotic therapy is recommended as initial prophylaxis against recurrent otitis media. Pressure-equalizing tubes may be used in patients who have more than one episode of breakthrough otitis media while receiving antibiotics. Adenoidectomy should be reserved for use when pressure-equalizing tubes fail or when insertion of a second set of tubes is required.     

Diagnostic issues in the clinical management of pericarditis

Aims: To review the current major diagnostic issues on the diagnosis of acute and recurrent pericarditis. Methods: To review the current available evidence, we performed a through search of several evidence‐based sources of information, including Cochrane Database of Systematic Reviews, Clinical Evidence, Evidence‐based guidelines from National Guidelines Clearinghouse and a comprehensive Medline search with the MeSH terms ‘pericarditis’, ‘etiology’ and ‘diagnosis’. Results: The diagnosis of pericarditis is based on clinical criteria including symptoms, presence of specific physical findings (rubs), electrocardiographical changes and pericardial effusion. Although the aetiology may be varied, most cases are idiopathic or viral, even after an extensive diagnostic evaluation. In such cases, the course is often benign following anti‐inflammatory treatment, and management would be not affected by a more precise diagnostic evaluation. A triage of pericarditis can be safely performed on the basis of the clinical and echocardiographical presentation. Specific diagnostic tests are not warranted if no specific aetiologies are suspected on the basis of the epidemiological background, history and presentation. High‐risk features associated with specific aetiologies or complications include: fever > 38 °C, subacute onset, large pericardial effusion, cardiac tamponade, lack of response to aspirin or a NSAID. Conclusions: A targeted diagnostic evaluation is warranted in acute and recurrent pericarditis, with a specific aetiological search to rule out tuberculous, purulent or neoplastic pericarditis, as well as pericarditis related to a systemic disease, in selected patients according to the epidemiological background, presentation and clinical suspicion.     

Acute post-MI pericarditis.

The diagnosis of acute pericarditis may be difficult to establish in patients with recent transmural infarcts as the symptomatology frequently mimics the clinical presentation associated with recurrent ischemia. A careful assessment of the postmyocardial infarction patient may reveal the clinical and electrocardiographic features associated with developing pericarditis. Prompt and accurate recognition of this complication is critical in order to institute definitive therapy, minimize the risk of hemodynamic compromise, and provide psychological support.     

Acute Pericarditis Resulting From an Endocardial Active Fixation Screw-In Atrial Lead

We examined the occurrence of acute pericarditis after pacemaker implantation in 123 consecutive patients (61 males, 62 females, ages 17–87 years) in whom a newer atrial active fixation bipolar lead was inserted endocardially in the right atrium for dual chamber pacing. The atrial leads were positioned to obtain the best possible pacing and sensing thresholds, after an initial attempt was made for insertion into the right atrial appendage or medially into the right atrial septum. Six patients (4.9%) developed acute symptomatic pericarditis with effusion within 24 hours of implantation. Of these six patients, four had leads screwed into the lateral waH. and the other two had leads placed in the anterolateral wall. The lead implantation parameters between patients with pericarditis and those without did not show any significant difference in the atrial P wave amplitude (2.3 ± 0.4 vs 2.9 ± 0.9 mV), pacing threshold (1.1 ± 0.2 vs 1.1 ± 0.4 V), or resistance (524 ± 112 vs 480 ± 94 ohms). All symptomatic patients were treated with nonsteroidal anti-inflammatory drugs with symptoms resolving in 1–2 weeks. We condude that: (1) a significant number of patients (4.9%) developed acute symptomatic pericarditis after insertion of this type of atrial fixation lead: (2) because of the lead design, the implantation parameters coud not be taken to predict the occurrence of pericarditis: and (3) caution should be taken for the insertion of this lead into the thin atrial wall.     

A strategy for early detection of recurrent hepatocellular carcinoma following initial remission by transcatheter arterial chemoembolization

OBJECTIVES: To find a better surveillance method in detecting recurrent HCCs, patterns of recurrences following initial remission by transcatheter arterial chemoembolization (TACE) were evaluated. METHODS: Of 235 consecutive HCC patients who underwent TACE, 69 with initial remission were followed for >12 months. We compared the recurrence rates according to the characteristics of original HCCs and analyzed the locations of recurrent HCCs. We also evaluated the diagnostic efficacies of CT scan with serum AFP, angiography and Lipiodol CT scan in detecting recurrent HCCs. RESULTS: In 37 of 69, recurrent HCCs were detected after a median period of 17 months. Multinodular HCCs recurred more frequently than single-nodular HCCs. All of 5 patients with portal vein thrombosis recurred. Although 46% of recurrences were adjacent to original tumors, 62% were separated from them (8% at both). HCC with heterogeneous lipiodol uptake frequently recurred adjacent to original tumors. Only 18 of 37 recurrent HCCs were initially detected by serum AFP and CT scans; 17 by angiography, 2 only by lipiodol CT scan. CONCLUSIONS: Regular angiography may be valuable in detecting recurrent HCCs, especially in multinodular HCC. HCC with heterogeneous lipiodol uptake should be treated in combination with local ablation therapy.     

A case of transient left ventricular apical ballooning syndrome in a child: clinical features and imaging findings

Transient left ventricular apical ballooning syndrome (TLVABS) is an acute cardiac disease that is characterized by transient left ventricular systolic dysfunction involving the apical region. The symptoms and electrocardiographic changes of TLVABS mimic those observed in acute myocardial infarction while obstructive coronary arterial lesions are not seen in patients with TLVABS. TLVABS usually occurs in elderly women after physical or emotional stress. However, it is very rare in children and so it not well known to pediatricians. Accordingly, TLVABS in children can be misdiagnosed as myocarditis or cardiomyopathy. We report here on a case of child who showed the typical findings of TLVABS in association with pericarditis. He presented with dyspnea and pericardial effusion, which required pericardiocentesis. After pericardiocentesis, he showed the typical echocardiographic and electrocardiographic findings of TLVABS. The MRI findings at 14 days after the initial symptoms showed normal coronary arteries and normal left ventricular function without any wall motion abnormalities. In addition, no delayed hyper enhancement was found on delayed-enhanced (DE)-MRI. We also reviewed the other reported cases of TLVABS in patients who were under the age of 40.     

Heart Rhythm During Syncope and Presyncope: Results of Implantable Loop Recorders

NIEROP, P.R., et al. : Heart Rhythm During Syncope and Presyncope: Results of Implantable Loop Recorders. Ambulatory ECG monitoring in patients with recurrent syncope is nondiagnostic in the majority of cases. Recently, an ECG implantable loop recorder (ILR) has been introduced. The ILR performs continuous ECG monitoring over a period of at least 14 months. From February 1997 to September 1999, 35 patients underwent implantation of an ILR. During a mean follow-up of  11 ± 8 months  , 24 (69%) patients had recurrent syncope or presyncope events. Four (11%) patients were not capable of activating the ILR to save the event. A symptom-rhythm correlation could be studied in 20 (83%) of 24 patients. Forty of 44 recurrences were captured by the ILR. There were 14 (40%) patients with at least one syncopal episode. An arrhythmic cause for syncope was found in eight of them (bradycardia in four and tachycardia in four). In the other six patients the heart rhythm was normal. In 17 (49%) patients with 1-year follow-up, the mean syncope event rate 12 months before ILR implantation was  4.7 ± 2.4  , whereas the mean syncope event rate 12 months after ILR implantation was  1.3 ± 0.7  (  P < 0.01  ). Resolution of symptoms was observed in 6 (17%) patients. These patients were significantly younger than patients without resolution (  50 ± 18 vs 69 ± 14 years, p < 0.01  ) and five were women. Three (9%) patients died during follow-up, all of them were noncompliant during their follow-up. In conclusion, the ILR made symptom—rhythm correlation possible in 83% of patients with recurrent syncope. Syncope recurrences decreased significantly after implantation of the device, especially in the younger patients. Noncompliant patients had a high mortality rate.     

The optimal treatment of venous thrombosis: current status and future perspectives

Acute deep venous thrombosis (DVT) of the lower extremities is a serious and potentially fatal disorder, which often complicates the course of hospitalized patients but may also affect ambulatory and otherwise healthy people. Venous thrombosis is uncommon in young individuals and becomes more frequent with advancing age. The clinically important problems associated with venous thrombosis are death from pulmonary embolism, morbidity resulting from the acute event, recurrent venous thromboembolic events, the post-thrombotic syndrome, and the inconvenience and side-effects of investigations and treatment. The main objectives of treatment of DVT are prevention of (both fatal and nonfatal) pulmonary embolism and thrombus extension in the acute phase of the disease, prevention of recurrences of venous thromboembolism in the months following the acute episode, and prevention of late sequelae (post-thrombotic syndrome). These objectives are satisfactorily achieved with anticoagulant drugs (heparin and vitamin K antagonists), which therefore are the mainstays of DVT treatment. Other therapeutic options have a more limited application.     

Adult rhinosinusitis: diagnosis and management

Rhinosinusitis can be divided among four subtypes: acute, recurrent acute, subacute and chronic, based on patient history and a limited physical examination. In most instances, therapy is initiated based on this classification. Antibiotic therapy, supplemented by hydration and decongestants, is indicated for seven to 14 days in patients with acute, recurrent acute or subacute bacterial rhinosinusitis. For patients with chronic disease, the same treatment regimen is indicated for an additional four weeks or more, and a nasal steroid may also be prescribed if inhalant allergies are known or suspected. Nasal endoscopy and computed tomography of the sinuses are reserved for circumstances that include a failure to respond to therapy as expected, spread of infection outside the sinuses, a question of diagnosis and when surgery is being considered. Laboratory tests are infrequently necessary and are reserved for patients with suspected allergies, cystic fibrosis, immune deficiencies, mucociliary disorders and similar disease states. Findings on endoscopically guided microswab culture obtained from the middle meatus correlate 80 to 85 percent of the time with results from the more painful antral puncture technique and is performed in patients who fail to respond to the initial antibiotic selection. Surgery is indicated for extranasal spread of infection, evidence of mucocele or pyocele, fungal sinusitis or obstructive nasal polyposis, and is often performed in patients with recurrent or persistent infection not resolved by drug therapy.     

Early Recurrence of First Unprovoked Seizures in Children

Objectives

The risk of early seizure recurrences after first unprovoked seizures in children is largely unknown. We aimed to determine the rate of seizure recurrence within 14 days of first unprovoked seizures in children and identify associated risk factors. Secondarily, we aimed to determine the risk of recurrence at 48 hours and 4 months.

Methods

We conducted a secondary analysis of a multicenter cohort study of children 29 days to 18 years with first unprovoked seizures. Emergency department (ED) clinicians completed standardized histories and physical examinations. The primary outcome, recurrent seizure at 14 days, and the secondary outcomes, recurrence at 48 hours and 4 months, were assessed by telephone follow‐up and medical record review. For each recurrence time point, we excluded those patients for whom no seizure had recurred but chronic antiepileptic drugs had been initiated.

Results

A total of 475 patients were enrolled in the parent study. Of evaluable patients for this secondary analysis, 26 of 392 (6.6%, 95% confidence interval [CI] = 4.4%–9.6%) had recurrences within 48 hours of the incident seizures, 58 of 366 (15.8%, 95% CI = 12.3%–20.0%) had recurrences within 14 days, and 107 of 340 (31.5%, 95% CI = 26.6%–36.7%) had recurrences within 4 months. On logistic regression analysis, age younger than 3 years was independently associated with a higher risk of 14‐day recurrence (adjusted odds ratio [OR] = 2.1, 95% CI = 1.2–3.7; p = 0.01). Having had more than one seizure within the 24 hours prior to ED presentation was independently associated with a higher risk of seizure recurrence at 48 hours (adjusted OR = 4.3, 95% CI = 1.9–9.8; p < 0.001).

Conclusions

Risk of seizure recurrence 14 days after first unprovoked seizures in children is substantial, with younger children at higher risk. Prompt completion of an electroencephalogram and evaluation by a neurologist is appropriate for these children.     

Attacks of pericarditis as a manifestation of familial Mediterranean fever (FMF)

Familial Mediterranean fever (FMF) is characterized by recurrent attacks of febrile serositis. While arthritis, pleuritis and peritonitis are common in FMF, no association of pericarditis with FMF has been described in detail. We retrospectively studied about 4000 FMF patients, using a computer chart review. Pericarditis was diagnosed when patients sustained attacks of pleuritic retrosternal chest pain and had typical findings in the electrocardiogram, echocardiogram or chest radiogram. The incidence and features of pericarditis in FMF were compared to published data. Over a period of 20 years, one or more episodes of pericarditis were recorded in 27 patients, a significantly higher incidence than in the general population (68 vs. 6 per 10(5) per year, p < 0.001). Each patient experienced 1-3 pericarditis attacks, lasting a mean of 4.2 days, accompanied by high temperature and symptoms of FMF attack at another site. The pericarditis attack resolved spontaneously and left no sequelae. FMF patients with pericarditis were comparable to other FMF patients in most demographic and clinical parameters. Pericarditis may be considered another rare manifestation of FMF.      

MR imaging findings in 76 consecutive surgically proven cases of pericardial disease with CT and pathologic correlation

To describe findings of patients with surgically confirmed pericardial disease on state of the art MR sequences. Retrospective review was performed for patients who underwent pericardiectomy and preoperative MR over a 5 year period ending in 2009. Patients' records were reviewed to confirm the diagnosis of chronic recurrent pericarditis, constrictive pericarditis, or pericardial tumor. MR imaging findings of pericardial thickness, IVC diameter, presence or absence of pericardial or pleural effusion, pericardial edema, pericardial enhancement, and septal "bounce" were recorded. Patients with constriction had a larger IVC diameter (3.1 ± 0.4 cm) than patients with recurrent pain and no constriction (2.0 ± 0.4 cm). Mean pericardial thickness for the 16 patients with chronic recurrent pericarditis but no evidence of constriction was 4.8 ± 2.9 mm. Mean pericardial thickness for patients with constriction was 9.2 ± 7.0 cm with calcification, and 4.6 ± 2.1 cm without calcification. 94% of patients with chronic recurrent pericarditis had gadolinium enhancement of the pericardium, while 76% of patients with constriction had pericardial enhancement. Septal "bounce" was present in 19% of chronic recurrent pericarditis cases and 86% of constriction cases. 5 patients had a pericardial neoplasm, 1 of which was not identified preoperatively. State of the art MR techniques can identify significant and distinct findings in patients with chronic recurrent pericarditis, constrictive pericarditis, and pericardial tumors.     

Pericarditis associated with ulcerative colitis and Crohn's disease.

Extracolonic manifestations of inflammatory bowel disease are common and diverse. However, cardiac complications are unusual and we therefore wish to report two cases in which pericarditis occurred. The first was a patient with Crohn's disease of the colon, in whom the pericarditis developed postoperatively. In the second case an acute pericarditis came on simultaneously with the initial presentation of ulcerative colitis.     

Sex difference in risk of recurrent venous thrombosis and the risk profile for a second event

Summary Background: The risk of recurrent venous thrombosis is higher in men than in women, and this is so far unexplained. We set out to determine the influence of age, time between first and second event, type of first event, oral contraception, pregnancy and surgery. Methods: We performed a prospective follow‐up study of 474 patients with a first objective diagnosis of deep vein thrombosis, aged 18–70 years (Leiden Thrombophilia Study cohort). Results: During 3477 person‐years of follow‐up, 90 recurrences occurred. The overall incidence rates of recurrence (IRs) were 40.9 per 1000 person‐years in men and 15.8 per 1000 person‐years in women. Men with an unprovoked first event had the highest risk of recurrence, with almost one‐third experiencing a second unprovoked event within 8 years (IR 41.2 per 1000 person‐years). This risk was three‐fold lower in women [IR 14.2 per 1000 person‐years; hazard ratio 2.8 (95% confidence interval 1.4–5.7)]. Age at diagnosis had little effect on recurrence rate, and nor had time elapsed since the first event. In women, almost half of the recurrences were provoked and were mainly related to oral contraceptive use or pregnancy. Conclusions: The higher recurrence rate in men than in women is not the result of differences in the environmental or transient risk factors that we studied. The risk profile for a second thrombotic event is clearly different from that of a first.     

胃癌根治术后复发区域的研究

目的分析胃癌根治术后复发区域及规律,初步探讨术后放疗范围。方法回顾分析2005年1月—2006年12月胃癌术后复发的220例病人的临床资料,总结复发区域与肿瘤原发部位的关系。结果胃癌原发部位不同,其术后复发区域不完全相同,22例胃底贲门部癌腹膜后淋巴结复发20例,局部复发4例;54例胃体部癌腹膜后淋巴结复发40例,局部复发4例;144例胃窦部癌腹膜后淋巴结复发110例,肝脏转移48例。结论胃癌根治术后放疗靶区应包括腹膜后淋巴结引流区。     

Management of the Child after Enema-Reduced Intussusception: Hospital or Home?

Background

Standard practice has been to admit children for an observation period after enema-reduced intussusception. However, the utility of such routine practice has not been clearly justified.

Study Objectives

The main objective was to determine the rate and timing of recurrent intussusception after successful enema reduction and describe any associated complications.

Methods

The study was a retrospective chart review identifying children with enema-reduced intussusception during a 7-year period from 2002 through 2008. Subjects were children ages 0 to 17 years presenting to the Emergency Department (ED) of a tertiary care, free-standing children’s hospital with confirmed and uncomplicated enema-reduced intussusception.

Results

During the study period there were 98 children with successful enema reduction of intussusception. There were 10 episodes of recurrence in 7 patients, for an overall recurrence rate of 7.1%. Three patients had two recurrences each, and the remainder had single recurrences. Two patients had early recurrences (<48h) at 3 and 5 h, for an early recurrence rate of 2.0%. The late recurrence rate (>48h) was 5.1%. No adverse events were noted in any of the recurrences.

Conclusions

Given the low early recurrence rate for enema-reduced intussusception and the minimal risk of adverse outcomes, ED observation for a 6-h period seems to be a safe alternative to inpatient management. These results support previous work and suggest that these patients can be managed on an outpatient basis.     

Recurrent pericarditis after thoracic surgery

Acute pericarditis is a frequent complication after cardiac and/or thoracic surgery. Recurrent acute pericarditis with multiple episodes is an uncommon phenomenon, however. Patients typically have chest pain and/or pericardial inflammation as shown by electrocardiography and echocardiography. Treatment presents a clinical challenge due to the condition's rarity and lack of multicenter comparative treatment studies. Numerous therapeutic modalities, including nonsteroidal anti-inflammatory agents (NSAIDs), corticosteroids, immunosuppressants, and pericardiectomy, have been used without overwhelming evidence of a standard protocol. We report a case in which 32 episodes of recurrent acute pericarditis occurred, emphasizing the need for multicenter trials comparing therapeutic modalities in the future.