Immune reconstitution inflammatory syndrome in HIV and sporotrichosis coinfection: report of two cases and review of the literature

We report 2 cases of patients with immune reconstitution inflammatory syndrome (IRIS) associated with cutaneous disseminated sporotrichosis and human immunodeficiency virus (HIV) coinfection. The patients received specific treatment for sporotrichosis. However, after 4 and 5 weeks from the beginning of antiretroviral therapy, both patients experienced clinical exacerbation of skin lesions despite increased T CD4+ cells (T cells cluster of differentiation 4 positive) count and decreased viral load. Despite this exacerbation, subsequent mycological examination after systemic corticosteroid administration did not reveal fungal growth. Accordingly, they were diagnosed with IRIS. However, the sudden withdrawal of the corticosteroids resulted in the recurrence of IRIS symptoms. No serious adverse effects could be attributed to prednisone. We recommend corticosteroid treatment for mild-to-moderate cases of IRIS in sporotrichosis and HIV coinfection with close follow-up.

Surgical treatment of type I neuritis in a teenage boy with borderline tuberculoid leprosy

Exacerbation of the immune response against Mycobacterium leprae can lead to neuritis, which is commonly treated via immunosuppression with corticosteroids. Early neurolysis may be performed concurrently, especially in young patients with a risk of functional sequelae. We report the case of a young patient experienced intense pain in the left elbow one year after the treatment of tuberculoid-tuberculoid leprosy. The pain was associated with paresthesias in the ulnar edge and left ulnar claw. After evaluation, the diagnosis was changed to borderline tuberculoid leprosy accompanied with neuritis of the left ulnar nerve. Early neurolysis resulted in rapid reduction of the pain and recovery of motor function.

Cystic fibrosis with normal sweat chloride concentration: case report

Cystic fibrosis is a genetic disease usually diagnosed by abnormal sweat testing. We report a case of an 18-year-old female with bronchiectasis, chronic P. aeruginosa infection, and normal sweat chloride concentrations who experienced rapid decrease of lung function and clinical deterioration despite treatment. Given the high suspicion ofcystic fibrosis, broad genotyping testing was performed, showing a compound heterozygous with deltaF508 and 3849+10kb C->T mutations, therefore confirming cystic fibrosis diagnosis. Although the sweat chloride test remains the gold standard for the diagnosis of cystic fibrosis, alternative diagnostic tests such as genotyping and electrophysiologic measurements must be performed if there is suspicion of cystic fibrosis, despite normal or borderline sweat chloride levels.

Genetic diversity in rosewood saplings (Aniba rosaeodora ducke, Lauraceae): an ecological approach

This article takes an ecological approach to the genetic diversity of Rosewood (Aniba rosaeodora Ducke) in a central Amazonian terra firme forest north of Manaus. Planted Rosewood setting, under partial shaded canopy, were assessed in terms of fruiting production, frugivory, and seed dispersal. Using RAPD molecular analysis procedures, the influence of the spatial distribution of adult trees on the genetic diversity (polymorphism) of saplings was assessed with genetic samples from 34 reproductive trees and 60 saplings. The density and distribution patterns the reproductive trees did not modify the sapling"s diversity (1.86%, AMOVA). Two types of adult tree dispersion were identified; i) clumped and ii) more widely dispersed. Polymorphism (77.5%) and gene flow were high between these. Although more sapling genetic variability in areas with a higher density of mature plants was not as high as expected, density did not affect the genetic diversity of samplings, indicating a high incidence of gene flow amongst trees. In planted Rosewood population (surrounded by low disturbed forest), fruiting trees experienced a high level of removal of seeds by toucans (Rhamphastidae), about of 50%. The high gene flow found among native trees suggested that toucans, promoting seed rain at short and long distances from maternal trees, actively contribute to the maintenance of genetic diversity within wild rosewood populations.

Laser assisted extraction of pacemaker and implantable defibrillator leads

OBJECTIVE: To analyze the results of laser-assisted extraction of permanent pacemaker and defibrillator leads. METHODS: We operated upon 36 patients, whose mean age was 54.2 years, and extracted 56 leads. The reasons for extracting the leads were as follows: infection in 19 patients, elective replacement in 13, and other causes in 4 patients. The mean time of catheter placement was 7.5±5.5 years. Forty-seven leads were from pacemakers, and 9 were from defibrillators. Thirty-eight leads were in use, 14 had been abandoned in the pacemaker pocket, and 4 had been abandoned inside the venous system. RESULTS: We successfully extracted 54 catheters, obtaining a 96.4% rate of success and an 82.1% rate for complete extraction. The 2 unsuccessful cases were due to the presence of calcium in the trajectory of the lead. The mean duration of laser light application was 123.0±104.5 s, using 5,215.2±4,924.0 pulses, in a total of 24.4±24.2 cycles of application. Thirty-four leads were extracted from the myocardium with countertraction after complete progression of the laser sheath, 12 leads came loose during the progression of the laser sheath, and the remaining 10 were extracted with other maneuvers. One patient experienced cardiac tamponade after extraction of the defibrillator lead, requiring open emergency surgery. CONCLUSION: The use of the excimer laser allowed extraction of the leads with a 96% rate of success; it was not effective in 2 patients who had calcification on the lead. One patient (2.8%) had a complication that required cardiac surgery on an emergency basis.

Diagnostic value of the tilt-table test for the assessment of syncope in children and adolescents

OBJECTIVE - To assess the diagnostic value, the characteristics, and feasibility of tilt-table testing in children and adolescents. METHODS - From August 1991 to June 1997, we retrospectively assessed 94 patients under the age of 18 years who had a history of recurring syncope and presyncope of unknown origin and who were referred for tilt-table testing. These patients were divided into 2 groups: group I (children) - 36 patients with ages ranging from 3 to 12 (mean of 9.19±2.31) years; group II (adolescents) - 58 patients with ages ranging from 13 to 18 (mean of 16.05±1.40) years. We compared the positivity rate, the type of hemodynamic response, and the time period required for the test to become positive in the 2 groups. RESULTS - The positivity rates were 41.6 % and 50% for groups I and II, respectively. The pattern of positive hemodynamic response that predominated in both groups was the mixed response. The mean time period required for the test to become positive was shorter in group I (11.0±7.23 min) than in group II (18.44±7.83 min). No patient experienced technical difficulty or complications. CONCLUSION - No difference was observed in regard to feasibility, positivity rate, and pattern of positive response for the tilt-table test in children and adolescents. Pediatric patients had earlier positive responses.

Useful clinical features for the selection of ideal patients with strial fibrillation for mapping and catheter ablation

OBJECTIVE: To identify useful clinical characteristics for selecting patients eligible for mapping and ablation of atrial fibrillation. METHODS: We studied 9 patients with atrial fibrillation, without structural heart disease, associated with: 1) antiarrhythmic drugs, 2) symptoms of low cardiac output, and 3) intention to treat. Seven patients had paroxysmal atrial fibrillation and 2 had recurrent atrial fibrillation. RESULTS: In the 6 patients who underwent mapping (all had paroxysmal atrial fibrillation), catheter ablation was successfully carried out in superior pulmonary veins in 5 patients (the first 3 in the left superior pulmonary vein and the last 2 in the right superior pulmonary vein). One patient experienced a recurrence of atrial fibrillation after 10 days. We observed that patients who had short episodes of atrial fibrillation on 24-hour Holter monitoring before the procedure were those in whom mapping the focus of tachycardia was possible. Tachycardia was successfully suppressed in 4 of 6 patients. The cause of failure was due to the impossibility of maintaining sinus rhythm long enough for efficient mapping. CONCLUSION: Patients experiencing short episodes of atrial fibrillation during 24-hour Holter monitoring were the most eligible for mapping and ablation, with a final success rate of 66%, versus the global success rate of 44%. Patients with persistent atrial fibrillation were not good candidates for focal ablation.

Biventricular pacing improves clinical behavior and reduces prevalence of ventricular srrhythmia in patients with heart failure

PURPOSE: To analyze the influence of biventricular pacing (BP) on clinical behavior, ventricular arrhythmia (VA) prevalence, and left ventricular ejection fraction (LV EF) by gated ventriculography. METHODS: Twenty-four patients with left bundle branch block (LBBB) and NYHA class III and IV underwent pacemaker implantation and were randomized either to the conventional or BP group, all receiving BP after 6 months. RESULTS: Sixteen patients were in NYHA class IV (66.6%) and 8 were in class III (33.4%). After 1-year follow-up, 14 patients were in class II (70%) and 5 were in class III (25%). Two sudden cardiac deaths occurred. A significant reduction in QRS length was found with BP (p=0.006). A significant statistical increase, from a mean of 19.13 ± 5.19% (at baseline) to 25.33 ± 5.90% (with BP) was observed in LVEF Premature ventricular contraction prevalence decreased from a mean of 10,670.00 ± 12,595.39 SD or to a mean of 3,007.00 ± 3,216.63 SD PVC/24 h with BP (p<0.05). Regarding the hospital admission rate over 1 year, we observed a significant reduction from 60. To 16 admissions with BP (p<0.05). CONCLUSION: Patients with LBBB and severe heart failure experienced, with BP, a significant NYHA class and LVEF improvement. A reduction in the hospital admission rate and VA prevalence also occurred.

Consequences of the Prolonged Waiting Time for Patients Candidates for Heart Surgery

OBJECTIVE - To assess mortality and the psychological repercussions of the prolonged waiting time for candidates for heart surgery. METHODS - From July 1999 to May 2000, using a standardized questionnaire, we carried out standardized interviews and semi-structured psychological interviews with 484 patients with coronary heart disease, 121 patients with valvular heart diseases, and 100 patients with congenital heart diseases. RESULTS - The coefficients of mortality (deaths per 100 patients/year) were as follows: patients with coronary heart disease, 5.6; patients with valvular heart diseases, 12.8; and patients with congenital heart diseases, 3.1 (p<0.0001). The survival curve was lower in patients with valvular heart diseases than in patients with coronary heart disease and congenital heart diseases (p<0.001). The accumulated probability of not undergoing surgery was higher in patients with valvular heart diseases than in the other patients (p<0.001), and, among the patients with valvular heart diseases, this probability was higher in females than in males (p<0.01). Several patients experienced intense anxiety and attributed their adaptive problems in the scope of love, professional, and social lives, to not undergoing surgery. CONCLUSION - Mortality was high, and even higher among the patients with valvular heart diseases, with negative psychological and social repercussions.

Late Clinical Outcomes of the Fontan Operation in Patients with Tricuspid Atresia

OBJECTIVE: Evaluation of the long-term clinical results of the Fontan operation in patients with tricuspid atresia. METHODS: A retrospective analysis was made at the Instituto de Cardiologia do Rio Grande do Sul (Institute of Cardiology of Rio Grande do Sul), from August 1980 through January 2000, of 25 patients with a long-term follow-up, out of a series of 36 patients who underwent the Fontan operation or one of its variants due to tricuspid atresia. Their mean age at surgery was 5.4±3.1 years, and their mean weight was 15.8±6.1 kg, the majority of them (63.9%) being males. Four patients underwent the classical Fontan operation, 12 the Kreutzer variant, 6 the Björk variant, 9 total cavopulmonary shunt with a fenestrated tube, and 5 total cavopulmonary shunt with a nonfenestrated tube. RESULTS: The patients were followed-up on an outpatient basis, with a mean long-term survival time of 5.5±4.2 years (50 days to 17.8 years) and a late mortality rate of 8%. Arterial saturation increased from 77.2±18.8% in the preoperative period to 91±6.7% upon the last outpatient visit (p>0.05). At the final check, most (67%) patients were asymptomatic and 87% could tolerate exercise. Ten (40%) patients experienced some kind of complication during the long-term follow-up, such as cardiac arrhythmia, cyanosis, protein-losing enteropathy, neurological events, right heart failure, intolerance to exercise and reoperation. CONCLUSION: The results indicate that, once the immediate postoperative period is over, during which the adaptations to the new circulatory physiology occur, the evolution of patients with tricuspid atresia who underwent the Fontan operation is satisfactory, in spite of a low, yet significant, morbidity.

Can patients with left main coronary artery disease wait for myocardial revascularization surgery?

OBJECTIVE: To assess the occurrence of cardiac events in patients diagnosed with left main coronary artery disease on diagnostic cardiac catheterization and waiting for myocardial revascularization surgery. METHODS: All patients diagnosed with left main coronary artery disease (stenosis > or = 50%) consecutively identified on diagnostic cardiac catheterization during an 8-month period were selected for the study. The group comprised 56 patients (40 males and 16 females) with a mean age of 61±10 years. The cardiac events included death, nonfatal acute myocardial infarction, acute left ventricular failure, unstable angina, and emergency surgery. RESULTS: While waiting for surgery, patients experienced the following cardiac events: 7 acute myocardial infarctions and 1 death. All events occurred within the first 60 days after the diagnostic cardiac catheterization. More patients, whose indication for diagnostic cardiac catheterization was unstable angina, experienced events as compared with those with other indications [p=0.03, relative risk (RR) = 5.25, 95% confidence interval = 1.47 - 18.7]. In the multivariate analysis of logistic regression, unstable angina was also the only factor that independently contributed to a greater number of events (p = 0.02, OR = 8.43, 95% CI =1.37 - 51.7). CONCLUSION: Unstable angina in patients with left main coronary artery disease acts as a high risk factor for cardiac events, emergency surgery being recommended in these cases.

Hemarthrosis of the knees following streptokinase therapy for acute myocardial infarction

A sixty-four-year-old male patient was studied who had acute coronary syndrome with ST segment elevation experienced bilateral hemarthrosis of the knees after administration of streptokinase and acetylsalicylic acid.

Initial experience in Brazil with the Helex septal occluder for percutaneous occlusion of atrial septal defects

OBJECTIVE: To evaluate the initial clinical experience with the Helex septal occluder for percutaneous closure of atrial septal defects. METHODS: Ten patients underwent the procedure, 7 patients with ostium secundum atrial septal defects (ASD) with hemodynamic repercussions and 3 patients with pervious foramen ovale (PFO) and a history of stroke. Mean age was 33.8 years and mean weight was 55.4 kg. Mean diameter by transesophageal echocardiography and mean stretched ASD diameter were 11.33 ± 3.3mm, and 15.2 ± 3.8mm, respectively. The Qp/Qs ratio was 1.9 ± 0.3 in patients with ASD. RESULTS: Eleven occluders were placed because a patient with 2 holes needed 2 devices. It was necessary to retrieve and replace 4 devices in 3 patients. We observed immediate residual shunt (< 2mm) in 4 patients with ASD, and in those with patent foramen ovale total occlusion of the defect occurred. No complications were noted, and all patients were discharged on the following day. After 1 month, 2 patients with ASD experienced trivial residual shunts (1mm). In 1 patient, we observed mild prolapse in the proximal disk in the right atrium, without consequences. CONCLUSION: The Helex septal occluder was safe and effective for occluding small to moderate atrial septal defects. Because the implantation technique is demanding, it requires specific training of the operator. Even so, small technical failures may occur in the beginning of the learning curve, but they do not involve patient safety.

Debranching Solutions in Endografting for Complex Thoracic Aortic Dissections

Background: Conventional surgical repair of thoracic aortic dissections is a challenge due to mortality and morbidity risks. Objectives: We analyzed our experience in hybrid aortic arch repair for complex dissections of the aortic arch. Methods: Between 2009 and 2013, 18 patients (the mean age of 67 ± 8 years-old) underwent hybrid aortic arch repair. The procedural strategy was determined on the individual patient. Results: Thirteen patients had type I repair using trifurcation and another patient with bifurcation graft. Two patients had type II repair with replacement of the ascending aorta. Two patients received extra-anatomic bypass grafting to left carotid artery allowing covering of zone 1. Stent graft deployment rate was 100%. No patients experienced stroke. One patient with total debranching of the aortic arch following an acute dissection of the proximal arch expired 3 months after TEVAR due to heart failure. There were no early to midterm endoleaks. The median follow-up was 20 ± 8 months with patency rate of 100%. Conclusion: Various debranching solutions for different complex scenarios of the aortic arch serve as less invasive procedures than conventional open surgery enabling safe and effective treatment of this highly selected subgroup of patients with complex aortic pathologies.

Alternatives to Aerobic Exercise Prescription in Patients with Chronic Heart Failure

Background: Exercise is essential for patients with heart failure as it leads to a reduction in morbidity and mortality as well as improved functional capacity and oxygen uptake (v̇O2). However, the need for an experienced physiologist and the cost of the exam may render the cardiopulmonary exercise test (CPET) unfeasible. Thus, the six-minute walk test (6MWT) and step test (ST) may be alternatives for exercise prescription. Objective: The aim was to correlate heart rate (HR) during the 6MWT and ST with HR at the anaerobic threshold (HRAT) and peak HR (HRP) obtained on the CPET. Methods: Eighty-three patients (58 ± 11 years) with heart failure (NYHA class II) were included and all subjects had optimized medication for at least 3 months. Evaluations involved CPET (v̇O2, HRAT, HRP), 6MWT (HR6MWT) and ST (HRST). Results: The participants exhibited severe ventricular dysfunction (ejection fraction: 31 ± 7%) and low peak v̇O2 (15.2 ± 3.1 mL.kg-1.min-1). HRP (113 ± 19 bpm) was higher than HRAT (92 ± 14 bpm; p < 0.05) and HR6MWT (94 ± 13 bpm; p < 0.05). No significant difference was found between HRP and HRST. Moreover, a strong correlation was found between HRAT and HR6MWT (r = 0.81; p < 0.0001), and between HRP and HRST (r = 0.89; p < 0.0001). Conclusion: These findings suggest that, in the absence of CPET, exercise prescription can be performed by use of 6MWT and ST, based on HR6MWT and HRST