Primary left atrial haemangioendothelioma

We describe the case of a 21-year-old female patient with epithelioid haemangioendothelioma of the roof of the left atrium. The patient underwent radical resection of the tumour with large disease-free margins, which required reconstruction of left and right atrial superior walls. The prognosis is unpredictable, but life expectancies ranging from 1 to 20 years have been described. There is no single treatment that can be prescribed but, because of their biologic behaviour, epithelioid haemangioendotheliomas must be regarded as fully malignant neoplasms and ought to be resected radically to prevent metastatic disease, and to improve life expectancy and quality of life

Surgery of chest wall deformities

OBJECTIVE: To evaluate the medium-term results of 77 surgical corrections in patients with chest wall deformities, 53 (68.8%) with pectus excavatum and 24 with pectus carinatum, operated upon from 1985 to 1994. METHODS: The mean age of the patients was 14.7 years (4-39 years) and 77% were younger than 15 years of age. There were 59 male (76.7%) and 18 female patients. Only four had a family history of the malformation. Seven patients (9.1%) presented with asthma-like symptoms, and 13 (16.9%) referred dyspnea and tiredness for small efforts. The remainder (74.2%) were asymptomatic, but most were psychologically disturbed by the deformity and postural abnormality. Two patients had other skeletal abnormalities. The modified surgical technique used in all cases consisted of subperichondrial resection of the abnormal costal cartilages, transverse and longitudinal osteotomies of the sternum and internal stabilization with a steel rod which was generally removed between 6 and 12 months postoperatively. RESULTS: There was neither early nor late mortality. One patient had a pneumothorax which required chest tube drainage. The mean admission time was 10.5 days (8-14 days). Follow-up was complete, and 90% of the patients had increased effort tolerance. Five of the seven patients (72%) with 'asthmatic' symptoms showed a decrease in the frequency of the crises. Two patients had recurrence of the depression by 3 and 8 months, respectively. The remaining 75 patients (97.3%) were satisfied with the cosmetic result of the surgery. CONCLUSIONS: Surgical treatment of chest wall deformities using this technique leads to good cosmetic, orthopedic and psychological results. We believe that the operations should be performed at any age in patients who have at least a moderate deformity

Surgery for pulmonary aspergiloma: curative?

Introduction.In lhe last few decades, there has been an increase in fungal diseases, especially in those caused by Aspergillus.The aim of this retrospective study was to confirm or ascertain whether surgical intervention to pulmonary aspergiloma can result in a cure or long term palliative treatment with improvement of quality of life. Methods and materiais. From 1989 to 2001, 23 patients with mean age of 44.1 years (18-69 years) were submitted to pulmonary surgery for excision of aspergilloma. Sixteen patients were mate (70%). The most frequent indication for surgery was haemoptysis in 16 patients (70%) followed by abundant sputum in 3 patients (3%). Four patients (17%) were asymptomatic. Old tuberculosis lesions (87%) or pulmonary abscesses (13%) were lhe basic conditions for lhe aspergilloma. Pre-operative evaluation of respiratory function showed a mean vital capacity of 69.8% (61-84% limits) and lhe mean Fevl was 66% (53-82% limits). Results. This group of patients were submitted to 18 lobectomies (82%), 2 bilobectomies (7%), 2 wedge resections and 1 pneumonectomy (4%). There was no operative mortality and lhe morbidity in lhe post-operative period was: persistent air leak in 7 patients (30%), post-operative bleeding in 2 patients (7%) and residual cavities in 2 patients (7%). The mean time of follow-up was 7.2 years (limits 1.5-14 years) and 3 deaths were registered. Two deaths were related to intestinal neoplasia and 1 related to lhe original disease, 5 years after surgery. All surviving patients referred good improvement of symptoms and quality of life. Conclusions. The resection of pulmonary aspergilloma could be performed with a low morbidity and mortality. The patients referred good improvement of symptoms and quality of life after surgery. Consequently, we suggest that surgical therapy is an option for both symptomatic and asymptomatic patient

Mediastinitis after aorto-coronary bypass surgery

OBJECTIVES: To identify risk factors in 60 cases of mediastinitis amongst 2512 patients (2.3%) subjected to isolated coronary bypass surgery from March 1988 through December 1995, treated by a closed irrigation/drainage system. PATIENTS AND METHODS: The mean age of the 60 patients was 56.9 +/- 6.8 years (45-81 years) and 55 (91.6%) were male. Early mediastinal reexploration was performed in all cases immediately after the diagnosis of mediastinitis, with debridement of necrosed tissues, followed by implantation of a closed-circuit irrigation system of the mediastinum constituted by irrigation catheter and drain, closure of the sternum and skin, and specific systemic antibiotic therapy. The mean interval between the original surgery and reexploration was 9.4 days (range 6-14 days). No patient required more extensive procedures, namely omental or muscular flaps. Twenty potential risk factors in patients with mediastinitis, including diabetes mellitus, obesity, coexistence of peripheral vascular disease, decreased LV function, use of inotropes, mediastinal blood drainage and utilization of double IMA, were compared with the group without mediastinitis. RESULTS: Mean cardiopulmonary bypass time was 74.1 +/- 8.1 min, anesthetic time 3.5 +/- 0.8 h and postoperative mechanical ventilation 18 +/- 3 h. A total of 23 patients (38.3%) received one IMA and 35 (58.3%) two IMAs. In the postoperative period, 7 of the 60 patients (11.6%) had required inotropes because of low output. Mediastinal blood loss was 1112cc +/- 452cc and 9 patients (15%) were transfused. Cultures were positive in 40 cases (66.6%) and the most frequent infecting agent was the Staph. epidermidis in 25 cases (62.5%), followed by Candida albicans and Enterobacter and Serratia species (7.5% each); 1 patient (1.7%) died and 9 (15%) had renal failure. The irrigation/drainage was maintained for a mean of 9.1 days (5-83 days). Patients with mediastinitis had a significantly higher prevalence of diabetes (41.6% vs. 18.8%; P < 0.01), obesity (48.3% vs. 15.2%; P < 0.001), peripheral vascular disease (11.6% vs. 4.0%; P < 0.05), but a lower incidence of poor LV function (18.3% vs. 32.7%; P < 0.05). A double IMA was used more frequently in patients who had mediastinitis (58.3% vs. 23.5%; P < 0.001) CONCLUSIONS: Diabetes mellitus, obesity, co-existence of peripheral vascular disease and use of double IMA are risk factors for mediastinitis after coronary artery surgery. The efficacy of the closed method of treatment with a mediastinal irrigation/drainage system was increased with early diagnosis and reintervention

Diaphragmmatic eventration: long-term follow-up and results of open-chest plicature

Objective: Diaphragmmatic eventration is a relatively uncommon entity with a simple surgical correction technique - plication of the diaphragm. This study aims to assess the clinical and ventilatory impact of this technique. Materials: From April 1988 to February 2007, we operated on 20 patients (12 men) with diaphragmmatic eventration using the postero-lateral approach and correction by radial plication. The mean age of the patients studied was 56.3+/-15.6 years (range: 13-74 years). A traumatic cause was identified in 13 patients; one patient had a congenital cause and the remainder were of idiopathic origin. Chronic obstructive pulmonary disease and arterial hypertension were present in one-half of the study group, while diabetes mellitus was present in three patients. Dyspnoea was the most common complaint in 75% of the patients, and thoracic pain was present in 25%. The mean forced expiratory volume in 1s (FEV(1)) and vital capacity (VC) were 66.2+/-15.3% and 70.4+/-16% of the predicted values, respectively. Results: There was no operative mortality. Apart from a patient with moderate/severe pain and another who had pneumonia, there were no other important perioperative complications. Average drainage time was 3.3+/-1.6 days (range: 2-7 days). Hospitalisation time was 6.2+/-1.6 days (5-10 days). Follow-up was complete, for a mean of 59.6+/-55.1 months (4-206 months). There were three late deaths (one sudden, one stroke and one trauma). Eight of the 17 survivors (47%) are asymptomatic. According to the MRC/ATS grading system, the dyspnoea score was 2.06+/-0.97 preoperatively and 1.06+/-1.14 postoperatively (p=0.007). At follow-up, the FEV(1) was 76.1+/-20.1% and the VC was 78.4+/-17.3% (p>0.1). Two patients had chronic pain. Conclusion: Plication of the diaphragm is a safe and efficient procedure. Most patients experienced significant clinical improvement with enhancement of the FEV(1) and VC. Chronic surgical pain still remains a potential problem with the classical approach

Lung metastases from colorectal cancer: surgical resection and prognostic factors

OBJECTIVE: To analyse our experience with excision of lung metastases from colorectal carcinoma (CRC), and to evaluate clinically relevant prognostic factors, identifying the cluster of patients who would benefit from this procedure. METHODS: Sixty-one patients, 42 men (69%), with primary CRC who underwent 94 curative resections of pulmonary metastases were retrospectively reviewed. Age was 30-80 years (mean 61.2+/-15). Population was analysed for age, sex, disease-free interval (DFI), prethoracotomy carcinoembryonic antigen (CEA) level, location and histology of primary tumour, number of lung lesions (and size of largest resected metastasis), type of lung resection, nodal involvement (hilar/mediastinal), use of adjuvant treatment, morbid-mortality and immediate and follow-up survival. RESULTS: Mean DFI was 29+/-22 months (range 5-132 months). There was no hospital mortality and significant morbidity occurred in five patients (8.2%). Mean follow-up was 39+/-4 months (range 4-173 months). Mean overall survival and disease-free survival were 67+/-16 months and 52+/-6 months, respectively. Three-, 5- and 10-year survival rates from date of primary colorectal resection were 83%, 71% and 43%, respectively. Three-, 5- and 10-year survival rates from date of lung resection were 61%, 48% and 11%, respectively. Five-year survival was 57% in patients with normal prethoracotomy CEA levels and 18% for those with high levels (>5 ng/ml) (p=0.039). CONCLUSIONS: Pulmonary metastasectomy has potential survival benefit for patients with metastatic colorectal carcinoma. Low morbidity and mortality rates, contrasting with lack of any other effective therapy, justify aggressive surgical management. Single deposits, DFI >36 months and normal prethoracotomy serum CEA were significant independent prognostic factors

Diagnostic value of surgical lung biopsy: comparison with clinical and radiological diagnosis

OBJECTIVE: To determine overall and disease-related accuracy of the clinical/imagiological evaluation for pulmonary infiltrates of unknown aetiology, compared with the pathological result of the surgical lung biopsy (SLB) and to evaluate the need for the latter in this setting. METHODS: We conducted a retrospective review of the experiences of SLB in 366 consecutive patients during the past 5 years. The presumptive diagnosis was based on clinical, imagiological and non-invasive or minimally invasive diagnostic procedures and compared with the gold standard of histological diagnosis by SLB. We considered five major pathological groups: diffuse parenchymal lung disease (DPLD), primitive neoplasms, metastases, infectious disease and other lesions. Patients with previous histological diagnosis were excluded. RESULTS: In 56.0% of patients (n=205) clinical evaluation reached a correct diagnosis, in 42.6% a new diagnosis was established (n=156) by the SLB, which was inconclusive in 1.4% (n=5). The pre-test probability for each disease was 85% for DPLD, 75% for infectious disease, 64% for primitive neoplasms and 60% for metastases. Overall sensitivity, specificity, positive and negative predictive values for the clinical/radiological diagnosis were 70%, 90%, 62% and 92%, respectively. For DPLD: 67%, 90%, 76% and 85%; primitive neoplasms: 47%, 90%, 46% and 90%; metastases: 99%, 79%, 60% and 99%; infectious disease 38%, 98%, 53% and 96%. CONCLUSIONS: Despite a high sensitivity and specificity of the clinical and imagiological diagnosis, the positive predictive value was low, particularly in the malignancy group. SLB should be performed in pulmonary infiltrates of unknown aetiology because the clinical/imagiological assessment missed and/or misdiagnosed an important number of patients

Eight common genetic variants associated with serum dheas levels suggest a key role in ageing mechanisms

Dehydroepiandrosterone sulphate (DHEAS) is the most abundant circulating steroid secreted by adrenal glands-yet its function is unknown. Its serum concentration declines significantly with increasing age, which has led to speculation that a relative DHEAS deficiency may contribute to the development of common age-related diseases or diminished longevity. We conducted a meta-analysis of genome-wide association data with 14,846 individuals and identified eight independent common SNPs associated with serum DHEAS concentrations. Genes at or near the identified loci include ZKSCAN5 (rs11761528; p = 3.15×10-36), SULT2A1 (rs2637125; p = 2.61×10-19), ARPC1A (rs740160; p = 1.56×10-16), TRIM4 (rs17277546; p = 4.50×10-11), BMF (rs7181230; p = 5.44×10-11), HHEX (rs2497306; p = 4.64×10-9), BCL2L11 (rs6738028; p = 1.72×10-8), and CYP2C9 (rs2185570; p = 2.29×10-8). These genes are associated with type 2 diabetes, lymphoma, actin filament assembly, drug and xenobiotic metabolism, and zinc finger proteins. Several SNPs were associated with changes in gene expression levels, and the related genes are connected to biological pathways linking DHEAS with ageing. This study provides much needed insight into the function of DHEAS

Signaling Cascades Modulate the Speed of Signal Propagation through Space

Cells are not mixed bags of signaling molecules. As a consequence, signals must travel from their origin to distal locations. Much is understood about the purely diffusive propagation of signals through space. Many signals, however, propagate via signaling cascades. Here, we show that, depending on their kinetics, cascades speed up or slow down the propagation of signals through space, relative to pure diffusion.We modeled simple cascades operating under different limits of Michaelis-Menten kinetics using deterministic reaction-diffusion equations. Cascades operating far from enzyme saturation speed up signal propagation; the second mobile species moves more quickly than the first through space, on average. The enhanced speed is due to more efficient serial activation of a downstream signaling module (by the signaling molecule immediately upstream in the cascade) at points distal from the signaling origin, compared to locations closer to the source. Conversely, cascades operating under saturated kinetics, which exhibit zero-order ultrasensitivity, can slow down signals, ultimately localizing them to regions around the origin.Signal speed modulation may be a fundamental function of cascades, affecting the ability of signals to penetrate within a cell, to cross-react with other signals, and to activate distant targets. In particular, enhanced speeds provide a way to increase signal penetration into a cell without needing to flood the cell with large numbers of active signaling molecules; conversely, diminished speeds in zero-order ultrasensitive cascades facilitate strong, but localized, signaling

Portuguese recommendations for the use of biological therapies in patients with psoriatic arthritis - 2015 update

OBJECTIVE: To update recommendationsforthe treatment of psoriatic arthritis with biological therapies, endorsed by the Portuguese Society of Rheumatology (SPR). METHODS: These treatment recommendations were formulated by Portuguese rheumatologists based on literature evidence and consensus opinion. At a national meeting the 16 recommendations included in this document were discussed and updated. The level of agreement among Portuguese Rheumatologists was assessed using an online survey. A draft of the full text of the recommendations was then circulated and suggestions were incorporated. A final version was again circulated before publication. RESULTS: A consensus was achieved regarding the initiation, assessment of response and switching biological therapies in patients with psoriatic arthritis (PsA). Specific recommendations were developed for several disease domains: peripheral arthritis, axial disease, enthesitis and dactylitis. CONCLUSION: These recommendations may be used for guidance in deciding which patients with PsA should be treated with biological therapies. They cover a rapidly evolving area oftherapeutic intervention.Asmore evidence becomes available and more biological therapies are licensed, these recommendations will have to be updated