Coarctação Isolada da Aorta: Experiência em 100 Doentes Consecutivos

INTRODUCTION: Coarctation of the aorta (CoA) is a stenosis usually located in the descending aorta. Treatment consists of surgical or percutaneous removal of the obstruction and presents excellent immediate results but significant residual problems often persist. OBJECTIVES: To describe the presentation, treatment and long-term evolution of a population of 100 unselected consecutive patients with isolated CoA in a single pediatric cardiology center. METHODS: This was a retrospective study of all patients with isolated CoA treated during4 the last 21 years (1987-2008). RESULTS: The patients (n=100, 68.3% male) were diagnosed at a median age of 94 days (1 day to 16 years). The clinical presentation differed between patients aged less or more than one year, the former presenting with heart failure and the latter being asymptomatic with evidence of hypertension (88 and 63%, respectively; p < 0.01). Treatment, a median of 8 days after diagnosis, was surgical in 79 cases (20 end-to-end anastomosis, 31 subclavian flap, 28 patch) and percutaneous in the remaining 21 (15 balloon angioplasty, 6 with stenting). The mean age of surgical patients was younger than in those treated percutaneously (3.4 vs. 7.5 years; p < 0.01). Immediate mortality was 2% and occurred in the surgical group. There was no late mortality, in a mean follow-up of 7.2 +/- 5.4 years. Recoarctation occurred in 8 patients (6 surgical, 2 percutaneous). There are 46 patients who currently have hypertension (19 at rest, 27 with effort), their median age at diagnosis being older than the others (23 vs. 995 days; p < 0.01). CONCLUSIONS: Isolated CoA has an excellent short-term prognosis but a significant incidence of long-term complications, and should thus no longer be seen as a simple obstruction in the descending aorta, but rather as a complex pathology that requires careful follow-up after treatment. Its potentially insidious presentation requires a high level of clinical suspicion, femoral pulse palpation during physical examination of newborns and older children being particularly important. Delay in treatment has an impact on late morbidity and mortality. Taking into account the data currently available on late and immediate results, the final choice of therapeutic technique depends on the patient's age, associated lesions and the experience of the medical-surgical team. Hypertension should be closely monitored in the follow-up of these patients, as well as its risk factors and complications.

Será que a Definição de Síndroma Metabólica pela International Diabetes Federation Melhora a Capacidade Preditora para Doença Coronária e do Espessamento da Íntima-Média Carotídea?

BACKGROUND: Metabolic syndrome (MS) is associated with increased incidence of diabetes and atherosclerotic complications. The new definition of the International Diabetes Federation (IDF) increases the population with this entity, compared to the NCEP ATP III definition. OBJECTIVES: To study the prevalence of coronary artery disease (CAD) and carotid intima-media thickness (IMT) in patients with and without MS, according to the NCEP ATP III and IDF definitions, and the predictive ability of carotid IMT for CAD. METHODS: We studied 270 consecutive patients admitted for elective coronary angiography due to suspicion of CAD. All patients underwent ultrasound study of the carotid arteries to measure IMT (the highest value between the right and left common carotid arteries was used in the analysis). Coronary stenosis of > or =70% (or 50% for the left main coronary artery) was considered significant. RESULTS: By the ATP III definition, 14% of the patients had MS, and these patients had a higher prevalence of CAD (87% vs. 63%, p = 0.004), but no significant difference was found for carotid IMT (1.03 +/- 0.36 mm vs. 0.95 +/- 0.35 mm, p=NS). With the IDF definition, 61% of the patients had MS; this group was slightly older and included more women. There were no differences in terms of CAD (68% vs. 63%) or carotid IMT (0.97 +/- 0.34 vs. 0.96 +/- 0.39 mm). On multivariate analysis, the ATP III definition of MS predicts CAD (OR 4.76, 95% CI 1.71-13.25, p = 0.003), but the IDF definition does not (OR 1.29, 95% CI 0.74-2.27, p = 0.37). On ROC curve analysis, an IMT of > or = 0.95 mm predicts CAD (AUC 0.66, p < 0.001), with a sensitivity of 52% and specificity of 75%. CONCLUSIONS: The new IDF definition increases the population with MS, decreasing the capacity to predict the presence of CAD. In our population, neither the ATP III nor the IDF definition showed differences in terms of carotid IMT. Carotid IMT can predict CAD, but with only modest sensitivity.

Cutaneous anthrax in Lima, Peru: retrospective analysis of 71 cases, including four with a meningoencephalic complication

Anthrax is a zoonosis produced by Bacillus anthracis, and as an human infection is endemic in several areas in the world, including Peru. More than 95% of the reported naturally acquired infections are cutaneous, and approximately 5% of them can progress to meningoencephalitis. In this study we review the clinical and epidemiological characteristics of the patients with diagnosis of cutaneous anthrax evaluated between 1969 and 2002 at the Hospital Nacional Cayetano Heredia (HNCH) and the Instituto de Medicina Tropical Alexander von Humboldt in Lima, Peru. Seventy one patients were included [49/71 (69%) of them men], with a mean age of 37 years. The diagnoses were classified as definitive (44%) or probable (56%). The most common occupation of the patients was agriculture (39%). The source of infection was found in 63 (88.7%) patients. All the patients had ulcerative lesions, with a central necrosis. Most of the patients (65%) had several lesions, mainly located in the upper limbs (80%). Four patients (5.6%) developed meningoencephalitis, and three of them eventually died. In conclusion, considering its clinical and epidemiological characteristics, cutaneous anthrax must be included in the differential diagnosis of skin ulcers. A patient with clinical suspicion of the disease should receive effective treatment soon, in order to avoid neurological complications which carry a high fatality rate.

Síndrome de Churg-Strauss, Aneurismas Femorais Venosos e Tromboembolismo Pulmonar

OBJECTIVE: Venous aneurysms of the lower limbs are rare. When they involve the deep venous system, they can be associated with deep venous thrombosis and pulmonary embolism. The authors report the case of a 63-year-old women evaluated by duplex imaging and venography, in the context of a pulmonary embolism, and detected bilateral large aneurysms of the femoral veins (5 cm in the right femoral vein and 4 cm in the left femoral vein). The aneurysms were located at the bifurcation of the common femoral vein with the superficial and profound veins. METHOD: The right aneurysm was partially thrombosed. The patient was asymptomatic in the lower-limb extremities. After a period of anticoagulation, the authors proceeded to surgically excise the right aneurysm and replaced the venous system using a polytetrafluoroethylene (PTFE) graft with the construction of an a-v fistula. RESULT: The patient had no complications in the postoperative period and her 8-month follow-up examination revealed no symptoms, not even leg edema. The duplex scan showed patency of the graft. She continued with oral anticoagulation. The histologic examination revealed an eosinophilic inflammatory reaction of the vein wall, compatible with the diagnosis of Churg-Strauss syndrome. CONCLUSION: The authors conclude that they have probably described the first case in the international literature of a venous aneurysm with a Churg-Strauss syndrome and pulmonary embolism.

Lesões Traumáticas Iatrogénicas das Veias Femorais no Decurso da Cirurgia de Varizes

Traumatic lesions of the femoral veins during varicose veins surgery are very uncommon and they raise several therapeutic difficulties. They occur in 1/1000 cases of varicose veins operated. The authors describe four cases of lesions of this type that were seen and treated for the last six years, probably because there was an increase in the number of varicose veins operated on in the Lisbon area. The authors discuss the surgical options and they concluded that these complications can be minimized with good anatomical and surgical skills, specially of the saphenous-femoral and saphenous-popliteal junctions.

Co-infection between influenza virus and flagellated bacteria

Trypsin is required in the hemagglutinin (HA) cleavage to in vitro influenza viruses activation. This HA cleavage is necessary for virus cell entry by receptor-mediated endocytosis. Bacteria in the respiratory tract are potential sources of proteases that could contribute to the cleavage of influenza virus in vivo. From 47 samples collected from horses, pigs, and from humans, influenza presence was confirmed in 13 and these samples demonstrated co-infection of influenza with flagellated bacteria, Stenotrophomonas maltophilia from the beginning of the experiments. Despite treatment with antibiotics, the bacteria remained resistant in several of the co-infected samples (48.39%). These bacteria, considered opportunistic invaders from environmental sources, are associated with viral infections in upper respiratory tract of hosts. The protease (elastase), secreted by Stenotrophomonas maltophilia plays a role in the potentiation of influenza virus infection. Proteolytic activity was detected by casein agar test. Positive samples from animals and humans had either a potentiated influenza infectivity or cytopathic effect (CPE) in MDCK and NCI H292 cells, Stenotrophomonas maltophilia were always present. Virus and bacteria were observed ultrastructurally. These in vitro findings show that microbial proteases could contribute to respiratory complications by host protease activity increasing inflammation or destroying endogenous cell protease inhibitors.

Tumores do Corpo Carotídeo. Experiência de um Serviço no Diagnóstico e Tratamento nos Últimos 10 Anos

INTRODUCTION: Carotid body tumours (CBT) are neoplasms that develop from paragangionic cells of this structure. They are rare, with an estimated incidence of 1:30000 and can be associated with other neuro-endocrine neoplasia. The authors report their experience in the management of the disease, in the last 10 years. MATERIAL AND METHODS: Eight patients (with eight tumours) were treated, all submitted to tumour resection. 75% were female and the mean age was 56 years. We report a 12,5% incidence of neurological sequelae from surgery, and no mortality. In the follow-up (which varied between 1 and 10 years), no local or contralateral recurrence or metastasis were registered. Also, we did not found family cases of this disease. CONCLUSIONS: The authors noticed an unusually high proportion of female patients. The tumour resection was curative in all patients, with a rate of neurological complications inferior to that reported in other published series. These neurological sequelae were reported in patients with large tumours, thus reinforcing the outmost importance of an early diagnosis. Pre-operative selective embolization of these tumours can be helpful in the resection of large tumours, allowing a potential reduction in neurological complications.

Abdominal angiostrongyliasis: a case with severe evolution

A case of acute abdomen disease caused by abdominal angiostrongyliasis is reported. A 42-year-old otherwise healthy patient presented with a complaint of nine days of abdominal pain, constipation, disury, fever and right iliac fossa palpable mass. Exploratory laparotomy was performed. After surgical treatment the patient presented serious complications.

Cystoscopy in the diagnosis and follow-up of urinary schistosomiasis in Brazilian soldiers returning from Mozambique, Africa

The assessment of urinary schistosomiasis in individuals coming from endemic areas often requires diagnostic resources not used in areas of exposure in order to determine complications or to establish more precise criteria of cure. Cystoscopy and 24-hour urine examination were performed, after treatments with praziquantel 40 mg/kg body weight, single dose, on 25 Brazilian military men who were part of a United Nations peace mission to Mozambique in 1994. The median age of the individuals was 29 years and all presented a positive urine parasitological exam. The alterations detected by cystoscopy were hyperemia and granulomas in the vesical submucosa in 59.1% of the individuals and only granulomas in 40.9%. A vesical biopsy revealed granulomas in all patients and viable eggs in 77.3% even after a period during which the patients no longer excreted eggs in urine. Cystoscopy after treatment, followed by biopsy and histopathological evaluation, performed in areas where the evolution of the disease can be better monitored, was found to be a safe criterion of parasitological cure.

Pulmonary thromboembolism in AIDS patient with chronic venous insufficiency, pulmonary tuberculosis and breast cancer: a case report and pathophysiology review

Recent literature reports thrombotic episodes occurring in patients with HIV infection associated with other abnormalities including neoplasms and infections predisposing to a hypercoagulable state. We report a 47-year-old woman who developed pulmonary thromboembolism in association with HIV infection, pulmonary tuberculosis and breast cancer. She was treated with rifampin, isoniazid, pyrazinamide; heparin, phenprocoumon, zidovudine, lamivudine and efavirenz. Acid fast bacilli were visualized in a sputum smear and three months after, Mycobacterium tuberculosis was isolated from lymph node biopsy during a episode of immune reconstitution. The isolated mycobacteria showed sensitivity to all first-line drugs. HIV infection, breast cancer and pulmonary tuberculosis have several mechanisms that induce hypercoagulable state and can lead to thromboembolic complications. Pulmonary thromboembolism in this patient was a diagnostic challenge because of all the other severe diseases that she experienced at the same time.

Echovirus 4 associated to hand, foot and mouth disease

Hand, foot and mouth disease (HFMD) is a contagious enteroviral infection occurring primarily in children and characterized by vesicular palmoplantar eruptions and erosive stomatitis. Echovirus 4 (EV-4) has been commonly associated with aseptic meningitis. The association of HFMD with EV-4 has not been reported previously. Two samples of a 14-month child who presented mild fever, sores in the mouth, rash with blisters on the palm of hands and soles of feet were sent to Enteric Viruses Laboratory of Adolfo Lutz Institute. Clinical samples were inoculated in three different cell lines, and those which presented cytopathic effect (CPE), were submitted to Indirect Immunofluorescence Assay (IFA) and "one step" RT-PCR. Agarose gel electrophoresis from RT-PCR product, showed a product with 437 bp, which is characteristic of Enterovirus group. Echovirus 4 was identified by IFA. Although HFMD is a viral infection associated mainly with Enterovirus 71 (HEV-71) and Coxsackievirus A16 (CV-A16), our results demonstrate a diversity of serotype related to HFMD and stress the importance of epidemiological surveillance to this disease and its complications.

Brain abscess secondary to frontal osteomyelitis

Frontal osteomyelitis is a rare complication of sinusitis. Common intracranial complications of the frontal osteomyelitis are meningitis, epidural empyema, subdural empyema and brain abscess. We described a case of frontal osteomyelitis with brain abscess caused by Staphylococcus aureus with improve after needle aspiration and antibiotics to brain abscess for eight weeks and for chronic osteomyelitis for four months.

Origem Anómala da Artéria Coronária Esquerda a Partir da Artéria Pulmonar

OBJECTIVE: To assess the frequency and severity of the anomalous origin of the left coronary artery (ALCA) from the pulmonary artery (PA). DESIGN OF THE STUDY: Prospective study of case series between March 1991 and December 1994. SETTING: Referral-based Paediatric Cardiology Department of a Tertiary Care Center. PATIENTS AND METHODS: Five consecutive patients (pts) with anomalous origin of the LCA from the PA; there were three infants aged 4 months and two children one 8 year and one 9 year old. There were three girls and two boys. All pts had clinical and 2D-echo and Doppler investigation prior to cardiac catheterization (CC). Indication for CC was based in the association of symptoms and signs of myocarditis or dilated cardiomyopathy of acute or subacute onset and electrocardiographic (ECG) signs of ischemia in infants. In older patients (pts) diagnosis was suspected mainly from ECG. During CC in all pts, aortograms and when necessary selective coronary angiograms were performed. Surgical correction was performed in all children. In two pts stress exercise ECG and stress Thallium studies before and after surgery were performed. RESULTS: two pts had "adult" an three had "infantile" type of ALCA from the PA. CC was performed and diagnosis was confirmed at surgery in all cases. In one child, correct diagnosis was made by ECO prior to CC and in one case LCA to PA fistula was suspected on Colour-Doppler study. No complications were attributed to CC. Several types of surgery were performed: reimplantation of the ALCA from the PA to the aorta (three pts); tunnel connection of the aorta to the ALCA via the PA (one pt) and left internal mammary to LCA anastomosis (one pt). Two infants died intraoperatively due to extensive myocardial infarction and poor left ventricular function. All the three survivors are asymptomatic after a mean follow up of 34 months. Two oldest pts are currently in New York Heart Association functional class I with normal ECG and improved myocardial perfusion on Thallium scan despite almost total occlusion of LCA at the site of implantation in the aorta as diagnosed on coronary angiogram. CONCLUSIONS: ALCA from PA is associated with major morbidity and mortality. Diagnosis should be suspected in pts with unexplained myocardial ischemia on ECG and even more if it is associated to clinical signs of dilated cardiomyopathy or myocarditis. Careful assessment on ECO and pulsed Doppler and colour flow mapping should make the diagnosis in most cases. Although surgery can be performed based only on ECO diagnosis, we strongly advise for angiography in all cases as in our experience there are false negative diagnosis by ECO. Preoperative Thallium studies can be useful for the selection of the type of surgery as pts with very little viable myocardium will not survive the establishment of a direct systemic to coronary blood flow and may be candidates for heart transplantation.

A Diabetes Mellitus e suas Complicações. Relembrar para Prevenir

Após breve referência aos principais programas de controlo das complicações da Diabetes Mellitus (Diabetes Control and Complications Trial, Declaração de St. Vincent, DiabCare), apresentam-se algumas propostas de revisão da sua classificação e dos critérios de diagnóstico. Abordam-se alguns conceitos actuais da sua fisiopatologia, descrevendo-se em seguida cada uma das suas complicações com ênfase nos aspectos essenciais da prevenção, diagnóstico precoce e terapêutica.