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.

Demographic and Clinical Characteristics of Human Immunodeficiency Virus-Infected Patients Receiving Dialysis in Portugal: a Nationwide Multicentre Survey

Background: Data on human immunodeficiency virus (HIV) infected patients receiving dialysis in Portugal is scarce. Methods: This nationwide epidemiological survey retrospectively evaluates HIV-infected patients on chronic dialysis in Portugal between 1997 and 2002. Results: Sixty-six patients were evaluated (mean age: 39.1±1.6 years, 47 men, 35 black African). Sixty-two patients started dialysis and 4 patients who were receiving dialysis had HIV seroconversion. Eighty-five percent of patients were treated in Lisbon. The annual incidence of HIV-infected patients on chronic dialysis was 0.5% in 1997 and 0.9% in 2002. Seventy-eight percent of patients were HIV-1 infected , 13% had hepatitis B and 31% hepatitis C. Sexual contact was the mode of transmission of HIV in 53% of cases. Four patients had biopsy-proved HIV-associated nephropathy. Ninety-five percent of patients were on chronic hemodialysis. Fifty percent of patients had acquired immunodeficiency syndrome. At follow-up, 12 patients died. HIV-infected CKD patient survival after starting dialysis was 80% at 3 years. Conclusion: The incidence of HIV-infected patients on chronic dialysis in Portugal has almost doubled. Widespread use of highly active antiretroviral therapy and the increasing number of black Africans from former overseas Portuguese colonies now living in Portugal are possible reasons for this large increase.

Neuroendocrine Tumours of the Female Genital Tract: a Case-Based Imaging Review with Pathological Correlation

BACKGROUND: Both primary and secondary gynaecological neuroendocrine (NE) tumours are uncommon, and the literature is scarce concerning their imaging features. METHODS: This article reviews the epidemiological, clinical and imaging features with pathological correlation of gynaecological NE tumours. RESULTS: The clinical features of gynaecological NE tumours are non-specific and depend on the organ of origin and on the extension and aggressiveness of the disease. The imaging approach to these tumours is similar to that for other histological types and the Revised International Federation of Gynecology and Obstetrics (FIGO) Staging System also applies to NE tumours. Neuroendocrine tumours were recently divided into two groups: poorly differentiated neuroendocrine carcinomas (NECs) and well-differentiated neuroendocrine tumours (NETs). NECs include small cell carcinoma and large cell neuroendocrine carcinoma, while NETs account for typical and atypical carcinoids. Cervical small cell carcinoma and ovarian carcinoid are the most common gynaecological NE tumours. The former typically behaves aggressively; the latter usually behaves in a benign fashion and tends to be confined to the organ. CONCLUSION: While dealing with ovarian carcinoids, extra-ovarian extension, bilaterality and multinodularity raise the suspicion of metastatic disease. NE tumours of the endometrium and other gynaecological locations are very rare. TEACHING POINTS: • Primary or secondary neurondocrine (NE) tumours of the female genital tract are rare. • Cervical small cell carcinoma and ovarian carcinoids are the most common gynaecological NE tumours. • Cervical small cell carcinomas usually behave aggressively. • Ovarian carcinoids tend to behave in a benign fashion. • The imaging approach to gynaecological NE tumours and other histological types is similar.