Takayasu Arteritis as a Secondary Cause of Arterial Hypertension

Abstract Takayasu arteritis (TA) is a rare type of vasculitis that affects mainly the aorta and its major branches. It is highly similar to giant cell arteritis (GCA), and differentiation between them may not be achieved even by histological examination. Arterial hypertension is typical of TA and is caused by stenosis of the renal arteries. Here we report the case of a 59-year-old woman, with a history of dyslipidemia and anemia, seen in the Internal Medicine department for resistant hypertension. Evaluation of secondary causes led to stenosis of the renal arteries. Assessment of target organ involvement was performed by computed tomography angiograph which revealed ectasia of the aortic arch and ascending aorta, tortuous course of the brachiocephalic trunk and the proximal portion of the right common carotid artery; positron-emission tomography which showed diffuse increased uptake in the ascending aorta, compatible with large vessels vasculitis. The patient was submitted to aortic valve replacement with a biological prosthesis combined with myocardial revascularization (Bentall-De Bono procedure). Aortic biopsy specimens showed anatomical and pathological features of GCA and TA. Due to persistently uncontrolled hypertension, prednisone 60 mg was initiated,with significant improvement in patient's condition.

Hansen's disease - a forgotten disease?

Hansen's disease, also known as leprosy, is an infection caused by the bacteria Mycobacterium leprae. The authors present the case of a 52-year-old man, born in Tondela and living in Espinho, with no pathological antecedents. The clinical picture began in April 2017, when macular lesions appeared in the lower limbs and rapidly progressed to the trunk and upper limbs, associated with complaints of pruritus but without alterations in the analytical study. After several topical and systemic treatments with glucocorticoids, antifungals, antibacterials and unsuccessful antihistamines, he was referred to an external consultation of Dermatology. He performed a biopsy of one of the lesions that revealed the definitive diagnosis: "Lepromatous Leprosy". After the biopsy result, he started triple treatment with rifampicin, clofazimine and dapsone with improvement of the condition.

Investigating Febrile Polyserositis: An Unusual Case of Idiopathic Hypereosinophilic Syndrome.

Hypereosinophilic syndrome (HES) is a heterogenous group of diseases characterized by abnormal accumulation of eosinophils in the blood or peripheral tissues. It can affect all organs and therefore clinical manifestations are highly variable. We describe the case of a 38-year-old man admitted for febrile polyserositis. He developed cardiac tamponade requiring pericardiocentesis complicated by left ventricle perforation which was successfully repaired. He presented mild peripheral eosinophilia. Bronchoalveolar lavage evidenced eosinophilic alveolitis, and pleural and pericardium histopathology revealed the presence of abundant eosinophils. All other causes of tissue eosinophilia were excluded and the diagnosis of idiopathic HES was made. The patient was started on glucocorticoids with resolution of symptoms. This case report describes a rare but potentially fatal presentation of HES and demonstrates the difficulty and delay in diagnosis when peripheral hypereosinophilia is absent. LEARNING POINTS: Hypereosinophilic syndrome (HES) is characterized by abnormal accumulation of eosinophils in the blood or peripheral tissues.The clinical manifestations of HES are highly variable.It may be difficult to diagnose HES when peripheral hypereosinophilia is absent.

Catastrophic antiphospholipid syndrome presented as ruptured papillary muscle during puerperium in a patient with systemic lupus erythematosus.

INTRODUCTION: Catastrophic antiphospholipid syndrome (CAPS) is a rare and serious phenomenon that requires prompt recognition and treatment. CASE PRESENTATION: The authors present the case of a puerperal woman with systemic lupus erythematosus (SLE) admitted to the emergency room with headache, blurred vision, thoracic pain, and purpuric lesions on both ears. Echocardiogram revealed global decrease in left ventricular function while cardiac and inflammatory markers were elevated. Three days after admission she developed cardiogenic shock due to rupture of mitral papillary muscle which required emergent cardiac surgery, with replacement of the mitral valve; treatment with anticoagulation, high-dose glucocorticoids, and intravenous immunoglobulins were initiated. Cardiac and brain MRI revealed signs of ischemic lesions in both organs. Histopathology analysis of the placenta and papillary muscle showed signs of ischemia secondary to microvascular thrombosis. Based on the clinical demonstration of thrombosis in three organs, and the presence of lupus anticoagulant antibodies, a diagnosis of probable CAPS was established. CONCLUSION: This case highlights the importance of a high level of suspicion of CAPS, particularly in patients with risk factors, and the value of immediate adequate treatment. Moreover, the rupture of a papillary muscle with histologically consistent signs of antiphospholipid syndrome expands the spectrum of involvement of this disease and should be considered as a rare but life-threatening possibility in patients with myocardial injury.

Prehospital Anticholinergic Burden Is Associated With Delirium but Not With Mortality in a Population of Acutely Ill Medical Patients.

OBJECTIVES: Anticholinergic drugs have several side effects, and they have been associated with adverse outcomes, particularly in older patients. The aim of this study was to analyze anticholinergic burden and its relationship to delirium and mortality in older acutely ill medical patients. DESIGN: Cohort study. SETTING AND PARTICIPANTS: Patients 65 years of age and older who were admitted to an Internal Medicine ward between August 1 and December 31, 2016. METHODS: Anticholinergic drug use, outpatient and inpatient, was assessed using the Anticholinergic Cognitive Burden Scale (ACB). Prevalent delirium was diagnosed by the Short Confusion Assessment Method (Short-CAM). RESULTS: Of the 198 patients, 28.3% developed delirium. Mortality rate was 13.6% in-hospital and 45.6% at 12 months. In multivariate analysis, outpatient ACB was associated with delirium, with an odds ratio (OR) of 1.65 [95% confidence interval (CI) 1.09-2.51]. Those with delirium had longer hospital stays (median 13 vs 8 days; P = .01), received more drugs (median 18 vs 15; P = .02), and presented a higher inpatient ACB (mean 3.9 vs 3.1; P = .034). No increased risk was found for in-hospital or 12-month mortality with drug use, ACB, or delirium. DISCUSSION: In the population studied, we found an association between anticholinergic burden as measured by the ACB and the presence of delirium, but not with mortality at 12 months. A very high 12-month mortality rate might have been an obstacle for association recognition. CONCLUSIONS AND IMPLICATIONS: Clinician awareness of possible drug side effects, especially in older populations, is crucial. As part of medication reconciliation at the time of hospitalization, ACB of prehospitalization medications should be routinely calculated by inpatient pharmacy services and made available to medical teams.

Breech Presentation: Vaginal Versus Cesarean Delivery, Which Intervention Leads to the Best Outcomes?

INTRODUCTION: The best route of delivery for the term breech fetus is still controversial. We aim to compare maternal and neonatal outcomes between vaginal and cesarean term breech deliveries. MATERIAL AND METHODS: Multicentric retrospective cohort study of singleton term breech fetuses delivered vaginally or by elective cesarean section from January 2012 - October 2014. Primary outcomes were maternal and neonatal morbidity or mortality. RESULTS: Sixty five breech fetuses delivered vaginally were compared to 1262 delivered by elective cesarean. Nulliparous women were more common in the elective cesarean group (69.3% vs 24.6%; p < 0.0001). Gestational age at birth was significantly lower in the vaginal delivery group (38 ± 1 weeks vs 39 ± 0.8 weeks; p = 0.0029) as was birth weight (2928 ± 48.4 g vs 3168 ± 11.3 g; p < 0.0001). Apgar scores below seven on the first and fifth minutes were more likely in the vaginal delivery group (1st minute: 18.5% vs 5.9%; p = 0.0006; OR 3.6 [1.9 - 7.0]; 5th minute: 3.1% vs 0.2%; p = 0.0133; OR 20.0 [2.8 - 144.4]), as was fetal trauma (3.1% vs 0.3%: p = 0.031; OR 9.9 [1.8-55.6]). Neither group had cases of fetal acidemia. Admission to the Neonatal Intensive Care Unit, maternal postpartum hemorrhage and the incidence of other obstetric complications were similar between groups. DISCUSSION: Although vaginal breech delivery was associated with lower Apgar scores and higher incidence of fetal trauma, overall rates of such events were low. Admission to the neonatal intensive care unit and maternal outcomes were similar. CONCLUSION: Both delivery routes seem equally valid, neither posing high maternal or neonatal complications' incidence.

Introdução: A melhor via de parto do feto pélvico de termo ainda é controversa. Pretendemos comparar desfechos maternos e neonatais entre partos vaginais e cesarianas de fetos pélvicos de termo. Material e Métodos: Estudo de coorte, multicêntrico e retrospetivo, incluindo gestações de feto único de termo, em apresentação pélvica, que terminaram em parto vaginal ou cesariana eletiva entre janeiro de 2012 e outubro de 2014. Os desfechos primários foram a morbilidade e mortalidade maternas e neonatais. Resultados: Sessenta e cinco partos vaginais foram comparados com 1262 cesarianas eletivas. As nulíparas foram mais frequentes no grupo das cesarianas (69,3% vs 24,6%; p < 0,0001). A idade gestacional foi inferior nos partos vaginais (38 ± 1 semanas vs 39 ± 0,8 semanas; p = 0,0029), verificando-se o mesmo para o peso ao nascer (2928 ± 48,4 g vs 3168 ± 11,3 g; p < 0,0001). Índices de Apgar < 7 foram mais frequentes nos partos vaginais (1º minuto: 18,5% vs 5,9%; p = 0,0006; OR 3,6 [1,9 - 7,0]; 5º minuto: 3,1% vs 0,2%; p = 0,0133; OR 20,0 [2,8 - 144,4]). Verificou-se também uma maior incidência de traumatismo fetal neste grupo (3,1% vs 0,3%; p = 0,031; OR 9,9 [1,8 - 55,6]). Nenhum grupo teve casos de acidemia fetal. As taxas de internamento na unidade de cuidados intensivos neonatais, de hemorragia materna pós-parto ou de outras complicações obstétricas foram idênticas. Discussão: O parto vaginal associou-se a índices de Apgar inferiores e a uma maior incidência de traumatismo fetal, com taxas globalmente baixas. Não se verificaram diferenças nas taxas de internamento neonatal nem nos desfechos maternos. Conclusão: Ambas as vias de parto parecem adequadas, nenhuma condicionando incidências elevadas de complicações maternas ou neonatais.