Hospital de Santa Maria
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Childhood hypopigmented mycosis fungoides: a commonly delayed diagnosis
Get PDFPrimary cutaneous lymphomas (PCLs) are exceedingly rare in children and adolescents, with mycosis fungoides (MF) being the most frequent PCL diagnosed in childhood. There are numerous unusual clinical variants of MF, including the hypopigmented type form (HMF). HMF is exceptional overall, but comparatively common among children. We present an 8-year-old boy with a 3-year history of progressive, generalised, scaly, hypopigmented round patches and few erythematous papules. He was first diagnosed with pityriasis alba (PA), and moisturisers were prescribed with no improvement. Skin biopsy showed typical features of MF, and the patient was successfully treated with narrowband ultraviolet B. HMF may simulate atopic dermatitis, PA, pityriasis lichenoides, tinea versicolour, vitiligo, postinflammatory hypopigmentation or leprosy. Therefore, persistent and unusual hypopigmented lesions should be biopsied to rule out this rare variant of MF
A decade of cardiac transplantation in Coimbra: the value of experience
No full textINTRODUCTION AND OBJECTIVES:
To analyze the experience gained in 10 years of the heart transplantation program of the University Hospital of Coimbra.
METHODS:
Between November 2003 and December 2013, 258 patients with a mean age of 53.0±12.7 years (3-72 years) and predominantly male (78%) were transplanted. Over a third of patients had ischemic (37.2%) and 36.4% idiopathic cardiomyopathy. The mean age of donors was 34.4±1.3 years and 195 were male (76%), with gender difference between donor and recipient in 32% of cases and ABO disparity (non-identical groups but compatible) in 18%. Harvest was distant in 59% of cases. In all cases total heart transplantation with bicaval anastomoses, modified at this center, was used. Mean ischemia time was 89.7±35.4 minutes. All patients received induction therapy.
RESULTS:
Early mortality was 4.7% (12 patients) from graft failure and stroke in five patients each, and hyperacute rejection in two. Thirteen patients (5%) required prolonged ventilation, 25 (11.8%) required inotropic support for more than 48 hours, and seven required pacemaker implantation. Mean hospital stay was 15.8±15.3 days (median 12 days). Ninety percent of patients were maintained on triple immunosuppressive therapy including cyclosporine, the remainder receiving tacrolimus. In 23 patients it was necessary to change the immunosuppression protocol due to renal and/or neoplastic complications and humoral rejection. All but two patients have been followed in the Surgical Center. Fifty patients (19.4%) subsequently died from infection (18), cancer (10), vascular (eight), neuropsychiatric (four), cardiac (two) or other causes (eight). Forty-six patients (17.8%) had episodes of cellular rejection (>2 R on the ISHLT classification), eight had humoral rejection (3.1%), and 22 have evidence of graft vascular disease (8.5%). Actuarial survival at 1, 5, and 8 years was 87±2%, 78±3% and 69±4%, respectively.
CONCLUSION:
This 10-year series yielded results equivalent or superior to those of centers with wider and longer experience, and have progressively improved following the introduction of changes prompted by experience. This program has made it possible to raise and maintain the rate of heart transplantation to values above the European average
Long-term follow-up of asymptomatic or mildly symptomatic patients with severe degenerative mitral regurgitation and preserved left ventricular function
Get PDFOBJECTIVES:
The timing for mitral valve surgery in asymptomatic patients with severe mitral regurgitation and preserved left ventricular function remains controversial. We analyzed the immediate and long-term outcomes of these patients after surgery.
METHODS:
From January 1992 to December 2012, 382 consecutive patients with severe chronic degenerative mitral regurgitation, with no or mild symptoms, and preserved left ventricular function (ejection fraction ≥ 60%) were submitted to surgery and followed for up to 22 years (3209 patient-years). Patients with associated surgeries, other than tricuspid valve repair, were excluded. Cox proportional-hazard survival analysis was performed to determine predictors of late mortality and mitral reoperation. Subgroup analysis involved patients with atrial fibrillation or pulmonary hypertension.
RESULTS:
Mitral valvuloplasty was performed in 98.2% of cases. Thirty-day mortality was 0.8%. Overall survival at 5, 10, and 20 years was 96.3% ± 1.0%, 89.7% ± 2.0%, and 72.4% ± 5.8%, respectively, and similar to the expected age- and gender-adjusted general population. Patients with atrial fibrillation/pulmonary hypertension had a 2-fold risk of late mortality compared with the remaining patients (hazard ratio, 2.54; 95% confidence interval, 1.17-4.80; P = .018). Benefit was age-dependent only in younger patients (<65 years; P = .016). Patients with atrial fibrillation/pulmonary hypertension (hazard ratio, 4.20, confidence interval, 1.10-11.20; P = .037) and patients with chordal shortening were at increased risk for reoperation, whereas patients with P2 prolapse (hazard ratio, 0.06; confidence interval, 0.008-0.51; P = .037) and patients with myxomatous valves (hazard ratio, 0.072; confidence interval, 0.008-0.624; P = .017) were at decreased risk.
CONCLUSIONS:
Mitral valve repair can be achieved in the majority of patients with low mortality (<1%) and excellent long-term survival. Patients with atrial fibrillation/pulmonary hypertension had compromised long-term survival, particularly younger patients (aged <65 years), and are at increased risk of mitral reoperation.info:eu-repo/semantics/publishedVersio
Pro-inflammatory triggers in childhood obesity: correlation between leptin, adiponectin and high-sensitivity C-reactive protein in a group of obese Portuguese children
Get PDFINTRODUCTION:
Pediatric obesity is increasingly prevalent in the Portuguese population. Adipocyte dysfunction results in the expression of pro-inflammatory mediators that are responsible for the low-grade inflammatory process that characterizes obesity.
OBJECTIVES:
The aim of this study was to investigate the relationship between markers of adiposity, inflammation and adipokines in a Portuguese obese pediatric population.
METHODS:
One hundred and twenty children of both sexes, aged 6-17 years, were included in this study. The control group consisted of 41 healthy normal-weight children. The variables analyzed were age, gender, body mass index, waist circumference, fat mass percentage, high-sensitivity C-reactive protein (hs-CRP), leptin and adiponectin.
RESULTS:
There were significant differences between controls and obese children for all parameters analyzed. In the obese group, after controlling for age and gender, hs-CRP (p=0.041), adiponectin (p=0.019) and leptin (p<0.001) still showed significant statistical differences. A direct correlation was found between hs-CRP, leptin, body mass index and waist circumference, the strongest being with leptin (r=0.568; p<0.001). This trend remained statistically significant, regardless of gender or pubertal age.
CONCLUSIONS:
Considering the role of leptin, adiponectin and hs-CRP in the genesis of endothelial dysfunction, they may be used in clinical practice for risk stratification, as well as in the assessment of weight control programs
Alergia de Contacto a Metais num Período de 20 Anos no Centro de Portugal: Implicações das Directivas Europeias
Get PDFINTRODUCTION:
Metals are a common cause of allergic contact dermatitis. After the introduction of the EU Nickel Directive (1994/27/CE; 2004/96/EC) and, more recently, the Cement Directive (2003/53/EC) there has been a significant decrease in sensitization to metals mainly in the Nordic countries. The applicability of these directives and their impact in the Portuguese population is unknown.
MATERIAL AND METHODS:
A retrospective study (1992-2011) was carried out in our patch test clinic to assess the temporal trend of metal sensitization (nickel [Ni], cobalt [Co] and chromium [Cr]) along the last 20 years, particularly considering age, sex and its relation with occupational activity.
RESULTS:
Out of 5 250 consecutively patch-tested patients, 1 626 (31%) were reactive to at least one metal (26.5% to Ni; 10.0% to Co and 7.9% to Cr). Women had a higher prevalence of sensitization to Ni (34.4% vs 8.9%) whereas men were more reactive to Cr (11.5% vs 5.0%). Nickel sensitization did not decrease significantly over the years, although in recent years among women sensitized to nickel the percentage of younger patients (16-30 years-old) is significantly lower (p < 0.001). Chromium sensitization significantly decreased, particularly in men (r = -0.535), and mainly in the construction workers (r = -0.639). Chromium reactivity associated with the shoe dermatitis has remained stable.
DISCUSSION:
We emphasize the higher and stable percentage of nickel sensitized individuals suggesting, so far, a low impact from the EU Ni directive, although a decreasing percentage in the the younger group among Ni sensitized women may suggest a beneficial effect is becoming evident is this age group. On the contrary, the impact of the directive regarding the modification of Cr in cement seems to be effective. There is now a need to regulate chromium content in leather products, namely in shoes.
CONCLUSIONS:
The regulation of interventional measures related either to the manufacture and trade of adornments or professional use will better protect the population of allergy to metals.info:eu-repo/semantics/publishedVersio
Evolução dos resultados perinatais em gestações triplas
Get PDFPURPOSE:
To evaluate the obstetric and perinatal outcomes evolution of triplet pregnancies.
METHODS:
A prospective observational study was conducted in triplet pregnancies delivered over 16 years in a tertiary obstetric center with differentiated perinatal support. Evaluation of demographic factors, obstetric complications, gestational age at delivery, mode of delivery, birth weight and immediate newborn outcome were done over a 16 years period. A global characterization of the sample was performed considering the listed parameters. Variables were categorized in three groups according to year of occurrence: 1996-2000, 2001-2006, 2007-2011, and all parameters were compared.
RESULTS:
Of the 33 triplets included, 72.7% resulted from induced pregnancies. All except one patient received prenatal corticosteroids and five received tocolytics. All women delivered prenatally and no significant differences were seen in the mean gestational age at delivery or birth weight towards time. There were three intrauterine fetal deaths. Neonatal immediate outcomes were not significantly different over the years.
CONCLUSION:
Despite remarkable progresses in perinatal and neonatal cares, no noticeable impact in triplet gestations' outcomes was seen, sustaining that triplets should be avoided due to their great risk of prematurity and neonatal morbidities, either by limiting the numbers of embryos transferred or by fetal reduction.info:eu-repo/semantics/publishedVersio
Obesity as a risk factor for Alzheimer's disease: the role of adipocytokines
Get PDFAlzheimer's disease is the leading cause of dementia and the most prevalent neurodegenerative disease. It is an aging-related multi-factorial disorder and growing evidence support the contribution of metabolic factors to what was formerly thought to be a centrally mediated process. Obesity has already been recognized as an important player in the pathogenesis of this type of dementia, independently of insulin resistance or other vascular risk factors. Although the exact underlying mechanisms are still unknown, adipocyte dysfunction and concomitant alteration in adipocyte-derived protein secretion seem to be involved, since these adipocytokines can cross the blood-brain barrier and influence cognitive-related structures. Very few studies have assessed the role of adipocytokines dysfunction on cognitive impaired patients and yielded contradictory results. Interestingly, extensive research on the central effects of leptin in Alzheimer's disease-transgenic mice has demonstrated its capacity to enhance synaptic plasticity and strength, as well as to prevent beta-amyloid deposition and tau phosphorylation. In addition, adiponectin, the most abundant adipocytokine whose levels are inversely correlated to adiposity, has shown to be neuroprotective to hippocampal cells. Many other adipose-derived cytokines have mainly pro-inflammatory properties, being able to trigger and/or enhance central inflammatory cascades and also to influence the secretion of other adipocytokines involved in cognition. This paper pretends to review the existing evidence on the contribution of adipocytokines dysfunction to the increased risk of dementia associated with mid-life obesity, unraveling its insulin-independent effects on cognition.info:eu-repo/semantics/publishedVersio
Hepatectomy and liver regeneration: from experimental research to clinical application
Get PDFBACKGROUND:
The mechanisms and kinetics of hepatic growth have continuously been investigated. This study concerns liver regeneration in animal and patients who underwent partial hepatectomy evaluated by the hepatic extraction fraction (HEF) calculated through radioisotopic methods.
METHODS:
Thirty normal Wistar rats were submitted to an 85% hepatectomy, and 95 patients with primary and secondary liver tumours were included. In animal study, the liver regeneration kinetics was assessed by HEF using 99mTc-mebrofenin, the ratio liver/bodyweight and by using bromodeoxyuridine deoxyribonucleic acid incorporation. In patient study, the liver regeneration was evaluated by calculation of HEF before surgery, 5 and 30 days after hepatectomy.
RESULTS:
In animal, we verified a positive correlation between HEF kinetics and liver/bodyweight ratio or hepatocyte proliferation evaluated by bromodeoxyuridine deoxyribonucleic acid staining after 85% hepatectomy. In the clinical arm, no statistical differences of the HEF before hepatectomy, 5 and 30 days after hepatectomy, were observed.
CONCLUSIONS:
Our results support the view that human liver regeneration commences early, is fast, non-anatomical and functionally complete 5 days after hepatectomy. The fast functional liver regeneration may have a high clinical impact particularly concerning the post-operative oncological therapeutic approaches.info:eu-repo/semantics/publishedVersio
