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São João da Madeira, Portugal

Silva D.,Centro Hospitalar Of Sao Joao | Ansotegui I.,Hospital Quiron Bizkaia | Morais-Almeida M.,CUF Descobertas Hospital | Morais-Almeida M.,University of Porto
World Allergy Organization Journal

The majority of drugs prescribed have not been tested in children and safety and efficacy of children's medicines are frequently supported by low quality of evidence. Therefore, a large percentage of prescriptions for children in the clinical daily practice are used off label. Despite the several recent legislation and regulatory efforts performed worldwide, they have not been successful in increasing availability of medicines adapted to children. Moreover, if we consider that 30% of the prescribed drugs for children are for the respiratory field and only 4% of new investigation projects for children research were proposed to access drugs for respiratory and allergy treatment, there is a clear imbalance of the children needs in this therapeutic area. This narrative review aimed to describe and discuss the off-label use of medicines in the treatment and control of respiratory and allergic diseases in children. It was recognized that a large percentage of prescriptions performed for allergy treatment in daily clinical practice are off label. The clinicians struggle on a daily basis with the responsibility to balance risk-benefits of an off-label prescription while involving the patients and their families in this decision. It is crucial to increase awareness of this reality not only for the clinician, but also to the global organizations and competent authorities. New measures for surveillance of off-label use should be established, namely through population databases implementation. There is a need for new proposal to correct the inconsistency between the priorities for pediatric drug research, frequently dependent on commercial motivations, in order to comply to the true needs of the children, especially on the respiratory and allergy fields. © 2014 Silva et al.; licensee BioMed Central Ltd. Source

Ferreira J.P.,Abel Salazar Biomedical Sciences Institute | Santos M.,Abel Salazar Biomedical Sciences Institute | Almeida S.,University of Lisbon | Marques I.,Abel Salazar Biomedical Sciences Institute | And 2 more authors.
European Journal of Internal Medicine

Background/objectives: Mineralocorticoid receptor antagonist (MRA) use in acutely decompensated chronic heart failure (ADCHF) may improve congestion through diuretic effect and prevent neurohormonal activation. We aimed to evaluate the clinical effect and safety of spironolactone in ADCHF. Methods: Prospective, experimental, single-center, and single-blinded trial. Patients were treated with: standard ADCHF therapy or oral spironolactone 50-100 mg/d plus standard ADCHF therapy. Results: During a 1 year period, 100 patients were enrolled, 50 included in the treatment group. Mean (SD) spironolactone dose (mg) at day 1 was 94.5 ± 23.3 and at day 3 was 62.7 ± 24.3. Worsening renal function (increase in pCr ≥ 0.3 mg/dL from day 1 to day 3) was more likely to occur in control group (20% vs. 4%; p = 0.038), serum potassium did not differ between groups, and plasma NTproBNP had a significant decrease in spironolactone group at day 3 (median [IQR], 2488 [4579] vs. 1555 [1832]; p = 0.05). Furthermore, a greater proportion of patients in the treatment group were free of congestion at day 3: less edema, rales, jugular venous pressure (JVP) and orthopnea (all, p < 0.05). In addition, a significantly higher proportion of patients were on oral furosemide at day 3 (44% vs. 82%; p < 0.001). Conclusions: Our study supports the safety of high dose spironolactone in ADCHF and suggests a positive impact in the resolution of congestion. The important findings of our pilot study need to be confirmed in larger trials. © 2013 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved. Source

Da Corte F.C.,Centro Hospitalar Of Sao Joao | Neves N.,University of Porto
European Journal of Orthopaedic Surgery and Traumatology

Rheumatoid arthritis (RA) is the most common inflammatory disease of the cervical spine (CS). After hands and feet, CS is the most commonly involved segment, being present in more than half of the patients with RA. Especially in the CS, RA may cause degeneration of ligaments, leading to laxity, instability and subluxation of the vertebral bodies. This is often asymptomatic or symptoms are erroneously attributed to peripheral manifestations. Otherwise, this may cause compression of spinal cord (SC) and medulla oblongata leading to severe neurologic deficits and even sudden death. Owing to its potentially debilitating and life-threatening sequelae, inevitable progression once neurologic deficits occur and the poor medical condition of afflicted patients, CS involvement remains a priority in the diagnosis and its treatment will remain a challenge. The surgical approach aims a solid fixation of the upper cervical spine, giving stability, preventing neurologic deterioration and injury to the SC, leading to improved neurologic function, vascular integrity and maintenance of sagittal balance. The recent advances in surgical techniques, complete understanding of the anatomy and precise preoperative evaluation led to safer and more effective procedures that have decreased complication rates. Based on the fact that when a patient becomes myelopathic the rate of long-term mortality increases and the chance of neurologic recovery decreases, many authors agree that early surgical intervention, before the onset of neurologic deficits, gives a more satisfactory outcome. However, the timing when a prophylactic stabilization should occur is poorly defined, and so, patients with radiographic instability but without evidence of neurologic deficit are still the most difficult to manage. © 2013 Springer-Verlag France. Source

Oliveira C.,University of Porto | Pinheiro H.,University of Porto | Figueiredo J.,University of Porto | Seruca R.,University of Porto | And 2 more authors.
Progress in Molecular Biology and Translational Science

The only gastric cancer (GC) syndrome with a proven inherited defect is designated as hereditary diffuse gastric cancer (HDGC) and is caused by germline E-cadherin/CDH1 alterations. Other E-cadherin-associated hereditary disorders have been identified, encompassing HDGC families with or without cleft-lip/palate involvement, isolated early-onset diffuse GCs, and lobular breast cancer families without GC. To date, 141 probands harboring more than 100 different germline CDH1 alterations, mainly point mutations and large deletions, have been described in these different settings. A third of all HDGC families described so far carry recurrent CDH1 alterations. Full screening of CDH1 is recommended in patients fulfilling the HDGC criteria and total prophylactic gastrectomy is the only reliable intervention for carriers of pathogenic alterations. In this chapter, we discuss CDH1-associated syndromes, frequency and type of CDH1 germline alterations, clinical criteria, and guidelines for genetic counseling, molecular pathology, and available animal/cell line models of the disease. © 2013 Elsevier Inc. Source

Carneiro F.,University of Porto | Carneiro F.,Centro Hospitalar Of Sao Joao

In 1998, Guilford et al. identified the hereditary diffuse gastric cancer (HDGC) syndrome, caused by germline alterations at the CDH1 (E-cadherin) gene. To date, 141 probands harboring more than 100 different germline CDH1 alterations, mainly point mutations and large deletions, have been described. In mutation-positive individuals prophylactic total gastrectomy is recommended. The systematic histological study of prophylactic gastrectomies shows intramucosal signet-ring cell carcinoma and pre-invasive lesions including in situ signet ring carcinoma with pagetoid spread of signet ring cells. In 2011, a new hereditary gastric cancer syndrome was identified: gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS). GAPPS is a unique gastric polyposis syndrome with a significant risk of gastric adenocarcinoma, characterized by the autosomal dominant transmission of fundic gland polyposis, with areas of dysplasia or intestinal-type GC, restricted to the proximal stomach, with no evidence of colorectal or duodenal polyposis or other heritable gastrointestinal cancer syndromes. © 2012 Springer-Verlag Berlin Heidelberg. Source

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