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Browsing by Author "Alves, R"

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  • Achieving K/DOQI Targets with Cinacalcet in Dialysis Patients with Secondary Hyperparathyroidism. A Portuguese Observational Study
    Publication . Macário, F; Frazão, JM; Ferreira, A; Weigert, A; Mota, M; Machado, D; Birne, R; Neto, R; Baldaia Moreira, A; Soares, C; Ribeiro, S; Mendes, T; Alves, R; Gomes, H; Raposo, H
    Secondary hyperparathyroidism is a common complication of chronic kidney disease. The elevated serum intact parathyroid hormone, phosphorus, calcium and calcium x phosphorus product have been independently associated with an increased relative risk of mortality. The standard therapy for secondary hyperparathyroidism, including active vitamin D analogues and phosphate binders, is often insufficient to allow patients to achieve the recommended Kidney Disease Outcomes Quality Initiative targets for bone and mineral metabolism. Randomised controlled phase III clinical studies in chronic kidney disease patients with secondary hyperparathyroidism have shown that cinacalcet treatment increases the proportion of patients achieving the recommended Kidney Disease Outcomes Quality Initiative targets for intact parathyroid hormone, phosphorus, calcium and calcium x phosphorus product. Aims: This observational multicentre study aims to evaluate cinacalcet’s ability to achieve and maintain Kidney Disease Outcomes Quality Initiative targets in a population with secondary hyperparathyroidism on chronic haemodialysis in Portugal. Patients and Methods: Patients on chronic dialysis that received cinacalcet during a free sampling programme were enrolled. Retrospective and prospective monthly data were collected from 3 months before until 6 months after the beginning of cinacalcet treatment. Additional assessment included a 12 month evaluation of all parameters. Results: 140 dialysis patients with secondary hyperparathyroidism were enrolled, 60% male, mean age 57.4±14.1 years. The mean intact parathyroid hormone, calcium, phosphorus, and calcium x phosphorus product values at baseline were 751.7±498.8 pg/ml, 9.7±3.8 mg/dl, 5.5±1.5 mg/dl, and 52.7±25.3 mg2/dl2, respectively. After 6 months’ cinacalcet treatment, 26.2%, 53.6%, 59.3%, and 81.0% of the patients achieved the Kidney Disease Outcomes Quality Initiative recommended levels for intact parathyroid hormone, calcium, phosphorus, and calcium x phosphorus product, respectively. The mean dose of cinacalcet at 6 months was 57.1±29.7 mg/day. Conclusions: The use of cinacalcet in clinical practice is an effective option for the treatment of secondary hyperparathyroidism in chronic dialysis patients, allowing more patients to reach and maintain the Kidney Disease Outcomes Quality Initiative targets.
    2009Journal article Open access Show more
  • Acidentes com Fogo de Artifício: Lesões da Mão na Criança
    Publication . Alves, R; Costa, MJ; Leal, MJ
    Análise da experiência e resultados da Instituição no trammento e reabilitação de lesões da mão provocadas pela explosão de artefactos de fogo de artifício. Casuística do HDE entre Janeiro de 1990 e Janeiro de 1997 constituída por 8 doentes estudados por um protocolo uniforme de avaliação. Na presente série todos os doentes são do meio rural. Verificou-se uma predominância do sexo masculino com 87%. A idade mais atingida foi a dos 10 anos. As lesões foram unilaterais em 87%. Registou-se uma prevalência da mão esquerda em 55%. O primeiro dedo foi o mais atingido com 25,5%. Nas lesões residuais, a amputação foi a mais frequente com 75%, seguida da brida com 62,2% e da alteração da sensibilidade táctil em 50% dos casos. O tratamento cirúrgico e de reabilitação precoce é importante para o prognóstico funcional do doente, mas é na prevenção destes acidentes que está a melhor solução para estes casos.
    1998Journal article Open access Show more
  • Chronic Gastric Volvulus
    Publication . Santos, E; Morão, S; Knoblich, M; Alves, R
    2021Journal article Open access Show more
  • Coexistence of Pheochromocytoma and Renal Artery Stenosis in a Pediatric Patient with Hypertension
    Publication . Serras, I; Baeta Baptista, R; Francisco, T; Casimiro, A; Lito, D; Alves, R; Abranches, M
    Pheochromocytoma and renal artery stenosis are surgically treatable causes of hypertension. Although rare, the coexistence of pheochromocytoma and renal artery stenosis has been described in case reports. Common pathophysiological mechanisms other than extrinsic compression may be involved in this association, such as catecholamine-induced vasospasm. The early recognition of the association of pheochromocytoma with renal artery stenosis is essential for appropriate treatment planning. We present the case of a previously healthy tenyear- old boy who presented with hypertensive encephalopathy, tachycardia and diaphoresis. Hypertension was found to be secondary to a catecholamine-producing tumor associated with coexisting renal artery stenosis. Hypertension resolved a few months after successful pheochromocytoma excision, without renal artery revascularization.
    2018Journal article Open access Show more
  • Collagen Type IV-Related Nephropathies in Portugal: Pathogenic COL4A5 Mutations and Clinical Characterization of 22 Families
    Publication . Nabais Sá, MJ; Sampaio, S; Oliveira, A; Alves, S; Moura, CP; Silva, SE; Castro, R; Araújo, JA; Rodrigues, M; Neves, F; Seabra, J; Soares, C; Gaspar, MA; Tavares, I; Freitas, L; Sousa, TC; Henriques, AC; Costa, FT; Morgado, E; Sousa, FT; Sousa, JP; da Costa, AG; Filipe, R; Garrido, J; Montalban, J; Ponce, P; Alves, R; Faria, B; Carvalho, MF; Pestana, M; Carvalho, F; Oliveira, JP
    Alport syndrome (AS) is caused by pathogenic mutations in the genes encoding α3, α4 or α5 chains of collagen IV (COL4A3/COL4A4/COL4A5), resulting in hematuria, chronic renal failure (CRF), sensorineural hearing loss (SNHL) and ocular abnormalities. Mutations in the X-linked COL4A5 gene have been identified in 85% of the families (XLAS). In this study, 22 of 60 probands (37%) of unrelated Portuguese families, with clinical diagnosis of AS and no evidence of autosomal inheritance, had pathogenic COL4A5 mutations detected by Sanger sequencing and/or multiplex-ligation probe amplification, of which 12 (57%) are novel. Males had more severe and earlier renal and extrarenal complications, but microscopic hematuria was a constant finding irrespective of gender. Nonsense and splice site mutations, as well as small and large deletions, were associated with younger age of onset of SNHL in males, and with higher risk of CRF and SNHL in females. Pathogenic COL4A3 or COL4A4 mutations were subsequently identified in more than half of the families without a pathogenic mutation in COL4A5. The lower than expected prevalence of XLAS in Portuguese families warrants the use of next-generation sequencing for simultaneous COL4A3/COL4A4/COL4A5 analysis, as first-tier approach to the genetic diagnosis of collagen type IV-related nephropathies.
    2015-11Journal article Open access Show more
  • Colon Stricture after Hemolytic Uremic Syndrome
    Publication . Sacras, ML; Ladeira, C; Pereira, SC; Alves, R
    2021Journal article Open access Show more
  • Curso sobre Alergia Alimentar da EAACI
    Publication . Rosa, S; Alves, R; Piedade, S; Reis, R; Geraldes, L; Figueira Santos, A; Costa, C; Silva, AT; Romeira, AM; Borrego, LM
    2006Journal article Open access Show more
  • Duplicação Tubular Colorretal
    Publication . Morão, S; Ladeira, C; Knoblich, M; Mendes, PP; Alves, R
    2018Journal article Open access Show more
  • Enfisema Lobar Congénito: Uma Apresentação Multilobar
    Publication . Moutinho, A; Seves, G; Gaspar, M; Alves, R; Sá Couto, H; Oliveira Santos, J
    O enfisema lobar congénito é umamalformação rara do tracto respiratório inferior detectada habitualmente nos primeiros meses de vida. Apresenta-se o caso de um lactente, do sexo masculino, de dois meses de idade, internado por suspeita de pneumonia, no qual a segunda radiografia de tórax revelou hipertransparência arredondada no lobo inferior esquerdo, sugestiva de enfisema lobar congénito. A tomografia computorizada torácica mostrou uma imagem quística,multilobar, e a broncoscopia excluiu obstrução brônquica. Foi programada cirurgia para os seis meses de idade, mas aos três meses foi submetido a intervenção cirúrgica urgente por pneumotórax hipertensivo. O exame anatomo-patológico da peça operatória permitiu o diagnóstico de enfisema lobar congénito. Destaca-se a apresentação multilobar e a complicação por pneumotórax.
    2011Journal article Open access Show more
  • Enterocolite Necrosante com Perfuração e Drenagem Peritoneal no Recém-Nascido de Muito Baixo Peso: Um Caso Clínico de Evolução Favorável
    Publication . Leal, I; Duarte, L; Alves, R; Magalhães, J; Videira-Amaral, J
    A enterocolite necrosante (ECN) constitui o problema gastrointestinal mais grave e mais frequente no recém-nascido (RN) de baixo peso. A melhoria na taxa de sobrevivência tem sido atribuída ao diagnóstico mais precoce e à experiência adquirida no tratamento do recém-nascido pré-termo em estado crítico. Desde 1977 que a drenagem peritoneal como actuação prioritária nos quadros de ECN tem sido preconizada nos recém- -nascidos de peso inferior a 1500 g com perfuração intestinal, e nos de peso superior a 1500 g com instabilidade hemodinâmica. Neste artigo relata-se o caso de um recém-nascido, com 1473 g de peso e 30 semanas de idade gestacional, ECN, sinais de perfuração intestinal e de instabilidade hemodinâmica, o qual foi submetido a drenagem peritoneal com evolução favorável e sem sequelas. Na discussão faz-se referência especial, de acordo com dados de literatura, aos mecanismos que explicam os bons resultados do procedimento em cerca de 2/3 dos casos de ECN com perfuração, os quais estão relacionados com as características particulares da cicatrização nos tecidos imaturos. Em conclusão, admite-se que a drenagem peritoneal deverá constituir a forma de actuação prioritária nos casos de ECN com perfuração e instabilidade hemodinâmica em RN pré-termo de muito baixo peso.
    1999Journal article Open access Show more