Browsing by Author "Vieira, P."
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- BK virus nephropathy in kidney transplantation - A literature review following a clinical casePublication . Barreto, P.; Almeida, M.; Dias, L.; Vieira, P.; Pedroso, S.; Martins, L.; Castro-Henriques, A.; Cabrita, A.Over the last 15 years, better immunosuppressive drugs have decreased acute rejection rates in kidney transplantation but have also led to an increase in the incidence and impact of BK virus nephropathy. The authors report the case of a 62 -year -old man submitted to a renal transplant of a deceased donor with an immunosuppression regimen free of rabbit anti -thymocyte globulin and tacrolimus, in whom BK nephropathy was diagnosed at seven weeks post -transplant. Intravenous human immunoglobulin (IVIG) was administered after immunosuppression reduction. Instituted treatment was successful. This clinical case highlights the importance of a high index of suspicion for an atypical presentation of BK nephropathy in renal transplant recipients and strengthens the need for other therapeutic interventions beyond the reduction of immunosuppression. It was the starting point for a review of BK virus nephropathy in kidney transplantation with a focus on risk factors, diagnosis and treatment.
- Um caso clínico de pseudohipoparatiroidismoPublication . Vieira, H.; Vieira, P.Introdução: O pseudohipoparatiroidismo engloba um grupo heterogéneo de doenças que se caracterizam por resistência dos órgãos alvo à acção da hormona paratiroideia. Caso clínico: Descreve-se o caso clínico de uma criança de nove anos, com história de convulsões generalizadas e câimbras, de aparecimento aos quatro e seis anos, respectivamente. Apresentava dismorfia facial, cataratas, atraso na erupção das peças dentárias, sinais de Chvostek e Trousseau, atraso do desenvolvimento psicomotor e baixa estatura. Na tomografia cranio-encefálica evidenciavam-se calcificações intraparenquimatosas. A presença de hipocalcemia, hiperfosfatemia e aumento da hormona paratiroideia permitiu estabelecer o diagnóstico de pseudohipoparatiroidismo. O fenótipo de osteodistrofia hereditária de Albright associado a alterações da função tiroideia é sugestivo de pseudohipoparatiroidismo do tipo Ia, por ser o mais frequente. O doente manteve irregular adesão à terapêutica, o que motivou múltiplas descompensações metabólicas. Conclusão: Salienta-se que a hipocalcemia deve ser considerada no diagnóstico diferencial das convulsões em idade pediátrica. O diagnóstico atempado e o correcto cumprimento da terapêutica, permitem prevenir as complicações da hipocalcemia. ABSTRACT Introduction: The pseudohypoparathyroidism includes a heterogeneous group of diseases characterized by parathyroid hormone action resistance of target organs. Case report: We report the case of a nine year-old child with generalized convulsions and cramps since the age of four and six years, respectively. He presented dysmorphic features, cataracts, delayed teeth eruption, Chvostek and Trousseau signs, psychomotor delay and short stature. Brain tomographic scan revealed cerebral intraparenquimatous calcifications. The presence of hypocalcemia, hyperphosphatemia and increased parathyroid hormone established the diagnosis of pseudohypoparathyroidism. The phenotype of Albright hereditary osteodystrophy associated with changes in thyroid function are suggestive of pseudohypoparathyroidism Ia, the most frequent type. The patient had irregular adherence to therapy, which caused several metabolic decompensation. Conclusion: We emphasize that hypocalcemia should be considered in the differential diagnosis of children seizures. Early diagnosis and proper compliance to therapy should prevent damage associated with hypocalcemia.
- Doença de Addison em idade pediátrica - um caso clínicoPublication . Franco, L.; Vieira, H.; Vieira, P.; Rebelo, I.Introdução: A insuficiência supra-renal primária de etiologia auto-imune, também designada Doença de Addison, é uma patologia rara, sobretudo em idade pediátrica. Caso clínico: Apresenta-se o caso de uma adolescente de 14 anos, internada por vómitos, diarreia e prostração de agravamento progressivo, com desidratação hiponatrémica grave associada a hipercaliemia e hipocloremia. Referia queixas de anorexia, astenia e perda ponderal com três meses de evolução. No exame objectivo apresentava-se obnubilada, mal perfundida e com ligeira hiperpigmentação cutânea e das mucosas. O estudo realizado confirmou o diagnóstico de insuficiência supra-renal primária, de etiologia auto-imune. Iniciou terapêutica com hidrocortisona e fludrocortisona, com boa evolução clínica. Discussão: A insuficiência supra-renal primária apresenta-se frequentemente de forma insidiosa, sendo os seus sintomas e sinais iniciais muitas vezes inespecíficos. A forma de apresentação aguda constitui uma verdadeira emergência médica, pelo que o grau de suspeição clínica deve ser elevado. ABSTRACT Introduction: The autoimmune primary adrenal insufficiency, also known as Addison disease, is a rare condition, especially in paediatric age. Case report: We present the case of a fourteen-year old adolescent, admitted in the sequence of vomiting, diarrhoea and progressive prostration, accompanied with severe hyponatremic dehydration, hyperkalemia and hypochloremia. She complained of anorexia, weakness and weight loss for the past three months. In the clinical examination she was obnubilated, with inadequate peripheral perfusion and slightly hyperpigmented skin and mucous membranes. The investigation confirmed the diagnosis of autoimmune primary adrenal insufficiency. She began treatment with hydrocortisone and fludrocortisone, with favourable clinical response. Discussion: The primary adrenal insufficiency often presents insidiously, with nonspecific signs and symptoms. Its acute presentation is a true medical emergency, so the degree of clinical suspicion should be high.
- Malignancy after renal transplantation: a single-centre experiencePublication . Vieira, P.; Bareto, P.; Pedroso, S.; Almeida, M.; Martins, L.; Dias, L.; Castro-Henriques, A.; Cabrita, A.Introduction: Malignancy management in renal transplant recipients is becoming a major factor affecting long‑term patient survival. Thus, we intended to evaluate both incidence and prognosis of malignant diseases following renal transplantation at a single centre in Portugal. Methods: We studied retrospectively the 2,358 patients who underwent kidney transplantation (KT) between 1983 and 2014. Apart from descriptive analysis, both demographic and clinical characteristics of cancer and non‑cancer cancer patients were compared. Results: During a median follow‑up of 118 (IQR 57‑179) months, 139 patients (5.8%) developed 158 de novo malignancies, with a median time from KT to diagnosis of 76..5 (IQR 21.0‑132.0) months. When compared to non‑cancer patients, they were older at KT date, had longer graft survival and a lower living donor recipients’ prevalence. As for post-transplant malignancies analysis, the most common were non‑cutaneous non‑lymphomatous cancers (49.4%, n=78), skin cancers (35.4%, n=56) and post‑transplant lymphoproliferative disorders (9.5%, n=15). Considering specific diagnosis, squamous cell carcinoma and basal cell carcinoma with 17.1% and 16.5% respectively, and non‑Hodgkin lymphomas with 7.6%, were the most frequent. Global mortality among cancer patients was 36.0%, with a median time of 9.7 (IQR 1.9‑17.5) months from time of diagnosis to death. As for survival analysis, cancer patient survival was significantly lower while censored graft survival was significantly higher in this group. Conclusion: Incidence and characteristics of malignancy following renal transplantation in our unit are similar to those globally described, despite some traits probably a result of specific ethnic and environmental characteristics.
- The first ABO-incompatible kidney transplantation performed in PortugalPublication . Barreto, P.; Vieira, P.; Dias, L.; Almeida, M.; Pedroso, S.; Martins, L.; Castro-Henriques, A.; Bini, M.; Cabrita, A.Kidney transplantation is the optimal treatment of end-stage renal disease (ESRD) improving survival and quality of life for most recipients. In our country, potential living donors have been refused due to the ABO incompatibility barrier. However, ABO -incompatible living donor kidney transplant is presently common practice in several countries with good outcomes. The authors describe a case of a 49-year-old female patient, with chronic kidney disease due to autosomal dominant polycystic kidney disease, who had started haemodialysis 10 months before and with blood group O. The living donor was a 53-year-old sister with blood group B. The desensitization protocol was based on rituximab and plasmapheresis. The induction protocol used was basiliximab, tacrolimus, mofetil mycophenolate and metilprednisolone. Five days post -transplant she presented a normal graft function that remained during the eight months follow -up. This case reveals the first ABO incompatible living donor kidney transplant performed in Portugal with excellent outcome