SNEF - Serviço de Nefrologia
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Browsing SNEF - Serviço de Nefrologia by Author "Aguiar, P."
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- Biomarkers and Imaging Findings of Anderson-Fabry Disease-What We Know NowPublication . Beirão, I.; Cabrita, A.; Torres, M.; Silva, F.; Aguiar, P.; Laranjeira, F.; Gomes, A.Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder, caused by deficiency or absence of the alpha-galactosidase A activity, with a consequent glycosphingolipid accumulation. Biomarkers and imaging findings may be useful for diagnosis, identification of an organ involvement, therapy monitoring and prognosis. The aim of this article is to review the current available literature on biomarkers and imaging findings of AFD patients. An extensive bibliographic review from PubMed, Medline and Clinical Key databases was performed by a group of experts from nephrology, neurology, genetics, cardiology and internal medicine, aiming for consensus. Lyso-GB3 is a valuable biomarker to establish the diagnosis. Proteinuria and creatinine are the most valuable to detect renal damage. Troponin I and high-sensitivity assays for cardiac troponin T can identify patients with cardiac lesions, but new techniques of cardiac imaging are essential to detect incipient damage. Specific cerebrovascular imaging findings are present in AFD patients. Techniques as metabolomics and proteomics have been developed in order to find an AFD fingerprint. Lyso-GB3 is important for evaluating the pathogenic mutations and monitoring the response to treatment. Many biomarkers can detect renal, cardiac and cerebrovascular involvement, but none of these have proved to be important to monitoring the response to treatment. Imaging features are preferred in order to find cardiac and cerebrovascular compromise in AFD patients.
- [Is it Fabry Disease? Diagnostic and Follow-Up Approach]Publication . Beirao, I.; Cabrita, A.; Torres, M.; Silva, F.; Aguiar, P.; Gomes, A.
- Overhydration prevalence in peritoneal dialysis - A 2 year longitudinal analysisPublication . Aguiar, P.; Santos, O.; Teixeira, L.; Silva, F.; Azevedo, P.; Vidinha, J.; Ferrer, F.; Carvalho, M.; Cabrita, A.; Rodrigues, A.Hypervolemia is a major concern in dialysis patients, and is associated with increased cardiovascular risk and death. Cross sectional analysis have previously demonstrated that peritoneal dialysis (PD) patients are not more overhydrated when compared to haemodialysiś ones. This study was designed to evaluate longitudinal trends in hydration status and corporal composition in a PD population.
- Pancreas-Kidney Transplantation: Analysis of 150 patients from one Centre in PortugalPublication . Martins, La Salete; Fonseca, Isabel; Aguiar, P.; Rocha, A.; Costa, R.; Santos, C.; Malheiro, J.; Pedroso, S.; Almeida, M.; Dias, L.; Castro-Henriques, A.; Cabrita, A.; Davide, J.Introduction: Simultaneous pancreas-kidney transplantation (SPKT) outcomes are conditioned in the short-term mostly by post-operative complications. In the long-term, cardiovascular (CV) disease and immunological loss are the main limitations to transplant survival. Aims: To analyse retrospectively the results from 150 SPKT performed at our centre. Patients and Methods: The 81 females and 69 males had a mean age of 35±6 years; they were diabetic for 24±6 years and had been on dialysis for 30±21months (except 5 preemptive). Anti-lymphocyte globulin, tacrolimus, mycophenolate and steroids were used as immunosuppressive therapy. Deceased-donor mean age was 28±11 years. In 28.7% the transplant was performed with 6 HLA-mismatches. Results: Acute rejection’s incidence was 16%. Ten SPKT patients died; infection was the leading cause of death (five cases), followed by Cardiovascular/cerebrovascular disease (three cases). In 21 patients the pancreas failed, mainly due to thrombosis or bleeding (11 cases), and infection (five cases); in two it was due to late acute rejection. In four patients only the kidney failed, due to chronic rejection. Five patients lost both grafts, from late acute rejection in four and thrombosis in one. We analyzed the 110 SPKT patients (73.3%) with both grafts functioning. Their mean serum creatinine was 1.2±0.4mg/dl; creatinineclearance was 76±24 ml/min; fasting glycaemia was 81±10mg/dl; and HbA1c was 5.3±0.4%. Hypertension has been treated in 47.2% of patients, in the majority (28.2%) with only one drug. Hyperlipidaemia was observed in 19.1% and excessive weight (>25kg/m2) in 17.3%. Conclusions: From our cohort of SPKT, 93.3% of patients are alive, 73.3% have both grafts functioning. Rejection was the main cause of late pancreas loss. Early mortality was due to infection (3.3%). CV/cerebrovascular disease was the main cause of late mortality (2%). The prevalence of hyperlipidaemia and overweight was inferior to 20%. Hypertension was the most frequently found CV risk factor.
- Tacrolimus, a forgotten agent in kidney transplant leukopeniaPublication . Azevedo, P.; Freitas, C.; Silva, H.; Aguiar, P.; Santos, T.; Cabral, J.; Rocha, G.; Almeida, M.; Pedroso, S.; Martins, L.; Dias, L.; Castro-Henriques, A.; Cabrita, A.Leukopenia in kidney transplant patients is frequent, it causes potentially life-threatening complications, but it is often poorly characterized. Opportunistic infections, immunologic disturbances and drug-related toxicity are principal causes of single or multilineage cytopenias. Tacrolimus-induced leukopenia is a less recognized but frequent complication. We describe one patient with leukopenia developing within seven months after renal transplant. After excluding other potential causes, tacrolimus was switched to cyclosporine, with recovery of white blood cell count. Based on the clinical report, the authors reviewed causes of post-transplant leukopenia, focusing on the diagnostic investigation. Early diagnosis and interventions are fundamental to improve prognosis.