Browsing by Author "Campos, A."
Now showing 1 - 10 of 10
Results Per Page
Sort Options
- Acute kidney injury with active urinary sediment analysis, a positive ANCA test and hypocomplememtemia: A tough situationPublication . Campos, A.; Vizcaíno, J.; Coelho, A.; Freitas, C.; Rocha, G.
- Anderson-Fabry disease: Ten-year outcome of enzyme replacement therapy in a renal transplant patientPublication . Santos, S.; Campos, A.; Beirao, I.Anderson‑Fabry disease (AFd) is a rare disorder characterised by the deficiency or absence of lysosomal enzymatic alpha‑galactosidase A activity (α‑Gal A) that leads to progressive and systemic accumulation of glycosphingolipids. The clinical manifestations are variable but kidney disease usually manifests before the fourth decade of life and chronic renal failure rapidly progresses to end‑stage renal disease (ESRD), requiring dialysis and kidney transplantation (KT). In patients with a definite diagnosis, enzyme replacement therapy (ERT) is recommended as soon as there are early clinical signs of kidney, heart or brain involvement. We present a case of a kidney transplant patient who was diagnosed with AFd nine years after KT, confirming the difficulty that may exist in na early diagnosis of this disease even among high‑risk groups. At this stage, in addition to renal damage, the patient already had advanced disease and established organ injury, including ocular, pulmonary, cerebrovascular and cardiac. He started agalsidase beta (Fabrazyme®) intravenously every two weeks at a dose of 1 mg/kg body weight. During ten years of treatment no major adverse events were reported and our experience indicates that ERT is a safe and effective treatment for extra‑renal Fabry manifestations in KT patients
- Autologous stem cell transplantation in a patient with severe systemic sclerosisPublication . Vaz, C.; Almeida, I.; Guedes, M.; Rosário, C.; Branca, R.; Campilho, F.; Roncon, S.; Vasconcelos, C.; Campos, A.Systemic Sclerosis (SSc) is a chronic disease of the connective tissue, whose pathogenesis involves abnormalities of the immunological system. It has a variable course and there is a subgroup of patients with rapidly progressive disease or unresponsive to conventional treatment. Thesepatients can benefit from intensive immunosuppression and autologous hematopoietic stem cell transplant. Clinical case: 19-year-old (y.o.) woman diagnosed with SSc at the age of 13 y.o. with cutaneous, vascular and articular involvement with initial response to me thotrexate. Three years later the disease progressed with severe digestive involvement (dysphagia, delayed gastric emptying and weight loss) needing gastrostomy for nutritional support. She was treated with cyclophosphamide without improvement. In May 2012 she had an autologous transplant with myeloablative regimen (BEAM): carmustine 300 mg/m2 x1day; etoposide 120 mg/kg x4days; cytarabine 120 mg/kg 12/12:h x4days; melphalan 140 mg/m2 x1day. A year and a half after transplantation she is asymptomatic, without any signs or symptoms of the disease, feeds by mouth and the gastric emptying study is normal. Currently she is free of medication.
- Espondilolise e espondilolistese ístmica de grau I no adolescente: Análise retrospectiva de 30 casosPublication . Oliveira, V.; Rodrigues-Pinto, R.; Costa, L.; Encarnação, A.; Campos, A.; Oliveira, A.Objectivo: a espondilolise e a espondilolistese ístmica grau I são causas de lombalgia nos adolescentes e associam-se a fractura de fadiga da pars interarticularis por movimentos repetidos de hiperextensão. Na literatura não existe consenso quanto aos benefícios do tratamento cirúrgico nestes doentes, muitas vezes atletas, e a controvérsia prolonga-se sobre qual a melhor técnica cirúrgica. Os autores descrevem a técnica cirúrgica que sistematicamente utilizam e analisam os resultados obtidos.
- Fgf-23 and vascular calcification in a peritoneal dialysis population with residual renal functionPublication . Santos, S.; Carlos Oliveira, José; Barra, T.; Campos, A.; carvalho, M.; Malheiro, J.; Fonseca, Isabel; Cabrita, A.; Adragão, T.; Rodrigues, AnabelaIntroduction and Aims: Fibroblast growth factor 23 (FGF-23) induces phosphaturia. Its clinical impact is beyond mineral bone disease in chronic kidney disease (CKD), being coupled with vascular calcification and mortality. Residual renal function (RRF) is associated with significant capacity to excrete phosphate in peri- toneal dialysis (PD). Besides testing whether FGF-23 is still related with glomerular filtration rate (GFR) and phosphate excretion in this late stage of CKD (5d), we aimed to explore its link with vascular calcification.Subjects and Methods: FGF-23 (C terminal) was measured in forty prevalent PD patients with RRF, aged 61.5 (51.0-67.0) years old, in renal replacement therapy (RRT) for 43.5 (23-80.0) months; 36.6% were female, 19.5% had diabetes mellitus and 37.5% were under automated PD regimen; 80% were on PD first, and only 20% had previous RRT. Relevant variables including dietary phosphate (P) intake, CKD-bone laboratory parameters, serum 25-hydroxyvitamin D, magnesium (Mg) levels, GFR, urinary phosphate, fractional excretion of phosphorus (FEP), albumin, proBNP and Adragão vascular calcification score were explored. Results: Median levels (25-75% range) of serum variables were: FGF-23 1997 (1623-2149) RU/mL, Mg 0.94 (0.8-1.0) mmol/L, 25-hydroxyvitamin D 30 (18-47) nmol/L, calcium 2.2 (2.0-2.37) mmol/L, phosphorus 1.69 (1.30-1.90) mmol/L, PTH 429 (309-626) pg/mL. FGF-23 correlated positively with serum phosphate (r = 0.39, p = 0.013) and negatively with urine volume (r = -0.48, p = 0.001), phosphaturia (r = -0.594, p < 0.0001) and GFR (r =-0.61,p < 0.0001). However, FGF-23 was not significantly correlated with age, total time of RRT, dietary P, FEP, Mg, nor 25-hydroxyvitamin D. High FGF-23 group had higher FEP. GFR was the single inde- pendent predictor of increased FGF-23. On the other hand, neither FGF-23 nor low FEP/FGF-23 ratio were significantly associated with the vascular calcification score. Only albumin (lower), magnesium (lower) and proBNP (higher) levels significantly differed in calcified versus non-calcified patients (all with p < 0.05). Conclusions: In our population, FGF-23 was not associated with vascular calcification. GFR was the single independent predictor of increased FGF-23 in patients with diuresis. Increment of FGF-23 in PD patients signalizes an active endocrine phosphaturic process compensating renal function loss, as expressed by higher fractional excretion of phosphorus. It alerts for dietetic and therapy optimization. However, its link with vascular calcification still lacks validation.
- Impact of preformed donor-specific antibodies against HLA class I on kidney graft outcomes: Comparative analysis of exclusively anti-Cw vs anti-A and/or -B antibodiesPublication . Santos, S.; Malheiro, J.; Tafulo, S.; Dias, L.; Carmo, R.; Sampaio, S.; Costa, M.; Campos, A.; Pedroso, S.; Almeida, M.; Martins, L.; Henriques, C.; Cabrita, A.AIM: To analyze the clinical impact of preformed antiHLA-Cw vs antiHLA-A and/or -B donor-specific antibodies (DSA) in kidney transplantation. METHODS: Retrospective study, comparing 12 patients transplanted with DSA exclusively antiHLA-Cw with 23 patients with preformed DSA antiHLA-A and/or B. RESULTS: One year after transplantation there were no differences in terms of acute rejection between the two groups (3 and 6 cases, respectively in the DSA-Cw and the DSA-A-B groups; P = 1). At one year, eGFR was not significantly different between groups (median 59 mL/min in DSA-Cw group, compared to median 51 mL/min in DSA-A-B group, P = 0.192). Moreover, kidney graft survival was similar between groups at 5-years (100% in DSA-Cw group vs 91% in DSA-A-B group, P = 0.528). The sole independent predictor of antibody mediated rejection (AMR) incidence was DSA strength (HR = 1.07 per 1000 increase in MFI, P = 0.034). AMR was associated with shortened graft survival at 5-years, with 75% and 100% grafts surviving in patients with or without AMR, respectively (Log-rank P = 0.005). CONCLUSION: Our data indicate that DSA-Cw are associated with an identical risk of AMR and impact on graft function in comparison with "classical" class I DSA.
- Kidney transplantation in a patient with preformed and exclusively anti-HLA-Cw donor specific antibodyPublication . Santos, S.; Castro, A.; Campos, A.; Pedroso, S.; Dias, L.; Castro-Henriques, A.We report a patient who had received a first kidney transplant and had preformed DSA anti-HLA-Cw, developing AMR C4d+ soon after transplant. Classically anti-HLA-Cw are considered less immunogenic and are not considered in many organ allocation systems or immunologic risk stratification algorithms, including in Portugal. However, data from literature confirms that their presence is as deleterious as DSA anti-HLA A/B/DR/DQ. Thus we should take HLA-C typing and respective antibody identification into account in sensitized patients, in order to access risk stratification and establish the need for correct induction or desensitization therapies.
- Mesalazine induced tubulointersticial nephritisPublication . Campos, A.; Santos, S.; Santos, J.; Malheiro, J.; Lobato, L.; Vizcaíno, J.; Cabrita, A.Inflammatory bowel disease and its various treatments may affect the kidney in several ways tubulointersticial nephritis is a rare but serious complication of longer-term mesalazine use. There are few cases reported in the literature. We report the first two cases of mesalazine-induced tubulointersticial nephritis, recently diagnosed in our department. The first one refers to a patient with ulcerous colitis and the second one to a patient with Crohn’s disease. Then the authors present a review of literature about the renal involvement in the inflammatory bowel disease. New cases of mesalazine nephrotoxicity should be reported to allow more accurate incidence estimation of this serious adverse effect. Routine monitoring of renal function is simple, inexpensive and allows an early diagnosis of this complication
- Peritoneal dialysis dropouts in different age and era cohorts: focus on the elderlyPublication . Campos, A.; Malheiro, J.; Teixeira, L.; Carvalho, M.; Cabrita, A.; Rodrigues, A.Introduction and Aims: Peritoneal dialysis (PD) is an efficient renal replacement therapy (RRT), but still remains underutilized at any age. Clinicians fear the rate of dropouts and lower technique survival, particularly in elderly patients. The authors aimed to explore such outcomes over the past 3 decades, in different age and era cohorts. Methods: Consecutive incident patients starting PD were identified from an ongoing registry-base prospective study of quality assessment. In order to control for an era effect, patients were assigned to 6 cohorts (5 years interval) according to the admission year between 1985 and 2014. Regression models taking competing risks into account were performed to identify potential prognostic factors for death and transfer to haemodialysis (HD) (adjusted for age, gender, diabetes, cohort era, automated peritoneal dialysis (APD) use, and first treatment modality – PD first, PD after HD, PD after renal transplant (RT). Then the patients were studied according to age at enrolment in the programme: A (18 44 years; n = 193); B (45 64 years; n = 176) and C (≥ 65 years old; n = 75). The HD transfer rates using Poisson analysis were evaluated. The incidence of dropout rates was compared at different times and between age groups, focusing particular attention on the elderly. Results: A total of 525 patients were evaluated: 211 male (40.2%), aged 48 ± 15.7 years old, on PD for 23 (IQR 9 – 41.5) months. The major cause of dropout technique was transfer to HD (35.4%), followed by renal transplantation (27.6%) and death (21.7%). The probability of technical failure and renal transplantation at 2 and 5 years was 19.2% and 18.1% and 34.2%; 27.4%, respectively. Probability of death at 2 and 5 years was 12.7%, and 21.8%, respectively. The contemporary cohort was associated with a lower risk of mortality and lower risk of transfer to haemodialysis, with greater access to renal transplantation. The regression model Fine & Gray showed that older age was associated with increased mortality, but was not associated with greater technical failure. Transfer to HD occurred in the elderly at a rate of 11epy/100 patient year (in comparison to 15 and 14 epy/100 patient-year in non-elderly groups A and B, respectively P= 0.33). The proportions of specific causes of technique failure did not change significantly according to age cohort. The dropout rates due to access-related-infection and ultrafiltration failure decreased in the elderly group in the more contemporary cohort, despite the differences were not statistically significant. Conclusions: The dropout by technique failure decreased significantly in the recent decade. Age at admission in peritoneal dialysis did not show to be a compromising factor of the technique survival
- Rare case of bilateral anterolateral and symmetrical bowing of tibia successfully treated with a distal tibial opening wedge osteotomyPublication . Miranda, M.; Afonso, C.; Martins, C.; Carvalho, J.; Campos, A.The anterolateral bowing of the tibia is closely associated with the development of its pseudarthrosis. Roughly, all deformities are unilateral so the shortening and angulation are easy to identify. We present a 6-year-old boy with an exuberant bilateral anterolateral bowing of tíbia. He has short stature, disturbed gait and callosity at the lateral border of the foot. Deformity was successfully treated by opening wedge tibia osteotomy and filled the remaining gap with structural fibular graft. It was fixed with two crossed K-wires and cast immobilization for 6 weeks. We decided to correct it before skeletal maturity due to the significant disturbance of the gait and esthetic impairment. It was obtained a satisfactory morphological and functional result with a simple and fast technique.