Browsing by Author "Santos, S."
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- Acute tubulointersticial nephritis with uveitis: A report of two casesPublication . Silva, F.; Correia, S.; Castro, A.; Moreira, C.; Santos, S.; Malheiro, J.; Santos, J.; Martins, L.; Cabrita, A.Tubulointersticial nephritis and uveitis syndrome is an idiopathic and rare cause of acute kidney injury that should not overlooked, because it usually requires specific therapeutic interventions. We report two distinct cases: a young and an elder female. Both cases presented with unspecific constitutional symptoms but had different onset of renal and ocular involvement. Both were treated with topical and systemic corticoids and although there was a good initial response in both cases, an early relapse after steroids taper was observed in the younger patient and a persistent renal dysfunction in the older one. A high clinical suspicion and understanding of this disease is necessary for an adequate management and treatment of these patients. Recent data associates a worse renal prognosis when the disease appears in advanced age. In both of our cases the outcome was good but we had a short follow-up. The histological presentation of this disease in our older patient was similar to that reported in the literature, with a high percentage of fibrosis and chronicity of renal tissue that can contribute to the higher grade of renal dysfunction in this type of patients.
- 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
- Diagnosis of monoclonal gammopathy of renal significancePublication . Correia, S.; Santos, S.; Martins, L.; Santos, J.Monoclonal gammopathies are a heterogeneous group of disorders characterized by clonal proliferation of immunoglobulin produced by B-lymphocytes or plasma cell clone. The term monoclonal gammopathy of renal significance (MGRS) was introduced to distinguish monoclonal gammopathies that result in the development of kidney disease from those that are benign. Screening for monoclonal immunoglobulin and an appropriate hematologic workup are fundamental and sometimes a difficult challenge, with therapeutic and prognostic implications. Kidney biopsy is essential to determine the exact nature of the lesion and to evaluate the severity of renal disease. In this review we discuss the clinical and pathologic features of MGRS, highlighting the most diagnostic difficulties and current therapeutic options.
- Eritema multiforme major a Herpes simplex 1 no adultoPublication . Brochado, B.; Santos, S.; Sousa, F.; Faria, R.
- 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.
- Impacto da doença crónica na adolescênciaPublication . Santos, S.; Santos, E.; Ferrão, A.; Figueiredo, C.RESUMO Introdução: A adolescência, as suas alterações biopsicossociais e a doença crónica influenciam-se mutuamente, com especial relevância no comportamento social, rendimento académico e comportamento sexual. Objectivos: Avaliar se a doença crónica influencia comportamentos de risco nesta idade. Material e Métodos: Estudo transversal e comparativo, realizado entre 15 de Julho e 30 de Setembro de 2008, a jovens entre os 13-20 anos com doença crónica (DC) seguidos em consultas do Hospital São Teotónio de Viseu (HSTV) e a adolescentes saudáveis de duas escolas. Foi aplicado um inquérito específico para avaliação de dados sociodemográficos, história da doença, comportamentos de risco, auto-estima e relacionamento amoroso. Os testes estatísticos utilizados para o tratamento de dados foram o teste X2 e de t-student. Resultados: A amostra foi constituída por 125 sujeitos do grupo adolescentes com DC e 135 do grupo de adolescentes saudáveis. Comparando os dois grupos, constatou-se que os adolescentes com DC reprovam mais que os adolescentes saudáveis. Todos os 22 sujeitos sexualmente activos com DC referiam o uso de preservativo. Registou-se um menor consumo de hábitos tabágicos e alcoólicos no grupo de DC. Nenhum adolescente com DC admitiu o consumo de outras drogas. Discussão: A literatura refere que os adolescentes com doença crónica têm a mesma ou maior tendência para comportamentos de risco. No entanto, no actual estudo, os adolescentes com DC apresentam menos comportamentos de risco.
- Incontinentia Pigmenti – caso clínicoPublication . Santos, S.; Oliveira, R.; Bastos, V.; Matos, J.; Andrade, I.Introdução: A Incontinentia pigmenti (IP) é uma rara genodermatose neuroectodérmica, com uma incidência de 1:50.000 nascimentos, sendo tipicamente letal no sexo masculino, in utero. Caso Clínico: Os autores apresentam o caso de um recém-nascido, com lesões papulo-vesiculosas desde o nascimento sugestivas de IP. A evolução, a anatomo-patologia e o estudo genético permitiram estabelecer o diagnóstico. Conclusão: A IP é uma entidade clínica potencialmente grave que requer um diagnóstico precoce e um seguimento multidisciplinar. A suspeita clínica é fundamental para chegar ao diagnóstico. ABSTRACT Introduction: Incontinentia pigmenti (IP) is a rare genodermatosis neuroectodermal multisystem disorder. This disease has an incidence of 1:50.000 births and is typically lethal in males, in utero. Case report: The authors present a clinical case of IP in a newborn with papulovesiculous eruptions observed on the first day of life. The evolution, anatomopathology and genetic study fi ndings established the diagnosis. Conclusion: The IP is a potentially serious clinical entity, which requires an early diagnosis and multidisciplinary follow-up.
- 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