Browsing by Author "Silva, Gil"
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- Blood pressure and phosphate level in diabetic and non-diabetic kidney disease: Results of the cross-sectional "Low Clearance Consultation" studyPublication . Mendes, Margarida; Resende, Luis; Teixeira, Alves; Correia, João; Silva, GilA statistically significant and moderate positive correlation was verified between systolic blood pressure and plasma phosphate concentration in the whole sample (diabetic and non-diabetic), due to the diabetic chronic kidney disease group.A statistically significant and moderate positive correlation was verified between the diastolic blood pressure and plasma phosphate concentration in the whole sample (diabetic and non-diabetic), due to the diabetic chronic kidney disease group.A statistically significant and strong negative correlation was found between diastolic blood pressure and estimated glomerular filtration rate in the diabetic chronic kidney disease group, but there was no statistically significant correlation in the whole sample (diabetic and non-diabetic).
- Blood pressure and phosphate level in diabetic and non-diabetic kidney disease: Results of the cross-sectional “Low Clearance Consultation” studyPublication . Mendes, Margarida; Resende, Luis; Teixeira, Alves; Correia, João; Silva, GilA statistically significant and moderate positive correlation was verified between systolic blood pressure and plasma phosphate concentration in the whole sample (diabetic and non-diabetic), due to the diabetic chronic kidney disease group.A statistically significant and moderate positive correlation was verified between the diastolic blood pressure and plasma phosphate concentration in the whole sample (diabetic and non-diabetic), due to the diabetic chronic kidney disease group.A statistically significant and strong negative correlation was found between diastolic blood pressure and estimated glomerular filtration rate in the diabetic chronic kidney disease group, but there was no statistically significant correlation in the whole sample (diabetic and non-diabetic).
- Celiac Disease as a Rare Cause of Membranous Nephropathy: A Case ReportPublication . Pestana, Nicole; Vida, Carlota; Durães, José; Silva, Gil; Vieira, PedroMembranous nephropathy is the most common cause of nephrotic syndrome in adults. A non-negligible number of cases are associated with systemic conditions. We report a case of a 50-year-old man who presented with nephrotic syndrome six months after being diagnosed with celiac disease. Although the patient showed disappearance of circulating immunoglobulin A (IgA) anti-tissue transglutaminase antibodies following a gluten-free diet, he had a sudden onset of nephrotic syndrome presenting with severe hypoalbuminemia. Other secondary causes were promptly excluded leading to the assumption of celiac disease-associated membranous nephropathy with remission after treatment with angiotensin system blockade and a gluten-free diet. The goal of this case report is to alert the clinic towards this rare association aiming for an early diagnosis and adequate selection of long-term therapy.
- Echocardiography and cardiovascular risk:The relationship in the renal transplant recipientPublication . Neto, Micaela; Gonçalves, Miguel; Resende, Luis; Vieira, Pedro; Gomes, Susana; Durães, José; Rosa, Nuno; Teixeira, José Alves; Silva, GilIntroduction: Cardiovascular disease (CVD) is the major cause of death among renal transplant recipientes (RTR). It is not known whether echocardiographic abnormalities are useful to identify RTR at high risk of CVD. Methods: Retrospective review of RTR with functioning and stable graft and an echocardiography performed in the last year. Risk of major adverse cardiac events (MACE) and death using a risk calculator specific for RTR. Results: Among 107 patients (57.9% males, 50.4±13.9 years), 7-year risk of MACE was >10% in 30.9% of patients and 7-year risk of death >10% in 56.1%. Left ventricular hypertrophy (LVH) was found in 55.1%, diastolic dysfunction in 39.3%, dilated left atrium (LA) in 53.3%, high pulmonary artery systolic pressure (PASP) in 9.0%, valvular calcifications in 22.4% and moderate to severe mitral regurgitation (MR) in 3.7%. Mean Ejection fraction was 68.36±6.87%. Univariate analysis showed an increased risk of MACE and death in patients with LVH, diastolic dysfunction, dilated LA, high PASP, valvular calcifications and MR. Multivariate analysis identified an independente association between the risk of MACE >10% and valvular calcifications and high PASP. Risk of death>10% in multivariate analysis had an independent association with diastolic dysfunction and elevated PASP. Conclusion: Echocardiographic abnormalities identify RTR at increased risk of MACE and death. Valvular calcifications and high PASP are predictors of MACE whereas diastolic dysfunction and high PASP predict death.
- Fabry disease in patients under dialysis: A screening study and identification of a novel mutationPublication . Silva, Francisca Gomes; Pestana, Nicole; Durães, José; Silva, GilFabry disease (FD) is a rare systemic disease, with a large spectrum of disease severity. A GLA gene mutation in X‑chromosome leads to progressive accumulation of globotriaosylceramide (Gb3) in various organs. We screened all patients under dialysis from a single center for GLA gene changes. Enzymatic activity of alpha galactosidase A (α‑Gal A) and concentration of lyso‑Gb3 were determined in dried blood spots. Genetic study was performed in male patients with low α‑Gal A activity and in all female subjects. For all positive patients, a complete family study was performed. A total of 72 dialysis patients were screened. Sequence analysis was carried out in 53 patients (25 males). Heterozygous variants of the GLA gene were found in 4 patients (7.5%): c.937G>T (D313Y) in exon 6; c.352C>T (R118C) in exon 2; c.870G>C (M290I) in exon 6 and c.580A>G (T194A) in exon 4. Family screening was performed in a total of 17 subjects, with a GLA genetic variant prevalence of 58.8%. Unlike p.D313Y and p.R118C, well‑known non‑pathogenic polymorphisms, p.M290I is a controversial poorly described mutation. Reports about its phenotypic expression are crucial for a better understanding of its behavior. The recognition of the novel mutation p.T194A is importante for better knowledge of FD and its spectrum of clinical manifestations. These affected patients are expected to develop a classic and life‑threatening FD phenotype and an early diagnosis is essential for their treatment success.
- IgA dominant glomerulonephritis associated to staphylococcus infection: a peculiar case reportPublication . Pestana, Nicole; Vieira, Pedro; Silva, Francisca; Figueira, José Ricardo; Silva, Gil; Durães, JoséIgA dominant glomerulonephritis associated to Staphylococcus infection is a rare clinical entity that has been described mainly in case reports. Biopsy features can resemble other disease entities mainly IgA nephropathy and Henoch‑Schönlein purpura nephritis. Treatment of IgA dominant glomerulonephritis associated to staphylococcal infection is based on antibiotics for the underlying infection, controlling hypertension and edema and may resort to concomitant use of steroids in selected cases. Prognosis markers such as hypertension, diabetes and interstitial fibrosis may influence treatment as they are associated with poor renal outcomes. We report a case of a 63‑year‑old man with known hypertension, pre‑diabetes and recent history of methicillin‐sensitive staphylococcus aureus bacteremia associated to prostatitis, who presented with a one‑month history of edema, arthralgia and foamy urine. Over this period he progressed to anasarca and nephrotic range proteinuria with concomitant rise in creatinine levels being documented. The renal biopsy showed segmental endocapillary proliferation and IgA segmental dominant staining associated to C3 and lambda in minor distribution. On completion of two months of steroid therapy the patient partially recovered his renal function and proteinuria. After nine months of tapering steroids, he presented with acute inflammatory arthritis supporting an inflammatory background disease. To our knowledge this case describes an unusual entity such as IgA dominant glomerulonephritis associated to staphylococcal infection co‑presenting with an associated reactive arthritis.
- A new millennium for women and kidney diseasePublication . Pestana, Nicole; Vieira, Pedro; Silva, GilIntroduction: Taking advantage of this year’s synergy, as World Kidney Day (WKD) and International Women’s Day fall on the same day, the theme chosen for WKD 2018 was “Kidneys and Women’s health”, drawing the attention of the nephrology community to the special features of diseases of the kidney as they affect women. When focusing on this subject, we come to see the major gaps in this area of knowledge. On one hand, it is now comprehensively accepted that there are unique biological and behavioral differences resulting in sex/gender variances, albeit mostly in favour of women. However, on the other, despite mounting evidence in multiple medical disciplines, these disparities have not been so well explored in nephrology, and so we try, in this editorial, to review current knowledge n this field.
- Obstructiva Acute Kidney Injury with maintenance of diuresis: A Case of Ureter BifidityPublication . Silva, Francisca Gomes da; Rosa, Nuno; Pestana, Nicole; Silva, GilPercutaneous kidney biopsy in transplanted kidneys remains an essential and commonly performed procedure required for diagnostic and prognostic information. Hemorrhage is the main complication of renal graft biopsy. We report a case of a 47-year-old caucasian woman admitted to perform an ultrasound (US)-guided biopsy of the renal graft. Six hours later, she presented with macroscopic hematuria which improved after urethral catheterization and intravenous hydration. However the hematuria reappeared associated with anemia and worsening of the serum creatinine value. Te US study, revealed hydronephrosis with high Doppler derived renal resistive index compatible with clot obstruction. Despite the vesical lavage nd intravenous hydration. However the hematuria reappeared associated with anemia and worsening of the serum creatinine value. Te US study, revealed hydronephrosis with high Doppler derived renal resistive index compatible with clot obstruction. Despite the vesical lavage with drainage of several clots, the patient rapidly progressed to hemorrhagic shock with worsening of renal function. Pelvic computed tomography (CT) revealed calyx and pelvis duplicity and ureter bifdity which merged into a single ureter and inserted into the right anterolateral wall of the bladder. Te inferior ureter was enlarged due to an obstructive clot. Most acute obstructive uropathies are associated with signifcant pain or the abrupt diminution of urine fow. Te presence of ureter bifdity in the CT study explained the maintenance of signifcant diuresis despite obstruction, located only to the lower ureter but with sufcient functional impact to condition acute kidney injury (AKI).
- Rare aetiology of obstructive kidney injury: Bilateral ureteral endometriosisPublication . Correia, Maria Inês; Vieira, Pedro; Gonçalves, Miguel; Durães, José M; Santos, Dina; Rosa, Nuno G; Silva, GilEndometriosis is highly prevalent in women at the reproductive age, ranging from 6-10%, though ureteral involvement is rare, only seen in 0.1% of the cases, but may lead to urinary tract obstruction with potential renal function loss. As endometriosis has a non-specific symptomology, diagnosis may be elusive, though it must be considered in order to prevent irreversible kidney injury and resultant morbidity. Here we present a 29-year-old Caucasian female with a past medical history of infertility and dysmenorrhoea who presented with a stage II acute kidney injury. On initial diagnostic approach, moderate bilateral ureterohydronephrosis was detected by ultrasound, assuming extrinsic compression, but no specific cause was recognizable. In order to diagnose the obstruction’s aetiology, Magnetic Resonance Imaging was used. This did not reveal an objective cause. Given the circumstances, workup ended in an explorative laparotomy, revealing a “frozen pelvis” due to endometriosis, confirmed histologically. Due to difficult ressecability, a conservative approach was taken through hormonal reatment and bilateral ureteral pigtail placement, given the necessity to salvage renal function and decrease disease burden.
- Renal Disease in Madera Islands: twenty years experience of native kidney biopsiesPublication . Vieira, Pedro; Goncalves, Miguel; Durães, José; Rosa, Nuno; Resende, Luís; Silva, Gil; Teixeira, Alves; Rodrigues, Fernando; Galvão, Maria; Brinca, Ana; Viana, Helena; Carvalho, Fernanda; Araújo, JoséRenal biopsy plays an essential role either in the diagnosis or in the prognosis of patients with renal disease. In order to assess its epidemiology and evolution in Madeira Islands, we analysed twenty-seven years of native kidney biopsies. Methods: We performed a retrospective analysis of clinical records, including histological revision from 1986 to 2012, totalling 315 native kidney biopsies. They were assessed regarding the temporal evolution both for the quality/indications for renal biopsy and for the patterns of kidney disease. Results: A total of 315 native kidney biopsies were analysed. The patients’ mean age was of 40.8 ± 18.4 years and 50.5%(n = 159) were males. The most common indications for renal biopsy were nephrotic syndrome (36.2%, n = 114) and acute kidney injury (20.0%, n = 63). Among primary glomerular diseases (41.5%, n = 115) the most common were IgA nephropathy (26.1%, n = 30) and focal-segmental glomerulosclerosis (17.4%, n = 20) and among secondary glomerular diseases (31.4%, n = 87), lupus nephritis (51.7%, n = 45) and amyloidosis (20.7%, n = 18). Statistical analysis revealed significant correlation between gender and major pathological diagnosis (Fisher’s exact test, p <.01) and between indications for renal biopsy and major pathological diagnosis (χ2, p <.01). Regarding the temporal evolution, no statistically significant differences were found in the number of renal biopsies (χ2, p =.193), number of glomeruli per sample (Fisher’s exact test, p =.669), age (Kruskal-Wallis, p =.216), indications for renal biopsy (χ2, p =.106) or major pathological diagnosis groups (χ2, =.649). However, considering the specific clinico-pathological diagnoses and their temporal variation, a statistically significant difference (Fisher’s exact test, p <.05) was found for lupus nephritis and membranous nephropathy with an increasing incidence and for amyloidosis with an opposite tendency. Discussion: The review of the native kidney biopsies from a population with particular characteristics, geographically isolated, such as those from Madeira Islands, showed parallel between epidemiological numbers referring to other