Browsing by Author "Silvestre, F."
Now showing 1 - 2 of 2
Results Per Page
Sort Options
- Biópsia Renal Percutânea: experiência de oito anosPublication . Castro, R.; Sequeira, M.; Faria, M.; Belmira, A.; Sampaio, S.; Roquete, P.; Silvestre, F.; Rocha, C.; Morgado, T.A biópsia renal constitui um instrumento fundamental para o diagnóstico e prognóstico de diversas patologias nefrológicas e sistémicas. No nosso Hospital a sua realização iniciou-se em 1994, tendo sido biopsado um doente com Doença de Berger. Até à data foram efectuadas 91 biópsias renais percutâneas com a seguinte distribuição anual: 1994 (n=3), 1995 (n=3), 1996 (n=3), 1997 (n=15), 1998 (n=5), 1999 (n=23), 2000 (n=13), 2001 (n=26) em 57 homens e 34 mulheres. Foi utilizada orientação ecográfica e na maioria dos casos a agulha de Vim Silverman (14G). Apenas em cinco casos se utilizou uma pistola automática BARD. Era nosso objectivo, em cada biópsia, a colheita de pelo menos dois fragmentos, um para microscopia óptica e outro para imunoflurescência. Os grandes síndromas nefrológicos que conduziram a este exame foram: síndroma nefrótico (n=27), anormalidades urinárias assintomáticas (n=25), insuficiência renal aguda ou rápidamente progressiva (n=18), insuficiência renal crónica (n=15), hipertensão arterial (n=4) e glomerulonefrite aguda (n=2). Em 92.3% (84/91) dos casos foi possível efectuar um diagnóstico histológico por microscopia óptica. Se considerarmos, no entanto, sete casos com suspeita clínica de nefropatia IgA em que o fragmento colhido para imunoflurescência não continha glomérulos, a eficácia diminuiu para 84.6% (77/91). O número médio de glomérulos por amostra foi de 18.3 ± 14.2 [0-80]. Os diagnósticos histológico obtidos foram os seguintes: doença de Berger (n=24), diversas formas de síndroma nefrótico primário (n=18), nefrite lúpica (n=8), glomerulonefrite mesangioproliferativa, sem glomérulos na imunoflurescência (n=6), ausência de tecido renal ou de glomérulos nas amostras (n=6), síndroma nefrótico secundário (n=4), nefrite túbulo-intersticial ou necrose tubular aguda (n=4), nefropatia diabética (n=3), rim de mieloma (n=3), glomerulonefrite crescêntica sem depósitos imunes (n=3), nefroangiosclerose hipertensiva (n=2), glomerulonefrite mesangioproliferativa IgM (n=2) e outros (n=8). A hematúria macroscópica revelou-se como a complicação mais frequente (n=9; 9.9%). Apenas em três casos se verificou a existência de hematoma renal ecograficamente (3.3%). A saída de sangue pelo mandril da agulha de biópsia surgiu em quatro casos (4.4%) e foi necessário proceder à transfusão de concentrado de glóbulos rubros em três doentes (3.3%). Registamos uma punção acidental de baço. Em nenhum caso foi necessário efectuar nefrectomia por hemorragia incontrolável. Identificamos, como índices de mau prognóstico relativamente à evolução para insuficiência renal crónica avançada (n=2) ou terminal (n=15), o maior número de glomérulos esclerosados (30% vs 8%; p<0.01) e de lesões túbulo-intersticiais (100% vs 63%; p<0.01). Em conclusão, a biópsia renal efectuada com orientação ecográfica permitiu a obtenção 21 de amostras com valor diagnóstico em 84.6% dos casos. A taxa de complicações foi relativamente baixa comparando com outras séries. Verificamos um progressivo aumento de qualidade das amostras renais colhidas, em relação directa com uma coordenação técnica crescente entre os nefrologistas e radiologistas intervenientes.nephrological and systemic pathologies. At our institution the first patient submitted to this technique, at 1994, showed Berger disease. Until 2002 we have performed 91 renal biopsies (57 men and 34 women) with the following annual distribution: 1994 (n=3), 1995 (n=3), 1996 (n=3), 1997 (n=15), 1998 (n=5), 1999 (n=23), 2000 (n=13) and 2001 (n=26). Ultrasound guidance was always used and in most of cases the technique was performed with Vim-Silverman (14G) needle. BARD automatic system was employed in only five patients. The clinical diagnosis that lead to renal biopsy were: nephrotic syndrome (n=27), asyntomatic urinary abnormalities (n=25), acute or rapidly progressive renal failure (n=18), chronic renal failure (n=15), hypertension (n=4) and acute nephritis (n=2). The efficacy for optic histological diagnosis was 92.3% (84/91). However, if we include seven cases of presumed IgA nephropathy that don’t included fragment for immunofluorescence (IF) analysis the efficacy declined to 84.6% (77/91). The mean number of glomeruli per fragment was 18.3 ± 14.2 [0-80]. Histological diagnosis were the following: Berger disease (n=24), idiopathic nephrotic syndrome (n=18), lupus nephritis (n=8), mesangial proliferative glomerulonephritis without glomeruli in the IF fragment (n=6), without glomeruli (n=6), secondary nephrotic syndrome (n=4), tubulointerstitial nephritis or acute tubular necrosis (n=4), diabetic nephropathy (n=3), myeloma kidney (n=3), pauci-imune and crescentic glomerulonephritis (n=3), hypertensive nephropathy (n=2), IgM mesangial proliferative glomerulonephritis (n=2) and various (n=8). Gross hematuria appeared in 9 patients (9.9%). Only in three of these patients it was showed, by ecography, the existence of kidney haematoma. Bleeding throughout the mandrill in four cases, leaded to transfusion in only three patients. We have registered one accidental spleen puncture. Nephrectomy for incontrollable bleeding was never needed. Higher glomerulosclerosis (30% vs 8%; p<0.01) and also a greater extent of tubulointersticial lesions (100% vs 63%; p<0.01), were predictors of progression into end-stage or advanced renal failure. Concluding, renal biopsy with ultrasound guidance was valuable for diagnosis in 84.6% of our proceedings. Our serie is similar to others concerning serious complications. Nephrologists and radiologists improved progressively their coordination performing this technique, improving the results during this period of 8 years.
- Familial ATTR amyloidosis: microalbuminuria as a predictor of symptomatic disease and clinical nephropathyPublication . Lobato, L.; Beirao, I.; Silva, M.; Bravo, F.; Silvestre, F.; Guimaraes, S.; Sousa, A.; Noel, LH.; Sequeiros, J.Familial ATTR amyloidosis: microalbuminuria as a predictor of symptomatic disease and clinical nephropathy. Lobato L, Beirão I, Silva M, Bravo F, Silvestre F, Guimarães S, Sousa A, Noël LH, Sequeiros J. SourceDepartment of Nephrology and Centro de Estudos de Paramiloidose, Hospital Geral de Santo António and Institute for Molecular and Cell Biology, Porto, Portugal. llobato@netcabo.pt Abstract BACKGROUND: Portuguese type familial amyloid polyneuropathy (FAP) is a neuropathic amyloidosis caused by a mutant transthyretin (TTR). Varying degrees of renal involvement have been reported. Our aim was to assess the value of microalbuminuria (MA) for predicting clinical neurological disease and overt nephropathy in TTR-related amyloidosis. METHODS: All subjects had the TTR Val30Met mutation, and were recruited between 1993 and 1999. We have prospectively evaluated 22 asymptomatic gene carriers (7 male, 15 female; mean age 41.6+/-9.6 years) and 32 patients with neuropathy (14 male, 18 female; 36.8+/-8.8 years, on average, 33.0+/-9.3 years at the onset of neuropathy). We measured urinary albumin excretion every year, if asymptomatic, or every 6 months if already affected. Kidney biopsies were performed in patients with normal urinary albumin excretion, MA, and overt nephropathy, respectively. RESULTS: In asymptomatic carriers, persistent MA was detected in eight (36%) subjects. The presence of MA in asymptomatic gene carriers, compared with those having normal urinary albumin excretion, conferred a 4.8-fold risk of developing neuropathy, usually within the subsequent 3 years. Once neurological signs appeared, nephropathy, manifested as MA, progressed to overt nephropathy in one-half of subjects. In patients with neuropathy, 24 (75%) had MA during follow-up: evolution towards clinical renal disease occurred in 14 (58%) and renal failure occurred in five (21%), always after a course of MA. Proteinuria or renal failure without prior persistent MA were never observed in the present patient cohort. Histopathological evaluation did not reveal glomerular lesions other than amyloid deposits to explain abnormal urinary albumin excretion. The amount of mesangial and vascular-pole amyloid deposits was correlated with the degree of albuminuria. CONCLUSIONS: Microalbuminuria represents the first stage of clinical TTR amyloid nephropathy and is premonitory of neuropathy. Its presence identifies a subgroup of patients who are more prone to develop overt nephropathy. Screening of MA may be important to assess disease onset and to recommend liver transplantation in individuals at risk.