Browsing by Author "Lobato, L."
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- Comparative study of the degree of patient satisfaction in intermittent catheterization with Lofric and polyvinyl chloride cathetersPublication . Pereira, P.; Urrutia, M.; Lobato, L.; Rivas, S.; Monereo, E.Actas Urol Esp. 2001 Nov-Dec;25(10):725-30. [Comparative study of the degree of patient satisfaction in intermittent catheterization with Lofric and polyvinyl chloride catheters]. [Article in Spanish] López Pereira P, Martínez Urrutia MJ, Lobato L, Rivas S, Jaureguizar Monereo E. SourceUnidad de Urología Infantil, Hospital Universitario La Paz, Madrid. Abstract PURPOSE: To assess the grade of satisfaction in children on intermittent catheterization with the use of LoFric and PVC conventional catheters. MATERIAL AND METHODS: A total of 40 p with experience in CIC were included in this study. An anonymous questionnaire was sent to all patients after 2-months using the LoFric catheter. Patients were divided in 3 groups (bladder augmentation, artificial sphincter, Mitrofanoff) because of major differences in CIC discomfort between these groups. RESULTS: The questionnaire was completed by 87.5% of the patients (35 p). In 86% (30 p) LoFric catheter training was easy or very easy but in 14% (5 p) it was difficult. Four patients had some difficulty during conventional catheter insertion, in 3 (75%) the difficulty disappeared with the use of LoFric catheter. Of the 51% (18 p) who reported some discomfort during the insertion of conventional catheter, 72% said it was eliminated when the LoFric catheter was used. Of 6 p with some discomfort when removing the conventional catheter, 5 (83%) said it disappeared with the new catheter. Th LoFric catheter was favored by 70% of patients because it reduced the discomfort caused by conventional catheters, bladder insertion was easier and smoother, and gel lubrication was not needed. The 17% of patients reported some difficulty dealing with this slippery catheter. CONCLUSIONS: The use of the LoFric catheter could be justified in patients who report with conventional catheters have some discomfort. It can also be recommended in patients with artificial sphincter, bladder augmentation and Mitrofanoff procedure, in whom any complication related to CIC would have serious consequences.
- End-Stage Renal Disease in Familial Amyloidosis ATTR Val30Met: APublication . Lobato, L.; Ventura, A.; Beirão, I.; Miranda, H.P.; Seca, R.; Henriques, A.C.; Teixeira, M.; Sarmento, A.M.; Pereira, M.C.Transplant Proc. 2003 May;35(3):1116-20. End-stage renal disease in familial amyloidosis ATTR Val30Met: a definitive indication to combined liver-kidney transplantation. Lobato L, Ventura A, Beirão I, Miranda HP, Seca R, Henriques AC, Teixeira M, Sarmento AM, Pereira MC. Department of Nephrology, and Liver Transplantation Program, Hospital Geral de Santo António, Largo Professor Abel Salazar, 4050, Porto, Portugal. llobato@nctcabo.pt PMID: 12947881 [PubMed - indexed for MEDLINE]
- Familial amyloid polyneuropathy type I (Portuguese): distribution and characterization of renal amyloid deposits.Publication . Lobato, L.; Beirao, I.; Guimaraes, S.; Droz, D.; Guimaraes, S.; Grunfeld, J.; Noel, L.
- 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.
- Homozygosity for the E526V Mutation in Fibrinogen A Alpha-Chain Amyloidosis: The First ReportPublication . Tavares, I.; Lobato, L.; Matos, C.; Santos, J.; Moreira, P.; Saraiva, M.; Castro Henriques, A.Systemic hereditary amyloidoses are autosomal dominant diseases associated with mutations in genes encoding ten different proteins. The clinical phenotype has implications on therapeutic approach, but it is commonly variable and largely dependent on the type of mutation. Except for rare cases involving gelsolin or transthyretin, patients are heterozygous for the amyloidogenic variants. Here we describe the first patient identified worldwide as homozygous for a nephropathic amyloidosis, involving the fibrinogen variant associated with the fibrinogen alpha-chain E526V (p.Glu545Val) mutation. In 1989, a 44-year-old woman presented with hypertension, hepatosplenomegaly, nephrotic syndrome, and renal failure. She started hemodialysis in 1990 and 6 years later underwent isolated kidney transplantation from a deceased donor. Graft function and clinical status were unremarkable for 16 years, despite progressively increased left ventricular mass on echocardiography. In 2012, 4 months before death, she deteriorated rapidly with severe heart failure, precipitated by Clostridium difficile colitis and urosepsis. Affected family members developed nephropathy, on average, nearly three decades later, which may be explained by the gene dosage effects on the phenotype of E526V (p.Glu545Val) fibrinogen A alpha-chain amyloidosis.
- 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
- Neutrophil gelatinase-associated lipocalin in kidney transplantation is an early marker of graft dysfunction and is associated with one-year renal functionPublication . Fonseca, Isabel; Carlos Oliveira, José; Almeida, M.; Cruz, M.; Malho, A.; Martins, La Salete; Dias, L.; Pedroso, S.; Santos, J.; Lobato, L.; Castro-Henriques, A.; Mendonça, D.Urinary neutrophil gelatinase-associated lipocalin (uNGAL) has been suggested as potential early marker of delayed graft function (DGF) following kidney transplantation (KTx). We conducted a prospective study in 40 consecutive KTx recipients to evaluate serial changes of uNGAL within the first week after KTx and assess its performance in predicting DGF (dialysis requirement during initial posttransplant week) and graft function throughout first year. Urine samples were collected on post-KTx days 0, 1, 2, 4, and 7. Linear mixed and multivariable regression models, receiver-operating characteristic (ROC), and areas under ROC curves were used. At all-time points, mean uNGAL levels were significantly higher in patients developing DGF (n = 18). Shortly after KTx (3-6 h), uNGAL values were higher in DGF recipients (on average +242 ng/mL, considering mean dialysis time of 4.1 years) and rose further in following days, contrasting with prompt function recipients. Day-1 uNGAL levels accurately predicted DGF (AUC-ROC = 0.93), with a performance higher than serum creatinine (AUC-ROC = 0.76), and similar to cystatin C (AUC-ROC = 0.95). Multivariable analyses revealed that uNGAL levels at days 4 and 7 were strongly associated with one-year serum creatinine. Urinary NGAL is an early marker of graft injury and is independently associated with dialysis requirement within one week after KTx and one-year graft function.
- Portuguese‐type amyloidosis (transthyretin amyloidosis, ATTR V30M).Publication . Lobato, L.J Nephrol. 2003 May-Jun;16(3):438-42. Portuguese-type amyloidosis (transthyretin amyloidosis, ATTR V30M). Lobato L. SourceDepartment of Nephrology, Santo António General Hospital, Porto, Portugal. llobato@netcabo.pt Abstract Portuguese-type amyloidosis (transthyretin amyloidosis, ATTR V30M) is the most common form of systemic hereditary amyloidosis, inherited in autosomal dominant mode. The disease, also called familial amyloid polyneuropathy type I (FAP-I), is caused by a mutant transthyretin (TTR) protein, which is synthesized by the liver. A single amino acid substitution of methionine for valine at position 30 of the TTR molecule (TTR V30M) was found in Portuguese patients. The clinical disease usually manifests as a peripheral sensory, motor and autonomic neuropathy starting in the 3rd or 4th decade of life. Renal manifestations of ATTR V30M, like other amyloidoses, are different levels of proteinuria and renal insufficiency. In ATTR V30M a large amyloid deposition in the medullary zone of the kidney and tubules is characteristic. A more extensive glomerular and vascular involvement is present only in patients with renal manifestations. A prospective survey in the north of Portugal showed that a stage of microalbuminuria (MA) could precede nephropathy and neurological disease. Nephropathy in FAP-I is present in one-third of affected patients and tends to aggregate in families. The progression towards end-stage renal disease (ESRD) affects 10% of the patients, and the survival after initiation of dialysis is a mean of 21 months. Patients who progress to ESRD have a late onset of neuropathy and lower prevalence of clinical disease in their families. Liver transplantation is a widely accepted treatment for FAP-I, and combined liver-kidney transplantation is also an option for selected patients with FAP-I and ESRD.
- Renal amyloidosis: classification of 102 consecutive casesPublication . Tavares, I.; Vaz, R.; Moreira, L.; Pereira, P.; Sampaio, S.; Vizcaíno, J.; Costa, P.P.; Lobato, L.Amyloidoses are a group of heterogeneous diseases classified according to the nature of their causative amyloid proteins. Commonly, paraffin-embedded tissue is used for the typing of amyloid by immunohistochemistry. DNA analysis should always be considered if hereditary amyloidosis is suspected. Since the kidneys are one of the organs that are most commonly involved in amyloid deposition in systemic amyloidoses, we screened 102 consecutive cases with biopsy-proven amyloid disease by immunohistochemistry. DNA analysis was performed to confirm a diagnosis of hereditary amyloidosis. Demographic characteristics, underlying disease and clinical data at the time of renal biopsy were obtained by retrospective review of medical records. The amyloidosis type according to immunohistochemical amyloid protein identification was AA in 60 (58.8%) patients, AL in 21 (20.6%), AFib in four (3.9%), ATTR in two (2.0%), AApoAI in one (1.0%), ALys in one (1.0%) and combined AL and AA in one (1.0%). The type of protein could not be classified in 12 (11.7%) patients: eight (7.8%) because of negative immunohistochemistry and four (3.9%) due to the lack of adequate tissue. DNA analysis confirmed AFib and ATTR cases by the identification of the point mutations FGA p.Glu545Val and TTR p.Met51Val, respectively. Mean age at diagnosis was 53.3 years (49.4 for AA, 63.0 for AL and 53.9 for AFib). Chronic infections were the most frequent disorder associated with AA amyloidosis, mainly tuberculosis, and only one patient had familial AA associated with Muckle-Wells syndrome. Nephrotic syndrome was the most frequent clinical manifestation, independently of the amyloid type. In our series, AA amyloidosis is still the most frequent type of systemic amyloidoses. Six patients had unequivocal hereditary amyloidosis. Immunohistochemistry did not establish the precursor protein in almost 8% of patients; however, an improvement could be obtained using a wider panel of amyloid antibodies.
- Urinary Biomarkers for Kidney Disease in ATTR AmyloidosisPublication . Rocha, A.; Bravo, F.; Beirão, I.; Vizcaíno, J.; Oliveira, J.; Lobato, L.Aim: The detection and prognosis of nephropathy in transthyretin amyloidosis depends on albuminuria and renal function. Knowing that urinary levels of alpha-1 microglobulin and beta-2 microglobulin reflect tubular dysfunction while urinary alpha-2 macroglobulin implies glomerular damage, we decide investigate the diagnostic value of these markers in the patients with transthyretin amyloidosis. Methods: Serum and urinary samples collected from 30 patients and 11 asymptomatic carriers were tested for alpha-1 microglobulin, beta-2 microglobulin, alpha-2 macroglobulin, albumin, creatinine and cystatin C. Results: Pathological urinary alpha-1 microglobulin was detected in 17 patients, beta-2 microglobulin in 6 and alpha-2 macroglobulin in 5; 5 patients had albuminuria (mg/g creatinine) 30-300 and in 20 patients values >300 were present. Asymptomatic carriers did not present pathological excretion of these biomarkers and albuminuria was >30 in 1 individual. The excretion rates of alpha-1 microglobulin and beta-2 microglobulin were positively correlated with albuminuria (P<0.001), serum creatinine (P<0.05) and cystatin C (P<0.001). Urinary alpha-2 macroglobulin was almost exclusively found in the presence of albuminuria, although their levels do not correlate. Conclusion: Urinary biomarkers emerge as a potential approach to detect renal disease but unexpectedly, urinary alpha-2 macroglobulin was not a marker of the severity of albuminuria.