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Revista / Livro: Pediatric Nephrology ×

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  • article 2 Citação(ões) na Scopus
    International cohort of 382 children with lupus nephritis - presentation, treatment and outcome at 24 months
    (2023) MUTIIS, Chiara De; WENDERFER, Scott E.; BASU, Biswanath; BAGGA, Arvind; ORJUELA, Alvaro; SAR, Tanmoy; AGGARWAL, Amita; JAIN, Avinash; YAP, Hui-Kim; TEO, Sharon; ITO, Shuichi; OHNISHI, Ai; IWATA, Naomi; KASAPCOPUR, Ozgur; YILDIZ, Mehmet; LAURENT, Audrey; MASTRANGELO, Antonio; OGURA, Masao; SHIMA, Yuko; RIANTHAVORN, Pornpimol; SILVA, Clovis A.; TRINDADE, Vitor; GIANVITI, Alessandra; AKINORI, Miyazono; HAMADA, Riku; FUJIMURA, Junya; MINAMIKAWA, Shogo; KAMIYOSHI, Naohiro; KAITO, Hiroshi; ISHIMORI, Shingo; IANNUZZELLA, Francesco; TULLUS, Kjell
    Background Children with lupus have a higher chance of nephritis and worse kidney outcome than adult patients. Methods We retrospectively analyzed clinical presentation, treatment and 24-month kidney outcome in a cohort of 382 patients (<= 18 years old) with lupus nephritis (LN) class >= III diagnosed and treated in the last 10 years in 23 international centers. Results The mean age at onset was 11 years 9 months and 72.8% were females. Fifty-seven percent and 34% achieved complete and partial remission at 24-month follow-up, respectively. Patients with LN class III achieved complete remission more often than those with classes IV or V (mixed and pure). Only 89 of 351 patients maintained stable complete kidney remission from the 6(th) to 24(th) months of follow-up. eGFR >= 90 ml/min/1.73 m(2) at diagnosis and biopsy class III were predictive of stable kidney remission. The youngest and the oldest age quartiles (2y-9y, 5m) (14y, 2m-18y,2m) showed lower rates of stable remission (17% and 20.7%, respectively) compared to the two other age groups (29.9% and 33.7%), while there was no difference in gender. No difference in achieving stable remission was found between children who received mycophenolate or cyclophosphamide as induction treatment. Conclusion Our data show that the rate of complete remission in patients with LN is still not high enough. Severe kidney involvement at diagnosis was the most important risk factor for not achieving stable remission while different induction treatments did not impact outcome. Randomized treatment trials involving children and adolescents with LN are needed to improve outcome for these children.
  • article 3 Citação(ões) na Scopus
    Chronic kidney disease in patients with childhood-onset systemic lupus erythematosus
    (2023) SAKAMOTO, Ana P.; SILVA, Clovis A.; ISLABAO, Aline G.; V, Glaucia Novak; MOLINARI, Beatriz; NOGUEIRA, Paulo K.; PEREIRA, Rosa M. R.; SAAD-MAGALHAES, Claudia; CLEMENTE, Gleice; PIOTTO, Daniela P.; AIKAWA, Nadia E.; PITTA, Ana C.; TRINDADE, Vitor C.; APPENZELLER, Simone; CARVALHO, Luciana M.; RABELO-JUNIOR, Carlos N.; FONSECA, Adriana R.; SZTAJNBOK, Flavio R.; SANTOS, Maria C.; BICA, Blanca E.; SENA, Evaldo G.; MORAES, Ana J.; FRAGA, Melissa M.; ROBAZZI, Teresa C.; SPELLING, Paulo F.; SCHEIBEL, Iloite M.; CAVALCANTI, Andre S.; MATOS, Erica N.; GUIMARAES, Luciano J.; SANTOS, Flavia P.; MOTA, Licia M. H.; BONFA, Eloisa; TERRERI, Maria T.
    Background Lupus nephritis (LN) is a frequent manifestation of childhood-onset systemic lupus erythematosus (cSLE) with a potential risk for kidney failure and poor outcomes. This study aimed to evaluate stages III, IV, and V of chronic kidney disease (CKD) and investigate risk factors for CKD in cSLE patients. Methods We performed a nationwide observational cohort study in 27 pediatric rheumatology centers, including medical charts of 1528 cSLE patients. Data were collected at cSLE diagnosis, during follow-up, and at last visit or death, between September 2016 and May 2019. Results Of 1077 patients with LN, 59 (5.4%) presented with CKD, 36/59 (61%) needed dialysis, and 7/59 (11.8%) were submitted for kidney transplantation. After Bonferroni's correction for multiple comparisons (p < 0.0013), determinants associated with CKD were higher age at last visit, urinary biomarker abnormalities, neuropsychiatric involvement, higher scores of disease activity at last visit and damage index, and more frequent use of methylprednisolone, cyclosporine, cyclophosphamide, and rituximab. In the regression model analysis, arterial hypertension (HR = 15.42, 95% CI = 6.12-38.83, p < 0.001) and biopsy-proven proliferative nephritis (HR = 2.83, 95%CI = 1.70-4.72, p <= 0.001) increased the risk of CKD, while children using antimalarials had 71.0% lower CKD risk ((1.00-0.29) x 100%) than children not using them. The Kaplan-Meier comparison showed lower survival in cSLE patients with biopsy-proven proliferative nephritis (p = 0.02) and CKD (p <= 0.001). Conclusions A small number of patients manifested CKD; however, frequencies of dialysis and kidney transplantation were relevant. This study reveals that patients with cSLE with hypertension, proliferative nephritis, and absence of use of antimalarials exhibited higher hazard rates of progression to CKD.