E-viri
Recenzirano
Odprti dostop
-
Malakasioti, Georgia; Iancu, Daniela; Milovanova, Anastasiia; Tsygin, Alexey; Horinouchi, Tomoko; Nagano, China; Nozu, Kandai; Kamei, Koichi; Fujinaga, Shuichiro; Iijima, Kazumoto; Kang, Hee Gyung; Sinha, Rajiv; Basu, Biswanath; Morello, William; Montini, Giovanni; Waters, Aoife; Boyer, Olivia; Yıldırım, Zeynep Yürük; Yel, Sibel; Dursun, İsmail; McCarthy, Hugh J.; Vivarelli, Marina; Prikhodina, Larisa; Besouw, Martine T.P.; Chan, Eugene Yu-hin; Huang, Wenyan; Kemper, Markus J.; Loos, Sebastian; Prestidge, Chanel; Wong, William; Zlatanova, Galia; Ehren, Rasmus; Weber, Lutz T.; Chehade, Hassib; Hooman, Nakysa; Tkaczyk, Marcin; Stańczyk, Małgorzata; Miligkos, Michael; Tullus, Kjell
Kidney international, 20/May , Letnik: 103, Številka: 5Journal Article
While 44-83% of children with steroid-resistant nephrotic syndrome (SRNS) without a proven genetic cause respond to treatment with a calcineurin inhibitor (CNI), current guidelines recommend against the use of immunosuppression in monogenic SRNS. This is despite existing evidence suggesting that remission with CNI treatment is possible and can improve prognosis in some cases of monogenic SRNS. Herein, our retrospective study assessed response frequency, predictors of response and kidney function outcomes among children with monogenic SRNS treated with a CNI for at least three months. Data from 203 cases (age 0-18 years) were collected from 37 pediatric nephrology centers. Variant pathogenicity was reviewed by a geneticist, and 122 patients with a pathogenic and 19 with a possible pathogenic genotype were included in the analysis. After six months of treatment and at last visit, 27.6% and 22.5% of all patients respectively, demonstrated partial or full response. Achievement of at least partial response at six months of treatment conferred a significant reduction in kidney failure risk at last follow-up compared to no response (hazard ratio 95% confidence interval 0.25, 0.10-0.62). Moreover, risk of kidney failure was significantly lower when only those with a follow-up longer than two years were considered (hazard ratio 0.35, 0.14-0.91). Higher serum albumin level at CNI initiation was the only factor related to increased likelihood of significant remission at six months (odds ratio 95% confidence interval 1.16, 1.08-1.24). Thus, our findings justify a treatment trial with a CNI also in children with monogenic SRNS. Display omitted
Avtor
![loading ... loading ...](themes/default/img/ajax-loading.gif)
Vnos na polico
Trajna povezava
- URL:
Faktor vpliva
Dostop do baze podatkov JCR je dovoljen samo uporabnikom iz Slovenije. Vaš trenutni IP-naslov ni na seznamu dovoljenih za dostop, zato je potrebna avtentikacija z ustreznim računom AAI.
Leto | Faktor vpliva | Izdaja | Kategorija | Razvrstitev | ||||
---|---|---|---|---|---|---|---|---|
JCR | SNIP | JCR | SNIP | JCR | SNIP | JCR | SNIP |
Baze podatkov, v katerih je revija indeksirana
Ime baze podatkov | Področje | Leto |
---|
Povezave do osebnih bibliografij avtorjev | Povezave do podatkov o raziskovalcih v sistemu SICRIS |
---|
Vir: Osebne bibliografije
in: SICRIS
To gradivo vam je dostopno v celotnem besedilu. Če kljub temu želite naročiti gradivo, kliknite gumb Nadaljuj.