Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome (OMIM 304790) is a rare hereditary disorder of the immune regulatory system caused by FOXP3 mutations. The clinical features of this syndrome include a wide spectrum of severe autoimmune diseases and renal involvement, mostly due to tubulointerstitial diseases, in some patients. Glomerulopathy of membranous nephropathy (MN) and minimal change nephrotic syndrome (MCNS), however, have also been reported. We encountered two children with IPEX syndrome from the same family. Interestingly, they had different glomerular lesions: one had MN and the other had MCNS. Herein we describe the cases of these siblings and review the possible mechanisms for the development of two different renal lesions.
Agnes Trautmann, Sven Schnaidt, Beata S. Lipska‐Ziętkiewicz, Monica Bodria, Fatih Özaltın, Francesco Emma, Ali Anarat, Anette Melk, Marta Azócar, Jun Oh, Bassam Saeed, Alaleh Gheisari, Salim Çalışkan, Jutta Gellermann, Lina María Serna-Higuita, Augustina Jankauskienė, Dorota Drożdż, Sevgı Mır, Ayşe Balat, Maria Szczepańska, Dušan Paripović, Aleksandra Żurowska, Radovan Bogdanović, Alev Yılmaz, Bruno Ranchin, Esra Baskın, Özlem Erdoğan, Giuseppe Remuzzi, Piotr Adamczyk, Elżbieta Kuźma-Mroczkowska, Mieczysław Litwin, Luisa Murer, Marcin Tkaczyk,
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