[CIS PIDD] [cis-pidd] kidney disease in WAS

[CIS-PIDD]

Dear all,

I am caring for a 21 year old WAS patient (R86C mutation) who had a very mild course of disease until age 19 when he developed renal disease. Probably IgA-nephritis but biopsy has not been performed, because nephrology said it wouldn't change their therapeutic approach and they were afraid of the bleeding risk. He was put on Ramipril and did quite well for about a year but his renal failure is now progressing to stage IV with a GFR of only 28ml/min and a creatinine of 2.9mg/dl. Nephrology have now suggested to begin preparing him for a renal allograft.



I would like to explore whether there was any more unpublished experience in the community about:



-          kidney transplantation in WAS



-          prognosis of autoimmune nephritis after HSCT in WAS



-          whether a kidney biopsy would help to evaluate the degree of organ damage or help to predict the effect of either immunosuppressive therapy or HSCT



Looking forward to hear your thoughts.

Best,

Michael





PD Dr. med. Michael Albert

Attending physician / Head Pediatric SCT Pediatrics / Pediatric Hematology/Oncology



Dr. von Hauner Children's Hospital

Klinikum der Universität München

Lindwurmstr. 4, 80337 München

Germany



Tel.: +49 89 4400-52811 (Reception)

Tel.: +49 89 4400-52785 (Office)

Fax: +49 89 4400-57171

E-Mail: michael.albert at med.uni-muenchen.de<mailto:michael.albert at med.uni-muenchen.de<mailto:michael.albert at med.uni-muenchen.de%3cmailto:michael.albert at med.uni-muenchen.de>>





www.klinikum.uni-muenchen.de<http://www.klinikum.uni-muenchen.de/<http://www.klinikum.uni-muenchen.de%3chttp:/www.klinikum.uni-muenchen.de/>>


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