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Deprecated: Implicit conversion from float 219.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 J+Investig+Med+High+Impact+Case+Rep 2018 ; 6 (ä): ä Nephropedia Template TP
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Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment #MMPMID29619395
Langevin C; Chapdelaine H; Picard JM; Poitras P; Leduc R
J Investig Med High Impact Case Rep 2018[Jan]; 6 (ä): ä PMID29619395show ga
Cronkhite-Canada syndrome is a rare syndrome consisting of extensive gastrointestinal polyposis and ectodermal changes including cutaneous hyperpigmentation, alopecia, and onychodystrophy. We report the case of a 45-year-old Caucasian male patient who failed multiple treatments over 2 years including steroids, azathioprine, adalimumab, and cyclosporine. He had recurrent and prolonged hospitalizations because of diarrhea, abdominal pain, weight loss, and malnutrition. Sirolimus was initiated with a significant clinical and endoscopic benefit apparent within, respectively, 2 and 8 weeks. An ongoing remission was achieved and maintained for over 6 months after prednisone tapering. We review the current evidence on treatment of Cronkhite-Canada syndrome and suggest the incorporation of sirolimus in that algorithm.