Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment
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 includin...
Main Authors: | , , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
SAGE Publishing
2018-03-01
|
Series: | Journal of Investigative Medicine High Impact Case Reports |
Online Access: | https://doi.org/10.1177/2324709618765893 |
id |
doaj-0015f47bd7f04f82ae63c7032687c10f |
---|---|
record_format |
Article |
spelling |
doaj-0015f47bd7f04f82ae63c7032687c10f2020-11-25T03:34:11ZengSAGE PublishingJournal of Investigative Medicine High Impact Case Reports2324-70962018-03-01610.1177/2324709618765893Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard TreatmentCatherine Langevin MD0Hugo Chapdelaine MD1Jean-Maxime Picard MD2Pierre Poitras MD3Raymond Leduc MD, FRCPC4Centre Hospitalier de l’Université de Montréal, Montreal, Quebec, CanadaMontreal Clinical Research Institute, Montreal, Quebec, CanadaChicoutimi Hospital, Chicoutimi, Quebec, CanadaCentre Hospitalier de l’Université de Montréal, Montreal, Quebec, CanadaCentre Hospitalier de l’Université de Montréal, Montreal, Quebec, CanadaCronkhite-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.https://doi.org/10.1177/2324709618765893 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Catherine Langevin MD Hugo Chapdelaine MD Jean-Maxime Picard MD Pierre Poitras MD Raymond Leduc MD, FRCPC |
spellingShingle |
Catherine Langevin MD Hugo Chapdelaine MD Jean-Maxime Picard MD Pierre Poitras MD Raymond Leduc MD, FRCPC Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment Journal of Investigative Medicine High Impact Case Reports |
author_facet |
Catherine Langevin MD Hugo Chapdelaine MD Jean-Maxime Picard MD Pierre Poitras MD Raymond Leduc MD, FRCPC |
author_sort |
Catherine Langevin MD |
title |
Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment |
title_short |
Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment |
title_full |
Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment |
title_fullStr |
Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment |
title_full_unstemmed |
Sirolimus in Refractory Cronkhite-Canada Syndrome and Focus on Standard Treatment |
title_sort |
sirolimus in refractory cronkhite-canada syndrome and focus on standard treatment |
publisher |
SAGE Publishing |
series |
Journal of Investigative Medicine High Impact Case Reports |
issn |
2324-7096 |
publishDate |
2018-03-01 |
description |
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. |
url |
https://doi.org/10.1177/2324709618765893 |
work_keys_str_mv |
AT catherinelangevinmd sirolimusinrefractorycronkhitecanadasyndromeandfocusonstandardtreatment AT hugochapdelainemd sirolimusinrefractorycronkhitecanadasyndromeandfocusonstandardtreatment AT jeanmaximepicardmd sirolimusinrefractorycronkhitecanadasyndromeandfocusonstandardtreatment AT pierrepoitrasmd sirolimusinrefractorycronkhitecanadasyndromeandfocusonstandardtreatment AT raymondleducmdfrcpc sirolimusinrefractorycronkhitecanadasyndromeandfocusonstandardtreatment |
_version_ |
1724560149924806656 |