Schulte, Sigrid, Kuetting, Fabian, Mertens, Jessica, Kaufmann, Thomas, Drebber, Uta, Nierhoff, Dirk, Toex, Ulrich and Steffen, Hans-Michael (2019). Case report of patient with aCronkhite-Canada syndrome: sustained remission after treatment with corticosteroids and mesalazine. BMC Gastroenterol., 19. LONDON: BMC. ISSN 1471-230X

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Abstract

BackgroundCronkhite-Canada syndrome is a rare disease of unknown etiology and the optimal treatment for this syndrome is unknown.Case presentationWe present the case of a man who at the age of 66.0years was diagnosed with Cronkhite-Canada syndrome (CCS). In addition to watery diarrhea, alopecia, and a complete loss of toenails and fingernails, the patient had been suffering from dysgeusia and rapid weight loss of more than 10.0kg within a few months. The patient had recently incurred a distal radius fracture. During the initial endoscopy an extensive polyposis of the stomach and jejunum was found. The diagnosis of CCS was made and after initiation of a steroid therapy his diarrhea improved immediately. A discontinuation of the steroid therapy was not possible and mesalazine (1000mgt.i.d.) was added to prednisolone (10.0mg/d). This therapy led to a remission within 6.0months with weight gain and normalization of serum albumin levels. The prednisolone dose was reduced to 7.5mg/d. During the following year, the steroids could be further reduced and nails had regrown again. Within three years, all polyps had disappeared and the steroid therapy was finished while the dosage of mesalazine was reduced in a stepwise fashion. Four years later, the mesalazine was stopped and more than 14.0years after the initial diagnosis the patient is still in complete remission without any treatment.ConclusionThe optimal treatment for CCS is unknown. In our case, the initial combination therapy of corticosteroids plus mesalazine followed by a mesalazine monotherapy has led to a remarkable long-lasting remission with complete resolution of all intestinal polyps.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Schulte, SigridUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kuetting, FabianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mertens, JessicaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kaufmann, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Drebber, UtaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Nierhoff, DirkUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Toex, UlrichUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Steffen, Hans-MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-156250
DOI: 10.1186/s12876-019-0944-x
Journal or Publication Title: BMC Gastroenterol.
Volume: 19
Date: 2019
Publisher: BMC
Place of Publication: LONDON
ISSN: 1471-230X
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
5-AMINOSALICYLIC ACID; COLON-CANCER; CRONKHITE; MANAGEMENT; CARCINOMA; RELEASEMultiple languages
Gastroenterology & HepatologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/15625

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