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Cronkhite–Canada syndrome
Anita Nagy1, Levente Tóth1, János Theisz1
11 Szent Imre Egyetemi Oktatókórház, Gasztroenterológia Profil, Budapest, Tétényi út 12-16., 1115.
Insights
Cronkhite-Canada syndrome is a rare noninherited disorder causing gastrointestinal polyposis and protein-losing enteropathy. This case report details a 71-year-old male diagnosed and treated for this condition.
Area of Science:
- Gastroenterology
- Internal Medicine
- Rare Diseases
Background:
- Cronkhite-Canada syndrome (CCS) is an extremely rare, noninherited disorder.
- It is characterized by gastrointestinal polyposis, protein-losing enteropathy, and ectodermal abnormalities.
- The etiology is largely unknown, with autoimmune mechanisms suspected.
Observation:
- A case of a 71-year-old male patient presenting with symptoms suggestive of CCS.
- Diagnosis was confirmed through patient history, physical examination, endoscopic findings, and histology.
- This represents the first reported case of CCS in Hungary.
Findings:
- The patient was diagnosed with Cronkhite-Canada syndrome.
- Treatment involved a combination of proton-pump inhibitor, corticosteroids, and mesalazine.
- Nutritional therapy was also administered as part of the management plan.
Implications:
- This case highlights the importance of recognizing and diagnosing rare conditions like CCS.
- The presented treatment regimen, based on literature, offers a potential therapeutic approach.
- Further research into the etiology and optimal management of CCS is warranted.
Abstract:
Összefoglaló. A Cronkhite-Canada-szindróma egy extrém ritka, nem öröklődő, gyomor-bél rendszeri polyposissal, fehérjevesztő enteropathiával és ectodermalis elváltozásokkal járó megbetegedés. A világon eddig összesen körülbelül 500 esetet jegyeztek fel. Az etiológia pontosan nem tisztázott, hátterében elsősorban autoimmun folyamatot feltételeznek. A diagnózis a páciens kórtörténetén, a fizikális vizsgálaton, az endoszkópos képen és a szövettani leleten alapul. A jelen közleményben egy 71 éves férfi beteg esetét mutatjuk be. A klinikai kép és az elvégzett vizsgálatok alapján a tünetek hátterében Cronkhite-Canada-szindrómát igazoltunk, majd a szakirodalomban leggyakrabban alkalmazott kombinált protonpumpagátló, kortikoszteroid és meszalazin adását vezettük be, illetve táplálásterápiát alkalmaztunk. Tudomásunk szerint Cronkhite-Canada-szindrómás beteg esete Magyarországon elsőként kerül ismertetésre. Orv Hetil. 2021; 162(11): 432-438. Summary. Cronkhite-Canada syndrome is an extremely rare, noninherited disease, characterized by gastrointestinal polyposis, protein-losing enteropathy and ectodermal abnormalities. Approximately 500 cases have been reported worldwide. The aetiology is unknown, most probably autoimmune mechanisms may be involved. The diagnosis is based on patient history, physical examination, endoscopic findings and histology. Here we report the case of a 71-year-old male, diagnosed with Cronkhite-Canada syndrome. The treatment consisted of proton-pump inhibitor, corticosteroids, mesalazin and nutritional therapy. To the best of our knowledge, this is the first report of Cronkhite-Canada syndrome in Hungary. Orv Hetil. 2021; 162(11): 432-438.
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