| Abstract [eng] |
Cronkhite–Canada syndrome is a rare non-hereditary disease. It occurrs worldwide at a rate of 1 case per 1 million people per year (1–3). The etiology of the disease is still unknown, but due to the positive response to steroid therapy and, often, positive immunological markers, it is presumed to be an autoimmune disorder (1,4–20). Manifestation age of the syndrome is over 50 years old and it occurs predominantly in males (21,22). Since 1955, only more than 500 articles have been published (5,20,22–24). The main presenting symptoms are multiple gastrointestinal polyps of various morphologies and ectodermal features: alopecia, skin hyperpigmentation, and nail abnormalities (2,12,25,26). Diagnosis of the syndrome is based on clinical symptoms, laboratory, endoscopic, and histopathological results of gastrointestinal polyps. Treatment consists of glucocorticosteroids, often prescribed with immunomodulators, dietary supplements, electrolytes, microelements, fluid therapy, acid blockers and antibiotics (16). Combined therapy is administered to achieve long-term disease remission and reduce the frequency of recurrences (19). The first case of Cronkhite-Canada syndrome in Lithuania was published in 2013 (28). This paper presents the second case of this disease in Lithuania. It describes a Lithuanian male patient who has been successfully treated and consistently monitored at Vilnius University Hospital Santaros Clinics since 2014. The prognosis and overall survival of patients with Cronkhite-Canada syndrome are gradually improving worldwide but it is still crucial to deepen knowledge about the disease itself, improve diagnostics and enhance treatment effectiveness. |
