BACKGROUND Cronkhite–Canada syndrome(CCS)is a rare sporadic polyposis syndrome that presents with gastrointestinal and ectodermal symptoms in addition to nutritional deficiencies.CCS combined with hypothyroidism is a...BACKGROUND Cronkhite–Canada syndrome(CCS)is a rare sporadic polyposis syndrome that presents with gastrointestinal and ectodermal symptoms in addition to nutritional deficiencies.CCS combined with hypothyroidism is an even rarer condition,with no standard treatment guidelines.CASE SUMMARY The present study described 2 patients with CCS:A 67-year-old woman with concomitant hypothyroidism and 68-year-old man treated with endoscopic mucosal resection(EMR).Both patients had multiple gastrointestinal symptoms and ectodermal changes,along with multiple gastrointestinal polyps.Microscopic examination showed that the mucosa in both patients was hyperemic and edematous,with pathologic examination showing distorted,atrophic,and dilated glands.Patient 1 had concomitant hypothyroidism and was treated with levothyroxine.Due to her self-reduction of hormone dose,her disease relapsed.Patient 2 underwent EMR,but refused further hormonal or biological treatments.Subsequently,he was treated with an oral Chinese medical preparation.CONCLUSION Pharmacotherapy can induce and maintain remission in CCS patients,with adjuvant EMR,long-term follow-up,and endoscopic surveillance being necessary.Case 1:Based on the aforementioned findings,Patient 1 was diagnosed with CCS and hypothyroidism.Case 2:Based on the aforementioned findings,Patient 2 was diagnosed with CCS.展开更多
Cronkhite-Canada syndrome(CCS)is a rare nonfamilial polyposis syndrome characterized by epithelial disturbances in the gastrointestinal tract and skin.The aim of this study was to investigate the clinical features and...Cronkhite-Canada syndrome(CCS)is a rare nonfamilial polyposis syndrome characterized by epithelial disturbances in the gastrointestinal tract and skin.The aim of this study was to investigate the clinical features and potential therapies for CCS.Six patients with CCS admitted from December 1992 to July 2008 to Peking Union Medical College Hospital were evaluated.All patients had clinical manifestation of nonhereditary gastrointestinal polyposis with diarrhea,skin hyperpigmentation,alopecia,and nail dystrophy.Fecal occult blood was positive in all six cases.Serum hemoglobin,potassium,calcium and protein were below the normal range in two cases.Anti-Saccharomyces cerevisiae and antinuclear antibodies were present in three cases.Multiple polyps were found in all patients by gastroscopy and colonoscopy,with only one in the esophagus.Histologically,there were hyperplastic polyps in five cases,tubular adenoma in three,and juvenile polyp in one with chronic inflammation and mucosal edema.Comprehensive treatment led by corticosteroids can result in partial remission of clinical symptoms,and longterm follow-up is necessary.展开更多
We report a case of Cronkhite-Canada syndrome(CCS)associated with myelodysplastic syndrome(MDS).A 54-year-old woman,diagnosed as MDS the prior year after evaluation of anemia,visited our hospital with the chief compla...We report a case of Cronkhite-Canada syndrome(CCS)associated with myelodysplastic syndrome(MDS).A 54-year-old woman,diagnosed as MDS the prior year after evaluation of anemia,visited our hospital with the chief complaint of epigastric discomfort.She also had dysgeusia,alopecia,atrophic nail change,and pigmentation of the palm,all of which began several months ago.Blood tests revealed severe hypoalbuminemia.Colonoscopy(CS)showed numerous,dense,red polyps throughout the colon and rectum.Biopsy specimens showed stromal edema,infi ltration of lymphocytes,and cystic dilatation of the crypt.Her clinical manifestations and histology were consistent with CCS.We prescribed corticosteroids,which dramatically improved her physical findings,laboratory data,and endoscopic fi ndings.This is the first report of CCS in a patient with MDS.展开更多
Cronkhite-Canada syndrome (CCS) is a rare, non-inherited polyposis syndrome, characterized by diffuse gastrointestinal (GI) hamartomatous polyposis with unique dermatologic changes including alopecia, skin hyperpigmen...Cronkhite-Canada syndrome (CCS) is a rare, non-inherited polyposis syndrome, characterized by diffuse gastrointestinal (GI) hamartomatous polyposis with unique dermatologic changes including alopecia, skin hyperpigmentation, and nail dystrophy. Patients can typically present with diarrhea, weight loss, protein-losing enteropathy, and nutritional deficiency. However, it can demonstrate diverse other clinical features, usually with poor prognosis. Currently, there are no specific diagnostic criteria established yet. The etiology of CCS is still obscure, but an autoimmune process has been suggested. Here we present an 81-year-old Caucasian female who had clinical presentations, physical exam, imaging, endoscopy and pathology findings that were all consistent with the diagnosis of CCS. We also include a detailed literature review of the other gastrointestinal polyposis syndromes (hamartomatous, adenomatous, hyperplastic and inflammatory polyposis). A high index of suspicion and recognition of the characteristic clinical, endoscopic as well as histopathological findings of CCS, as well as different gastrointestinal polyposis conditions, can help clinicians with more timely and correct diagnosis.展开更多
基金Jilin Provincial Science and Technology Department Project,No.20200201343JCScience and Technology Development Program of Jilin Province,No.20210402013GH.
文摘BACKGROUND Cronkhite–Canada syndrome(CCS)is a rare sporadic polyposis syndrome that presents with gastrointestinal and ectodermal symptoms in addition to nutritional deficiencies.CCS combined with hypothyroidism is an even rarer condition,with no standard treatment guidelines.CASE SUMMARY The present study described 2 patients with CCS:A 67-year-old woman with concomitant hypothyroidism and 68-year-old man treated with endoscopic mucosal resection(EMR).Both patients had multiple gastrointestinal symptoms and ectodermal changes,along with multiple gastrointestinal polyps.Microscopic examination showed that the mucosa in both patients was hyperemic and edematous,with pathologic examination showing distorted,atrophic,and dilated glands.Patient 1 had concomitant hypothyroidism and was treated with levothyroxine.Due to her self-reduction of hormone dose,her disease relapsed.Patient 2 underwent EMR,but refused further hormonal or biological treatments.Subsequently,he was treated with an oral Chinese medical preparation.CONCLUSION Pharmacotherapy can induce and maintain remission in CCS patients,with adjuvant EMR,long-term follow-up,and endoscopic surveillance being necessary.Case 1:Based on the aforementioned findings,Patient 1 was diagnosed with CCS and hypothyroidism.Case 2:Based on the aforementioned findings,Patient 2 was diagnosed with CCS.
文摘Cronkhite-Canada syndrome(CCS)is a rare nonfamilial polyposis syndrome characterized by epithelial disturbances in the gastrointestinal tract and skin.The aim of this study was to investigate the clinical features and potential therapies for CCS.Six patients with CCS admitted from December 1992 to July 2008 to Peking Union Medical College Hospital were evaluated.All patients had clinical manifestation of nonhereditary gastrointestinal polyposis with diarrhea,skin hyperpigmentation,alopecia,and nail dystrophy.Fecal occult blood was positive in all six cases.Serum hemoglobin,potassium,calcium and protein were below the normal range in two cases.Anti-Saccharomyces cerevisiae and antinuclear antibodies were present in three cases.Multiple polyps were found in all patients by gastroscopy and colonoscopy,with only one in the esophagus.Histologically,there were hyperplastic polyps in five cases,tubular adenoma in three,and juvenile polyp in one with chronic inflammation and mucosal edema.Comprehensive treatment led by corticosteroids can result in partial remission of clinical symptoms,and longterm follow-up is necessary.
文摘We report a case of Cronkhite-Canada syndrome(CCS)associated with myelodysplastic syndrome(MDS).A 54-year-old woman,diagnosed as MDS the prior year after evaluation of anemia,visited our hospital with the chief complaint of epigastric discomfort.She also had dysgeusia,alopecia,atrophic nail change,and pigmentation of the palm,all of which began several months ago.Blood tests revealed severe hypoalbuminemia.Colonoscopy(CS)showed numerous,dense,red polyps throughout the colon and rectum.Biopsy specimens showed stromal edema,infi ltration of lymphocytes,and cystic dilatation of the crypt.Her clinical manifestations and histology were consistent with CCS.We prescribed corticosteroids,which dramatically improved her physical findings,laboratory data,and endoscopic fi ndings.This is the first report of CCS in a patient with MDS.
文摘Cronkhite-Canada syndrome (CCS) is a rare, non-inherited polyposis syndrome, characterized by diffuse gastrointestinal (GI) hamartomatous polyposis with unique dermatologic changes including alopecia, skin hyperpigmentation, and nail dystrophy. Patients can typically present with diarrhea, weight loss, protein-losing enteropathy, and nutritional deficiency. However, it can demonstrate diverse other clinical features, usually with poor prognosis. Currently, there are no specific diagnostic criteria established yet. The etiology of CCS is still obscure, but an autoimmune process has been suggested. Here we present an 81-year-old Caucasian female who had clinical presentations, physical exam, imaging, endoscopy and pathology findings that were all consistent with the diagnosis of CCS. We also include a detailed literature review of the other gastrointestinal polyposis syndromes (hamartomatous, adenomatous, hyperplastic and inflammatory polyposis). A high index of suspicion and recognition of the characteristic clinical, endoscopic as well as histopathological findings of CCS, as well as different gastrointestinal polyposis conditions, can help clinicians with more timely and correct diagnosis.
