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Nakamura AR, Yamamoto S, Chigusa Y, Mandai M, Mogami H. Infliximab treatment for Cronkhite-Canada syndrome in pregnancy: A case report. Case Rep Womens Health 2025; 46:e00706. [PMID: 40236588 PMCID: PMC11999527 DOI: 10.1016/j.crwh.2025.e00706] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/14/2025] [Revised: 03/25/2025] [Accepted: 03/26/2025] [Indexed: 04/17/2025] Open
Abstract
Cronkhite-Canada syndrome (CCS) is a rare nonhereditary disorder characterized by gastrointestinal polyps and protein-losing enteropathy. While an increasing number of CCS cases have been reported worldwide, no documented cases involving pregnant patients could be found. Consequently, optimal management strategies for CCS during the preconception period and pregnancy remain unclear., The present report concerns the case of a 36-year-old woman with steroid-refractory CCS stabilized with gastrointestinal surgeries and infliximab, an anti-tumor necrosis factor-α (TNF-α) agent, who became pregnant. Infliximab was continued throughout pregnancy and postpartum. Despite persistent hypoalbuminemia, sh delivered a healthy infant weighing 2518 g vaginally at 38 weeks and 2 days without CCS exacerbation. Both the patient and her infant experienced an uneventful postpartum course. This case suggests that maintaining disease control with anti-TNF-α therapy in pregnant patients with CCS may contribute to optimizing maternal and neonatal outcomes.
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Affiliation(s)
- Ayano Rosemary Nakamura
- Department of Gynecology and Obstetrics, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan
| | - Shuji Yamamoto
- Department of Gastroenterology and Hepatology, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan
| | - Yoshitsugu Chigusa
- Department of Gynecology and Obstetrics, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan
| | - Masaki Mandai
- Department of Gynecology and Obstetrics, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan
| | - Haruta Mogami
- Department of Gynecology and Obstetrics, Graduate School of Medicine, Kyoto University, 54 Shogoin Kawahara-cho, Sakyo-ku, Kyoto 606-8507, Japan
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He ML, Zheng Y, Tian SX. Cronkhite-Canada syndrome complicated with pulmonary embolism: A case report. World J Clin Cases 2024; 12:4820-4826. [PMID: 39070830 PMCID: PMC11235495 DOI: 10.12998/wjcc.v12.i21.4820] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/17/2024] [Revised: 05/23/2024] [Accepted: 06/11/2024] [Indexed: 06/30/2024] Open
Abstract
BACKGROUND Cronkhite-Canada syndrome (CCS) is a rare disease, that causes gastrointestinal polyps, ectodermal abnormalities, and gastrointestinal symptoms. CCS is prone to thromboembolism, but clinical workers have not yet established a clinical consciousness of preventing thromboembolism. The present case illustrates pulmonary embolism (PE) complicated by CCS. CASE SUMMARY A 46-year-old male patient presented with mucus, purulent, and bloody stool. Ectodermal changes included skin pigmentation, alopecia, and nail dystrophy. Colonoscopy revealed the presence of multiple polyps. After a comprehensive evaluation, the patient was diagnosed with CCS. During the disease, he was also diagnosed with pulmonary embolism, Riehl's melanosis, and intestinal flora imbalance. After symptomatic treatment with omeprazole, mesalazine, rivaroxaban, nutritional support, and regulation of intestinal flora, the patient's symptoms were significantly relieved. CONCLUSION CCS complicated with PE was reported for the first time in China in this study. Despite the fact that CCS is extremely rare, patients with CCS should be classified as a high-risk venous thromboembolism (VTE) population, and emphasis should be placed on venous thromboembolism risk assessment and stratification, deep venous thromboembolism screening, prevention of VTE, and careful long-term follow-up.
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Affiliation(s)
- Mao-Lang He
- College of Medicine, Shihezi University, Shihezi 832099, Xinjiang Uygur Autonomous Region, China
| | - Yong Zheng
- Department of Gastroenterology, The First Affiliated Hospital of Shihezi University, Shihezi 832008, Xinjiang Uygur Autonomous Region, China
| | - Shu-Xin Tian
- Department of Gastroenterology, The First Affiliated Hospital of Shihezi University, Shihezi 832008, Xinjiang Uygur Autonomous Region, China
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Kim JH, Oh EH, Han DS. Polyposis of gastrointestinal tract after COVID-19 mRNA vaccination: a report of two cases. Clin Endosc 2024; 57:402-406. [PMID: 38605688 PMCID: PMC11133996 DOI: 10.5946/ce.2023.268] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/19/2023] [Revised: 12/05/2023] [Accepted: 12/06/2023] [Indexed: 04/13/2024] Open
Abstract
Cronkhite-Canada syndrome is a rare gastrointestinal polyposis syndrome with distinctive clinical features and endoscopic findings. Diagnosis can be challenging without suspicion, and the disease carries high mortality due to complications such as infection, gastrointestinal bleeding, and malignancies. This paper presents two cases of Cronkhite-Canada syndrome occurring after coronavirus disease 2019 (COVID-19) mRNA vaccination. Both cases exhibited typical clinical findings, including hypogeusia, onychodystrophy, alopecia, and weight loss. Typical polyposis in the gastrointestinal tract was confirmed through endoscopies. As symptomatic treatment did not improve the symptoms, corticosteroids were administered, and symptoms and laboratory test results improved immediately. The patients improved upon corticosteroids tapering. These cases illustrate typical presentations of Cronkhite-Canada syndrome and the course of the disease following corticosteroid treatment. Additionally, they suggest the possibility that Cronkhite-Canada syndrome may be triggered by COVID-19 mRNA vaccination.
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Affiliation(s)
- Jun Ho Kim
- Department of Internal Medicine, Hanyang University Guri Hospital, Hanyang University College of Medicine, Guri, Korea
| | - Eun Hye Oh
- Department of Internal Medicine, Hanyang University Guri Hospital, Hanyang University College of Medicine, Guri, Korea
| | - Dong Soo Han
- Department of Internal Medicine, Hanyang University Guri Hospital, Hanyang University College of Medicine, Guri, Korea
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Khadarian K, Pai R, Samadder NJ. Cronkhite-Canada Syndrome: A Rare Cause of Gastrointestinal Polyposis With Response to Emerging Therapy. ACG Case Rep J 2024; 11:e01331. [PMID: 38586820 PMCID: PMC10997232 DOI: 10.14309/crj.0000000000001331] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/30/2023] [Accepted: 03/05/2024] [Indexed: 04/09/2024] Open
Abstract
A 70-year-old man presented to the clinic with a 6-month history of dysgeusia, followed by chronic, non-bloody diarrhea and 45 lb unintentional weight loss. Esophagogastroduodenoscopy discovered confluent nodularity in the gastric antrum and examined duodenum, but a normal esophagus. Colonoscopy uncovered patches of polypoid nodular mucosa throughout the entire colon. Biopsies of the nodular mucosa were consistent with hamartomatous polyps while biopsies of the intervening, normal-appearing mucosa demonstrated edema with crypt architectural distortion. Other hereditary polyposis syndromes were excluded with genetic testing, confirming a diagnosis of Cronkhite-Canada syndrome. Adalimumab therapy was initiated with clinical improvement after nonresponse to prednisone.
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Affiliation(s)
- Kevork Khadarian
- Division of Gastroenterology and Hepatology, Department of Medicine, Mayo Clinic, Phoenix, AZ
| | - Rish Pai
- Department of Pathology and Laboratory Medicine, Mayo Clinic, Scottsdale, AZ
| | - Niloy Jewel Samadder
- Division of Gastroenterology and Hepatology, Department of Medicine, Mayo Clinic, Phoenix, AZ
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Nguyen LC, Thi Pham T, Nguyen TT, Nguyen NH, Van Kieu T, Anh Do G, Thi-Ngoc Doan H, Van Tran C, Vu NT. A case report of Cronkhite-Canada syndrome first encounterd at a hospital in northern Vietnam. Ann Med Surg (Lond) 2023; 85:6134-6140. [PMID: 38098584 PMCID: PMC10718350 DOI: 10.1097/ms9.0000000000001334] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2023] [Accepted: 09/09/2023] [Indexed: 12/17/2023] Open
Abstract
Introduction and importance Cronkhite-Canada syndrome (CCS) is an extremely rare non-inherited syndrome first described in 1955 with only about 500 more cases reported so far. Since the aetiology of the disease remains unknown, there were no specific treatments in consensus. In many countries, CCS is a completely new condition that may confuse physicians at first encounter. Lessons should be learned from these cases by gastrointestinal specialists to be aware of this condition in any circumstances. Case presentation The authors reported a case study of a 45-year-old Vietnamese male with CCS diagnosis, which encountered at our centre for the first time. Clinical discussion The definitive diagnosis was provided by combining clinical characteristics, and endoscopic and histopathologic features, after excluding other causes of gastrointestinal polyposis. The patient responds to corticosteroids, proton pump inhibitors, and nutritional support right after treatment. After 1 year of treatment, his symptoms ameliorated completely although colon polyps insignificantly reduced. Conclusion Gastroenterologists should always be aware of patients with CCS with the following symptoms: gastrointestinal hamartomatous polyps, diarrhoea, and the dermatologic triad of alopecia, hyperpigmentation, and onychodystrophy.
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Affiliation(s)
- Long Cong Nguyen
- Gastroenterology and Hepatology Center
- Department of Internal Gastroenterology, University of Medicine and Pharmacy, Hanoi National University, Hanoi, Vietnam
| | | | | | - Nam Hoai Nguyen
- Gastroenterology and Hepatology Center
- Department of Internal Gastroenterology, University of Medicine and Pharmacy, Hanoi National University, Hanoi, Vietnam
| | | | | | | | | | - Nhung Thi Vu
- Pathology and Cytology Center, Bach Mai Hospital
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Dong J, Ma TS, Tu JF, Chen YW. Surgery for Cronkhite-Canada syndrome complicated with intussusception: A case report and review of literature. World J Gastrointest Surg 2022; 14:200-210. [PMID: 35317544 PMCID: PMC8908339 DOI: 10.4240/wjgs.v14.i2.200] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/18/2021] [Revised: 10/15/2021] [Accepted: 01/20/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Cronkhite-Canada syndrome (CCS) is a rare nonhereditary disease with a syndrome of multiple gastrointestinal polyps, skin pigmentation, hair loss, and fingernail/toenail dystrophy. Intussusception is a serious condition with an occurrence rate of 5% in adults, which is mainly caused by intestinal tumors or other intestinal occupations. CASE SUMMARY A 57-year-old woman was admitted to our hospital due to abdominal distension and pain for the past year. Her nausea and vomiting symptoms had been aggravated for the past month. Previous transoral enteroscopy results one year prior showed chronic erosive gastritis protuberans, duodenitis, and jejunitis. She had sparse body hair and brown pigmentation on the skin of her hands and bilateral anterior tibias. The nails of both hands were pale and lacked luster, and the fingernail of her ring finger was longitudinally cracked. Gastroscopy showed extensive diffuse polypoid lump changes in the gastric body and antrum, of 0.5-3 cm in size. Colonoscopy showed multiple polypoid mucosal bulges in the terminal ileum and multiple polyps (0.3-5 cm) throughout the colon. The patient was diagnosed with CCS and underwent partial excision of the polyps, but she refused hormone therapy. One month later, the patient complained of nausea and vomiting, accompanied by abdominal pain and inability to pass gas or stool. Contrast-enhanced computed tomography of the abdomen showed gastrointestinal polyposis and ileocecal intussusception. She underwent stomach and bowel surgery. CONCLUSION CCS, as a rare disease with poor prognosis, should be treated aggressively. Systematic steroids, immunosuppressive agents, and biological agents were not applied; thus, the patient's symptoms quickly progressed, and intussusception occurred. She had to undergo surgery. Improved compliance may lead to a better prognosis.
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Affiliation(s)
- Jie Dong
- Cancer Center, Department of Gastroenterology, Zhejiang Provincial People’s Hospital (Affiliated People’s Hospital, Hangzhou Medical College), Hangzhou 310014, Zhejiang Province, China
| | - Tian-Shi Ma
- Cancer Center, Department of Pathology, Zhejiang Provincial People’s Hospital (Affiliated People’s Hospital, Hangzhou Medical College), Hangzhou 310014, Zhejiang Province, China
| | - Jiang-Feng Tu
- Cancer Center, Department of Gastroenterology, Zhejiang Provincial People’s Hospital (Affiliated People’s Hospital, Hangzhou Medical College), Hangzhou 310014, Zhejiang Province, China
| | - You-Wei Chen
- Cancer Center, Department of Gastroenterology, Zhejiang Provincial People’s Hospital (Affiliated People’s Hospital, Hangzhou Medical College), Hangzhou 310014, Zhejiang Province, China
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