Department of Gastroenterology, Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan, China.
The North Sichuan Medical College, Nanchong, Sichuan, China.
Medicine (Baltimore). 2024 Oct 25;103(43):e40242. doi: 10.1097/MD.0000000000040242.
Cronkhite-Canada syndrome (CCS) is a nonhereditary, rare polyposis condition, first documented by Cronkhite and Canada in 1955. The primary distinct features of this syndrome include ectodermal abnormalities and diffuse gastrointestinal polyp changes accompanied by protein loss. The primary clinical manifestations of CCS include hair loss, excessive pigmentation of the skin, and malnourishment of fingernails or toenails. Other notable symptoms include weight loss, protein-losing enteropathy, diarrhea, abdominal pain, nausea, vomiting, taste abnormalities, and atrophic glossitis, which predominantly occur in middle-aged and older males. CCS is characterized by an extremely rare, nonfamilial hamartomatous polyposis syndrome, in which polyps are distributed in the stomach and colon (90%), small intestine(80%), and rectum (67%), while sparing the esophagus.
This report describes a 72-year-old female, initially treated for intestinal obstruction, followed by a small intestine resection. She reported diarrhea, emaciation, and loss of appetite across various hospitals.
Endoscopic examination of the stomach and colon, plus capsule endoscopy, revealed multiple polyps throughout her gastrointestinal tract, except in the esophagus.
Treatment included hormones with antiallergic medication, acid-suppressing drugs, salicylates, and nutritional support with zinc sulfate, adding trace elements and amino acids.
posttreatment, the patient demonstrated significant improvement in appetite and taste. Atrophic glossitis, upper limb pigmentation, and frequency of diarrhea also notably decreased. reexamination through endoscopy after 3 months of treatment revealed a substantial decrease in the number and size of gastrointestinal polyps.
In this case, from the lower esophageal sphincter to the rectum, there is an increasing trend of eosinophil and mast cell infiltration. These lesions can cause a positive IgG result. Pathological analysis indicates that the extent and severity of lesions in the middle and lower gastrointestinal tract are more substantial than in the upper tract. During treatment, endoscopic observations reveal that lesions in the middle and lower tract tend to resolve faster than those in the upper tract. Hormone therapy has demonstrated significant efficacy in treating this disease. Early treatment and regular follow-up for this disease can reduce the risk of cancerous changes and related complications.
克罗恩凯特-加拿大综合征(CCS)是一种非遗传性罕见的息肉病,于 1955 年由 Cronkhite 和 Canada 首次记录。该综合征的主要特征包括外胚层异常和弥漫性胃肠道息肉改变,并伴有蛋白质丢失。CCS 的主要临床表现包括脱发、皮肤过度色素沉着以及指甲或趾甲营养不良。其他显著症状包括体重减轻、蛋白丢失性肠病、腹泻、腹痛、恶心、呕吐、味觉异常和萎缩性舌炎,主要发生在中年和老年男性中。CCS 的特征是一种极为罕见的非家族性错构瘤性息肉病综合征,其中息肉分布于胃和结肠(90%)、小肠(80%)和直肠(67%),而食管不受累。
本报告描述了一位 72 岁女性患者,最初因肠梗阻接受治疗,随后进行了小肠切除术。她在多家医院报告腹泻、消瘦和食欲不振。
胃和结肠内镜检查以及胶囊内镜检查显示,除食管外,整个胃肠道均有多发性息肉。
治疗包括激素联合抗过敏药物、抑酸药物、柳氮磺胺吡啶和硫酸锌、微量元素和氨基酸的营养支持。
治疗后,患者的食欲和味觉明显改善。萎缩性舌炎、上肢色素沉着和腹泻频率也明显减少。治疗 3 个月后内镜复查显示,胃肠道息肉的数量和大小均显著减少。
在本例中,从食管下段括约肌到直肠,嗜酸性粒细胞和肥大细胞浸润呈递增趋势。这些病变可导致 IgG 呈阳性。病理分析表明,中、下消化道的病变程度和严重程度比上消化道更严重。治疗过程中,内镜观察显示,中、下消化道的病变较上消化道更快得到缓解。激素治疗对该疾病具有显著疗效。早期治疗和定期随访该疾病可以降低癌变和相关并发症的风险。