关键词: Case report Cronkhite-Canada syndrome Gastrointestinal polyposis Hormonal therapy Prognosis

Mesh : Male Humans Middle Aged Endoscopy, Gastrointestinal / adverse effects Glucocorticoids / therapeutic use Esophagus / pathology Intestinal Polyposis / complications diagnosis therapy Diarrhea / etiology

来  源:   DOI:10.3748/wjg.v30.i8.984   PDF(Pubmed)

Abstract:
BACKGROUND: Cronkhite-Canada syndrome (CCS) is a rare, noninherited disease characterized by gastrointestinal polyposis with diarrhea and ectodermal abnormalities. CCS polyps are distributed through the whole digestive tract, and they are common in the stomach and colon but very uncommon in the esophagus.
METHODS: Here, we present a case of a 63-year-old man with skin hyperpigmentation accompanied by diarrhea, alopecia, and loss of his fingernails. Laboratory data indicated anemia, hypoalbuminemia, hypocalcemia, hypokalemia, and positive fecal occult blood. Endoscopy showed numerous polyps scattered throughout the digestive tract, including the esophagus. He was treated with nutritional support and glucocorticoids with remission of his symptoms.
CONCLUSIONS: Comprehensive treatment led by hormonal therapy can result in partial or full remission of clinical symptoms. Treatment should be individualized for each patient according to their therapy response. Surveillance endoscopy is necessary for assessing mucosal disease activity and detecting malignant transformation.
摘要:
背景:Cronkhite-Canada综合征(CCS)是一种罕见的,非遗传性疾病的特征是胃肠道息肉病,腹泻和外胚层异常。CCS息肉分布于整个消化道,它们在胃和结肠中很常见,但在食道中却很少见。
方法:这里,我们介绍了一个63岁的男人,皮肤色素沉着过度伴有腹泻,脱发,还有他指甲的脱落.实验室数据显示贫血,低蛋白血症,低钙血症,低钾血症,和阳性粪便潜血。内窥镜检查显示许多息肉分散在整个消化道,包括食道.他接受了营养支持和糖皮质激素治疗,症状缓解。
结论:以激素治疗为主导的综合治疗可使临床症状部分或完全缓解。应根据每位患者的治疗反应进行个体化治疗。监测内窥镜检查对于评估粘膜疾病活动性和检测恶性转化是必要的。
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