三种胃肠道息肉综合征的内镜特点与临床病理联系

胃肠道息肉综合征为一组特殊的疾病症候群,以其特征性的胃肠道多发息肉及增加的癌症风险而受到关注。然而因其发病率相对较低及受到检查、治疗手段的限制,并未得到临床医师的充分认识。我们将以胃肠道息肉综合征中的Cronkhite—Canada综合征（Cronkhite—Canada syndrome,CCS）、Peutz—Jeghers综合征（Peutz—Jeghers syndrome,PJS）和家族性腺瘤性息肉病（familial adenomatous polyposis,FAP）的典型内镜表现为基础,探讨它们的临床病理联系。     

Cronkhite-Canada综合征2例报告并国内外文献评价

目的总结Cronkhite—Canada综合征（CCS）的临床表现、消化道息肉特点、病理改变及诊断治疗，以提高临床医生对本病的认识。方法对我院诊断明确的2例CCS病例及1990年以来72篇文献报告的31例病例的临床资料回顾性研究。结果符合CCS诊断标准的入组病例33例，男20例，女13例；年龄17～77岁，中位年龄51岁。临床表现为脱发、指（趾）甲改变、皮肤色素沉着、腹部不适及低蛋白血症。全部患者均有消化道多发息肉，累及胃、小肠、大肠。息肉病理无特异性，其中8例癌变。治疗方法包括激素治疗17例，内镜下息肉摘除2例，因癌变、消化道梗阻及蛋白丢失性肠病手术11例（3例同时激素治疗）和其他治疗6例，其中1年内激素治疗有效14例，最长1例随访7年无复发。结论Cronkhite—Canada综合征（CCS）是以消化道多发息肉伴外胚层改变为主要临床特点的综合征，诊断较困难，激素治疗能改善症状，长期疗效仍待进一步研究。     

4例Cronkhite Canada综合征患者临床分析并文献复习

目的探讨Cronkhite Canada综合征(Cronkhite Canada syndrome,CCS)患者的临床特点、治疗方法。方法回顾性分析2010年8月至2018年5月在中国人民解放军总医院第七医学中心消化内科诊断为CCS的4例患者的病例特征。结果 CCS患者常存在典型的消化道症状(腹泻、腹痛、味觉减退等)和外胚层病变[脱发、色素沉着、指(趾)甲营养不良],易合并便潜血阳性,低蛋白、低钾、低钙血症等;消化道息肉以胃、结肠最常见,病理多表现为管状腺瘤、炎性息肉。3例患者予塞来昔布治疗有效,1例患者予糖皮质激素治疗有效。结论 CCS血清Ig G4阴性患者可考虑塞来昔布治疗,推荐剂量400 mg/d。     

Cronkhite-Canada综合征的X线表现

1955年Cronkhite及Canada描述2例独特的伴有外胚层改变的非家族性胃肠道息肉,至今已有百余例报告。本文报告6例临床及病理均符合的Cronkh-ite-Canada综合征(CCS)患者,并复习文献。     

Cronkhite-Canada综合征国内文献复习

胃肠道息肉-色素沉着-秃发-指(趾)甲萎缩综合征(Cronkhite-Canada syndrome，CCS)是由Cronkhite和Canada于1955年首先报道的一组临床综合征。国内1985年首次报告本病，现就山西医科大学第二医院收治的1例患者及国内发表有关文献24篇加以总结分析。     

Cronkhite-Canada综合征1例

Cronkhite-Canada 综合征(Cronkhite-Canada syndrome, CCS),又称息肉-色素沉着-脱发-指(趾)甲营养不良综合征.该病最早在1955 年由Cronkhite和Canada首先报道[1], 1966年命名为Cronkhite-Canada syndrome[2],是临床上一类非...     

Cronkhite-Canada综合征

1955年,Cronkhite 和 Canada 首次描述2例消化道弥漫性息肉病伴皮肤色素沉着、脱发和指甲萎缩,目前称为 Cronkhite-Canada 氏综合征(CCS)。有关本病的若干方面意见尚不一致。本文复习文献中报道的55例 CCS。     

儿童肾病综合征治疗

儿童肾病综合征与成人患者不同,大多数为微小病变(MCNS),对激素治疗敏感,但少数病例(主要是局灶节段性肾小球硬化FSGS,口服激素治疗却无效.依据对激素治疗的反应性,儿童肾病综合征可分为激素敏感型和不敏感型两大类,二者在治疗和预后上亦有差异.许多医疗中心对儿童肾病综合征     

激素抵抗型肾病综合征早期预测因素的研究进展

原发性肾病综合征（idiopathic nephrotic syndrome，INS）是一组临床常见的肾脏疾病。糖皮质激素（glucoeorticoid，GC）是INS的首选和基础用药。激素抵抗型肾病综合征（steroid—resistant nephrotic syndrome，SRNS）是指患者使用常规剂量的GC治疗8—12周（局灶节段性肾小球硬化症为16周）无效，     

胃肠道息肉为特征的Cronkhite-Canada综合征临床分析

Cronkhite-Canada综合征（Cronkhite-Canada syndrome，CCS），又名多发性胃肠道息肉-色素沉着-脱发-指（趾）甲营养不良综合征，是由Cronkhite和Canada在1955年首先报道的一组非常罕见的临床综合征，其病因不明，主要表现为胃肠道多发息肉伴外胚层病变，如全身皮肤色素沉着、毛发脱落、指（趾）甲营养不良等。日本学者Goto［1］在1995年回顾了110例CCS患者的临床特征，报道其发病率约为1／100万，男女比例约3∶2，根据发病经过分为5型：1型，以腹泻为初发症状；2型，味觉异常为首发表现；3型，口腔内感觉异常及口渴；4型，腹部症状为主，但无腹泻；5型，毛发脱落为主要表现。国内外文献均以个案报道为主，诊断主要依靠典型的症状、体征和内镜所见，目前暂无统一的治疗方案。为提高对本病的认识，现就我院确诊的3例患者及1985年以来国内报道的65例CCS患者资料进行回顾分析。     

Cronkhite-Canada syndrome associated with myelodysplastic syndrome

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, infiltration 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 findings. This is the first report of CCS in a patient with MDS.     

Cronkhite-Canada syndrome with colon cancer, portal thrombosis, high titer of antinuclear antibodies, and membranous glomerulonephritis

A 64-year-old man, who came to us with diarrhea, presented with ectodermal changes such as hyperpigmentation, alopecia, and onychatrophy, and was affected by polyposis in the colorectum and stomach. The polyps were histologically consistent with those in Cronkhite-Canada syndrome (CCS). Interestingly, the patient also had colon cancer, as well as portal thrombosis and a high concentration of antinuclear antibody. Treatment with prednisolone ameliorated the symptoms and the gastrointestinal polyposis, while the cancer was successfully treated with a hemicolectomy. Six months after the surgery, the patient developed nephropathy, with nephrotic-range proteinuria, without recurrence of the cancer. The biopsied renal specimen showed membranous glomerulonephritis. This is a rare case of CCS associated with various complications such as colon cancer, portal vein thrombosis, a high titer of antinuclear antibodies, and membranous glomerulonephritis. Although the pathogenesis of CCS is essentially unknown, these complications might have been indicative of an underlying immunological abnormality.     

Steroid therapy during acute myocardial infarction. A cause of delayed healing and of ventricular aneurysm

Clinical and necropsy observations are described in a man who received large doses of corticosteroids for Dressler's syndrome which complicated acute myocardial infarction. Although the patient survived for 63 days, during 53 of which he received corticosteroids, the infarct histologically appeared to be only 10 to 14 days old. Thus, the healing of the infarct was clearly delayed. Experimental studies in dogs were cited in which healing of myocardial infarction was delayed by corticosteroid therapy.In our patient a large left ventricular aneurysm also developed. Study of previous reports of human subjects receiving corticosteroids during acute myocardial infarction disclosed that aneurysmal formation was also a common complication of corticosteroid therapy for the postmyocardial infarction syndrome.It is concluded that use of glucocorticosteroids for any reason during acute myocardial infarction may be hazardous, and that their use in patients with the postmyocardial infarction syndrome, a benign and self-limited condition, should be avoided.     

Cronkhite—Canada syndrome associated with perianal condyloma acuminatum with malignant transformation: A case report

Rationale:Cronkhite-Canada syndrome (CCS) is a rare non-familial polyposis syndrome characterized by multiple gastrointestinal polyps with the ectodermal triad. To date, many complications of CCS have been reported in the literature, but perianal condyloma acuminatum with malignant transformation has not been included.Patient concerns:This report presents the case of a 52-year-old Chinese man who presented with diarrhea, loss of appetite, and weight loss. He developed skin pigmentation and atrophy of the fingernails and toenails. Upper gastrointestinal endoscopy, colonoscopy, capsule endoscopy, and enteroscopy revealed diffuse polyps along the entire digestive tract. Histopathological examination revealed polyps of different pathological types dominated by hamartoma. Physical examination revealed a crissum cauliflower-like neoplasm (2.5 × 2.0 cm). After perianal tumor resection, pathology suggested that this was a perianal condylomatous lesion with malignant transformation, as well as well-differentiated squamous cell carcinoma.Diagnoses:These clinical features and endoscopic findings were consistent with CCS which associated with perianal condyloma acuminatum with malignant transformation.Intervention:Clinical remission was achieved with glucocorticoid, azathioprine, and nutritional support.Outcome:At the 4-year follow-up, the patient had no diarrhea or loss of appetite, had gained 13 kg in weight, and the perianal tumor had not recurred.Lessons:No previous report has described CCS in a patient with perianal condyloma acuminatum with malignant transformation. As both conditions are related to immune disorders, their occurrence may be correlated.     

Comparing the 2010 North American and European atrial fibrillation guidelines

This article compares the important differences in the American College of Cardiology Foundation (ACCF)/American Heart Association (AHA)/Heart Rhythm Society (HRS), Canadian Cardiovascular Society (CCS), and European Society of Cardiology (ESC) 2010 guidelines on atrial fibrillation (AF). All guidelines recommend more lenient targets for ventricular rate control although the CCS guidelines recommend a target heart rate at rest <100 bpm whereas the the ACCF/AHA/HRS and ESC guidelines accept a target heart rate at rest <110 bpm with provisos. All the guidelines recommend that the choice of antiarrhythmic drug for maintenance of sinus rhythm be based on the underlying cardiovascular disease state. However, the CCS guidelines do not recommend that the use of Class IC drugs or sotalol be restricted in the presence of left ventricular hypertrophy alone. All the guidelines have incorporated dronedarone into their recommendations of antiarrhythmic drug therapy for maintenance of sinus rhythm. However, the CCS guidelines do not make a specific recommendation that the use of dronedarone is reasonable to decrease the risk of hospitalization for cardiovascular causes in patients with AF. The ACCF/AHA/HRS update makes a strong recommendation for catheter ablation in patients with paroxysmal AF who have failed a single anti-arrhythmic drug whereas the CCS and ESC guidelines make this a conditional recommendation. The CCS guidelines are the only guidelines at present that recommend dabigitran for prevention of stroke in high risk patients and suggest that dabigatran is preferred to warfarin for stroke prevention in most patient groups.     

Steroid response in refractory asthmatics

Inhaled glucocorticosteroids are currently the most effective anti-inflammatory controller medications for treating persistent asthma. The efficacies of glucocorticoids include reducing asthma symptoms, reducing exacerbation frequency, improving quality of life, improving lung function, decreasing airway hyperresponsiveness, controlling airway inflammation, and reducing mortality. However, the treatment response to glucocorticosteroids in asthmatics varies, and certain subtypes of asthma, such as refractory asthma, respond poorly to high-dose inhaled glucocorticoid and systemic steroids. The medical costs of treating refractory asthmatics represent about 50% of the total healthcare cost for asthma. A thorough understanding of the mechanisms of glucocorticoid action, patient responses to glucocorticoids, and steroid resistance observed in refractory asthmatics is necessary for the targeted development of therapeutic drugs. This review discusses the characteristics of severe refractory asthmatics and the mechanisms of steroid response and resistance in asthma treatment.     

Polymyalgia rheumatica and giant-cell arteritis

Polymyalgia rheumatica and giant-cell arteritis are closely related disorders that affect people of middle age and older. They frequently occur together. Both are syndromes of unknown cause, but genetic and environmental factors might have a role in their pathogenesis. The symptoms of polymyalgia rheumatica seem to be related to synovitis of proximal joints and extra-articular synovial structures. Giant-cell arteritis primarily affects the aorta and its extracranial branches. The clinical findings in giant-cell arteritis are broad, but commonly include visual loss, headache, scalp tenderness, jaw claudication, cerebrovascular accidents, aortic arch syndrome, thoracic aorta aneurysm, and dissection. Glucocorticosteroids are the cornerstone of treatment of both polymyalgia rheumatica and giant-cell arteritis. Some patients have a chronic course and might need glucocorticosteroids for several years. Adverse events of glucocorticosteroids affect more than 50% of patients. Trials of steroid-sparing drugs have yielded conflicting results. A greater understanding of the molecular mechanisms involved in the pathogenesis should provide new targets for therapy.     

Biochemical assessment of niacin deficiency among carcinoid cancer patients

OBJECTIVE: Carcinoid cancer patients often have elevated levels of serotonin or its precursor 5-hydroxytryptophan. Normally, serotonin synthesis accounts for a small fraction of tryptophan catabolism, which should be directed along a pathway that allows partial conversion to niacin; hence, increased diversion of tryptophan toward serotonin could cause variable degrees of niacin deficiency in carcinoid patients. Therefore, the prevalence of niacin deficiency among carcinoid patients was investigated by clinical assessment of pellagra and biochemical assessment of whole blood niacin number, a ratio derived from two biologically active forms of niacin (NAD/NADP x 100). METHODS: Clinical and biochemical niacin status were assessed in a cohort of newly diagnosed carcinoid patients with carcinoid syndrome (CCS, n = 36), carcinoid patients without carcinoid syndrome (CWCS, n = 32) and noncarcinoid controls (n = 24) recruited at two primary care clinics. Other aspects of serotonin metabolism were measured by analyses of plasma serotonin and tryptophan and urinary excretion of 5-hydroxyindoleacetic acid. RESULTS: Biochemical niacin deficiency (niacin number < 130) was significantly more common in CCS patients (10 out of 36) compared to controls (p < 0.05, Fisher's exact test), while CWCS patients displayed an incidence that was not significantly elevated (4 out of 32). Only one CCS patient, who was also identified biochemically as niacin deficient, was clinically diagnosed with pellagra. CONCLUSION: Biochemical niacin deficiency is more prevalent among newly diagnosed CCS patients than in controls. Manifestation of pellagra is a less sensitive indicator, and dependence on this endpoint could lead to a lack of appropriate nutritional support for this group of patients.     

Cronkhite-Canada综合征1例报道

Cronkhite-Canada综合征(Cronkhite-Canada syndrome,CCS)发病罕见,病因不明,是以胃肠道多发息肉和外胚层三联征两大症候群为主,临床表现为慢性腹泻、腹痛、脱发、皮肤色素沉着、指(趾)甲萎缩脱落等。本文报道CCS 1例。