ISSN:8756-0437    

DOI:10.1002/ssu.2980030202

出版商:John Wiley&Sons, Inc.    

年代:1987

数据来源: WILEY

ISSN:8756-0437    

DOI:10.1002/ssu.2980030203

出版商:John Wiley&Sons, Inc.    

年代:1987

数据来源: WILEY

摘要:

AbstractThe emergence of familial polyposis coli from the general group of conditions with multiple polyps of the large intestine into a well‐defined separate entity started when histopathology became a science. The recognition of its two main features — inheritance as a Mendelian dominant characteristic and its high incidence of associated colorectal cancer — greatly helped in establishing a policy of treatment designed to prevent cancer. The removal of all or most of the large intestine before cancer had supervened has had considerable success in reducing the cancer incidence. However, the more recent awareness that the adenomas can frequently be found also in the upper gastrointestinal tract has introduced new problems, the solution of which is currently a matter of investig

ISSN:8756-0437    

DOI:10.1002/ssu.2980030204

出版商:John Wiley&Sons, Inc.    

年代:1987

数据来源: WILEY

摘要:

AbstractWhile the inheritance pattern of familial polyposis coli is established as an autosomal dominant pattern, the expression of the various extracolonic manifestations associated with the neoplastic polyposis is less well understood. The discrete polyp cancer syndrome may not be recognized unless both polyps and colon cancer are considered in the inheritance pattern. The hamartomatous polyps follow a Mendelian‐dominant inheritance pattern for the Peutz‐Jeghers syndrome, while the inheritance pattern for the juvenile polyposis syndromes is less clear. Cowden's disease appears in a Mendelian dominant pattern, but the occurrence of colonic polyps is less well documented. The ganglioneuromas follow a mendelian dominant inheritance pattern, while the relationship and occurrence of colonic polyps in association with Torre's syndrome is uncertain. The Mendelian dominant inheritance pattern for the cancer family syndrome is documented; the role of colon polyps in this syndrome is less well understood. A further understanding of the inheritance patterns of these various colon polyps will lead to more understanding of the basic disease and help in prevention and early detection for treatment and c

ISSN:8756-0437    

DOI:10.1002/ssu.2980030205

出版商:John Wiley&Sons, Inc.    

年代:1987

数据来源: WILEY

摘要:

AbstractDental and bone abnormalities of the maxilla and mandible are present in approximately 80% of patients with familial polyposis coli. The dental abnormalities include impacted teeth (other than third molars), supernumerary teeth, congenitally missing teeth, fused roots of first and second molars, and unusually long and tapered roots of posterior teeth. The bone lesions consist mostly of osteomas, either isolated or in clusters, in the maxilla and mandible or of exostoses with lateral and/or lingual extensions. Since dental and bone abnormalities are already present early in life there is a strong suggestion that they may be used as diagnostic features in the recognition of familial polyposis coli.

ISSN:8756-0437    

DOI:10.1002/ssu.2980030206

出版商:John Wiley&Sons, Inc.    

年代:1987

数据来源: WILEY

摘要:

AbstractIn this review we present diseases associated with hereditary polyposis syndromes. In addition to the known extracolonic manifestations of the so‐called Gardner's syndrome, other important diseases are carcinomas of the thyroid and of the small bowel and bile duct adenoma. The incidence of these diseases is discusse

ISSN:8756-0437    

DOI:10.1002/ssu.2980030207

出版商:John Wiley&Sons, Inc.    

年代:1987

数据来源: WILEY

摘要:

AbstractThe original concept of familial polyposis coli (FPC) as only a genetically determined premalignant disease of the colon changed in the 1950's, with the description of Gardner's syndrome. The extracolonic manifestations of osteoma and epidermoid cyst have since been shown to be only a small part of the spectrum of both benign and malignant extracolonic manifestations of the disease. The modern concept of this condition is that FPC is a genetically determined generalized growth disorder that gives rise to tumors in various parts of the body.

ISSN:8756-0437    

DOI:10.1002/ssu.2980030208

出版商:John Wiley&Sons, Inc.    

年代:1987

数据来源: WILEY

摘要:

AbstractThe ileal pouch‐anal procedure maintains enteric continence by preserving critical physiological mechanism

ISSN:8756-0437    

DOI:10.1002/ssu.2980030209

出版商:John Wiley&Sons, Inc.    

年代:1987

数据来源: WILEY

摘要:

AbstractThe history of familial polyposis coli and its various surgical treatment alternatives are presented. Each form of treatment has advantages and disadvantages. The menu of alternatives allows the surgeon the freedom to choose the best option for each patient.

ISSN:8756-0437    

DOI:10.1002/ssu.2980030210

出版商:John Wiley&Sons, Inc.    

年代:1987

数据来源: WILEY

摘要:

AbstractRegistry ascertainment of kindreds with adenomatous polyposis (AP) reduces the incidence of colorectal carcinoma by medical monitoring for offspring and siblings of affected patients. Due to its pleiomorphic genotype and a 50% risk for AP in each patient's progeny, early screening is mandatory. Flexible sigmoidoscopy, averaging from puberty to age 48, annually or every two years, is one diagnostic technique that does not result in excess patient discomfort or cost. Timely examination is facilitated when eye anomalies, osteomas, or epidermoid cysts are present in childhood as potential clinical markers for AP. Denial, misperceptions, and fear may impede surveillance in the absence of education by the registry health care team.

ISSN:8756-0437    

DOI:10.1002/ssu.2980030211

出版商:John Wiley&Sons, Inc.    

年代:1987

数据来源: WILEY