welcome to oneFAPvoice
- a positively charged Familial Adenomatous Polyposis community.- join today!
- login
Familial Adenomatous Polyposis in Children and Adolescents
source: Journal of pediatric gastroenterology and nutrition
year: 2010
authors: Alkhouri N, Franciosi J P, Mamula P
summary/abstract:BACKGROUND : Familial adenomatous polyposis (FAP) is the most common inherited polyposis syndrome characterized by the development of hundreds of colorectal adenomatous polyps. The aim of this study was to review cases of FAP diagnosed at The Children’s Hospital of Philadelphia in a 16-year period.
METHODS : Medical records of patients diagnosed as having FAP between 1990 and 2005 were reviewed. The collected data included disease presentation, genetic profile, extraintestinal manifestations, surveillance, and treatment.
RESULTS : We identified 12 patients with FAP. The age range at presentation was 7 to 18 years. Seven (68%) patients presented due to symptoms, the most common of which was rectal bleeding (6 patients, 86%). The youngest age at which polyps were detected was 7 years. Eight patients (67%) had positive family history. Three patients had Gardner syndrome and 1 presented in infancy with hepatoblastoma. Four patients had adenomatous polyposis coli gene mutation identified. One patient was diagnosed as having rectal carcinoma in situ. Six patients (50%) had gastric fundic gland polyposis and 6 had duodenal adenomatous changes. Capsule endoscopy was performed in 3 patients; 1 had multiple polyps in the duodenum and the jejunum. Seven patients (58%) underwent total colectomy with no serious complications.
CONCLUSIONS : FAP is a rare condition but with significant risk of cancer and comorbidity. In this series, patients commonly presented to medical attention due to their symptoms. The youngest patient with polyps detected was 7 years old. We identified 1 patient with rectal cancer in situ and high proportion of patients with duodenal adenomatous lesions. Majority of patients underwent early colectomy.
organization: Cleveland ClinicDOI: 10.1097/MPG.0b013e3181e1a224
read more full text source
expertly curated content related to this topic
-
Discussing Pediatric Familial Polyposis and Colorectal Cancerhttps://youtu.be/km3FYvoOV94...
-
diane’s storyI was devastated, I had made all my arra...
-
Gender-Specific Aspects of Lynch Syndrome and Familial Adenomatous PolyposisBACKGROUND: The most frequent hereditar...
-
Identification of 5 Novel Germline APC Mutations and Characterization of Clinical Phenotypes in Japanese Patients wi...BACKGROUND : Familial adenomatous polypo...
-
Evaluation of Management of Desmoid Tumours Associated with Familial Adenomatous Polyposis in Dutch PatientsBACKGROUND : The optimal treatment of de...
-
Sex and the Male Ostomate Fact SheetMany sex problems that male ostomates ex...
-
Evaluation of 28 Years of Surgical Treatment of Children and Young Adults with Familial Adenomatous PolyposisBACKGROUND : In this retrospective study...