Clinical analysis of Peutz-Jeghers syndrome:a report of 6 cases.
- Author:
Ke DONG
1
;
Bo LI
;
Ben-hai LI
;
Quan-lin GUAN
;
Yong-zhong HUO
Author Information
- Publication Type:Case Reports
- MeSH: Adolescent; Adult; Female; Humans; Intestine, Small; surgery; Male; Pedigree; Peutz-Jeghers Syndrome; diagnosis; genetics; surgery
- From: Chinese Journal of Gastrointestinal Surgery 2005;8(4):336-338
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVETo investigate the diagnostic methods and reasonable treatment of Peutz-Jeghers syndrome (PJS).
METHODSClinical data of six patients with PJS were reviewed.
RESULTSRepeated abdominal pain, intussusception and intestinal polyp with bleeding were main manifestations. Four patients father,three patients grandfather and one patients mother were diagnosed with PJS. Three patients had family history of cancer. Case 4 and case 5 underwent laparotomy for many times because of intussusceptions caused by polyps or recurrent abdominal pain. Case 1 and case 4 had polyps synchronous with adenoma, and case 2 had polyp with gastric cancer. Main treatment included polyp resection and partial small intestinal and colon resection.
CONCLUSIONSPatients with PJS have family history of cancer and a high incidence of polyp recurrence of small intestine. Surgical intervention is the first choice regimen. Surveillance should be emphasized on gastrointestinal tract and other potential malignant organs in PJS patients.