Peutz-Jeghers syndrome: its natural course and management
- PMID: 1117595
Peutz-Jeghers syndrome: its natural course and management
Abstract
Two hundred and twenty-two patients with Peutz-Jeghers syndrome were ascertained in Japan between 1961 and 1974 through two nationwide surveys, medical literature, and personal examinations. Genetic analysis was made of this group as well as 102 follow-up cases. The average age at diagnosis was 23 in males and 26 in females, with male to female ratio of 1:1.13. Presenting complaints of 170 patients included obstruction (42.8 per cent of patients), abdominal pain (23.4 per cent), rectal bleeding (13.5 per cent), extrusion of polyp (7.2 percent). Diagnosis of 52 patients was based on melanin pigmentation. Intussusception occurred in 46.9 per cent of the patients, most often in the small intestine. Polyps occurred in the stomach in 108 patients (48.6 per cent), small intestine, 142 patients (64 per cent), colon, 118 patients (53.2 per cent) and rectum, 71 patients (32 per cent). Among the 222 patients, cancer was histologically verified in 28. Fifteen early cancers occurred (3 gastric, 8 small intestine, 4 colon), and 11 advanced cancers (3 gastric, 1 small intestine, 6 colon, and 1 both colon and small intestine). Mortality was lower than in patients with familial polyposis coli but higher than in the general population. Conservative surgical management, planned medical follow-up, and the need for a national registration system are stressed.
Similar articles
-
Peutz-Jeghers syndrome: a clinical study of a large family in two generations.Z Kinderchir. 1986 Dec;41(6):364-8. doi: 10.1055/s-2008-1043380. Z Kinderchir. 1986. PMID: 3825304
-
[Clinical analysis of Peutz-Jeghers syndrome:a report of 6 cases].Zhonghua Wei Chang Wai Ke Za Zhi. 2005 Jul;8(4):336-8. Zhonghua Wei Chang Wai Ke Za Zhi. 2005. PMID: 16167257 Chinese.
-
Wireless capsule endoscopy for evaluation of phenotypic expression of small-bowel polyps in patients with Peutz-Jeghers syndrome and in symptomatic first-degree relatives.Endoscopy. 2004 Dec;36(12):1060-6. doi: 10.1055/s-2004-826038. Endoscopy. 2004. PMID: 15578295 Clinical Trial.
-
[Peutz-Jeghers syndrome: an account of 3 cases in the same family and a review of the literature].Chir Ital. 2005 Jul-Aug;57(4):425-36. Chir Ital. 2005. PMID: 16060180 Review. Italian.
-
Inherited gastrointestinal-polyposis syndromes.N Engl J Med. 1976 May 13;294(20):1101-4. doi: 10.1056/NEJM197605132942007. N Engl J Med. 1976. PMID: 768769 Review. No abstract available.
Cited by
-
Peutz-Jeghers syndrome: an unusual autopsy finding in pregnancy.Autops Case Rep. 2021 Apr 20;11:e2021279. doi: 10.4322/acr.2021.279. Autops Case Rep. 2021. PMID: 33968835 Free PMC article.
-
Findings from the Peutz-Jeghers syndrome registry of uruguay.PLoS One. 2013 Nov 19;8(11):e79639. doi: 10.1371/journal.pone.0079639. eCollection 2013. PLoS One. 2013. PMID: 24260271 Free PMC article.
-
Cancer problem in Peutz-Jeghers syndrome.Adv Biomed Res. 2013 Mar 30;2:35. doi: 10.4103/2277-9175.109721. eCollection 2013. Adv Biomed Res. 2013. PMID: 23977663 Free PMC article.
-
A new simple technique for performing intraoperative endoscopic resection of small-bowel polyps in patients with Peutz-Jeghers syndrome.Surg Today. 1999;29(6):581-3. doi: 10.1007/BF02482361. Surg Today. 1999. PMID: 10385381
-
Colonic polyposis syndromes--an experience from a tertiary centre in South India.Indian J Gastroenterol. 2015 May;34(3):233-9. doi: 10.1007/s12664-015-0558-3. Epub 2015 May 10. Indian J Gastroenterol. 2015. PMID: 25957234