Peutz-Jeghers syndrome (PJS) is an autosomally dominant disorder associated with mutations in tumor suppressor gene STK11, and characterized by gastrointestinal polyposis, mucocutaneous pigmentation, and cancer predisposition:
- up to 93% lifetime risk of any cancer in affected individuals, and cancer often develops around age 40-49 years; associated cancers include cancers of the small bowel, stomach, pancreas, breast, ovary, cervix, lung, and testes
- colon cancer risk is reported at 39%, and typical age of colorectal cancer diagnosis is 42-46 years in affected patients
- rarely, PJS may be idiopathic and not associated with STK11 mutations
Froghi F, et al. A randomised trial of post-discharge enteral feeding following surgical resection of an upper gastrointestinal malignancy. Clin Nutr. 2017 Dec;36(6):1516-1519.
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RESULTS: 44 patients (M:F, 29:15) were randomised, 23 received jejunal supplements. There were no differences between the groups. Percentage of calculated energy requirement received was greater in the supplemented group at weeks 3 and 6 (p < 0.0001). Oral energy intake was not different between the groups at any time period. After hospital discharge, there were no differences in MFI-20, EQ5D and QLQ-OES18 scores at any time point. From hospital discharge fatigue improved and plateaued at 6 weeks (p < 0.05 for both groups), independence at 12 weeks (p < 0.05 for both groups). No improvement was seen in quality of life until 24 weeks in the active group alone (p < 0.02) and not at all in the control group.
CONCLUSIONS: Addition of jejunal feeding is effective in providing patients with an adequate energy intake. Increased energy intake however, produced no obvious improvement in measures of fatigue, quality of life or health economics.