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Peutz-Jeghers syndrome-This video explains all of the salient options of Peutz-Jeghers syndrome together with pathogenesis, morphology, medical options, problems and therapy elements.
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#Pathology
#Peutz-Jeghers Syndrome
Normal Options:
Hamartomatous Polyps all through gut. Polyps might also happen in gall bladder, bladder and nasopharynx.
A number of areas of mucocutaneous melanin pigmentation on the face, arms, ft, buccal mucosa and genitalia.
Virtually all instances are linked to a germline mutation in LKB1 / STK11 at 19p13.3
Microscopic options: Hamartomatous mucosal polyps with attribute central core of branching easy muscle, easy muscle, lamina propria, and glands lined by normal-appearing intestinal epithelium
Dysplasia and adenocarcinoma can develop inside polyps
Scientific Options:
Mucocutaneous pigmented lesions are frequent and could be the first clue to a person having Peutz-Jeghers syndrome; nevertheless, this pigmentation could fade over time
Presenting signs generally embody GI bleeding, belly ache or intussusception
Equal female and male incidence with median age of 11-12 yrs.
Excessive danger of creating intercourse twine tumors of the testes, gastric , small intestinal cancers, colon, pancreatic, breast, lung, ovarian, and uterine cancers.
Remedy:
After an preliminary analysis of endoscopic options of the small and enormous bowel, repeat analysis and therapeutic intervention is required.
Extra frequent analysis of the GI tract is recommended after age 50, as most malignancies happen at the moment
Related Intussusception/obstruction, bleeding to be handled promptly.
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