1. A classical case of Peutz–Jeghers syndrome with brief review of literature
- Author
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Bodhisatwa Behera, Jayanti Nayak, Manoj Kumar Patro, and T Santosh
- Subjects
congenital, hereditary, and neonatal diseases and abnormalities ,medicine.medical_specialty ,business.industry ,Peutz–Jeghers syndrome ,Cancer ,Disease ,medicine.disease ,Gastroenterology ,Pathology and Forensic Medicine ,medicine.anatomical_structure ,Hamartomatous Polyp ,Intussusception (medical disorder) ,Internal medicine ,lcsh:Pathology ,otorhinolaryngologic diseases ,Medicine ,In patient ,Oral mucosa ,skin and connective tissue diseases ,business ,Melanin pigment ,Intussusception ,lcsh:RB1-214 - Abstract
PJS is an autosomal dominant genetic disease associated with melanin pigment spots on the oral mucosa, lips, nasal alae, palm and soles, as well as hamartomatous polyps in the alimentary canal. Polyps are often a cause of intussusception in the affected patients. Cancers of gastrointestinal system, uterus and breast are common in patients with PJS. Long-term follow-up is required to prevent intussusception in children and cancer in adults. We report a classical case of Peutz–Jeghers syndrome presenting with jejunoileal intussusception in a 9year old child.
- Published
- 2016
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