Hamartomatous Polyposis Syndromes: Management and Surveillance Strategies

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SEDIGHI SIMA; Jokar Mohammad Hassan; Moradzadeh Maliheh

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Journal Paper

Paper Information

Journal: Journal of Shahid Sadoughi University of Medical Sciences Year:2020 | Volume:28 | Issue:6 Page(s): 2720-2733

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Information Journal Paper

Title

Hamartomatous Polyposis Syndromes: Management and Surveillance Strategies

Author(s)

SEDIGHI SIMA | Jokar Mohammad Hassan | Moradzadeh Maliheh | Issue Writer Certificate

Keywords

Hamartomatous polyposis syndromesQ1

Juvenile polyposis syndromeQ1

Peutz-Jeghers syndromeQ1

Cowden SyndromQ1

Bannayan-Riley-Ruvalcaba SyndromeQ1

Abstract

Introduction: Hamartomatous polyposis syndromes (HPS) are a rare group of dominant autosomal inheritance, which is characterized by the development of hamartomatous polyps in the gastrointestinal tract. This syndrome included Juvenile polyposis syndrome (JPS), Peutz-Jeghers syndrome (PJS), and PTEN Hamartoma Tumour Syndrome (PHTS). PTEN Hamartoma Tumour Syndrome (PHTS) itself includes Cowden Syndrome, Bannayan-Riley-Ruvalcaba Syndrome (BRRS), and Proteus Syndrome. Diagnosis of Hamartum polyarthritis syndrome (HPS) is routinely made based on clinical criteria, but sometimes the diagnosis of this syndrome becomes problematic due to the varying severity of clinical symptoms even within a family. Therefore, today, genetic testing of germ-related mutations associated with this disease is used in the diagnosis process. Management strategy of the syndrome is different due to extra-intestinal symptoms and various cancers. Clinical awareness and early diagnosis of HPS is important, so it is recommended to patients and families at risk for genetic counseling and care. Surveillance in children with HPS might prevent intestinal and extra-intestinal disorders, and reduce the risk of intestinal cancer or breast cancer in adults.

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APA: Copy

SEDIGHI, SIMA, Jokar, Mohammad Hassan, & Moradzadeh, Maliheh. (2020). Hamartomatous Polyposis Syndromes: Management and Surveillance Strategies. JOURNAL OF SHAHID SADOUGHI UNIVERSITY OF MEDICAL SCIENCES, 28(6 ), 2720-2733. SID. https://sid.ir/paper/386478/en

Vancouver: Copy

SEDIGHI SIMA, Jokar Mohammad Hassan, Moradzadeh Maliheh. Hamartomatous Polyposis Syndromes: Management and Surveillance Strategies. JOURNAL OF SHAHID SADOUGHI UNIVERSITY OF MEDICAL SCIENCES[Internet]. 2020;28(6 ):2720-2733. Available from: https://sid.ir/paper/386478/en

IEEE: Copy

SIMA SEDIGHI, Mohammad Hassan Jokar, and Maliheh Moradzadeh, “Hamartomatous Polyposis Syndromes: Management and Surveillance Strategies,” JOURNAL OF SHAHID SADOUGHI UNIVERSITY OF MEDICAL SCIENCES, vol. 28, no. 6 , pp. 2720–2733, 2020, [Online]. Available: https://sid.ir/paper/386478/en

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