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Subject Area

Pediatrics

Article Type

Review

Abstract

Objective The aim was to review the studies on clinical presentation, diagnosis, and management of inherited platelet disorder (IPD). Materials and methods A systematic search of MEDLINE (PubMed, Medscape, Science Direct, EMF-Portal) and internet was conducted on all articles published from 1997 to 2016. English language reports of perianal fistula were assessed. The initial search presented 150 articles where 26 satisfied the inclusion criteria. Articles not reporting on IPD in the title or abstract were not included. A total of 11 independent investigators extracted data on methods. Comparisons were made by structured review, with the results tabulated. Eight authors emphasized clinical presentation of IPD, nine about diagnosis of IPD, and nine about management of IPD. Findings Common symptoms include ecchymosis, epistaxis, menorrhagia, and excessive bleeding with childbirth, surgery, dental procedures, and trauma. IPD diagnosis is straightforward in the major platelet function disorders such as Bernard-Soulier Syndrome (BSS) and Glanzmann thrombasthenia (GT). Establishing a conclusive molecular diagnosis is the bedrock of good hematological practice, because it informs optimal treatment and can provide clarity about disease progression. The management of patients with IPDs usually consists of general measures aimed at avoiding bleeding and the use of supportive therapy to control hemorrhagic episodes. Conclusion IPDs are important causes of bleeding that can quantitatively and qualitatively alter platelets, impairing their function. The management of patients with IPDs usually consists of general measures aimed at avoiding bleeding and the use of supportive therapy to control hemorrhagic episodes. Moreover, patients and their parents must be instructed about drugs that impair platelet functions.

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