A STUDY OF BERNARD-SOULIER SYNDROME IN TEHRAN, IRAN

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TOUGEH GH.R.; KEYHANI MANOUCHEHR; SHARIFIAN R.A.; SAFAEI REZA; EMAMI A.H.; DALILI H.

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Journal Paper

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Journal: ARCHIVES OF IRANIAN MEDICINE Year:2010 | Volume:13 | Issue:6 Page(s): 549-551

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Title

A STUDY OF BERNARD-SOULIER SYNDROME IN TEHRAN, IRAN

Author(s)

Keywords

BERNARD SOULIER SYNDROME

CLINICAL MANIFESTATION

CONSANGUINITY

GIANT PLATELET

Abstract

Background: BERNARD SOULIER SYNDROME (BSS) is a hemorrhagic disorder with an autosomal recessive pattern of inheritance. We describe the demographic and clinical characteristics of Iranian patients with BSS followed in a major teaching and tertiary care hospital in Tehran, Iran.Methods: We performed a retrospective medical record review of 97 patients with BSS who received care at the Imam Khomeini Hospital between 1969 and 2001. We collected data on the family history, clinical presentation, bleeding episodes, and lab profiles of these patients.Results: Among all patients, 78 (81%) had a family history of CONSANGUINITY. The most common presenting symptom was epistaxis, seen in 62 (63.9%) patients. Peripheral blood smears demonstrated GIANT PLATELETs on 67 (68.7%) of patients. Complete blood count demonstrated decreased platelets in 85 (87.4%) of patients ranging from 20,000/mL to 130,000/mL. Anemia was seen in 62 (64%) and 91 (93.8%) had prolonged bleeding time. The majority of patients (60%) had mild bleeding episodes, but 39 (40%) had at least one episode of severe bleeding in their past history.Conclusion: Our data are consistent with other reports regarding clinical presentation of BSS, but CONSANGUINITY seems to be more common.

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APA: Copy

TOUGEH, GH.R., KEYHANI, MANOUCHEHR, SHARIFIAN, R.A., SAFAEI, REZA, EMAMI, A.H., & DALILI, H.. (2010). A STUDY OF BERNARD-SOULIER SYNDROME IN TEHRAN, IRAN. ARCHIVES OF IRANIAN MEDICINE, 13(6), 549-551. SID. https://sid.ir/paper/280616/en

Vancouver: Copy

TOUGEH GH.R., KEYHANI MANOUCHEHR, SHARIFIAN R.A., SAFAEI REZA, EMAMI A.H., DALILI H.. A STUDY OF BERNARD-SOULIER SYNDROME IN TEHRAN, IRAN. ARCHIVES OF IRANIAN MEDICINE[Internet]. 2010;13(6):549-551. Available from: https://sid.ir/paper/280616/en

IEEE: Copy

GH.R. TOUGEH, MANOUCHEHR KEYHANI, R.A. SHARIFIAN, REZA SAFAEI, A.H. EMAMI, and H. DALILI, “A STUDY OF BERNARD-SOULIER SYNDROME IN TEHRAN, IRAN,” ARCHIVES OF IRANIAN MEDICINE, vol. 13, no. 6, pp. 549–551, 2010, [Online]. Available: https://sid.ir/paper/280616/en

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