Feminizing Genitoplasty in a young girl with Glanzmann’s thrombasthenia-management of haemostasis

Kashaf Khalid Turk; Zafar Nazir

doi:10.5455/JPMA.34911

Feminizing Genitoplasty in a young girl with Glanzmann’s thrombasthenia-management of haemostasis

Kashaf Khalid Turk, Zafar Nazir

Section of Paediatric Surgery, Department of Surgery, Medical College, Pakistan

Research output: Contribution to journal › Article › peer-review

Abstract

We report peri- and post-operative management of haemostasis in a 11-year old girl with Glanzmann Thrombasthenia (GT) who had feminizing genitoplasty for genital ambiguity due to Congenital Adrenal Hyperplasia (CAH-21 Hydroxylase deficiency). A blend of Glanzmann Thrombasthenia (GT) and DSD 46XX due to CAH is not reported in literature. Surgery particularly genitourinary reconstruction in patients with GT is challenging due to risk of intra and post-operative bleeding. Haemostasis can successfully be achieved with platelet transfusions, antifibrinolytic (Tranexamic acid) and judicious use of recombinant factor VIIa (rFVIIa) even in a resource limited setting.

Original language	English
Pages (from-to)	1084-1085
Number of pages	2
Journal	Journal of the Pakistan Medical Association
Volume	70
Issue number	6
DOIs	https://doi.org/10.5455/JPMA.34911
Publication status	Published - Jun 2020

Keywords

Disorders of Sexual Differentiation (DSD)
Feminizing Genitoplasty
Glanzmann's Thrombasthenia
Inherited Platelet disorders
Recombinant Factor VIIa (rFVIIa)
Urogenital Sinus

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title = "Feminizing Genitoplasty in a young girl with Glanzmann{\textquoteright}s thrombasthenia-management of haemostasis",

abstract = "We report peri- and post-operative management of haemostasis in a 11-year old girl with Glanzmann Thrombasthenia (GT) who had feminizing genitoplasty for genital ambiguity due to Congenital Adrenal Hyperplasia (CAH-21 Hydroxylase deficiency). A blend of Glanzmann Thrombasthenia (GT) and DSD 46XX due to CAH is not reported in literature. Surgery particularly genitourinary reconstruction in patients with GT is challenging due to risk of intra and post-operative bleeding. Haemostasis can successfully be achieved with platelet transfusions, antifibrinolytic (Tranexamic acid) and judicious use of recombinant factor VIIa (rFVIIa) even in a resource limited setting.",

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AU - Nazir, Zafar

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AB - We report peri- and post-operative management of haemostasis in a 11-year old girl with Glanzmann Thrombasthenia (GT) who had feminizing genitoplasty for genital ambiguity due to Congenital Adrenal Hyperplasia (CAH-21 Hydroxylase deficiency). A blend of Glanzmann Thrombasthenia (GT) and DSD 46XX due to CAH is not reported in literature. Surgery particularly genitourinary reconstruction in patients with GT is challenging due to risk of intra and post-operative bleeding. Haemostasis can successfully be achieved with platelet transfusions, antifibrinolytic (Tranexamic acid) and judicious use of recombinant factor VIIa (rFVIIa) even in a resource limited setting.

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KW - Feminizing Genitoplasty

KW - Glanzmann's Thrombasthenia

KW - Inherited Platelet disorders

KW - Recombinant Factor VIIa (rFVIIa)

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Feminizing Genitoplasty in a young girl with Glanzmann’s thrombasthenia-management of haemostasis

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Keywords

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