Abstract Number: PB0176
Meeting: ISTH 2020 Congress
Background: In 21st century hematological diagnostic tools are just amazing. But what should we do when we receive abnormal clotting tests in healthy child? There many guidelines about II,VII,X clotting factors deficiencies, but not so much about XII and V.
Aims: We want to find out the clinical significance of laboratory changes, management of pre-and post surgical treatment in children with a deficiency of XII and V clotting factors.
Methods: We have reviewed 22 child case histories with rare coagulation disorders. Study was carried out on the basis of the hematology department of Sant- Petersburg State Children Hospital in 2014-2019 years.
Results: All children had significant changes in basic coagulation tests, that were made because of planned surgery. They were sent to hematologist, who made more tests to clarify clotting deficiency. 19 children had mild and severe deficiency of XII factor (less 1-4%) and 3 children had lower level of V factor (21- 35%). No history of bleeding was seen in any of the patients. Parents of that children were scared and wanted to have hemostatic agents. All children were successfully surgical treated, without any bleeding episodes and use of any hemostatic medication. In all the cases good venous access and fresh frozen plasma were prepared in case of bleeding. The trust to hematologist after the procedures was 100%.
Conclusions: Despite the knowledge that deficiency of XII factor is asymptomatic there are difficulties in pre and post operative management in many hospitals in Russia. Parents are very scared when receive abnormal basic clotting tests. Children with “light” deficiency of V clotting factor do not require any hemostatic agents, but for safety of the surgeon good venous access and preparation of fresh frozen plasma is made in our hospital. More information of rare coagulation disorders must be provide to other specialties like pediatricians and surgeons.
To cite this abstract in AMA style:Sokolova N, Vaynyunskaya N. Rare Coagulation Disorders in Children – Is it Like to Buy a Pig in a Poke or Nothing Special? Experience of Saint Petersburg, Russia [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/rare-coagulation-disorders-in-children-is-it-like-to-buy-a-pig-in-a-poke-or-nothing-special-experience-of-saint-petersburg-russia/. Accessed October 1, 2023.
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