Central venous catheter fragmentation in boy with hemophilia A and inhibitors (CROSBI ID 718551)
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Podaci o odgovornosti
Jelić, Matej ; Šarić, Dalibor ; Mucavac, Lucija ; Dejanović- Bekić, Sara ; Pavlović, Maja ; Turudić, Daniel ; Bilić, Ernest
engleski
Central venous catheter fragmentation in boy with hemophilia A and inhibitors
Hemophilia A is characterized by deficiency in FVIII and approximately 30% of people with severe hemophilia A are affected by inhibitors. A 11- year-old boy was diagnosed with severe hemophilia A immediately after birth due to hematoma around right kidney and positive family history. He developed inhibitors on FVIII when he was 12 months of age and therapy was changed in activated prothrombin complex on demand. During next 9 years, recurrent bleeding in the both knees started to occur which was manifested with haemophilic arthropathy. After several attempt, immune tolerance induction (ITI) was approved by insurance company when he was 10 years old. We started with plasma-derived F VIII in dosage of 100 i.u. per kg BW daily. Titer of inhibitors was negative after 3 months of ITI, and half life of FVIII was normalised after 6 months. For ease of administering therapy, central venous catheter (CVC) was inserted in right subclavian vein. On the other hand, usage of CVC can be complicated by infections and thrombosis. One year after insertion of CVC, he became febrile and microbiology findings revealed that blood culture was contaminated with Staphylococcus aureus, Acinetobacter ursingii, Achromobacter xyloxidans and Fusarium proliferatum which was indication for emergency removal of CVC. Unfortunately, during that surgical intervention, breakage of a CVC occurred and the tip of the catheter was notably missing. Emergency MSCT of thorax showed tubular structure located in right ventricle with loop at the entrance of right atrium and descending into the inferior vena cava as well as confluence with hepatic veins. To avoid major surgery, the retained portion was successfully removed by the interventional cardiologist through a femoral vein. During procedure, patient received plasma- derived FVIII to maintain the value of FVIII above 80% and there was no bleeding. Prophylactic antibiotics (meropenem, teicoplanin, liposomal amphotericin B) were continued for 10 days. After three negative blood cultures, negative galactomannan (GM) and 1, 3-β-d-glucan (BDG) tests, he was released home and regularly controlled in day hospital. He is now using plasma-derived F VIII 1500 IU every other day as a prophylaxis of hemophilia A and is doing well without bleeeding and development of inhibitors.
central venous catheter ; hemophilia A
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Podaci o prilogu
A128-A128.
2021.
nije evidentirano
objavljeno
10.1136/archdischild-2021-europaediatrics.303
Podaci o matičnoj publikaciji
Archives of disease in childhood
0003-9888
1468-2044
Podaci o skupu
10th Congress of European Paediatric Association EPA/UNEPSA jointly held with 14 th Congress of Croatian Paediatric Society
poster
07.09.2021-09.10.2021
Zagreb, Hrvatska
Povezanost rada
Biotehnologija u biomedicini (prirodno područje, biomedicina i zdravstvo, biotehničko područje)