Naslov
Central venous catheter fragmentation in boy with hemophilia A and inhibitors

Autori
Jelić, Matej ; Šarić, Dalibor ; Mucavac, Lucija ; Dejanović- Bekić, Sara ; Pavlović, Maja ; Turudić, Daniel ; Bilić, Ernest

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
Archives of disease in childhood, 106 (2021), Suppl 2 / - , 2021, A128-A128

Skup
10th Congress of European Paediatric Association EPA/UNEPSA jointly held with 14 th Congress of Croatian Paediatric Society

Mjesto i datum
Zagreb, Hrvatska, 07.10.2021. - 09.10.2021

Vrsta sudjelovanja
Poster

Vrsta recenzije
Nije recenziran

Ključne riječi
central venous catheter ; hemophilia A

Sažetak
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.

Izvorni jezik
Engleski

Znanstvena područja
Biotehnologija u biomedicini (prirodno područje, biomedicina i zdravstvo, biotehničko područje)

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