ICNC Abstracts, ICNC 2018

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Importance of Influenza vaccination in Urea Cycle Disorders
Selda Bülbül, Cansu Çelik, Elif Gökçe Ünal

Last modified: 2018-09-15


Urea cycle disorders (UCDs) result in hyperammonemia and life-threatening metabolicdecompensations with degradation effect of infections. Survivors of the metabolicdecompensation frequently have severe neurologic injury. Therefore, the most critical aspectsof follow-up in these patients is to avoid infectious exposures. The aim of this paper was toreport the effects of viral infections in 3 UCD patients.Cases 1&2: 20 months old male twins (UT&UT) received the diagnosis of Argininosuccinate Lyase deficiency at 3 months of age. They were under control with proper dietand specific medicaments since then. However, unexpectedly they were applied withvomiting and lethargy 15 days ago. Their blood ammonia level was 657 and 625 Mmol/Lt,AST and ALT levels were 160 U/L and 142 U/L/ -538,71 U/L and 530,91 U/L respectively).Viral PCR evaluation revealed haemophilus influenzae infection (HII) . They were stabilizeda week later with N-asetylcystein (NAC) and high dose Vitamine C treatment.Case 3: 3 years old girl (EMY) received the diagnosis of Arginino succinate sentetasedeficiency after birth. She has been followed-up in our clinic for 21 months with slightneurological deficits. She appiled with vomiting and fever 6 months ago. Her bloodammonia level was 1136,9 Mmol/Lt, AST and ALT levels were 2602U/L and 2220,74U/L.Her viral PCR also showed HII and was stabilized 3 days later with the same treatment.All 3 patients were not vaccinated agianst HII, therefore we wanted to highlight theimportance of vaccination against HII in UCD patients to avoid hyperammonia.

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