Suksham JAIN, Deepak CHAWLA

Transient hyperammonemia of newborn

Yenidoğanın geçici hiperamonyemisi (YDGH) doğum sonrası ilk birkaç günde ağır hiperamonyemi, koma, organic asidüriinin bulunmayışı ve genellikle tam iyileşme ile karakterizedir. Bazı üre siklüs defekti kalıtsal hastalıklarının aksine karaciğerde spesififk bir yapısal değişiklik saptanamaz. Bu çalışmada 10-15 sn süreli yaygın klonik konvülziyonlar ve takip eden mekanik ventilasyon gerektirecek düzeyde solunum yetmezliği ile belirti veren, sonrasında tam iyileşme gösteren bir YDGH olgusu sunulmuştur

Yenidoğanın geçici hiperamonyemisi

Transient hyperammonemia of neonate (THAN) presents within first few days of postnatal life in form of severe hyperammonemia, a comatose state, absence of abnormal organic aciduria, normal activity of urea cycle enzymes and usually, completes recovery. In contrast to some of the hereditary disorders of the urea cycle, no specific structural changes could be found in THAN in liver. We herein reported a neonate with THAN presented with episode of a generalized clonic seizure of 10-15 secondds duration followed by respiratory failure needing mechanical ventilation.

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