Difficult Airway and Pulmonary Hypertension Coexistence in a Child With I-Cell Disease

Year 2016, Volume: 69 Issue: 1, 41 - 43, 03.06.2016

Ayhan Yaman , Fatma Tuba Eminoğlu , Tanıl Kendirli , Çağlar Ödek , Tayfun Uçar , Erdal İnce

Abstract

I-cell disease (mucolipidosis type II) is an autosomal recessive lysosomal enzyme targeting disorder leading to fatal outcome in childhood mostly due to respiratory insufficiency. The most common features of the condition are mental and physical retardation with typical orofacial features. Typical cardiac involvement includes thickening and deformation of mitral and aortic valves and dilated or hypertrophic cardiomyopathy. Mucopolysaccharidoses have been described as the worst airway problems in pediatric anesthesia, and there have been a number of previous reviews that have demonstrated a high incidence of airway problems. Here, we report the clinical course of an infant with confirmed I-cell disease (mucolipidosis type II) complicated by difficult airway and severe pulmonary hypertension, which is very rarely associated with this disorder.

Keywords

Children , Difficult Airway , I-cell Disease , Pulmonary Hypertension.

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Zor Hava Yolu ve Pulmoner Hipertansiyon Birlikteli􀃾i olan I-Cell Hastal􀃾 Olgusu

Abstract

cell hastal􀃾 (mukolidipoz tip 2) otozomal resesif kaltml, çocukluk ya􀄹 grubunda ço􀃾unlukla solunum yetmezli􀃾ine ba􀃾l ölümcül seyredebilen lizozomal enzim bozuklu􀃾udur. Hastal􀃾n en sk görülen bulgular mental ve motor retardasyon ile birlikte tipik orofasiyal anomalilerdir. Tipik kardiyak bulgular mitral kapak kalnla􀄹mas, aortik kapak kalnla􀄹mas, hipertrofik ve dilate kardiyomiyopatidir. Mukopolisakkaridozlar pediatrik anestezide görülen en zor hava yolu problemleri olarak tanmlanmaktadr ve bunu teyit eden daha önce yaynlanm􀄹 birçok yaynda hava yolu problemlerinin yüksek oldu􀃾u bildirilmi􀄹tir. Bu çal􀄹mada I-cell hastal􀃾 (mukolidipoz tip 2) tans alan, zor hava yolu ve bu hastalkla birlikte nadir bildirilen a􀃾r pulmoner
hipertansiyonu olan bir bebek sunulmu􀄹tur.

Keywords

Çocuk , I-cell hastal􀃾 , Pulmoner hipertansiyon , Zor hava yolu.

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References

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