Otolaryngological findings in mucopolysaccharidosis

Cemal CİNGİ, (Eskişehir Osmangazi Üniversitesi, Tıp Fakültesi, Kulak Burun Boğaz Hastalıkları Anabilim Dalı, Eskişehir, Türkiye)
NURAY BAYAR MULUK, (Kırıkkale Üniversitesi, Tıp Fakültesi, Kulak Burun Boğaz Hastalıkları Anabilim Dalı, Kırıkkale, Türkiye)
Deniz HANCI, (Liv Hastanesi, Kulak Burun Boğaz Hastalıkları Kliniği, İstanbul, Türkiye)
Ethem ŞAHİN, (Bayındır Göztepe Hastanesi, Kulak Burun Boğaz Hastalıkları Kliniği, İstanbul, Türkiye)
Mustafa ACAR (Boş)
Journal of Medical Updates
0 0
Yıl: 2014 Cilt: 4 Sayı: 3 Sayfa Aralığı: 122 - 129 Metin Dili: Türkçe İndeks Tarihi: 29-07-2022

Otolaryngological findings in mucopolysaccharidosis

Öz:
Bu derleme makalede MPS hastalarındaki otolarengolojik sorunları raporladık. Mukopolisakkaridozlar (MPS'ler) her biri mukopolisakkaritlerin parçalanmasında rol alan ve şimdilerde glikozaminoglikanlar denilen (GAG'ler) kalıtsal bir enzim eksikliği nedeniyle oluşan bir lizozom depo hastalıkları grubudur. Mukopolisakkaridozlar, mukopolisakkaridoz tip 1-VII diye bilinen 7 metabolizma hastalığı grubunu oluştururlar. Grupların tümünde klinik ve otolaringolojik belirtiler görülür. Mukopolisakkaridoz hastalarında sık görülen otolarengolojik bulgular olarak üst hava yolu obstrüksiyonu, obstrüktif uyku apnesi, ağız açmanın kısıtlanması, orta kulak efüzyonu, işitme ve soluma sorunları vb. bildirilmektedir. KBB uzmanları arasında MPS'ler konusunda farkındalığın artırılması çocuk doktorundan ziyade bir KBB uzmanına giden MPS'den kuşkulanılan hastalar için yaşam kurtarıcı bir çaba olacaktır. Mukopolisakkaridoz hastalarında kısa boyun nedeniyle trakeotomi yapmak zorlaşabilir. Ağız açmanın kısıtlanması nedeniyle tonsillektomi ve adsenoidektomi ameliyatlarından önce hastalar dikkatle değerlendirilmelidir. Anesteziden önce hava yolu sorunları değerlendirilmelidir. Tüm KBB uzmanlarının bu sorunların farkınnda olmaları konusunda dikkati çekilmelidir.
Anahtar Kelime:

Konular: Genel ve Dahili Tıp

Mukopolisakkaridoz hastalarındaki otolarengolojik bulgular

Öz:
In this review paper, we reported otolaryngological problems in patients with mucopolysaccharidoses (MPSs). Mucopolysaccharidoses are a group of lysosomal storage diseases, each of which is produced by an inherited deficiency of an enzyme involved in the degradation of acid mucopolysaccharides, now called glycosaminoglycans (GAGs). The mucopolysaccharidoses consist of a group of 7 metabolic disorders, known as mucopolysaccharidoses types I-VII. In all groups, there are clinical and otolaryngological manifestations. In MPS patients, upper airway obstruction, obstructive sleep apnea, restriction of mouth opening, middle ear effusion, hearing and breathing problems, etc. are reported as common otolaryngological findings. Increasing awareness of MPS's among ENT doctors will be a life saving attempt for MPS suspected patients who admit an ENT doctor rather than a pediatrician. In MPS patients, tracheotomy may be difficult due to short neck. Due to mouth opening restriction, patients should be evaluated carefully before tonsillectomy and adenoidectomy operations. Airway problems must be evaluated before anesthesia. All ENT doctors should be noticed to be aware of these problems.
Anahtar Kelime:

Konular: Genel ve Dahili Tıp
Belge Türü: Makale Makale Türü: Derleme Erişim Türü: Erişime Açık
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APA CİNGİ C, BAYAR MULUK N, hancı d, ŞAHİN E, ACAR M (2014). Otolaryngological findings in mucopolysaccharidosis. , 122 - 129.
Chicago CİNGİ Cemal,BAYAR MULUK NURAY,hancı deniz,ŞAHİN Ethem,ACAR MUSTAFA Otolaryngological findings in mucopolysaccharidosis. (2014): 122 - 129.
MLA CİNGİ Cemal,BAYAR MULUK NURAY,hancı deniz,ŞAHİN Ethem,ACAR MUSTAFA Otolaryngological findings in mucopolysaccharidosis. , 2014, ss.122 - 129.
AMA CİNGİ C,BAYAR MULUK N,hancı d,ŞAHİN E,ACAR M Otolaryngological findings in mucopolysaccharidosis. . 2014; 122 - 129.
Vancouver CİNGİ C,BAYAR MULUK N,hancı d,ŞAHİN E,ACAR M Otolaryngological findings in mucopolysaccharidosis. . 2014; 122 - 129.
IEEE CİNGİ C,BAYAR MULUK N,hancı d,ŞAHİN E,ACAR M "Otolaryngological findings in mucopolysaccharidosis." , ss.122 - 129, 2014.
ISNAD CİNGİ, Cemal vd. "Otolaryngological findings in mucopolysaccharidosis". (2014), 122-129.
APA CİNGİ C, BAYAR MULUK N, hancı d, ŞAHİN E, ACAR M (2014). Otolaryngological findings in mucopolysaccharidosis. Journal of Medical Updates, 4(3), 122 - 129.
Chicago CİNGİ Cemal,BAYAR MULUK NURAY,hancı deniz,ŞAHİN Ethem,ACAR MUSTAFA Otolaryngological findings in mucopolysaccharidosis. Journal of Medical Updates 4, no.3 (2014): 122 - 129.
MLA CİNGİ Cemal,BAYAR MULUK NURAY,hancı deniz,ŞAHİN Ethem,ACAR MUSTAFA Otolaryngological findings in mucopolysaccharidosis. Journal of Medical Updates, vol.4, no.3, 2014, ss.122 - 129.
AMA CİNGİ C,BAYAR MULUK N,hancı d,ŞAHİN E,ACAR M Otolaryngological findings in mucopolysaccharidosis. Journal of Medical Updates. 2014; 4(3): 122 - 129.
Vancouver CİNGİ C,BAYAR MULUK N,hancı d,ŞAHİN E,ACAR M Otolaryngological findings in mucopolysaccharidosis. Journal of Medical Updates. 2014; 4(3): 122 - 129.
IEEE CİNGİ C,BAYAR MULUK N,hancı d,ŞAHİN E,ACAR M "Otolaryngological findings in mucopolysaccharidosis." Journal of Medical Updates, 4, ss.122 - 129, 2014.
ISNAD CİNGİ, Cemal vd. "Otolaryngological findings in mucopolysaccharidosis". Journal of Medical Updates 4/3 (2014), 122-129.
Sayfa Oluşturulma Tarihi
22-04-2024 22:32

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