General Anesthesia Management in a Patient with Triple A Syndrome

Öz

Triple A (Allgrove, AAA) syndrome is a rare autosomal recessive disorder that coexists with achalasia, alacrimia and adrenocorticotropic hormone–resistant adrenal insufficiency. Neurological disorders and autonomic neuropathy are often conditions with this syndrome. Adrenal insufficiency; may cause an adrenal crisis in conditions such as surgery, infection, or trauma. This can put anesthetists in difficult situations during anesthesia for various surgeries. With this case, we aimed to share our experiences regarding the points to be considered in an anesthesia method with triple A syndrome.

Anahtar Kelimeler

• Achalasia
• Adrenal crisis
• Alacrimia

Triple A Sendromlu Hastada Genel Anestezi Yönetimi – Olgu Sunumu

Öz

Triple A (Allgrove, AAA) sendromu akalazya, alakrima ve adrenal yetmezliğin birlikte görüldüğü nadir otozomal resesif bir bozukluktur. Nörolojik bozukluklar ve otonom nöropati de bu sendromla birlikte sık görülen durumlardır. Adrenal yetmezlik; ameliyat, enfeksiyon veya travma gibi durumlarda adrenal krize sebep olabilir. Bu da çeşitli ameliyatlar için anestezi sırasında anestezistleri zor durumlarla karşı karşıya getirebilir. Bu olgu ile Triple A sendromlu bir çocuğun anestezi yönteminde dikkat edilmesi gereken noktalarla ilgili deneyimlerimizi paylaşmayı amaçladık.

Anahtar Kelimeler

• akalazya
• adrenal kriz
• alakrima
• Triple A syndrome.

Kaynakça

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