DOCK8 Eksikliği Sonucu Gelişen Çoklu Besin Alerjisi Olan Bir Olguda Kök Hücre Tedavisi Döneminde Karşılaşılan Nütrisyonel Sorunlar ve Uygulamalar

Yıl 2025, Cilt: 5 Sayı: 1, 46 - 54, 18.04.2025

Furkan Yolcu , Hayriye Özer Işık , Gülhan Samur

Öz

DOCK8 eksikliği, yüksek morbidite ve mortaliteye sahip nadir görülen bir kombine immün yetmezlik hastalığıdır. Klinik özellikleri arasında atopi, besin alerjisi, tekrarlayan enfeksiyonlar, otoimmünite riski ve kanser bulunmaktadır. Bilinen tek tedavisi Hematopoetik Kök Hücre Naklidir (HKHN). DOCK8 eksikliğinde HKHN süreci yan etkileri ve komplikasyonları fazla olan bir tedavi sürecidir. Bu süreçte hastalar nütrisyonel açıdan nakilin ilk gününden son gününe kadar izlenmelidir. Hastanın aldığı tedaviye, karşılaşılan semptom ve komplikasyonlara uygun olacak şekilde tıbbi beslenme tedavisi yapılmalıdır. Bu olgu sunumunda DOCK8 eksikliği tanısı ile izlenen olgunun tedavisinde tıbbi beslenme tedavisinin uygulanması ve önemi tartışılmıştır.

Anahtar Kelimeler

DOCK8 eksikliği, Hematopoetik kök hücre nakli, Beslenme

Nutritional Problems and Aplications in Stem Cell Transplantation Period in A Case With Multiple Food Allergies as a Result of DOCK8 Deficiency

Öz

DOCK8 deficiency is a rare combined immunodeficiency with high morbidity and mortality. Clinical features include atopy, food allergy, recurrent infections, risk of autoimmunity, and cancer. The only known treatment is Hematopoietic Stem Cell Transplant (HSCT). In DOCK8 deficiency, HSCT is a treatment process with many side effects and complications. In this process, patients should be monitored nutritionally from the first day of the transplant to the last day. Medical nutrition therapy should be performed in accordance with the treatment, symptoms and complications encountered. In this case report, the application and importance of medical nutrition therapy in the treatment of a case followed up with DOCK8 deficiency was discussed.

Anahtar Kelimeler

DOCK8 deficiency, Hematopoietic stem cell transplant, Nutrition

Kaynakça

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