Diyabetik ketoasidoz ve talasemi majorlu bir yenidogan: Nadir bir olgu/A newborn with diabetic ketoacidosis and thalassemia major: A rare case

ÖZET. Diyabetik ketoasidoz mutlak insülin yetersizligine bagli gelisen ve genellikle hiperglisemi, ketonemi, asidemi, glikozüri, ketoüri ile karakterize sistemik bir durumdur. Talasemi Major "beta globulin" zincirinin yapilamamasi veya az yapilmasi nedeniyle eritrosit ömrünün kisa olmasindan kaynaklanan, hayatin 3.-4. ayinda anemi bulgulari görülmeye baslayan ve zamanla sürekli kan nakli gerektiren çok ciddi kalitsal bir kan hastaligidir. Olgu sunumumuzda 24 yasindaki talasemi tasiyicisi bir baba ve 23 yasindaki talasemi tasiyicisi ve gestasyonel diyabetli bir anneden dogan, anemi, hiperglisemi, kusma ve asidoz tablosuyla servisimize yatirilan ketonüri olmadigi halde diyabetik ketoasidoz ve erken dönemde eritrosit transfüzyonu gerektiren talasemi majorlu 20 günlük nadir görülen bir olguyu sunduk. Yenidoganlarda diyabetik ketoasidoz tablosunun ketonüri olmadan da görülebilecegi ve talasemi major olgularinda hiperglisemi ve asidozla birlikte erken dönemde aneminin de ortaya çikabilecegine vurgu yapmak için olgu sunuldu. Anahtar kelimeler: Talasemi major, diyabetik ketoasidoz, yenidogan. ABSTRACT. Diabetic ketoacidosis is a systemic situation caused by absolute insulin deficiency and characterized by hyperglycemia, ketonemia, acidemia, glycosuria and ketonuria. Thalassemia Major is a very serious hereditary blood disorder due to low levels or absence of "beta globulin" chain, characterized by requiring a blood transfusion from 3-4. month of life due to the relatively short life of red cells. We, herein presented a rare case of 20 day-old newborn with anemia, hyperglycemia, vomiting, acidosis being diagnosed as thalassemia major that required blood transfusion in the early period of life and diabetic ketoacidosis without ketonuria who born from 24 year old father carrier of thalassemia and 23-year-old mother with carrier of thalassemia and gestational diabetes. The case was presented in order to emphasize that diabetic ketoacidosis can occur in newborns without ketonuria and thalassemia major may cause anemia in the early period of life due to hyperglycemia and acidosis. Key words: Thalassemia major, diabetic ketoacidosis, newborn.

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