Mahmut DEMİR, Ahmet GÜZELÇİÇEK, Hüseyin GÜMÜŞ, Halil KAZANASMAZ, Abdullah SOLMAZ

G6PD eksikliği olan hastada favizme bağlı aneminin spontan düzelmesi

Glukoz-6-fosfat dehidrogenaz (G6PD) enzim eksikliği en sık görülen enzim yetersizliğidir ve G6PD enzimi pentoz fosfat yolunun ilk basamağını katalize eden anahtar bir enzimdir. G6PD enzim eksikliği; klinik olarak yenidoğan hiperbilüribinemisi, akut veya kronik hemoliz gibi bir dizi bozukluklara neden olur. G6PD enzimi pentoz fosfat yolunda ilk adım olan yolu katalize eder ve hücreleri oksidatif hasardan koruyan antioksidanların oluşumuna neden olur. G6PD enzim eksikliği olan hastalarda bazı ilaçlar, bazı metabolik durumlar ve bazı enfeksiyonların etkileriyle kırmızı kan hücrelerinde gelişen oksidatif strese bağlı hemoliz olur. Bu yazıda, G6PD enzim eksikliği ve orak hücre anemisi taşıyıcılığı olan olgumuzda, bakla yeme sonrası gelişen hemolize bağlı oluşan aneminin bakla maruziyeti kesildikten sonra anemisi spontan düzelen bir olgu sunuldu.

Anahtar Kelimeler:

Glukoz-6-fosfat dehidrogenaz eksikliği, Orak hücreli anemi, Favizm, Hemolitik kriz

The anemia improved spontaneously dependent on the favism in patient with G6PD deficiency

Glucose-6-phosphate dehydrogenase (G6PD) enzyme deficiency is the most common enzyme deficiency and the G6PD enzyme is a key enzyme catalyzing the first step of the pentose phosphate pathway. Clinically, G6PD enzyme deficiency causes a series of disorders such as newborn hyperbilirubinemia, acute or chronic hemolysis. The G6PD enzyme catalyzes the first step of the pentose phosphate pathway and leading to the formation of antioxidants that protect cells from oxidative damage. In patients with G6PD enzyme deficiency some drugs, some metabolic conditions and some infections cause oxidative stress-related hemolysis in red blood cells. In this article, we presented a case in which the anemia spontaneously regresses after the exposure of the pine to the anemia due to haemolysis after the eating of the pod in our case with G6PD enzyme deficiency and sickle cell anemia carrier.

Keywords:

Glucose-6-phosphate dehydrogenase deficiency, Sickle cell anemia, Favism, Hemolytic crisis,

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