Orak Hücre Hastalıkları

Orak hücre hastalıkları (OHH), damar endoteli üzerine uzun süreli, yıkıcı bir süreçtir ve özellikle kılcal damar ağı düzeyinde hasara neden olur. Bu uzun süreli ve yıkıcı süreç doğumda başlar ve yaşamın erken dönemlerinde hızlanmış endotel hasarına bağlı çoklu organ yetmezlikleriyle sonlanır. Kronik tıkayıcı akciğer hastalığı, bacak yarası, çomaklaşma, priapizm, koroner kalp hastalığı, kronik böbrek hastalığı, çevresel damar hastalığı, siroz ve inme muhtemelen OHH’de sonlanma noktalarından sadece birkaçıdır. OHH’de gelişebilecek neredeyse tüm akut olaylar ve acillerde vücutta gelişen yaygın doku hipoksisinin önlenmesinde etkili tedavi yöntemi kırmızı küre desteği olmalıdır. Uzun dönem tedavide, OHH’de gelişebilecek sonlanma noktalarının tümüyle önlenmesinde olmasa da geciktirilmesinde ağızdan alınan hidroksiüre ucuz, güvenli ve etkili bir ilaçtır.Anahtar Kelimeler: Orak hücre hastalıkları, kronik endotel iltihabı

Anahtar Kelimeler:

Orak hücre hastalıkları, kronik endotel iltihabı

Sickle Cell Diseases

Sickle cell diseases (SCDs) are chronic destructive process on endothelium particularly at the capillary level. The chronic catastrophic process initiates at birth, and terminates with accelerated endotelial damage induced multiorgan failures in early years of life. Chronic obstructive pulmonary disease, leg ulcers, digital clubbing, priapism, coronary heart disease, chronic renal disease, peripheric artery disease, cirrhosis, and stroke are just some of the terminal end points of the diseases. Red blood cell transfusions should be the treatment of choice nearly in all acute events and emergencies of the diseases to prevent disseminated tissue hypoxia all over the body. In long term treatment, an oral hydroxyurea is cheap, safe, and effective drug, not to prevent but to delay the end points of the diseases.Key Words: Sickle cell diseases, chronic endothelial inflammation

Keywords:

Sickle cell diseases, chronic endothelial inflammation,

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