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Polisiteminin Nadir bir Nedeni: İdiyopatik Pulmoner Arteriyel Hipertansiyon

A Rare Cause of Polycythemia: Idiopathic Pulmonary Arterial Hypertension

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Abstract (2. Language): 
Idiopathic pulmonary arterial hypertension (IPAH) with an incidence of 1-2/million per-year. IPAH is a term used to define a variety of progressive conditions that have in common, increased pulmonary vascular resistance leading to right heart failure and death. Pulmonary arterial hypertension is presented with cyanosis, polyctemia and right heart failure. Increased prevelance of mortality is due to progression and late diagnosis of the disease. We present this case to alert physicians to keep in mind rare but serious disease IPAH in the differential diagnosis of polyctemia.
Abstract (Original Language): 
İdiyopatik pulmoner arteriyel hipertansiyon (İPAH), yıllık insidansı 1-2/milyon olan oldukça nadir görülen, tedavi edilmediğinde hızlı bir seyirle sağ kalp yetmezliği ve ölüme kadar giden morbidite ve mortalitesi yüksek bir hastalıktır. Klinik bulgular tüm pulmoner hipertansiyon tiplerinde olabileceği gibi siyanoz, polisitemi ve sağ kalp yetmezliği belirtileri ile seyredebilir. Mortalitenin yüksek olması, hastalığın seyrinden ve tanının sıklıkla geç evrede konmasından kaynaklanmaktadır. Bu vakayı, özellikle genç yaşta olan olgularda polisitemi etyolojisi araştırılırken nadir görülen İPAH hastalığına dikkat çekmek için sunduk.
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REFERENCES

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