Erişkin orak hücre anemili hastalarda pulmoner komplikasyonlar

Orak hücreli anemi (OHA) tekrarlayan ağrılı krizler, süregiden hemolitik anemi ve çoklu organ yetmezliğine yol açan kalıtımsal bir hemoglobin hastalığıdır. Önceleri OHA’lı bir hastanın tahmini yaşam süresinin dördüncü dekatı aşması beklenen bir durum değilken; günümüzde, gelişmiş sağlık bakım sistemleri olan ülkelerde hastaların çoğu erişkin yaşlara ulaşabilmektedir. Hastaların erişkin yaşlara ulaşmaları ile birlikte geri dönüşümsüz organ hasarları ile daha sık karşılaşılmaya başlanmıştır. Akciğerler OHA’da tutulan organlardan biridir. Pulmoner komplikasyonlar önemli bir morbidite ve mortalite nedeni olup, ölümlerden büyük oranda sorumludur. Bu konuyla ilgili bilgilerimizin son 10-15 yılda artmasına rağmen, OHA’lı hastalarda pulmoner komplikasyonların spesifik bir tedavisi henüz yoktur. Özellikle erişkin OHA’lı hastalarda sağlık bakımı konusunda sınırlı verilerin olması hem hastalar ve aileleri ve hem de sağlık bakım hizmeti verenler için sorun yaratır. Pulmoner komplikasyonların sıklığı ve ağırlığı, hekimlerin bu konuya daha çok ilgisini gerektirmektedir. Bu derlemede OHA’nın aşağıda belirtilen pulmoner komplikasyonlarına yer verilmiştir: a) akut göğüs sendromu, b) venöz tromboemboli, c) astım, d) solunum fonksiyon bozukluğu, e) uykuda solunumsal bozukluklar, f) pulmoner hipertansiyon.

Pulmonary complications in adult patients with sickle cell disease

Sickle cell disease (SCD) is an inherited hemoglobin disorder, associated with recurrent painful episodes, ongoing hemolytic anemia and progressive multi-organ failure. While survival beyond the fourth decade of life for a patient with SCD was previously considered unusual; nowadays, in countries with developed health care systems, patients with SCD could reach into adulthood. However, irreversible organ damages have become more prevalent along with the patients’ reaching adulthood. The lungs as well are among the organs involved in SCD. Pulmonary complications are significant causes of morbidity and mortality and are substantially responsible for deaths. Although our understanding of these conditions has improved over the past 10-15 years, there remains no specific treatment of pulmonary complications of SCD. Limited data on health care issues, particularly in older adults with SCD, poses multiple challenges to patients, their families and health care providers. The incidence and severity of pulmonary complications require the physicians’ further interest in this subject. The present review comprises the following pulmonary complications of SCD: i) acute chest syndrome, ii) venous thromboembolus, iii) asthma, iv) respiratory dysfunction, v) respiratory disorders during sleeping, vi) pulmonary hypertension.

Keywords:

Sickle cell disease, older adults pulmonary complication,

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