The Prevalence of Paroxysmal Nocturnal Hemoglobinuria Clone in Adult Patients with Idiopathic Pulmonary Hypertension

Aim: Paroxysmal nocturnal hemoglobinuria (PNH) a is a clonal disorder that may lead to several conditions such as thromboses, Budd-Chiari syndrome, renal failure, impotence, and pulmonary hypertension (pHT). Since the presentation of PNH may be occult, monitoring for clonal evolution is recommended in certain situations including aplastic anemia, Myelodysplastic syndrome, and unexplained cytopenia, and thrombosis. The prevalence of PNH clone in patients with idiopathic pHT is unknown. We designed a study to determine the prevalence of PNH clone in patients with idiopathic pHT, since it may be the first isolated presentation of the disease. Methods: A total of 45 patients with pHT were screened for PNH clone by proaerolysin conjugated with fluorescein. Results: Only two out of 45 patients had elevated lactate dehydrogenase (LDH) levels at presentation. PNH clone was detected in none of the patients. Conclusion: Screening for PNH clone in patients with pHT, who have normal LDH levels is unnecessary

İnfektif endokarditin epidemiyolojik, klinik ve prognostik sonuçları: 90 atağın retrospektif kohortu

To evaluate the epidemiological, clinical, microbiological, and echocardiographic features, as well as the prognosis and long- term outcome of patients with infective endocarditis. Methods: The clinical records and follow-up data of 90 endocarditis episodes in 86 patients diagnosed with definite and possible infec- tive endocarditis according to the modified Duke criteria in a tertiary university hospital, between 1998 and 2016, were reviewed. Results: Fifty-six patients were male (65.1%), and the mean age was 49.9 ± 14.3. Native valve endocarditis constituted 62.2% of the cases, while the remaining patients had prosthetic valve endocarditis. The aortic (34.4%) and mitral (24.4%) valves were infected more frequently. Streptococci (27.7%) and staphylococci (24.4%) were the most frequently isolated microorganisms. Embolic complications (35.5%) were the leading cause of morbidity, followed by valve insufficiency (28.8%) and heart failure (21.1%). Valve replacement surgery was performed in 28 patients (31%). The in-hospital mortality rate was 15.1% (n = 13). Chronic renal failure (P = .042) and degenerative valves (P = .036) were significantly associated with mortality. Among 43 of the 73 cases available for telephonic survey, 36 (83.7%) patients were alive and without disease, with a median follow-up of 52.9 (4-163) months. Twenty-five (69.4%) of these patients were younger than 55 years, and 24 (66.6%) had native valve endocarditis. Conclusion: Underlying cardiac conditions and chronic renal failure increase mortality in infective endocarditis, regardless of the patho- gen. Long-term survival seems promising in cases with native valve endocarditis and in younger patients with low rates of comorbidities.İnfektif endokardit tanısıyla izlenen hastaların epidemiyolojik, klinik, mikrobiyolojik, ekokardiyografik özellikleri, prognozu ve uzun dönem sonuçlarını değerlendirmek. Yöntemler: 1998 ve 2016 yılları arasında bir üniversite hastanesinde modifiye Duke kriterlerine göre kesin ve olası infektif endokardit tanısı konan 86 hastanın 90 endokardit atağının klinik kayıtları ve takip verileri retrospektif olarak incelendi. Bulgular: Çalışmamızda hastaların 56’sı (%65,1) erkek ve ortalama yaş 49,9 ± 14,3 idi. Doğal kapak endokarditi olguların %62,2’sini oluştururken, diğerlerinde protez kapak mevcuttu. En sık aort (%34,4) ve mitral (% 24,4) kapak tutulumu saptandı. Etken olarak strep- tokoklar (%27,7) ve stafilokoklar (%24,4) en sık izole edilen mikroorganizmalardı. Tüm komplikasyonlar içinde embolik komplikasyonlar (%35,5) ilk sırada yer almış, bunu kapak yetmezlikleri (%28,8) ve kalp yetmezliği (%21,1) izlemiştir. Hastaların 28’ine (%31) kapak replasman operasyonu yapılmış ve tüm hastalar içinde 13 hasta (%15,1) hastane takibinde kaybedilmiştir. Mortalite kronik böbrek yetmezliği (P = ,042) ve dejeneratif kapak hastalarında (P = ,036) istatistiksel olarak daha anlamlı bulundu. Taburculuk sonrası prognoz ve uzun dönem sonuçlar değerlendirildiğinde sağ kalan 73 hastanın 43’üne ulaşılabilmiş ve bu hastalardan 36’sının (%83,7) ortalama 52,9 ay (4-163) hayatta ve genel durumlarının iyi olduğu, 25 hastanın (%69,4) <55 yaş, 24’ünün (%66,6) doğal kapak endokarditi tanısıyla izlenmiş olduğu görülmüştür. Sonuç: İnfektif endokardit hastalarında altta yatan kalp hastalıkları ve kronik böbrek yetmezliği patojenden bağımsız olarak mortalit- eyi arttırmaktadır. Doğal kapak endokarditi ve komorbiditeleri daha az olan genç yaş hastalarda ise uzun dönem sağkalım umut verici görünmektedir

Six-minute walk test in pulmonary arterial hypertension

Exercise intolerance is the main characteristic of pulmonary arterial hypertension (PAH). The six-minute walk test (6MWT) and cardiopulmonary exercise test are widely used in assessing exercise capacity of PAH patients. Six-minute walk distance (6MWD) has been specified as the main clinical outcome in PAH and has been used as the primary end-point in many studies conducted for new PAH treatments. Using 6MWD as the end-point in clinical studies has many advantages. 6MWT is an inexpensive, easily applicable, and repeatable standardized test that is well-tolerated by PAH patients. Moreover, it is a valid measure of symptomatic improvement. It is correlated with variables of maximal cardiopulmonary exercise test as a measure of submaximal exercise capacity and disease severity markers such as functional class and pulmonary hemodynamics. It is widely used in clinical practice together with other invasive and non-invasive disease markers in assessing disease progression and response to treatment. In addition, it has prognostic importance and is a good prognostic marker. On the other hand, there are limitations to the use of 6MWD as the primary end-point in PAH treatment. It has decreased sensitivity in individuals with less severe disease and high 6MWD at baseline and decreased adequacy in assessing the effects of treatment in patients who are still under PAH treatment. Despite the limitations, 6MWD plays a key role in the evaluation and management of PAH patients