Serum apelin as a novel non-invasive marker for subclinical cardiopulmonary complications in children and adolescents with sickle cell disease.

BACKGROUND: Cardiovascular involvement represents a leading cause of mortality and morbidity in sickle cell disease (SCD). Apelin is a peptide involved in the regulation of cardiovascular function. AIM: To determine serum apelin among 40 children and adolescents with SCD compared with 40 healthy controls and assess its relation to markers of hemolysis, iron overload as well as cardiopulmonary complications. METHODS: SCD patients, in steady state and asymptomatic for heart disease, were studied stressing on hydroxyurea/chelation therapy, hematological profile, serum ferritin and apelin levels. Full echocardiographic study including assessment of biventricular systolic function and pulmonary artery pressure was done. RESULTS: Apelin levels were significantly lower in SCD patients compared with controls (P<0.001). Cardiopulmonary complications were encountered in 30% of patients. Apelin was significantly decreased among patients with cardiopulmonary disease (P=0.006) whether those at risk of pulmonary hypertension (P=0.018) or patients with heart disease (P=0.043). Hydroxyurea-treated patients had higher apelin levels than untreated ones (P=0.001). Apelin was negatively correlated to lactate dehydrogenase, indirect bilirubin, serum ferritin, end systolic diameter, tricuspid regurgitant jet velocity, right ventricle systolic pressure, pulmonary vascular resistance and tissue Doppler imaging S wave. Apelin cutoff value of 1650ng/L could significantly detect the presence of cardiopulmonary complications in SCD with 90.9% sensitivity and 72.4% specificity. CONCLUSION: Apelin is a promising marker for screening of SCD patients at risk of cardiopulmonary disease because it is altered during the early subclinical stage of cardiac affection. A combination of apelin and echocardiography provides a reliable method to assess cardiopulmonary affection in young SCD patients.

Chronic thromboembolic pulmonary hypertension (CTEPH) - potential role of multidetector-row CT (MD-CT) and MR imaging in the diagnosis and differential diagnosis of the disease.

Chronic thromboembolic pulmonary hypertension (CTEPH) can be defined as pulmonary hypertension (resting mean pulmonary arterial pressure of 25 mm Hg or more determined at right heart catheterization) with persistent pulmonary perfusion defects. It is a rare, but underdiagnosed disease with estimated incidences ranging from 0.5% to 3.8% of patients after an acute pulmonary embolism (PE), and in up to 10% of those with a history of recurrent PE. CTEPH is the only form of pulmonary hypertension that can be surgically treated leading to normalization of pulmonary hemodynamics and exercise capacity in the vast majority of patients. The challenges for imaging in patients with suspected CTEPH are fourfold: the imaging modality should have a high diagnostic accuracy with regard to the presence of CTEPH and allow for differential diagnosis. It should enable detection of patients suitable for PEA with great certainty, and allow for quantification of PH by measuring pulmonary hemodynamics (mPAP and PVR), and finally, it can be used for therapy monitoring. This overview tries to elucidate the potential role of ECG-gated multidetector CT pulmonary angiography (MD-CTPA) and MR imaging, and summarizes the most important results that have been achieved so far. Generally speaking, ECG-gated MD-CTPA is superior to MR in the assessment of parenchymal and vascular pathologies of the lung, and allows for the assessment of cardiac structures. The implementation of iodine maps as a surrogate for lung perfusion enables functional assessment of lung perfusion by CT. MR imaging is the reference standard for the assessment of right heart function and lung perfusion, the latter delineating typical wedge-shaped perfusion defects in patients with CTEPH. New developments show that with MR techniques, an estimation of hemodynamic parameters like mean pulmonary arterial pressure and pulmonary vascular resistance will be possible. CT and MR imaging should be considered as complementary investigations providing comprehensive information in patients with CTEPH.

[Investigation on response of the patient-reported outcome scale of the main-symptoms of chronic obstructive pulmonary disease complicated with pulmonary heart disease].

OBJECTIVE: To select appropriate descriptors for response of the patient-reported outcome (PRO) scale for the main symptoms of patients with chronic obstructive pulmonary disease (COPD) complicated with pulmonary heart disease. METHODS: A cross-sectional investigation was carried out. Five equidistant ordinal descriptive words in the PRO scale of main symptoms for COPD complicated with pulmonary heart disease were selected. There were 32 alternative words in the questionnaire. Thirty respondents were required to place each descriptive word on a 10-centimeter line according to where they considered each descriptive word should be placed. Then, the line was measured by ruler; average, standard deviation and median were calculated by excel software; the authors finally chose the five equidistant words which accurately reflect the degree of main symptoms. RESULTS: The five most appropriate descriptive words were selected; they were "never", "seldom", "half-partly sometimes", "very often" and "always". CONCLUSION: These selected decorated words are suitable for the PRO scale for patients with COPD complicated with pulmonary heart disease.

[RIGHT VENTRICULAR DIASTOLIC FUNCTION AND PERIPHERAL HEMODYNAMICS IN PATIENTS WITH CHRONIC COR PULMONALE RECEIVING VARIOUS THERAPY REGIMENS].

Right ventricular diastolic function, pulmonary hemodynamics, and peripheral endothelial vasoregulatory function were studied in patients with chronic cor pulmonale during complex treatment over time. The study confirmed the vasodilatory effect of ozone therapy and amlodipine during standard therapy, which appeared as lower blood pressure and better right ventricular diastolic function.

A history of pulmonary embolism and deep venous thrombosis.

Although enormous progress has been made in understanding the physiology of pulmonary embolism, developing new diagnostic modalities and strategies, and constant refinement in the use of heparin therapy and thrombolytic therapy, venous thromboembolism remains a common and lethal process. As the history of this disease illustrates, advances continue to be made and it is anticipated that with newer diagnostic studies and anticoagulants under development, diagnosis and treatment of pulmonary embolism will continue to improve.

Factores pronósticos en la EPOC / Prognostic factors in Chronic Obstructive Pulmonary Disease

Prolongar la supervivencia de los pacientes con enfermedad pulmonar obstructiva crónica (EPOC) es, y ha sido durante décadas, un objetivo complicado. Sin embargo, poco a poco esta percepción empieza a cambiar. De la mano de un mejor conocimiento etiopatogénico de la enfermedad, del análisis de las causas de muerte y sobre todo de la identificación de diversos factores pronósticos, se van dando pasos firmes que permiten afrontar el futuro con mayor optimismo. La presente revisión pretende analizar de forma pormenorizada los principales determinantes pronósticos que se han descrito en la literatura médica y valorar sus posibles implicaciones terapéuticas. Tradicionalmente se ha aceptado que el volumen espiratorio forzado en el primer segundo y su descenso acelerado a lo largo del tiempo era uno de los mejores predictores de mortalidad. Este hecho condicionó durante décadas el objetivo terapéutico en la EPOC. Sin embargo, hoy sabemos que la EPOC es una enfermedad con múltiples dimensiones, algunas de las cuales tienen importantes consecuencias pronósticas. La hiperinsuflación pulmonar, la tolerancia al esfuerzo, las exacerbaciones, la comorbilidad y las manifestaciones sistémicas, especialmente las resultantes de la esfera cardiovascular, son dimensiones que en los últimos años se han revelado como potentes predictores de muerte. El sustrato inflamatorio, tanto local como sistémico, parece estar detrás de muchas de estas manifestaciones y por ello merece especial consideración. Estos nuevos factores pronósticos tienen la virtud de ser potencialmente modificables, lo que subraya la necesidad de planificar intervenciones terapéuticas orientadas a revertir sus efectos cambiando de alguna forma la estrategia tradicional

Efforts over the last few decades to extend the life expectancy of patients with chronic obstructive pulmonary disease (COPD) have faced difficulties, but our perception of the problems involved is now starting to change. Improvements in our understanding of the pathogenic and etiological mechanisms of the disease, analysis of the causes of death, and, in particular, identification of the relevant prognostic factors have resulted in firm advances that allow us to face the future with greater optimism. The aim of this review is to provide a detailed analysis of the chief prognostic factors described in the literature and to evaluate the therapeutic implications of these findings. The traditional view maintained that the accelerated decline in forced expiratory volume in 1 second over time was one of the best predictors of mortality, and this belief has for decades determined the strategies used to treat COPD. However, we now know COPD to be a multidimensional disease and are aware that some of its other manifestations have important prognostic implications. Lung hyperinflation, exercise tolerance, exacerbations, comorbidity, and systemic manifestations­in particular those related to the cardiovascular system­have all been shown in recent years to be strong predictors of mortality. The inflammatory substrate, whether local or systemic, merits special consideration because it appears to be the cause of many of these manifestations. These newly identified prognostic factors are of great interest in that it may be possible to moderate their influence, a circumstance that highlights the need to change the traditional treatment approach and devise therapeutic interventions oriented towards reversing the effects of these factors

Right ventricular dysfunction in chronic obstructive pulmonary disease. Evaluation and management.

Cor pulmonale is an important consequence of COPD. Although the incidence is not precisely known, it is seen more frequently in patients with hypoxemia, CO2 retention and severely reduced FEV1. When present, it limits peripheral oxygen delivery, increases shortness of breath, and reduces exercise endurance. It is also associated with higher mortality rates independent of other prognostic variables. Numerous factors may contribute to the development of cor pulmonale in patients with COPD, but its primary cause is chronic alveolar hypoxia resulting in pulmonary vasoconstriction, vascular remodeling and pulmonary hypertension. The physical exam, chest radiograph and ECG may be helpful in detecting the presence of cor pulmonale, but because of anatomic changes that occur in the chest, these tests are often insensitive in patients with COPD. Noninvasive diagnostic techniques utilizing Doppler echocardiography and radionuclide angiography allow for detection of RV dysfunction at an earlier stage and in most cases, preclude the need for right heart catheterization. LTO2 is the only therapy shown to improve survival in patients with COPD. However, statistical proof correlating improvements in pulmonary hemodynamics with increased survival is lacking. Bronchodilators, such as the beta 2 agonists and especially theophylline, may have beneficial effects on pulmonary hemodynamics in addition to their effect on respiratory function and are useful in COPD when RV dysfunction is present. Diuretics and phlebotomy are also useful in improving symptoms in appropriate patients. Vasodilators such as calcium channel blockers and ACE-inhibitors may improve pulmonary hemodynamics acutely, but may lower arterial PO2 by worsening ventilation-perfusion matching or blunt the improvement in pulmonary hemodynamics seen with supplemental oxygen. The long-term benefits of these agents have not been proven and their routine use in patients with cor pulmonale due to COPD cannot be recommended.

[Chronic pulmonary heart disease and its risk factors among a worker population in Teheran]. / Le coeur pulmonaire chronique et ses facteurs de risque dans une population ouvrière de Téhéran

Because of the high frequency of chronic cor pulmonale in workers admitted to the cardiology department of the Khazaneh Hospital in Teheran, we studied the clinical aspect and the risk factors of this disease in 66 male patients. The average age of patients was 56.1 years and they often had a long history of bronchitis isolated or associated with emphysema. The ECG analysis showed that most abnormalities were localized on the QRST wave. Tobacco and a polluted working environment were the factors most frequently met in our patients. The opium habit probably acted as a risk factor for chronic bronchopneumopathy, but further studies are necessary to ascertain the fact.