Omic-signature of bronchopulmonary dysplasia associated pulmonary hypertension in <1500g-birth-weight-infants with hemodynamically significant intracardiac shunt.

Background: PDA and ASD are common intracardiac shunts noted in prematurely born infants. While there is evidence of persistent PDA and ASD associated with a higher risk for developing bronchopulmonary dysplasia (ICS-BPD) and pulmonary hypertension (ICS-BPD-PH), the underlying pathogenesis is poorly understood and hence challenging to identify at-risk infants. Our study goal was to evaluate transcriptomic expression and associated pathways in tracheal aspirates (TAs) of low-birth-weight infants with hemodynamically significant cardiac shunt (ICS) that develop bronchopulmonary dysplasia (ICS-BPD) and pulmonary hypertension (ICS-BPD-PH).

Multiomics endotyping of preterm infants with bronchopulmonary dysplasia and pulmonary hypertension-A pilot study.

Pulmonary hypertension associated with bronchopulmonary dysplasia is a severe complication of preterm birth resulting in high mortality of up to 50% within the first 2 years of life. There is a direct relationship between bronchopulmonary dysplasia severity and incidence of associated pulmonary hypertension. However, it is challenging to clinically characterize severe bronchopulmonary dysplasia with and without pulmonary hypertension and there is need for better understanding of the two entities.

The association of nocturnal hypoxia and an echocardiographic measure of pulmonary hypertension in children with sickle cell disease.

Background: Pulmonary hypertension (PH) is multifactorial in origin and may develop early in children with sickle cell disease (C-SCD). Potential etiologies are hemolysis-induced endothelial dysfunction, left ventricular (LV) dysfunction, and chronic hypoxia. Nocturnal hypoxia (NH) in C-SCD is known to be a sequela of obstructive sleep apnea (OSA).

An immunohistochemical analysis of folate receptor beta expression and distribution in giant cell arteritis - a pilot study.

Background: Giant cell arteritis (GCA) is a chronic vasculitis of large and medium vessels in which no targetable biomarkers exist to allow selective treatment, predict disease activity and monitor therapeutic responses. The accessibility of the temporal artery (TA) for biopsy allows morphologic studies to characterize macrophages and T cells in the microenvironment of the arterial wall. We evaluated the expression of folate receptor beta (FRB), a candidate diagnostic/therapeutic biomarker, compared its expression with key macrophage markers and correlated it with GCA severity.

Assessing pulmonary arterial hypertension in infants with severe chronic lung disease of infancy: a role for a pulmonary artery catheter?

The treatment of pulmonary arterial hypertension (PAH) associated with chronic lung disease of infancy (CLDI) is becoming commonplace. However, an optimal approach to the monitoring of this treatment has not been clearly established, and data suggest that such therapy may not be without risk. This study assessed the feasibility and safety of pulmonary artery catheter (PAC) placement and its role in the management of PAH associated with CLDI.

Cardiac tumor in juvenile onset Behçet's disease: case report and review of the literature.

Behçet's disease is a chronic multisystemic inflammatory disorder characterized by orogenital ulcerations, uveitis, and occasional cardiac involvement. This report describes an atypical presentation of pediatric Behçet's disease with pulmonary emboli and a cardiac mass. A 16-year-old boy with a 2-year history of oral ulcers presented with weight loss, fever, joint pain, and a large tumor adhering to the free wall of the heart's right ventricle.