OBJECTIVES Pulmonary arterial hypertension (PAH) is associated with rapid deterioration and poor prognosis in SLE, especially during pregnancy. The prevalence of PAH in SLE in non-tertiary centres is uncertain. This study aims to estimate the point prevalence of PAH and identify risk factors for PAH in a large cohort of SLE patients. METHODS A prospective cross-sectional study of 288 patients...

OBJECTIVE The aim of this study was to investigate whether there are differences in capillary nailfold changes in patients with systemic sclerosis (SSc) with and without pulmonary arterial hypertension (PAH), and whether these changes are associated with PAH severity and disease specificity. METHODS Capillary density and loop dimensions were studied in 21 healthy controls, 20 patients with id...

Human phenylalanine hydroxylase (PAH) is specifically expressed in the liver to convert L-phenylalanine to L-tyrosine. Deficiency of the PAH enzyme causes classic phenylketonuria, a common genetic disorder. The human PAH gene has a TATA-less promoter with multiple transcriptional initiation sites. A 9-kilobase DNA fragment 5'-flanking to the human PAH gene is sufficient to confer tissue- and de...

OBJECTIVE To explore the effects of renal sympathetic denervation (RSD) on pulmonary vascular remodeling in a model of pulmonary arterial hypertension (PAH). METHODS According to the random number table, 24 beagles were randomized into control, PAH and PAH+RSD groups (n=8 each). The levels of neurohormone, echocardiogram and dynamics parameters were measured. Then 0.1 ml/kg dimethylformamide ...

Background—Incidence and prevalence rates for pediatric pulmonary hypertension (PH) and pulmonary arterial hypertension (PAH) are unknown. This study describes the nationwide epidemiological features of pediatric PH in the Netherlands during a 15-year period and the clinical course of pediatric PAH. Methods and Results—Two registries were used to retrospectively identify children (0–17 years) w...

This statement of clinical guidelines, although at times subjective in its ratings of evidence, is valuable as an overview of the field of pulmonary hypertension and comprehensive in its review of the extant literature. Sections include diagnosis and management of pulmonary arterial hypertension (PAH); methodology and grading for pulmonary hypertension evidence review and guideline development;...

The objectives of this work assignment were to evaluate ELISA screening methods and determine whether these methods indicate effectively those microenvironments where high exposure to polycyclic aromatic hydrocarbons (PAH) or other semivolatile organic compounds (SVOC) is likely. Four commercially available assay kits for PAH, carcinogenic PAH (C-PAH), 2,4-D, and pentachlorophenol (PCP) were ev...

Pulmonary arterial hypertension (PAH) is characterized by obstruction of pre-capillary pulmonary arteries, which leads to sustained elevation of pulmonary arterial pressure. Identifying those at risk through early interventions, such as genetic testing, may mitigate disease course. Current practice guidelines recommend genetic counseling and offering genetic testing to individuals with heritabl...