In patients clinically known to have or suspected of having PH, cardiac MR-derived curvature ratio, as compared with RHC measurement, was an accurate and reproducible index for estimation of RVSP.
Eccrine angiomatous hamartoma (nevus) is a rare form of congenital tumorous malformation with proliferation of eccrine secretory coils and ducts, surrounding capillary angiomatous channels and occasionally other minor elements. To date, there have been only about 24 cases reported in the literature. We report an additional case with more detailed description of the microscopic findings, including immunohistochemical observations. The patient was a 28-year-old female who presented with painless, flesh- to reddish brown-colored, violaceous or bluish subcutaneous nodules on the extremities and trunk. The tumors did not show sweating following exertion. The histologic features were comparable to the previously reported cases. The hamartomatous eccrine sweat glands and ducts and a few apocrine glands demonstrated qualitatively diminished antigens commonly found in the eccrine sweat apparatuses, such as carcinoembryonic antigen (CEA) and S-100 protein. The findings of CD34, CD44, human nerve growth factor receptor and Ulex europaeus antigens have not been previously reported. The histologic features suggested a "hamartomatous" growth rather than a true neoplastic process.
Sixteen cases of schneiderian-type mucosal papillomas arising in the nasopharynx and oropharynx are reported. The patients include 11 men and 5 women ranging in age from 45 to 79 years (median 62 years). In 12 patients, the papilloma was discovered as an incidental finding, and 2 patients complained of nasal airway obstruction. In the remaining 2 cases, information regarding the cause that led to discovery was unavailable. None of the patients had a prior or concurrent history of sinonasal (schneiderian) papillomas. Histologically, all of the tumors were identical to sinonasal inverted papillomas. Transnasal or transoral surgical excision was the treatment of choice. In 4 of the patients, recurrent tumor occurred within 6 months of initial resection, necessitating additional surgery. Extended follow-up information was available in 9 cases. Eight of the 9 patients are alive and have been free of disease over periods ranging from 15 to 201 months (median 114 months) from diagnosis. One patient was found to have a separate nasopharyngeal squamous cell carcinoma 14 months after the diagnosis of the schneiderian-type papilloma. This patient died secondary to the squamous cell carcinoma 30 months after his initial evaluation.
The management of pulmonary arterial hypertension (PAH) has significantly evolved over the last decades in the wake of more sensitive diagnostics and specialized clinical programs that can provide focused medical care. In the current era of PAH care, 1-year survival rates have increased to 86%–90% from 65% in the 1980s, and average long-term survival has increased to 6 years from 2.8 years. The heterogeneity in the etiology and disease course has opened doors to focusing research in phenotyping the disease and understanding the pathophysiology at a cellular and genetic level. This may eventually lead to precision medicine and the development of medications that may prevent or reverse pulmonary vascular remodeling. With more insight, clinical trial designs and primary end-points may change to identify the true survival benefit of pharmacotherapy. Identifying responders from non-responders to therapy may help provide individualized patient-centered care rather than an algorithm-based approach. The purpose of this review is to highlight the latest advances in screening, diagnosis, and management of PAH.
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