Search results for "PERT"
showing 10 items of 7689 documents
Update on diagnostic strategies of pulmonary embolism
1999
Acute pulmonary embolism is a frequent disease with non-specific findings, high mortality, and multiple therapeutic options. A definitive diagnosis must be established by accurate, non-invasive, easily performed, cost-effective, and widely available imaging modalities. Conventional diagnostic strategies have relied on ventilation-perfusion scintigraphy complemented by venous imaging. If the results are inconclusive, pulmonary angiography, which is regarded as the gold standard, is to be performed. Recently, marked improvements in CT and MRI and shortcomings of scintigraphy led to an update of the diagnostic strategy. Spiral CT is successfully employed as a second-line procedure to clarify i…
Treatment of pulmonary hypertension in patients undergoing cardiac surgery with cardiopulmonary bypass: a randomized, prospective, double-blind study
2006
Pulmonary hypertension can already be present in patients undergoing cardiac surgery or can be exacerbated by cardiopulmonary bypass. Postoperative treatment is still a challenge for physicians. The aim of this study was to evaluate the effects of inhaled prostacyclin (iPGI2) and nitric oxide (iNO) compared with those of intravenous vasodilators.This prospective, randomized, double-blind study included 58 patients affected by severe mitral valve stenosis and pulmonary hypertension with high pulmonary vascular resistance (250 dynes x s x cm(-5)) and a mean pulmonary artery pressure25 mmHg. All patients were monitored by central venous, radial arterial and Swan-Ganz catheters. Data were recor…
Inhaled nitric oxide as a rescue therapy in a preterm neonate with severe pulmonary hypertension: a case report
2018
Abstract Background Inhaled nitric oxide (iNO) has been approved for the treatment of persistent pulmonary hypertension of the newborn (PPHN) in term and near-term newborns. Its role in the management of persistent pulmonary hypertension in preterm infants is not clear. Although guidelines do not exist, some studies have shown that iNO could be used as a rescue therapy in preterm neonate with severe pulmonary hypertension. Case presentation We describe the case of a preterm neonate, born at 30 + 1 weeks of gestation, with hypoxic respiratory failure not responding to maximal conventional therapy. On the third day of life echocardiography showed severe pulmonary hypertension with right to le…
Transposition of the great arteries and aortopulmonary window in the same patient: clinical report and follow-up.
2002
Trasnposition of great arteries (TGA) Has been reported in combination with several congenital defects. Only one case of TGA has been described in association with aortopulmonary window (APW).
Chronic thromboembolic pulmonary hypertension from the perspective of patients with pulmonary embolism
2018
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but feared long-term complication of acute pulmonary embolism (PE), although CTEPH may occur in patients with no history of symptomatic venous thromboembolism. It represents the most severe presentation of the so-called 'post-PE syndrome', a phenomenon of permanent functional limitations after PE caused by deconditioning after PE or ventilatory or circulatory impairment as a result of unresolved pulmonary artery thrombi. Because the post-PE syndrome may occur in up to 50% of PE survivors, and CTEPH tends to have an insidious and non-specific clinical presentation, CTEPH is often not diagnosed or diagnosed after a very long dela…
Case report supporting that the Barber-Say and ablepharon macrostomia syndromes could represent one disorder.
2009
We report on a 7-year-old girl with unequivocal features of Barber-Say syndrome (BSS): generalized hypertrichosis especially at the back, dry lax skin, macrostomia, thin lips, cup-shaped ears, bulbous nose, hypoplastic nipples, and abnormal external genitalia. She also demonstrated conductive hearing impairment and microblepharon. BSS has been reported with ectropion (not present in our patient), but ablepharon and microblepharon (i.e., absent or hypoplastic eyelids) have always been considered as hallmarks of ablepharon macrostomia syndrome (AMS). This is the first report of microblepharon in BSS. Other authors have discussed that BSS and AMS could possibly represent one syndrome, and our …
Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome
2015
Abstract Pulmonary agenesis is a rare congenital disorder with large variability in presentation and prognosis. We describe a full-term infant born with right-sided pulmonary agenesis who underwent thoracoscopic placement of a tissue expander. He ultimately died of pulmonary hypertension. Immunohistology showed intimal hyperplasia without the loss of endothelial caveolin-1 expression. A literature review revealed that while some of these patients have favorable outcome, many succumb despite therapy.
Latanoprost-induced iris darkening: a morphometric study of human peripheral iridectomies.
2003
Abstract Purpose. This microscopic study was undertaken to compare the melanocytes of peripheral iridectomy specimens from two eyes that had latanoprost-induced iris darkening (LIID) with iridectomies taken from the fellow untreated eyes. Methods. The two patients in this study were the ones who underwent LIID in the latanoprost treated eye from a series of 17 patients requiring bilateral trabeculectomy. The first trabeculectomy procedure provided a control peripheral iridectomy for each patient, whereas the second eye was treated with once daily 50 μg ml−1 latanoprost drops for 6 months. The four peripheral iridectomy specimens from the two LIID patients were subjected to quantitative morp…
Oral findings in Midline Syndrome: a case report and literature review
2010
We describe a female patient with a midline syndrome. The patient presents agenesis of the corpus callosum, encephalocele, iris coloboma, hypertelorism, submucosal cleft palate and dental anomalies. Despite being very characteristic, her phenotypical traits do not coincide exactly with those reported to date in the literature. The karyotype and the molecular cytogenetic study do not show mutations. We identify the presence of dental anomalies in the mother and other family members, not being identified MSX1 and PAX9 mutations that could the related with their etiology. Despite the fact that dental agenesis has been related to a large number of other malformation syndromes and congenital con…
Genome-wide association analyses identify multiple loci associated with central corneal thickness and keratoconus
2013
The author manuscript of this article is open access and is freely available online at PubMed Central