Inhaled recombinant GM-CSF reduces the need for whole lung lavage and improves gas exchange in autoimmune pulmonary alveolar proteinosis patients.

Rationale: Whole lung lavage (WLL) is a widely accepted palliative treatment for autoimmune pulmonary alveolar proteinosis (aPAP) but does not correct myeloid cell dysfunction or reverse the pathological accumulation of surfactant. In contrast, inhaled recombinant granulocyte-macrophage colony-stimulating factor (rGM-CSF) is a promising pharmacological approach that restores alveolar macrophage functions including surfactant clearance. Here, we evaluate WLL followed by inhaled rGM-CSF (sargramostim) as therapy of aPAP.

A mini-whole lung lavage to treat autoimmune pulmonary alveolar proteinosis (PAP).

Background: PAP is an ultra-rare respiratory syndrome characterized by the accumulation of surfactant within the alveoli. Whole lung lavage (WLL) is the current standard of care of PAP, however it is not a standardized procedure and the total amount of fluid used to wash each lung is still debated. Considering ICU hospitalization associated risks, a "mini-WLL" with anticipated manual clapping and reduced total infusion volume and has been proposed in our center.

The effectiveness of miniscrews in the three-dimensional control of a palatal impacted canine: "Canine Only" approach. Case report.

Treatment time and mechanics required to solve palatally impacted canines are usually challenging. Different biomechanics and protocol approaches are available, and among these, the use of skeletal anchorage as reactive unit can be noteworthy. The purpose of this article is to show a treatment of a palatally impacted canine by the means of a double miniscrew and cantilever system only.

Reproducibility of MRI Features of Uterine Leiomyomas: A Study on Interobserver Agreement and Inter-Method Agreement With Surgery.

Purpose: To evaluate interobserver agreement in the interpretation of different MRI features of uterine leiomyomas (UL) according to observers' experience, and to assess the inter-method reproducibility (MRI versus surgery) regarding the International Federation of Gynecology and Obstetrics (FIGO) classification.

Methods: Retrospective study including UL patients who underwent MRI and surgical treatment. Four blinded observers (2 >10 years of experience) assessed UL regarding dimensions and volume; inner and outer mantles; FIGO classification; vascularization; degeneration; and diffusion-weighted imaging features.

Congenital Anomalies of the Upper Urinary Tract: A Comprehensive Review.

The upper urinary tract is the most common human system affected by congenital anomalies. Congenital anomalies of the kidneys and ureters comprise a wide spectrum of disorders ranging from simple variants with no clinical significance to complex anomalies that may lead to severe complications and end-stage renal disease. They may be classified as anomalies of renal form, which are subclassified as structural anomalies (eg, persistent fetal lobulation, hypertrophied column of Bertin, and dromedary hump) and fusion anomalies (eg, horseshoe kidney and pancake kidney); anomalies of renal position (eg, renal malrotation, simple renal ectopia, and crossed renal ectopia) and renal number (eg, renal agenesis and supernumerary kidney); and abnormalities in development of the urinary collecting system (eg, pyelocaliceal diverticulum, megacalycosis, ureteropelvic junction obstruction, duplex collecting system, megaureter, ectopic ureter, and ureterocele).

Search strategies of Wikipedia readers.

The quest for information is one of the most common activity of human beings. Despite the the impressive progress of search engines, not to miss the needed piece of information could be still very tough, as well as to acquire specific competences and knowledge by shaping and following the proper learning paths. Indeed, the need to find sensible paths in information networks is one of the biggest challenges of our societies and, to effectively address it, it is important to investigate the strategies adopted by human users to cope with the cognitive bottleneck of finding their way in a growing sea of information.

Whole lung lavage therapy for pulmonary alveolar proteinosis: a global survey of current practices and procedures.

Background: Whole lung lavage (WLL) is the current standard of care treatment for patients affected by pulmonary alveolar proteinosis (PAP). However, WLL is not standardized and international consensus documents are lacking. Our aim was to obtain a factual portrayal of WLL as currently practiced with respect to the procedure, indications for its use, evaluation of therapeutic benefit and complication rate.

Assessment and management of pulmonary alveolar proteinosis in a reference center.

Pulmonary alveolar proteinosis (PAP) is a term defining an ultra-rare group of disorders characterised by a perturbation in surfactant homeostasis, resulting in its accumulation within airspaces and impaired gas transfer. In this report we provide data from a cohort of PAP patients (n=81) followed for more than two decades at the San Matteo University Hospital of Pavia, Italy. In agreement with other large series in PAP individuals, 90% of the study subjects were affected by autoimmune/idiopathic PAP, while the remaining subjects were divided as follow: congenital 1%, secondary 4% and PAP-like 5%.

Pulmonary alveolar proteinosis: diagnostic and therapeutic challenges.

Pulmonary Alveolar Proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals and in distinct clinical forms: autoimmune (previously referred to as the idiopathic form, represents the vast majority of PAP cases, and is associated with Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) auto-antibodies; GMAbs), secondary (is a consequence of underlying disorders), congenital (caused by mutations in the genes encoding for the GM-CSF receptor), and PAP-like syndromes (disorders associated with surfactant gene mutations).

In Lysinuric Protein Intolerance system y+L activity is defective in monocytes and in GM-CSF-differentiated macrophages.

Background: In the recessive aminoaciduria Lysinuric Protein Intolerance (LPI), mutations of SLC7A7/y+LAT1 impair system y+L transport activity for cationic amino acids. A severe complication of LPI is a form of Pulmonary Alveolar Proteinosis (PAP), in which alveolar spaces are filled with lipoproteinaceous material because of the impaired surfactant clearance by resident macrophages. The pathogenesis of LPI-associated PAP remains still obscure.

Corticospinal excitability during action observation in task-specific dystonia: a transcranial magnetic stimulation study.

Observation of actions performed by other individuals activates the onlooker's motor system in a way similar to real movement execution. The functioning of this mechanism in the pathological domain is not clear yet. The aim of this study was to explore whether action observation activates the motor system of patients affected by a task-specific form of dystonia, such as writer's cramp.

Physical properties, lung deposition modeling, and bioactivity of recombinant GM-CSF aerosolised with a highly efficient nebulizer.

Pulmonary alveolar proteinosis (PAP) is a rare condition characterized by the accumulation of lipoproteinaceous material within air spaces. Although whole lung lavage is the current standard of care, recent advances in our understanding of PAP pathophysiology suggest that the disorder may benefit from inhalation of recombinant granulocyte-macrophage colony-stimulating factor (rGM-CSF). The aim of this study was to determine the physical properties and bioactivity of rGM-CSF aerosolised by the highly efficient AKITA² APIXNEB® nebulizer system.

Therapy options in pulmonary alveolar proteinosis.

Pulmonary alveolar proteinosis is a rare condition characterized by the accumulation of lipoproteinaceous material within the airspaces, resulting in impaired gas transfer, and clinical manifestations ranging from asymptomatic to severe respiratory failure. To the best of the authors' knowledge, there are only a few conditions whose natural history has been so dramatically changed by the influence of advances in basic science, clinical medicine, and translational research in therapeutic approaches. Whole-lung lavage is the current standard of care and it plays a critical role as a modifier factor of the natural history of proteinosis.

Whole lung lavage: a unique model for ultrasound assessment of lung aeration changes.

Purpose: Whole lung lavage (WLL) pathophysiologically represents a human model of controlled lung de-aeration, resembling various pathological conditions encountered in the critically ill. Through one-lung ventilation and progressive alveolar flooding, it mimics, respectively, re-absorption atelectasis formation and lung consolidation. With re-ventilation of the treated lung, PEEP application and diuretics administration, it then creates a model of pulmonary edema and its progressive resolution.

Plasmapheresis for treatment of pulmonary alveolar proteinosis.

Whole lung lavage (WLL) is currently the standard therapy for pulmonary alveolar proteinosis (PAP). Nevertheless, some PAP patients respond poorly to WLL or require it frequently. The present paper reports a patient with autoimmune PAP with persistent disease despite three WLL treatments over 10 months.

Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case report.

Background: Pulmonary alveolar proteinosis (PAP) is a rare disease characterised by accumulation of lipoproteinaceous material within alveoli, occurring in three clinically distinct forms: congenital, acquired and secondary. Among the latter, lysinuric protein intolerance (LPI) is a rare genetic disorder caused by defective transport of cationic amino acids. Whole Lung Lavage (WLL) is currently the gold standard therapy for severe cases of PAP.

Peripheral blood progenitor cell mobilization and collection in 42 patients with primary systemic amyloidosis.

Background: High-dose chemotherapy followed by an inoculum of autologous peripheral blood progenitor cells (PBPCs) can improve survival in patients affected with primary systemic amyloidosis (AL). It has been documented, however, that the morbidity and mortality of PBPC mobilization and collection in this setting are higher than in patients with other diseases. To minimize the mobilization and collection-related risks, we developed a multidisciplinary approach involving different specialists to manage AL patients with predominant heart and renal involvement.

Long-term durable benefit after whole lung lavage in pulmonary alveolar proteinosis.

Whole lung lavage (WLL) is still the gold-standard therapy for pulmonary alveolar proteinosis (PAP). The few studies on the duration of the effect of WLL, belonging to a rather remote period, show significant but transient benefits. In 21 patients with idiopathic PAP, the duration of any benefit and, in 16 of them, the time course of lung function improvement (at baseline, 1 week, 6 months, 1 yr and then every 2 yrs after WLL) were evaluated.

Surfactant apoprotein A modulates interleukin-8 and monocyte chemotactic peptide-1 production.

Previous studies have shown that surfactant apoprotein A (SP-A) and natural or synthetic surfactant can modulate the release of pro-inflammatory cytokines from alveolar mononuclear phagocytes. The aim of this study was to assess whether SP-A or Surfactant (Surf) from patients with pulmonary alveolar proteinosis (PAP) can affect the release of two chemokines (interleukin (IL)-8 and monocyte chemtactic peptide (MCP)-1) from human monocytes and rat lung type-II cells. In addition IL-8 and MCP-1 levels were assessed in the brochoalveolar lavage fluid (BALF) of seven patients with PAP and compared with those in a group of control subjects (n=5).

Diagnostic value of exercise electrocardiography for predicting a positive scintigraphic test in patients with complete right bundle branch block.

Background: The diagnostic value of exercise electrocardiography (ECG) in patients with complete right bundle block (cRBBB) remains controversial. The aim of this study was to investigate the diagnostic accuracy of exercise ECG for predicting ischemia in the presence of cRBBB.

Methods: From a series of 1300 consecutive patients attending for exercise ECG and 99mTc-tetrofosmin single photon emission computed tomography (SPECT), we identified 38 male patients with cRBBB and 38 matched controls with normal intraventricular conduction.

Usefulness of platelet glycoprotein IIb/IIIa inhibitors in coronary stenting for reconstruction of complex lesions: procedural and 30 day outcome.

Background: Intracoronary stent implantation during percutaneous transluminal coronary angioplasty (PTCA) has shown favorable results, reducing acute complications associated with PTCA, such as coronary artery dissection and abrupt or threatened vessel closure. However, treatment of lesions with a complex morphology and diffuse disease, requiring long or multiple coronary stents, is still associated with a poorer outcome. We investigated the hypothesis that abciximab might lead to a different outcome in patients with complex coronary lesions, which require long or multiple stent implantation.