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Since its first clinical description (on his son) by William James West (1793-1848) in 1841, and the definition of the classical triad of (1) infantile spasms; (2) hypsarrhythmia, and (3) developmental arrest or regression as “West syndrome”, new and relevant advances have been recorded in this uncommon disorder. New approaches include terminology of clinical spasms (e.g., infantile (IS) vs. epileptic spasms (ES)), variety of clinical and electroencephalographic (EEG) features (e.g., typical ictal phenomena without EEG abnormalities), burden of developmental delay, spectrum of associated genetic abnormalities, pathogenesis, treatment options, and related outcome and prognosis. Aside the classical manifestations, IS or ES may present with atypical electroclinical phenotypes (e.g., subtle spasms; modified hypsarrhythmia) and may have their onset outside infancy. An increasing number of genes, proteins, and signaling pathways play crucial roles in the pathogenesis. This condition is currently regarded as a spectrum of disorders the so-called infantile spasm syndrome (ISs), in association with other causal factors, including structural, infectious, metabolic, syndromic, and immunologic events, all acting on a genetic predisposing background. Hormonal therapy and ketogenic diet are widely used also in combination with (classical and recent) pharmacological drugs. Biologically targeted and gene therapies are increasingly studied. The present narrative review searched in seven electronic databases (primary MeSH terms/keywords included West syndrome, infantile spasms and infantile spasms syndrome and were coupled to 25 secondary clinical, EEG, therapeutic, outcomes, and associated conditions terms) including MEDLINE, Embase, Cochrane Central, Web of Sciences, Pubmed, Scopus, and OMIM to highlight the past knowledge and more recent advances.

This study aims to assess the prevalence and clinical correlates of small airway obstruction (SAO) in patients with systemic sclerosis (SSc).

Sixty-nine consecutive patients with SSc (63 women and 6 men) were included. Lung function tests, including assessment of lung diffusing capacity, were performed in all patients. Patients were considered to have SAO when the maximal expiratory flow at 25% of the forced vital capacity (MEF

) was lower than 60% as predicted. High-resolution computed tomography (HRCT) of the lung was performed in all patients with MEF

< 60%. We assessed the relationship of SAO in our patients with large airway obstruction, decreased lung diffusing capacity, HRCT findings, disease duration, disease subtype, scleroderma-specific antibodies, and smoking.

SAO was noticed in 46/69 (66.6%) of patients with SSc. Restrictive lung disease was found in 4/69 (5.8%), obstruction of large airways in 18/69 (26.1%), and decreased lung diffusing capacity in 47/69 (68.1%) of patients. No differe (COPD), if isolated or associated with large airway obstruction, especially in tobacco smokers. On the other hand, SAO associated with decreased lung diffusing capacity was found to be not related to smoking, and may indicate a possible prominent bronchiolar involvement within SSc-related interstitial lung disease Key Points • Small airway obstruction in patients with systemic sclerosis can be considered a clinical feature of undiagnosed obstructive lung disease, if isolated or associated with large airway obstruction, especially in tobacco smokers. • Obstruction of small airways, associated with decreased lung diffusing capacity, may indicate a possible prominent bronchiolar involvement within systemic sclerosis-related interstitial lung disease.Rheumatoid arthritis is a systemic inflammatory disease which causes symmetric polyarthritis. Lungs are common site for extra-articular involvement. Rheumatoid lung nodules occur in about 32% of patients with rheumatoid arthritis. The appearance of a lung nodule, along with the blood vessel supplying it, is called “feeding-vessel” sign on computed tomography. It is most commonly seen in infections. However, it can also be present in metastases and pulmonary vasculitis. We describe a woman with long-standing rheumatoid arthritis with subcutaneous and pulmonary nodules. Computed tomography of the chest showed “feeding-vessel” sign. There was no evidence of infection, malignancy, or vasculitis. She was treated for rheumatoid lung nodulosis with rituximab with which she improved remarkably. To the best of our knowledge, “feeding-vessel” sign in rheumatoid lung nodules has never been reported before. This case highlights the fact that “feeding-vessel” sign is not specific for pulmonary infections. It can rarely be seen in rheumatoid lung nodulosis.Electrospun nanofibers of polyacrylonitrile/Ni-metal-organic framework 74 (PAN/Ni-MOF-74) were prepared and utilized as a novel sorbent for spin-column micro-solid-phase extraction (SC-μSPE) of atenolol (ATN) and captopril (CAP). The electrospun nanofibers were characterized by field emission scanning electron microscopy, energy-dispersive X-ray spectroscopy, Fourier transform infrared spectroscopy, and X-ray diffraction analysis. Ni-MOF-74 nanoparticles in the polymeric network of polyacrylonitrile considerably enhance the extraction efficiency of the electrospun sorbent due to providing hydrophobic, hydrogen bonding, and π-π interactions with the target analytes. The entire procedure, including sample loading, washing, and eluting of the target analytes was performed by centrifugation of the spin column. The extracted analytes were then quantified by high-performance liquid chromatography with a diode array detector. Various parameters affecting extraction efficiency were optimized using the one-variable-at-a-time method. Under optimum conditions, the calibration plots were linear in the range 0.5-500 ng mL-1 for ATN and 0.3-500 ng mL-1 for CAP with r2 > 0.999. Limits of detection of 0.15 and 0.13 ng mL-1 were obtained for ATN and CAP, respectively. read more The intra-assay relative standard deviation for five replicate measurements was ≤ 7.8. The relative recoveries for both drugs were within the range 82.6-98.9%. The applicability of the method was successfully investigated for measuring the target drugs in biological fluids and wastewater. The results indicate proper accuracy and analytical performance of the proposed method. Graphical abstract Schematic presentation of electrospun nanofibers of polyacrylonitrile/Ni-metal-organic framework 74 (PAN/Ni-MOF-74) which are used as the sorbent for spin-column microextraction (SC-μSPE) of atenolol (ATN) and captopril (CAP) prior to HPLC-DAD analysis.