Assessment of Right Heart Functions in Children with Mild Cystic Fibrosis.

BACKGROUND: Cystic fibrosis (CF) is a multisystemic disease that prevalently involves the lungs. Hypoxemia occurs due to the existing of progressive damage to the pulmonary parenchyma and pulmonary vessels. The condition may cause systolic and diastolic dysfunction to the right ventricle due to the effects of high pulmonary artery systolic pressure (PASP). The study aimed to determine echocardiographic alterations in PASP, right ventricle (RV) anatomy, and functions in mild CF children. MATERIALS AND METHODS: RV anatomy, systolic, and diastolic functions were evaluated with conventional echocardiographic measurements. Estimated PASP was used measured with new echocardiographic modalities, including pulmonary artery acceleration time (PAAT), right ventricular ejection time (RVET), and their ratio (PAAT/RVET). The obtained echocardiographic data were statistically compared between the patient group and the control group. RESULTS: The study consisted of 30 pediatric patients with mild CF and 30 healthy children with similar demographics. In patient group, conventional parameters disclosed differences in RV anatomy, both systolic and diastolic functions of RV compared with the healthy group. We did not compare the patient group with published standard data because of the wide range variability. However, new echocardiographic parameters showed notable increase in pulmonary artery pressure compared with values of control group and published standard data (p<0.001). CONCLUSION: Elevated PASP, RV failure, and Cor pulmonale usually begin early in children with mild CF. In addition to routine echocardiographic measurements to evaluate RV, we recommend the use of new echocardiographic modalities for routine examinations and in the follow up of children with mild CF.

Development of Myocardial Infarction in a 12-Year-Old Female after the Use of Inhaled Salbutamol.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) accounts for 0.023% of all cases reported in pediatric patients. According to literature, only a handful of ALCAPA patients are able to reach adulthood. Clinical manifestations of ALCAPA range from fatigue during exercise to sudden death in adulthood. Herein, we described a 12-year-old symptomatic patient with ALCAPA who had severe chest pain after using salbutamol treatment for presumed asthma. ALCAPA is one of the curable versions of myocardial ischemia and infarction in childhood. Due to clinical findings in conjunction with electrocardiogram and echocardiography, a computed tomography scan with coronary angiography was performed and the diagnosis of ALCAPA was confirmed. We presented this case because ALCAPA-related myocardial ischemia and infarction in children are rare with only sporadic cases reported. This case illustrated the need for close monitoring and surgery as the best treatment for ALCAPA associated with myocardial infarction.

Migraine or idiopathic intracranial hypertension: Magnetic resonance venography and magnetic resonance imaging findings.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a disease characterised by increased cerebral pressure without a mass or hydrocephalus. We aimed to differentiate migraine and IIH patients based on imaging findings. RESULTS: Patients with IIH (n = 32), migraine patients (n = 34) and control subjects (n = 33) were evaluated. Routine magnetic resonance imaging, contrast-enhanced 3D magnetic resonance venography and/or T1-weighted 3D gradient-recalled echo were taken with a 1.5 T magnetic resonance scanner. Optic-nerve sheath distention, flattened posterior globe and the height of the pituitary gland were evaluated in the three groups. Transverse sinuses (TS) were evaluated with respect to score of attenuation/stenosis and distribution. Pearson chi-square, Fisher's exact test and chi-square trend statistical analyses were used for comparisons between the groups. A p-value of <0.05 was considered statistically significant. Decreased pituitary gland height, optic-nerve sheath distention and flattened posterior globe were found to be statistically significant (p < 0.001) in IIH patients. Bilateral TS stenosis was also more common in IIH patients than in the control group and migraine group (p = 0.02). CONCLUSION: Decreased pituitary gland height, optic-nerve sheath distention, flattened posterior globe, bilateral stenosis and discontinuity in TS are significant findings in differentiating IIH cases from healthy individuals and migraine patients. Bilateral TS stenosis may be the cause rather than the result of increased intracranial pressure. The increase in intracranial pressure, which is considered to be responsible for the pathophysiology of IIH, is not involved in the pathophysiology of migraine.

Lyotropic liquid crystals and linear supramolecular polymers of end-functionalized oligosaccharides.

We synthesized permethylated maltoheptaose oligosaccharides, whose both ends, untrivially, have been functionalized with supramolecular binders 2-ureido-4[1H]-pyrimidinones (UPy) after single ring-opening of ß-cyclodextrin counterpart. In 1,1,1,3,3,3-hexafluoro-2-propanol (HFIP), they show lyotropic liquid crystallinity. In the dried state they allow linear saccharide-based supramolecular polymers by UPy-dimerization.

Thin Films with Perpendicular Tetragonally Packed Rectangular Rods Obtained from Blends of Linear ABC Block Terpolymers.

A binary blend of poly(isoprene-block-styrene-block-(2-vinylpyridine)) (ISP) triblock terpolymers, having the same chain length but different compositions, was used to achieve an ordered lattice with 4-fold symmetry of rectangular-shaped rods of poly(isoprene) (I) and poly(2-vinylpyridine) (P). In given conditions, the I and P domains were oriented perpendicularly to the substrate, providing an appealing type of templates for nanopatterning. Thin films were prepared by spin coating, exposed to solvent vapor (providing morphological reorganization), and then characterized by atomic force microscopy, transmission electron microscopy, and grazing-incidence small-angle X-ray scattering. Selective I and P identifications were carried out by AFM and TEM on a model ISP, as well as development of a technique of electronic contrast enhancement to better assign the self-assembly structure in GISAXS.

Time-Controllable Lipophilic-Drug Release System Designed by Loading Lipid Nanoparticles into Polysaccharide Hydrogels.

A hybrid hydrogel composed of solid lipid nanoparticles (LNPs) entrapped within chemically cross-linked carboxymethylcellulose (CMC) is developed to achieve localized and sustained release of lipophilic drugs. The analysis of LNP stability as well as the hydrogel swelling and mechanical properties confirm the successful incorporation of particles up to a concentration of 50% w/wCMC . The initial LNP release rate can be prolonged by increasing the particle diameter from 50 to 120 nm, while the amount of long-term release can be adjusted by tailoring the particle surface charge or the cross-linking density of the polymer. After 30 d, 58% of 50 nm diameter negatively charged LNPs escape from the matrix while only 17% of positively charged nanoparticles are released from materials with intermediate cross-linking density. A mathematical diffusion model based on Fick's second law is efficient to predict the diffusion of the particles from the hydrogels.